Section 1. Cutaneous Findings in the Newborn

Vernix Caseosa

Vernix, derived from the same root as varnish, is the whitish-gray covering on newborn skin and is composed of degenerated fetal epidermis and sebaceous secretions.

Epidemiology

Age Newborns.

Gender M = F.

Prevalence Seen in all infants.

Pathophysiology

The vernix caseosa is thought to play a protective role for the newborn skin with both water-barrier and antimicrobial properties.

Physical Examination

Skin Findings

Type of Lesion Adherent cheesy material which dries and desquamates after birth (Fig. 1-1).

Color Gray-to-white.

Distribution Generalized.

Differential Diagnosis

The vernix caseosa is generally very distinctive but must be differentiated from other membranous coverings such as collodion baby and harlequin fetus. Both of these are much thicker, more rigid, and more dry.

Course and Prognosis

In an otherwise healthy newborn, the vernix caseosa will fall off in 1 to 2 weeks.

Treatment

No treatment is necessary. Much of the vernix caseosa is usually wiped from the skin at birth. The rest of the vernix is shed over the first few weeks of life.

Current newborn skin care recommendations are as follows:

1. 1. Full immersion baths are not recommended until the umbilical stump is fully healed and detached.

   2. At birth, blood and meconium can be gently removed with warm water and cotton balls.

   3. Umbilical cord care and/or circumcision varies from hospital to hospital. Several methods include a local application of alcohol (alcohol wipes), topical antibiotic (bacitracin, Polysporin, or neosporin), or silver sulfadiazine cream (Silvadene) to the area(s) with each diaper change. The umbilical cord typically falls off in 7 to 14 days.

   4. Until the umbilical and/or circumcision sites are healed, spot cleaning the baby with cotton balls and warm water is recommended. After the open sites have healed, the baby can be gently immersed in lukewarm water and rinsed from head to toe.

   5. Avoiding perfumed soaps and bubble baths is best. Fragrance-free, soap-free cleansers are the least irritating. Such cleansers should be used only on dirty areas and rinsed off immediately.

   6. After bathing, newborn skin should be patted dry (not rubbed). The vernix caseosa may still be present and adherent for several weeks. Topical moisturizers are usually not recommended.

Cutis Marmorata

A physiologic red–blue reticulated mottling of the skin of newborn infants. It is seen as an immature physiologic response to cold with resultant dilatation of capillaries and small venules. Skin findings usually disappear with rewarming and the phenomenon resolves as the child gets older.

Insight

While cutis marmorata is essentially always normal, its analogue in adults, livedo reticularis, can be associated with connective tissue disease and vasculopathies.

Epidemiology

Age Onset during first 2 to 4 weeks of life; associated with cold exposure.

Gender M = F.

Prevalence More prevalent in premature infants.

Pathophysiology

Thought to be owing to the immaturity of the autonomic nervous system of newborns. Physiologically normal and resolves as child gets older.

History

Reticulated mottling of the skin resolves with warming.

Physical Examination

Skin Findings

Type Reticulated mottling (Fig. 1-2).

Color Reddish-blue.

Distribution Diffuse, symmetric involvement of the trunk and extremities.

Differential Diagnosis

Cutis marmorata is benign and self-limited. It can be confused with cutis marmorata telangiectatica congenita (CMTC), a more severe condition that can also present as reticulated vascular changes at birth. CMTC is a rare, chronic, relapsing, severe form of vascular disease that can lead to permanent scarring skin changes.

Course and Prognosis

Recurrence is unusual after 1 month of age. Persistence beyond neonatal period is a possible marker for trisomy 18, Down syndrome, Cornelia de Lange syndrome, hypothyroidism, or CMTC.

Treatment and Prevention

Usually self-resolving.

Neonatal Hair Loss

Neonatal hair at birth is actively growing in the anagen phase, but within the first few days of life converts to telogen (the rest period before shedding) hair. Consequently, there is normally significant hair shedding during the first 3 to 4 months of life.

Insight

Parents may complain that “back to sleep” is the cause of hair loss in their child; the sleep position only accentuates the normal loss and is not the cause.

Synonym Telogen effluvium of the newborn.

Epidemiology

Age Newborns, can be seen at 3 to 4 months of age.

Gender M = F.

Prevalence Affects all infants to some degree.

Pathophysiology

There are three stages in the hair life cycle:

1. 1. Anagen (the active growing phase that typically lasts from 2 to 6 years).

   2. Catagen (a short-partial degeneration phase that lasts from 10 to 14 days).

   3. Telogen (resting and shedding phase that lasts from 3 to 4 months).

At any given time in a normal scalp, 89% of the hair are in anagen phase, 1% are in catagen phase, and 10% are in telogen phase. Neonatal hair loss occurs because most of the anagen hair at birth simultaneously converts to telogen phase in the first few days of life, resulting in whole-scalp shedding of the birth hair in 3 to 4 months.

History

During the first 3 to 6 months of life, there will be physiologic shedding of the newborn hair. In some cases, the new hair balances the shedding hair and the process is almost undetectable.

Physical Examination

Skin Findings

Type of Lesion Nonscarring alopecia (Fig. 1-3).

Distribution Diffuse involving the entire scalp.

Differential Diagnosis

Neonatal hair loss is a normal physiologic process that is diagnosed clinically by history and physical examination. The alopecia may be accentuated on the occipital scalp because of friction and pressure from sleeping on the back.

Course and Prognosis

In an otherwise healthy newborn, the hair loss self-resolves by 6 to 12 months of age.

Treatment

No treatment is necessary. The parents should be reassured that neonatal hair loss is a normal physiologic process and that the hair will grow back by 6 to 12 months of age without treatment.

Miliaria, Newborn

Miliaria is a common neonatal dermatosis resulting from sweat retention caused by incomplete differentiation of the epidermis and its appendages. Obstruction and rupture of epidermal sweat ducts is manifested by a vesicular eruption.

Insight

In older children and adults, miliaria rubra is the most common form, usually seen in areas of moist occlusion, such as on the back of a bedridden patient with fever.

Synonyms Prickly heat, heat rash.

Epidemiology

Age Newborn.

Gender M = F.

Incidence Greatest in the first few weeks of life.

Prevalence Virtually, all infants develop miliaria.

Etiology Immature appendages and keratin plugging of eccrine duct leads to vesicular eruption.

Pathophysiology

Incomplete differentiation of the epidermis and its appendages at birth leads to keratinous plugging of eccrine ducts and subsequent leakage of eccrine sweat into the surrounding tissue. Staphylococci on the skin may play a role in the occlusion process as well.

Physical Examination

Skin Findings

Miliaria Crystallina (Sudamina)

Type Superficial pinpoint clear vesicles (Fig. 1-4).

Color Skin, pink.

Distribution Generalized in crops.

Sites of Predilection Intertriginous areas, commonly neck and axillae, or clothing-covered truncal areas.

Miliaria Rubra (Prickly Heat)

Type Pinpoint papules/vesicles.

Color Erythematous.

Sites of Predilection Covered parts of the skin, forehead, upper trunk, volar aspects of the arms, and body folds.

Differential Diagnosis

The diagnosis of miliaria is made based on observation of characteristic lesions. Vesicles may be ruptured with a fine needle, yielding clear, entrapped sweat. Miliaria rubra requires close inspection to ascertain its nonfollicular nature to distinguish it from folliculitis. Miliaria can also be confused with candidiasis and acne.

Laboratory Examinations

In miliaria crystallina, vesicles are often found superficially in the stratum corneum and serial sectioning demonstrates direct communication with ruptured sweat ducts. Miliaria rubra histologically demonstrates degrees of spongiosis and vesicle formation within the epidermal sweat duct.

Management

Prevention Avoid excessive heat and humidity. Lightweight and/or absorbent clothing, cool baths, and air-conditioning help prevent sweat retention.

Treatment Preventative measures only for newborns; in older patients, cool moist dressings, antibacterial cleansers, and cream-based emollients may speed healing.

Milia

Multiple 1- to 2 mm white/yellow superficial tiny cysts seen over the forehead, cheeks, and nose of infants. They may be present in the oral cavity as well where they are called Epstein’s pearls.

Insight

So-called secondary milia (milia after trauma to the skin) can be seen in patients with blistering disorders such as epidermolysis bullosa.

Epidemiology

Age All ages, especially newborns.

Gender M = F.

Prevalence Up to 40% of infants have milia on the skin, up to 85% of infants have the intraoral counterpart (Epstein’s pearls) on the palate.

Etiology May be related to trauma to the skin surface during delivery.

Pathophysiology

Milia and Epstein’s pearls are caused by the cystic retention of keratin in the superficial epidermis.

Physical Examination

Skin Findings

Type Few to numerous pinpoint white papules (Fig. 1-5).

Size 1 to 2 mm.

Color Yellow to pearly white.

Distribution Forehead, nose, cheeks, gingiva, midline palate (Epstein’s pearls), rarely on the penis, and at sites of trauma.

Differential Diagnosis

Milia must be differentiated from molluscum contagiosum and sebaceous hyperplasia. Molluscum contagiosum does not typically appear in the immediate neonatal period and is characterized by dome-shaped papules with central umbilication. Sebaceous hyperplasia is usually more yellow than white in color and, on close inspection, is found to be composed of tiny aggregates of micropapules with central umbilication. Epstein’s pearls can be differentiated from intraoral mucinous cysts by their typical midline palate location and spontaneous resolution.

Laboratory Examinations

Dermatopathology Superficial, tiny epithelial cysts containing keratin and developing in connection with the pilosebaceous follicle.

Course and Prognosis

Milia and Epstein’s pearls should spontaneously exfoliate during the first few weeks of life. Unusually, persistent milia or widespread milia can be seen in conjunction with more severe developmental problems, namely, oral-facial-digital syndrome, and hereditary trichodysplasia.

Treatment and Prevention

No treatment is necessary. Cosmetically, lesions may be incised and expressed.

Acne Neonatorum

Acne neonatorum is a benign, self-limited, acneiform eruption that develops within the first 30 days of life.

Insight

In severe cases that do not resolve, evaluation for underlying androgen excess is warranted.

Synonyms Transient cephalic neonatal pustulosis, neonatal cephalic pustulosis.

Epidemiology

Age Rare at birth, peaks between the age of 2 and 4 weeks of life.

Gender M = F.

Prevalence Up to 50% of infants.

Pathophysiology

Some, perhaps most, of these cases are thought to be related to Malassezia yeasts on the skin. Transient increases in circulatory androgens may also contribute to neonatal acne.

History

Multiple discrete papules develop between the age 2 and 4 weeks of life, evolve into pustules, and spontaneously resolve.

Physical Examination

Skin Findings

Type Inflammatory papules and pustules; comedones are rare (Fig. 1-6A).

Color Erythematous.

Distribution Face, chest, back, and groin.

Differential Diagnosis

Erythema toxicum, candidiasis, and staphylococcal infection can be considered.

Laboratory Examinations

Dermatopathology Increased number of sebaceous glands and keratin-plugged pilosebaceous orifices lead to rupturing and neutrophilic or granulomatous inflammation.

Course and Prognosis

Neonatal acne may persist up to 8 months of age. There is some suggestion that infants with extensive neonatal acne may experience severe acne as adults.

Treatment and Prevention

Neonatal acne typically resolves spontaneously. For severe involvement, 2% ketoconazole cream or 2.5% benzoyl peroxide gel applied twice daily may be used (Fig. 1-6C)

Erythema Toxicum Neonatorum

Benign transient, blotchy erythema with central vesiculation is seen in newborns.

Epidemiology

Age Newborns.

Gender M = F.

Prevalence Unclear, reports range from 4.5% to 70% of term infants. Less common in premature infants.

Pathophysiology

The cause of erythema toxicum is unknown. The eosinophil response is suggestive of a hypersensitivity reaction, but specific allergens have not been identified.

History

Macular erythema with central vesicles and pustules appear between 24 and 48 hours of life.

Physical Examination

Skin Findings

Type Blotchy erythematous macules 2 to 3 cm in diameter with a central 1- to 4-mm central papule, vesicle, or pustule (Fig. 1-7).

Color Erythematous.

Distribution Chest, back, face, and proximal extremities, sparing the palms and soles.

Diagnosis and Differential Diagnosis

Diagnosis Wright stain of a vesicle will reveal a predominance of eosinophils. Gram stain will be negative.

Differential Diagnosis Erythema toxicum must be differentiated from miliaria rubra and transient neonatal pustular melanosis. The lesions of erythema toxicum are usually larger than those of miliaria rubra and have wider erythematous halos surrounding them. Transient neonatal pustular melanosis has a predominance of neutrophils rather than eosinophils in the vesicles and typically heals with residual pigmentation. Bacterial and fungal culture of erythema toxicum lesions will be negative differentiating it from neonatal bacterial infections and congenital candidiasis. More worrisome considerations include herpes infection and Langerhans cell histiocytosis, these may require virologic testing and skin biopsy for definitive diagnosis and must not be overlooked.

Laboratory Examinations

Wright Stain A smear of a vesicle with Wright stain reveals numerous eosinophils.

Gram Stain Negative.

Dermatopathology Intraepidermal vesicle filled with eosinophils.

Hematologic Examination Peripheral eosinophilia of up to 20% may be seen in some cases.

Course and Prognosis

Lesions may occur from birth to the 10th day of life and individual lesions clear within 5 days. All lesions resolve by 2 weeks.

Treatment

No treatment is necessary.

Transient Neonatal Pustular Melanosis

A benign and self-limited condition characterized by blotchy erythema and superficial vesico-pustules of the newborn that heal with residual pigmentation.

Insight

Many times, particularly in postterm infants, no pustules or vesicles remain at birth and only pigmented macules are found.

Epidemiology

Age Newborns.

Gender M = F.

Race More common in black infants.

Prevalence 0.2% to 4% of the newborns.

Pathophysiology

The pathophysiology is unknown.

History

Idiopathic, superficial, sterile vesicles and pustules that are present at birth rupture in 24 to 48 hours and heal with pigmented macules that slowly fade over several months.

Physical Examination

Skin Findings

Type Tiny vesicles, pustules (Fig. 1-8), or ruptured lesions with a collarette of scale. There is generally minimal or no surrounding erythema.

Color Hyperpigmented macules may develop at the site of resolving vesicles and pustules.

Distribution Clusters on face, trunk, and proximal extremities, rarely palms and soles may be involved.

Diagnosis and Differential Diagnosis

Diagnosis Wright stain of a vesicle will reveal a predominance of neutrophils. Gram stain will be negative for bacteria.

Differential Diagnosis Includes erythema toxicum neonatorum, staphylococcal and other bacterial infections, candidiasis, herpes, and miliaria.

Laboratory Examinations

Wright Stain A smear of a vesicle with Wright stain reveals numerous neutrophils and an occasional eosinophil.

Gram Stain Negative.

Dermatopathology Vesicular lesions show intraepidermal vesicles filled with neutrophils. Macular hyperpigmented lesions show mild hyperkeratosis and basilar hyperpigmentation.

Course and Prognosis

The vesicles and pustules usually disappear by 5 days of age and the pigmented macules fade over 3 to 6 months.

Treatment

No treatment is necessary.

Neonatal Lupus Erythematosus

Neonatal lupus erythematosus (NLE) is an uncommon autoimmune disease caused by the transplacental antibodies from mother to fetus. The skin lesions are usually nonscarring and similar to those of subacute cutaneous lupus erythematosus (SCLE) in adults. The lesions may be accompanied by cardiac conduction defects, hepatobiliary disease, or hematologic disturbances.

Insight

If a newborn is thought to have “widespread ringworm,” neonatal lupus must be considered.

Epidemiology

Age Skin lesions may be present at birth or may appear within the first few months life. Lesions typically last several weeks to months, but typically resolve by 6 months of age. Heart block appears typically during gestation (at about 18 weeks of gestational age, but may occur later) and is generally irreversible.

Gender M = F.

Incidence Unknown. One estimate, based on the incidence of congenital heart block with NLE being the most frequent etiology, approximates the incidence of NLE to be near 1:20000 births.

Etiology and Transmission Maternal autoantibodies cross the placenta during pregnancy and are thought to produce both the cutaneous and systemic findings of NLE. Many mothers of neonatal lupus patients have no symptoms of connective tissue disease.

Pathophysiology

Mothers of babies with NLE possess autoantibodies (95% of cases possess anti-Ro/SSA, others may have anti-La/SSb or anti-U1RNP antibodies). These IgG autoantibodies pass through the placenta from mother to fetus and can be found in the neonate’s sera. It is felt that these autoantibodies are implicated in the development of cutaneous and systemic findings of NLE. The skin lesions are temporary and begin to resolve at or before the time that maternal autoantibodies are cleared from the child’s system (typically at 6 months of age). Conduction defects may be caused by anti-Ro/SSA-mediated interference of specific cardiac serotoninergic receptors.

Physical Examination

About half of the NLE cases report exhibited skin disease and about half exhibit congenital heart block. Approximately 10% have both skin disease and heart block.

Skin Findings

Type Scaly plaques, epidermal atrophy. Generally, no scarring or follicular plugging.

Color Pink, erythematous. May be hypopigmented.

Shape Round, elliptical, or annular.

Distribution Frequently on face and scalp. Lesions may be concentrated in the periorbital and malar areas. Widespread involvement may occur (Fig 1-9).

General Findings

Typically, disease presents with complete heart block that begins during gestation. Occasionally, lesser degrees of conduction abnormality are present. Fibrosis of the AV node (in rare instances, the SA node) may lead to bradycardia. NLE typically presents with isolated heart block and no associated cardiac anomalies. Other findings include liver disease, thrombocytopenia, and leukopenia.

Differential Diagnosis

The skin findings of the newborn may be confused with other scaly, erythematous plaques such as seborrhea, eczema, psoriasis, or tinea. Heart block may be detected during routine obstetrical examination and confirmed by fetal ultrasound. Finally, the diagnosis can be confirmed by serologic studies.

Laboratory Examinations

Histopathology Biopsy of lesional skin reveals findings similar to that of SCLE lesions. There is vacuolar basal cell degeneration in the epidermis and a sparse mononuclear cell infiltrate in the superficial dermis. Immunofluorescence studies demonstrate a granular deposition pattern of IgG in the dermoepidermal junction with variable C3 ang IgM deposition.

Serology Sera of mother and baby should be tested for autoantibodies. Ninety-five percent of cases are owing to anti-Ro/SSA antibodies. Anti-La/SSB antibodies may also be present but almost never without the presence of anti-Ro/SSA antibodies. Anti-U1RNP autoantibodies account for the NLE cases that do not have anti-Ro/SSA antibodies present.

Other Obstetrical detection of slowed fetal heart rate or ultrasound documentation of heart block may be found as early as 16 weeks of gestational age.

Course and Prognosis

Infants with NLE, who survive the neonatal period have a good prognosis and rarely go on to develop autoimmune disease. The estimated mortality rate is 10%, with deaths being secondary to intractable heart failure during the neonatal period. Mothers with babies, who develop NLE do not have an increased rate of spontaneous abortion but often develop autoimmune disease, on an average, 5 years after delivery. The risk of NLE in future pregnancies is approximately 25% for mothers who have had one child with NLE.

Skin Findings

The cutaneous lesions of NLE in the neonate are benign and transient, usually lasting weeks to months and regress by the age of 6 months, occasionally with residual postinflammatory hypopigmentation or atrophy that gradually improves.

General Findings

Despite complete heart block and a subsequent slowed heart rate, babies with cardiac involvement of NLE tend to be well compensated. Half of them do not require any treatment, and the other half need pacemakers. Approximately 10% do not respond despite pacemaker placement likely because of coexistent myocardial disease, and die of intractable heart failure.

Management

during Gestation

Testing for autoantibodies during pregnancy is appropriate for women with anti-Ro/SSA autoantibody symptomatology: Sjögren’s syndrome, SCLE, SLE, arthralgias, dry eyes, dry mouth, or photosensitivity. Women with previous births with heart block or other signs/symptoms suggestive of NLE should also be tested. It is important to keep in mind that 50% of women with babies with NLE are asymptomatic at delivery and conversely, most women with anti-Ro/SSA antibodies will have normal babies. It is estimated that only 1% to 2% of women with anti-Ro/SSA autoantibodies will have a baby with NLE. Risk factors that increase these percentages include a previous baby with NLE and mothers with the diagnosis of SLE. Screening tests should include fluorescent antibody tests for anti-Ro/SSA, anti-La/SSB, and anti-U1RNP autoantibodies.

Obstetrical screening for a slowed fetal heart rate is also important and if heart block is confirmed, the fetus should be monitored carefully for the development of hydrops fetalis. The delivery room should be equipped to manage a newborn with possible heart failure. The use of systemic steroids or plasmapheresis during gestation has been utilized in life-threatening circumstances.

Neonatal Period

Skin Findings Skin disease is generally benign and self-limited. Supportive care includes strict sun protection and topical steroids for more severe cutaneous involvement. Systemic treatment is not indicated.

General Findings Treatment of heart disease is not always indicated. For those children with heart failure because of a slowed heart rate, pacemaker implantation is the treatment of choice.

Aplasia Cutis Congenita

Aplasia cutis congenita is a congenital defect of the scalp characterized by localized loss of the epidermis, dermis, and sometimes, subcutaneous tissue.

Epidemiology

Age Present at birth.

Gender M = F.

Incidence Unclear; one estimate is 1 in 10000 births.

Etiology Unknown for most cases; whereas, some are related to genetic syndromes, fetus papyraceous, and teratogens.

Genetics Most cases are sporadic, some familial cases are reported.

Pathophysiology

The exact mechanism of this disorder is not known. It is hypothesized that aplasia cutis congenita results from incomplete neural tube closure or an embryonic arrest of skin development.

History

Aplasia cutis congenita presents as an asymptomatic ulceration of the scalp at birth that heals with scarring. Lesions from earlier in gestation may present as membranelike scars at birth.

Physical Examination

Skin Findings

Type of Lesion Ulceration that is replaced with scar tissue (Fig. 1-10). In the scar, no hair or appendages are present.

Size 1 to 3 cm, may be larger.

Shape Round, oval, or stellate.

Number Solitary lesion (70% of cases), two lesions (20% of cases), three or more lesions (10% of cases).

Color Pink to red healing to white–gray.

Distribution Scalp vertex, midline (50% of cases) or adjacent areas (30%). Can also rarely be seen on the face, trunk, or extremities.

General Findings

Typically, aplasia cutis congenita is an isolated skin finding. In rare instances, it may be seen with other developmental abnormalities such as skeletal, cardiac, neurologic, or vascular malformations.

Differential Diagnosis

The diagnosis of aplasia cutis congenita is made by history and physical examination. It should be differentiated from scalp electrode monitors, forceps, or other iatrogenic birthing injuries as well as neonatal herpes simplex virus infection.

Laboratory Examinations

Dermatopathology Skin biopsy reveals an absence of the epidermis and appendageal structures. There is a decrease in dermal elastic tissue, and, in some deep cases, a loss of all skin layers and subcutaneous tissues.

Course and Prognosis

The prognosis of cutis aplasia congenita as an isolated finding is good. The ulceration typically heals with scarring in a few weeks. The scarred area will persist as an asymptomatic lesion for life.

Treatment

Localized care of the ulcerated area at birth includes the following:

1. 1. Gentle cleansing of the area with warm water and cotton balls.

   2. A thin layer of topical antibiotic ointment (Bactroban, bacitracin, Polysporin, or neosporin) to prevent secondary infection.

   3. Protective coverings to prevent further trauma to the area. The area will heal with scarring and as the child grows, the scar usually becomes inconspicuous and covered with surrounding hair.

Management options:

1. 1. No treatment. The scarred area should be examined annually for any changes since scarred areas do have a higher risk of neoplastic transformation.

   2. Surgical correction of the area by excision or hair transplant.

Heterotopic Neural Nodules

Heterotopic neural nodules refer to a group of malformations that includes ectopic leptomeningeal or brain tissue in the dermis and subcutis. These lesions are present on the scalp at birth and may communicate directly with the CNS.

Insight

Thick, dark hair forming a ring around a scalp lesion is known as the “hair collar sign” and suggests underlying CNS malformations or ectopic neural tissue in the scalp.

Synonyms Primary cutaneous meningioma, atretic meningocele or encephalocele, heterotopic neural rest, cutaneous ectopic brain.

Epidemiology

Age Present at birth.

Gender M = F.

Incidence Rare.

Etiology Unknown.

Pathophysiology

Herniation of neural tissue during development with subsequent severing is thought to be the cause of most of these lesions. “Rests” are collections of surviving embryonic cells that appear to have been misdirected during development and may explain some types of these malformations.

History

Heterotopic neural nodules present on the scalp at or shortly after birth and persist. They may become secondarily infected and some may swell with crying or Valsava maneuvers.

Physical Examination

Skin Findings

Type of Lesion Papule, plaque, or cyst, without scalp hair (Fig. 1-11). Likely to have surrounding area of thick, coarse hair (hair collar sign). May also have associated capillary stain.

Size 2 to 4 cm.

Shape Round.

Number Solitary lesion.

Color Skin-colored, erythematous, or blueish.

Distribution Parietal and occipital scalp, almost always near midline.

General Findings

Typically, heterotopic neural nodules are an isolated skin finding. However, because there may be connection with the brain, infection can be transmitted in a retrograde fashion from the skin.

Differential Diagnosis

The diagnosis is generally made by history and physical examination. Radiologic imaging is essential to evaluate for intracranial connection. Dermoid cyst, meningocele, encephalocele, nevus, lipoma, aplasia cutis congenita, and vascular neoplasms must be considered in the differential diagnosis.

Laboratory Examinations

Dermatopathology Skin biopsy reveals thin central epidermis with dermal collagen surrounded by cystic spaces with meningothelial cells. Thickened hair follicles may be seen at the periphery associated with large apocrine and sebaceous glands.

Imaging Computed tomography (CT) scan or magnetic resonance imaging (MRI) may reveal intracranial extension.

Course and Prognosis

The prognosis of heterotopic neural nodules is good. With surgery, future risk of retrograde infection can be eliminated.

Treatment

Identification of the lesion as a heterotopic neural nodule and involving a neurosurgeon or plastic surgeon with experience in the area is paramount. Imaging is essential prior to surgery or manipulation of any sort. Complete excision is curative.

Accessory Tragus

Accessory tragi result when a branchial arch or cleft fails to fuse or close properly. Defects occur unilaterally or bilaterally and may have accompanying facial anomalies.

Synonym Preauricular skin tag.

Epidemiology

Age Newborn.

Gender M = F.

Incidence 4 in 1000.

Etiology May be related to an underlying genetic anomaly.

Pathophysiology

Accessory tragi is caused by first or second branchial arch fusion abnormality during embryonic development.

History

Noted at birth and persists asymptomatically for life. Usually, it is an isolated, congenital defect. Rarely, there may be other associated branchial arch abnormalities or other genetic syndromes present. The presence of preauricular tags are associated with urinary tract abnormalities such as vesicoureteral reflux. Accessory tragi are a feature of Goldenhar syndrome, Treacher–Collins, and VACTERL syndrome, among others.

Physical Examination

Skin Findings

Type Small, pedunculated papule (Fig. 1-12).

Color Skin color, tan to brown.

Size 1 to 7 mm.

Shape Pedunculated, round to oval lesions.

Sites of Predilection Preauricular area.

Differential Diagnosis

Differential diagnosis includes epidermal inclusion cyst, fibroma, adnexal tumor, or lipoma.

Laboratory Examinations

Dermatopathology Skin biopsy or removal would show a loose fibrous stroma with overlying thinned epidermis and numerous vellus hair. Cartilage is frequently present.

Course and Prognosis

Accessory tragi are typically asymptomatic but persist for life, occasionally becoming inflamed.

Management

Solitary, uncomplicated lesions may be tied off shortly after birth but otherwise can be excised during childhood for cosmetic purposes. Screening urinary tract ultrasonography is recommended by some authors.

Branchial Cleft Cyst

An epithelial cyst on the lateral neck caused by an incomplete obliteration of the branchial clefts during embryologic development.

Insight

Branchial cleft cysts are the most common etiology of congenital neck masses.

Synonyms Branchial cyst, branchial sinus.

Epidemiology

Age Newborn.

Gender M = F.

Prevalence Unknown.

Etiology Arises from failure of involution of the second or third branchial clefts. Remnants of the cleft membrane form cysts or sinuses.

Genetics Most are sporadic but case reports of autosomal dominant inheritance patterns exist.

Pathophysiology

Branchial cleft cysts are nonregressed remnants of the embryonic second and third branchial clefts. Similar lesions, thyroglossal ducts, and/or sinuses, can be seen more near the midline of the neck.

History

Onset Lesions are evident at birth or appear in early childhood as cystic swellings in the neck, deep to the sternomastoid muscle. Occasionally, they can become infected and painful.

Physical Examination

Skin Findings

Type Fluctuant papule or nodule.

Color Skin-colored.

Size 2 to 10 mm.

Distribution Unilateral or bilateral swellings in the neck, deep to the sternomastoid muscle (Fig. 1-13).

Differential Diagnosis

Branchial cleft cysts can be confused with other nodular lesions on the neck such as lymph nodes or epidermal or pilar cysts. The location and presence at birth can help distinguish them from other neck lesions.

Laboratory Examinations

Dermatopathology On skin biopsy, the cyst will have a stratified squamous epithelial lining.

Course and Prognosis

Cysts often form sinuses or fistulae, draining mucus internally to the pharynx and/or externally through the skin of the neck along the anterior edge of the sternomastoid muscle. Cysts and associated sinuses may also become infected.

Management

These lesions are benign, however, can be symptomatic with recurrent infections and swelling. Symptomatic lesions can be surgically corrected.

Accessory Nipple

An additional nipple appearing anywhere along an imaginary line drawn from the midaxilla to the inguinal area. Nipples may appear unilaterally or bilaterally and with or without areolae.

Synonyms Supernumerary nipple, polythelia.

Epidemiology

Age Newborn.

Gender M = F.

Prevalence Common.

Etiology Developmental defect with persistence of the embryonic milkline.

Pathophysiology

Accessory nipples represent persistent, fetal, embryonic milkline lesions.

History

Lesions are present at birth, asymptomatic, and persist for life.

Physical Examination

Skin Findings

Type Papule with or without surrounding areola.

Color Pink to brown.

Size Usually smaller than normal anatomically placed nipples.

Shape Often round to oval in shape.

Distribution Chest, abdomen, thigh, vulva.

Sites of Predilection Along a line from the midaxillae to the inguinal area (Fig. 1-14).

Differential Diagnosis

When occurring without an areola, the nipple may be misdiagnosed as a congenital nevus.

Laboratory Examinations

Dermatopathology Skin biopsy would reveal lactation ducts and glands, histologic findings of a normal nipple.

Diagnosis

The diagnosis of an accessory nipple can be made clinically, especially if it is present along the anterior milkline. Other ectopically placed lesions may need a biopsy confirmation.

Course and Prognosis

Accessory nipples are present at birth and persist asymptomatically for life. Malignant degeneration is rare.

Management

Accessory nipples do not need treatment but should be monitored, since those with associated breast tissue can have the same malignant potentials as normal breast tissue. Surgical removal can be performed for diagnostic, cosmetic, or therapeutic purposes.

Neonatal Herpes Simplex Virus Infection

Neonatal herpes simplex infection is a potentially fatal disease. Neonatal herpes has a broad clinical spectrum and may be categorized into three patterns: localized infection confined to the skin, eyes, or mouth; CNS disease; and disseminated disease.

Insight

Many infants with CNS and disseminated neonatal herpes do not develop cutaneous lesions.

Epidemiology

Age Neonatal herpes can present up to 4 weeks of age. Disseminated disease often presents during the first week of life, while localized infection typically presents later. CNS infection presents between 14 and 21 days of life.

Gender M = F.

Prevalence Approximately 1 in 3000 live births.

Etiology 75% result from herpes simplex virus-2 (HSV-2) infection, 25% result from herpes simplex virus-1 (HSV-1) infection.

Transmission Herpes virus is most commonly inoculated onto the baby’s mucous membranes during its passage through the birth canal; however, ascending infection and transmission in the perinatal period from the mouth or hands of caregivers may occur.

Pathophysiology

Herpes virus inoculation occurs mucocutaneously; systemic infection can ensue with disease limited to the brain or diffuse hematogenous dissemination.

History

Pregnant women with recurrent genital herpes are at very low risk of transmitting HSV to their babies (<5%) because of anti-HSV antibodies, which pass through the placenta and convey immunity to the baby. The majority of cases occur in asymptomatic mothers with primary genital herpes infection making it very difficult to predict those at risk.

Physical Examination

Skin Findings

Type of Lesion Skin lesions begin as 2- to 8-mm macules or papules that progress to single or grouped vesicles that then rupture leaving an erosion or ulcer, which crusts over and heals (Fig. 1-15).

Color Pink to erythematous.

Shape Round to oval.

Distribution Oral lesions are most frequently located on the tongue, palate, gingiva, lips, and buccal mucosa. Ocular lesions appear as erosions on the conjuctiva and cornea. Skin lesions occur at sites of inoculation and infant monitoring scalp electrodes may produce enough skin trauma to allow invasion by the herpes virus. At birth in vertex deliveries, the scalp is a common site for the development of initial herpetic lesions and conversely, in breech deliveries, the buttocks and perianal area frequently manifest the first lesions. The vesicles may become generalized in the disseminated pattern of the disease.

General Findings

Early in the infection, infants typically present with nonspecific symptoms such as lethargy, poor feeding, and fever. CNS involvement is manifested by isolated encephalitis with frequent seizures that are nonfocal in nature. Eye involvement is seen in 10% to 20% of these patients and is noted characteristically between age 2 days and age 2 weeks of life. Disseminated infection presents with irritability, respiratory distress, jaundice, and seizures from viral infection of the brain, lungs, liver, and adrenal glands.

Differential Diagnosis

The diagnosis of neonatal herpes is made by history and clinical presentation and confirmed by detection of the herpes virus. The differential diagnosis includes other blistering diseases of the newborn such as congenital varicella, bullous impetigo, pemphigus vulgaris, and other causes of neonatal sepsis.

Laboratory Examinations

Tzanck Preparation Cells scraped from the base of vesicular lesions are positive for multinucleated giant cells in 60% of culture proven HSV.

Histopathology Intraepidermal vesicles produced by ballooning degeneration. Inclusion bodies (eosinophilic structures with a surrounding clear halo) are frequently seen in the center of enlarged, round nuclei of balloon cells.

Direct Immunofluorescence Cells scraped from the base of vesicular lesions can be tested for the presence of herpes virus infection using HSV-1 or HSV-2 specific monoclonal antibodies. Sensitivity and specificity correlate highly with tissue culture results.

Serology Antibody measurement in the baby is of little value because the seropositivity rate for HSV-2 among all women of childbearing age exceeds 20%.

Culture HSV may be cultured within 2 to 5 days from infected samples of skin, throat, conjuctiva, cerebrospinal fluid, blood, urine, and stool.

Polymerase Chain Reaction Herpes simplex DNA can be detected in the CSF using the polymerase chain reaction, an extremely sensitive technique that is frequently used for CSF evaluation.

Course and Prognosis

Morbidity and mortality of neonatal herpes is strongly associated with disease classification.

Localized Infection Treated infants with cutaneous infection have virtually a 100% survival rate, although some will suffer a cutaneous relapse in the first year of life. Approximately 10% will manifest herpetic lesions in the oropharynx. Up to 40% will have persistent ocular pathology, although this is seen most frequently in children suffering from severe neurologic residua (see below).

CNS Infection Untreated cases have a mortality rate of 50% and treated cases have a mortality rate of 15% to 30%. HSV-2 causes significantly worse encephalitis as compared to HSV-1. Many of these cases sustain permanent neurologic impairment.

Disseminated These neonates frequently progress to cardiovascular compromise, coagulopathy, and, in the absence of therapy, some 80% of cases result in death. With antiviral therapy, the mortality rate is reduced to approximately 25%.

Management

In light of the varied clinical manifestations of neonatal herpetic infections and the severe consequences of untreated disease, immediate institution of antiviral chemotherapy has been recommended for any infant presenting with signs of severe, unidentified infection in the first month of life, even in the absence of any characteristic skin findings.

Antiviral therapy of IV acyclovir 60 mg/kg divided into three equal doses and administered every 8 hours for 14 days if limited disease, 21 days if disseminated or involving CNS. If ocular involvement is present, addition of topical 1% trifluridine drops can be instituted. Infants with CNS or disseminated disease that have been treated successfully may require long-term suppressive antiviral therapy to prevent subclinical CNS recurrences.

Congenital Varicella Zoster Virus

The majority of infants exposed to varicella zoster virus (VZV) infection during pregnancy are asymptomatic and normal. There are two exceptions: congenital VZV syndrome and neonatal varicella.

Congenital VZV Syndrome Occurs if a mother is infected in the first 20 weeks of pregnancy and transplacental infection of fetus occurs before maternal immunity can protect the infant. This acquired infection leads to severe fetal sequelae and mortality in up to 30%.

Neonatal Varicella Occurs if the mother is infected VZV within three weeks of delivery. Since the infant is born before maternal antibodies have been generated, the infant has no immunity and will have a severe infection in the first 12 days of life with pneumonitis, hepatitis, meningoencephalitis, and up to a 20% mortality.

Epidemiology

Age Newborn.

Gender M = F.

Incidence Both congenital VZV and neonatal VZV are very rare.

Etiology VZV.

Transmission Varicella causes maternal viremia, which can cause transplacental infection; ascending infection from the birth canal and transmission in the perinatal period also may occur.

Physical Examination

Skin Findings

CongenitalVZV Syndrome

Type Cicatricial scars, skin loss, limb contractures.

Distribution Sometimes in a dermatomal pattern.

General Findings Low-birth-weight, eye defects, encephalomyelitis, hypoplastic limbs, microcephaly, pneumonitis.

Neonatal Varicella

Type Monomorphic progression from macule, papules, vesicles to crust (Fig. 1-16).

Color Erythematous.

Distribution Generalized.

General Findings Pneumonitis, hepatitis, and meningoencephalitis.

Differential Diagnosis

Congenital HSV, other TORCH (toxoplasmosis, other [syphilis], rubella, CMV, HSV) infections, sepsis.

Laboratory Examinations

Direct immunofluorescence (DFA), polymerase chain reaction, or skin biopsy of cutaneous lesions can demonstrate VZV. Cultures of tissue or CSF have not been shown to reliably grow VZV in infants with congenital VJV syndrome. IgG and IgM antibodies to VZV can be demonstrated in the mother’s sera to determine either previous immunity or active infection to confirm the findings and VZV-specific IgG antibodies in the baby that persist older than 7 months of life (by which point any maternal antibodies should have disappeared).

Diagnosis

Based on history or maternal infection, clinical appearance of the newborn, and laboratory findings.

Course and Prognosis

Infants in utero of mothers with VZV are generally protected by the transplacental acquired antibodies generated during maternal VZV infection. Thus, most infants are unaffected and born normal. These infants may develop unusual manifestations of attenuated immunity later in life such as herpes zoster at an early age. Such children are immunocompetent, but had in utero primary VZV infection (chicken pox) and an attenuated immune response since maternal antibodies were present to help fight the infection. Infants born to mothers who develop varicella 5 days prior to delivery to 2-day postpartum are at highest risk for a fatal course because of insufficient time for maternal antibody acquisition.

Congenital VZV syndrome has up to a 30% mortality rate but long-term outcome can be good for surviving patients.

Herpes zoster (shingles) in a pregnant woman is generally thought to have minimal risk for sequelae for the fetus.

Management

Those pregnant women who have been exposed to varicella should have a VZV-IgG level measured if they do not have a history of varicella infection or vaccination. For those with negative histories or serologies, varicella zoster immune globulin (VZIG) is indicated at any point during pregnancy. Administration of acyclovir should be given at the first clinical sign of VZV infection. For VZV infections between 5 days before and 2-day postpartum, mothers should be given acyclovir and an attempt to delay the delivery may be made. The neonates should be given VZIG and systemic acyclovir immediately upon delivery and should be isolated and observed for 2 weeks.

Blueberry Muffin Baby

The “blueberry muffin baby” is so-named because of the baby’s clinical presentation, with disseminated red–blue papules and nodules representing islands of extramedullary hematopoiesis. The blueberry muffin baby can be seen with in utero infections with toxoplasmosis, varicella, cytomegalovirus (CMV), human immunodeficiency virus (HIV), and rubella.

Epidemiology

Age Newborn.

Gender M = F.

Incidence Rare.

Etiology Maternal infection during pregnancy and transplacental infection with toxoplasmosis, varicella, CMV, HIV, or rubella.

Physical Examination

Skin Findings

Type Purpuric macules, papules, and nodules (Fig. 1-17).

Color Bluish-red to purple.

Size 2 to 8 mm in diameter.

Shape Circular to oval.

Palpation Infiltrated, larger lesions are palpable 1 to 2 mm above the skin’s surface.

Distribution Generalized.

General Findings

Toxoplasmosis Lymphadenopathy, hepatosplenomegaly, hydrocephaly, microcephaly, cataracts, pneumonitis, chorioretinitis, convulsions.

Varicella See “Congenital and Neonatal Varicella.”

CMV Jaundice, hepatosplenomegaly, anemia, thrombocytopenia, respiratory distress, convulsions, and chorioretinitis.

HIV Hepatosplenomegaly, other opportunistic infections.

Rubella Cataracts, deafness, growth retardation, hepatosplenomegaly, cardiac defects, and meningoencephalitis.

Differential Diagnosis

The differential diagnosis of a blueberry muffin baby is typically the infectious causes of extramedullary dermal hematopoiesis: toxoplasmosis, varicella, CMV, HIV, and rubella. The diagnosis is typically made by maternal history and detection of one of the above-named infectious agents.

Laboratory Examinations

Dermatopathology Blueberry muffin lesions show aggregates of large nucleated and nonnucleated erythrocytes.

Toxoplasmosis

DNA Detection of parasite DNA in amniotic fluid or fetal blood.

Serology Infant antitoxoplasma IgM higher than maternal IgM is diagnostic of congenital infection.

Wright’s or Giemsa Stain CSF or lymph node, spleen, or liver demonstrating Toxoplasma gondii.

Skull Films Diffuse, punctate, comma-shaped, intracranial calcifications.

CMV

Culture Urine, liver, CSF, gastric washing, and pharynx will show characteristic large cells with intranuclear and cytoplasmic inclusions.

Head CT scan Intracranial calcifications may be present.

Rubella

Serology Elevated antirubella IgM in infant or persistent antirubella IgG in infants older than 6 months. Fourfold or greater increase in acute and convalescent sera is also diagnostic.

Culture Virus can be isolated from nasopharynx, urine, CSF, skin, stool, or eyes.

Course and Prognosis

Toxoplasmosis Infected infants may be stillborn, premature, or full term. At birth, they may present with malaise, fever, rash, lymphadenopathy, hepatosplenomegaly, convulsions, and chorioretinitis, though most are asymptomatic. Overall prognosis is poor, especially with infants that have liver and bone marrow involvement.

CMV Affected infants may have sensorineural hearing loss, mental retardation, learning disabilities, and seizures. Poor prognosis, especially with intracranial calcifications or hydrocephalus.

Rubella Rubella acquired during the first trimester may lead to low-birth-weight, microcephaly, mental retardation, cataracts, deafness, and heart abnormalities. Rubella acquired during second or third trimester can lead to hepatosplenomegaly, pneumonitis, myocarditis, encephalitis, osteomyelitis, or retinopathy.

Management

Toxoplasmosis 30+ days of sulfadiazine (150–200 mg/kg/d divided qid) plus pyrimethamine (1–2 mg/kg/d divided bid). Infants with chorioretinitis or high CSF protein levels may need systemic steroids.

CMV Ganciclovir can be used to treat retinitis and organ involvement.

Rubella Immune globulin administered to exposed pregnant mother within 22 hours of onset can prevent/reduce fetal infection. Neonatal rubella requires ophthalmologic and supportive care. However, infected infants can shed the virus for up to a year.

Congenital Syphilis

A prenatal spirochetal infection with characteristic early and late signs and symptoms. Clinical manifestations of early congenital syphilis (first several months of life) include anemia, fever, wasting, hepatosplenomegaly, lymphadenopathy, rhinitis (snuffles), mucocutaneous lesions (including rhagades: radial fissures and furrows around the mouth), and pseudoparalysis. Symptoms of late congenital syphilis, which appears after 2 years of age, include interstitial keratitis, Hutchinson teeth (notched central incisors), and eighth nerve deafness; these three compose the Hutchinson triad. Frontal bossing, mulberry molars, saddle nose deformity, Clutton joints (painless knee swelling), and anterior bowing of the shins are also features of late congenital syphilis.

Synonym Prenatal syphilis.

Epidemiology

Age Within first months of life and after the age of 2.

Gender M = F.

Incidence Incidence had dropped to insignificant levels in 1959, but since then has been increasing.

Etiology Spirochete Treponema pallidum crosses the placenta to infect the fetus.

History

Neonates with syphilis are often born without signs of disease at time of birth. Clinical manifestations in first month of life are common. Skin findings include macules, papules, papulosquamous, and vesicobullous lesions. Widespread desquamation can occur (Fig. 1-18). Mucocutaneous involvement of mouth and lips is common. Systemically, there is lymphadenopathy, hepatosplenomegaly, and pseudoparalysis.

Physical Examination

Early Congenital Syphilis Signs and symptoms appearing in first 2 months of life.

Skin Findings

Noted in one-third to one-half of affected infants. Skin lesions are infectious.

Type Macules, papules, or papulosquamous lesions on body. Patches on mucous membranes, raised verrucous plaques on anogenital, or oral membranes.

Color Bright pink to erythematous; fades to copper-colored or brownish.

Size and Shape Large, round, or oval.

Distribution Generalized or localized to any area.

Sites of Predilection Face, dorsal surface of trunk and legs, diaper area, palms, and soles.

General Findings

Hepatomegaly, lymphadenopathy (especially epitrochlear), splenomegaly, jaundice, anemia, thrombocytopenia, osteochondritis, and meningitis. “Barber-pole umbilical cord” spiral red, white, and black discoloration of necrotizing umbilical cord.

Late Congenital Syphilis Signs and symptoms appearing after 2 years of age.

Skin Findings

Hypersensitivity type reaction or scars and deformities related to infection. Skin lesions no longer infectious.

General Findings

Hutchinson’s triad: (1) interstitial keratitis, (2) Hutchinson teeth, (3) eighth nerve deafness; dental changes (mulberry molars or bony gums that progress to necrotic ulcers), Higoumenakia sign (unilateral, thickened inner one-third of clavicle), arthritis, Parrot lines (radial scars after rhagades heal), and eye changes (choroiditis, retinitis, and optic atrophy).

Differential Diagnosis

The diagnosis of congenital syphilis can be made based on clinical suspicion and confirmed by positive dark-field microscopy or fluorescent antibody tests of the umbilical cord, placenta or skin lesions, by radiologic bony changes, and positive serology for syphilis. A serologic titer in the newborn at least fourfold higher than the mother is diagnostic of congenital syphilis.

Laboratory Examinations

Dark-Field Microscopy of the umbilical cord, skin lesions, or mucous membranes for T. pallidum.

Radiologic Studies for bone abnormalities: widening of the epiphyseal line with increased density of the shafts.

Placental Changes include focal villositis, endovascular and perivascular proliferation in villous vessels, and relative immaturity of villi.

Course and Prognosis

Relative to time during pregnancy of maternal spirochetemia and subsequent fetal inoculation. Up to 4 months of gestation, spontaneous abortion is common. Infection after 4 months of gestation may result in stillborn or fatally ill neonate. Prognosis with contracted disease is dependent on early and proper diagnosis and adequate treatment.

Management

Treatment without CNS involvement:

1. 1. Aqueous crystalline penicillin G: (100000– 150000 units/kg) IM or (50000 units/kg IV q 8–12 hour) × 14 days.

   2. Procaine penicillin G: (50000 units/kg IM q 24 hour × 10–14 days).

   3. Benzathine penicillin G: (50000 units/kg IM × 1 dose).

Treatment with CNS involvement:

1. 1. Crystalline penicillin G: (30000–50000 units/kg) divided bid–tid × 3 weeks.

   2. Procaine penicillin G: (50000 units/kg) qid × 3 weeks.

Subcutaneous Fat Necrosis

A benign, self-limited panniculitis in which subcutaneous fat is injured and becomes inflamed, presenting as a firm, erythematous nodule or plaque in an otherwise healthy newborn.

Insight

Frequently, these children are the product of long, difficult labor and delivery.

Epidemiology

Age First several weeks of life.

Gender M = F.

Prevalence Rare.

Etiology Cold injury, trauma at delivery, asphyxia, ischemia, and other neonatal stressors may initiate the disease.

Pathophysiology

The fat of neonates contain more saturated fatty acids, which have a higher melting point than adult fatty acids. Once the temperature of the skin drops below the melting point of the fat, crystallization occurs with subsequent necrosis and granulomatous inflammatory response. This feature may play a role in enhancing physical or metabolic trauma leading to necrosis.

History

Asymptomatic single or multiple red nodules or plaques that begin within the first 2 weeks of life and resolve spontaneously over several weeks.

Physical Examination

Skin Findings

Type of Lesion Sharply demarcated indurated nodules coalescing into larger plaques, occasionally ulcerated (Fig. 1-19).

Color Reddish to purple.

Palpation Firm to palpation.

Distribution Cheeks, buttocks, back, arms, or thighs.

General Findings

Infrequently, hypercalcemia can present with irritability, vomiting, weight loss, and failure to thrive.

Differential Diagnosis

Subcutaneous fat necrosis must be differentiated from bacterial cellulitis, sclerema neonatorum, and congenital tumors such as a sarcoma. Babies with subcutaneous fat necrosis generally appear healthy, they are afebrile and feeding vigorously which is helpful in eliminating cellulitis and sclerema neonatorum from the differential.

Laboratory Examinations

Dermatopathology Foci of fat necrosis often with needle-shaped clefts surrounded by granulomatous inflammation. Areas of calcification may be present.

Laboratory Examination of Blood Hypercalcemia may be found infrequently, usually around the time of resolution of the skin lesions. Hypoglycemia, thrombocytopenia, and hypertriglyceridemia may also occur.

Course and Prognosis

Lesions evolve slowly over weeks-to-months from red nodules, to bruiselike discoloration, to a hard subcutaneous mass that usually resolves without atrophy or scarring. In the case of hypercalcemia left untreated, seizures, cardiac arrhythmias, renal failure, and death may result.

Management

No treatment is necessary for these self-resolving lesions in general. Fluctuant lesions may be aspirated for comfort and to minimize overlying epidermal necrosis.

The calcium level should be monitored at least biweekly in these infants as the skin lesions resolve, since hypercalcemia must be treated aggressively.

Sclerema Neonatorum

A diffuse skin hardening seen in severely ill newborns with multiple etiologies.

Epidemiology

Age Newborns, premature infants are more susceptible.

Gender Sclerema occurs slightly more frequently in males.

Incidence Rare.

Etiology Sclerema can result from a number of physiologic insults and is a nonspecific sign of poor prognostic outcome rather than a primary disease. In 25% of affected infants, the mother is severely ill at the time of delivery.

Pathophysiology

The fat of neonates contain more saturated fatty acids, which have a higher melting point than adult fatty acids. Once the temperature of the skin drops below the melting point of the fat, crystallization occurs. Vascular collapse, hypothermia, and metabolic derangements seen in severe illness may induce crystallization.

History

Onset typically in first week of life in severely ill infants with sepsis, hypoglycemia, hypothermia, or severe metabolic abnormalities.

Physical Examination

Skin Findings

Type of Lesion Diffuse woodlike hardening and thickening of the skin .

Color Yellow–white mottled appearance.

Palpation Stony, hard, and cool.

Distribution Symmetrical, beginning on the legs and progressing upward to involve the buttocks and trunk.

General Findings

Infant is severely ill with an underlying medical condition such as sepsis, cardiac or respiratory problems, hypothermia, or metabolic abnormality. Infants are weak, lethargic, and feed poorly.

Differential Diagnosis

Clinically, the diffuse skin thickening and hardening are characteristic of sclerema. These skin changes are similar morphologically to scleroderma which is not seen in this context. Sclerema neonatorum may be confused with subcutaneous fat necrosis of the newborn. Several distinguishing features include: (1) appearance of the infant: in sclerema neonatorum, the infant appears extremely ill; in subcutaneous fat necrosis, the infant appears remarkably well; (2) distribution of the lesions: in sclerema neonatorum, the sclerosis is diffuse and in subcutaneous fat necrosis, the lesions are localized; (3) morphology of the lesions: in subcutaneous fat necrosis, there is often significant erythema of the nodules, which may be mobile, while sclerema tends to be more pale and bound down.

Laboratory Examinations

Dermatopathology Edema and thickening of fibrous septa surrounding fat lobules, occasionally with needlelike clefts within fat cells; usually less necrosis than seen in subcutaneous fat necrosis of the newborn.

Course and Prognosis

The prognosis of sclerema neonatorum is poor. By the time the skin is diffusely hardened, the infant is usually very ill with high morbidity and mortality.

Treatment and Prevention

Careful neonatal monitoring of premature infants with precise temperature control, appropriate antibiotic therapy, and correction of metabolic abnormalities, possibly with repeated exchange transfusions or systemic corticosteroids may arrest and reverse the process.