75: Rash and Dermatologic Problems

A nurse calls you to tell you that an infant has a rash. A rash is any change of skin that affects its color, appearance, or texture. Although the majority of rashes in newborns are benign and require no treatment, certain rashes require a workup and intervention.

1. What are the characteristics of the rash? Morphology of the lesion aids differential diagnosis. Is it macular (flat lesion <1 cm), papular (raised up to 1 cm), nodular (raised up to 2 cm), vesicular (raised, <1 cm, filled with clear fluid), bullous (raised, >1 cm, with clear fluid), or pustular (raised with purulent fluid)?

2. Are there petechiae (tiny pinpoint red dots from broken blood vessels), purpura (large, flat area of blood under tissue), or ecchymosis (very large bruised area)? All can result from intradermal bleeding and need to be differentiated from erythema (redness of the skin). With erythema, the redness is cleared when pressed and returns when you release. With pressure, petechiae, purpura, and ecchymosis do not blanch. Petechiae on the lower body after a breech delivery or upper body with a vertex presentation can be normal. If widespread, petechiae are considered abnormal. Petechiae and purpura can signify thrombocytopenia and require a workup.

3. Is there a history of a congenital infection? Obtain a thorough maternal history. TORCH (toxoplasmosis, other, rubella, cytomegalovirus, herpes simplex virus) infections are known to cause rashes. The “blueberry muffin baby” has widespread purpura and papules and can be seen in rubella (the term was first used to describe infants infected with rubella in the epidemic of 1960), cytomegalovirus (CMV; most common viral infection), and syphilis. Cutaneous extramedullary hematopoiesis causes the blueberry muffin rash. The blueberry muffin rash has historically been associated with congenital viral infections but can also be seen in blood disorders (hemolytic disease of the newborn, twin-to-twin transfusion, hereditary spherocytosis), vascular disorders (multiple hemangiomas, blue rubber bleb nevus, multiple glomangiomas, multifocal lymphangioendotheliomatosis), and malignancies (neuroblastoma, congenital leukemia cutis, Langerhans cell histiocytosis, and congenital rhabdomyosarcoma). See Chapter 141 and disease-specific chapters.

4. Is the infant ill appearing? A well infant with a rash suggests a benign rash. A febrile or ill-appearing infant with a rash requires a thorough workup to search specifically for an infectious cause.

5. What medications did the mother receive during pregnancy and delivery? Is the mother breast-feeding and taking any medications? Medications are a rare cause of rash in infants. Methimazole, an antithyroid medication, has been associated with aplasia cutis, a localized absence of skin on the scalp.

6. Does the skin lesion make you think of a genetic disorder? Skin lesions can be associated with genetic syndromes. Blisters: epidermolysis bullosa. Brown, flat patches: Neurofibromatosis. Cutis marmorata: Cornelia de Lange syndrome and in trisomies 18 (Edward syndrome) and 21 (Down syndrome). Deficient hair and nails: ectodermal dysplasias. Scaly, thick skin: ichthyoses. Thin, fragile skin: collagen disorders and hypoplasia of the dermis. Unformed skin: aplasia cutis congenita, Adams-Oliver and epidermolysis bullosa. White skin and hair: piebaldism, tuberous sclerosis.

1. Absence of part of the skin. Aplasia cutis congenita consists of 9 groups of disorders based on the location and presence of other malformations that involve absence of part of the skin. It can be localized (most commonly on the scalp) or involve a large portion of the body (Plate 3).

2. Skin and soft tissue infections in the neonatal intensive care unit (NICU). These are common because preterm infants have fragile skin. Types of infections seen are cellulitis, abrasions, and abscesses. These usually occur where the skin has been traumatized (diaper areas, surgical incision sites, fetal scalp electrodes, venipuncture sites, heelstick sites, and others). The most common organism is Staphylococcus aureus. Methicillin-resistant strains are becoming more frequent. Skin and soft tissue infections involving surgical procedures (gastrointestinal [GI] tract) are caused more commonly by gram-negative rods and yeasts. Omphalitis, an infection of the umbilical stump and the area around it, can also occur and is usually caused by bacteria (gram negative or gram positive).

3. Benign skin disorders/rashes that usually require no workup or intervention. These rashes are very common.

   1. Erythema toxicum. The most common newborn rash, it consists of erythematous macules with a central papule or pustule. It can be present at birth, typically appears within the first 48 hours, and may appear at up to 2 weeks of age. It is more common in full-term infants and more common on the trunk, extremities, and perineum. New lesions can appear after the initial onset and usually disappear after a week (Plate 4).

   2. Transient neonatal pustular melanosis. These 2- to 5-mm pustules are usually present at birth on various sites, typically the face and sacrum of full-term infants. Pustules evolve and disappear within 48 hours, but can leave hyperpigmented macules that eventually resolve but may persist for months (Plate 5).

   3. Sebaceous gland hyperplasia. Tiny yellow papules usually occurring on the cheeks and nose. Smaller and more yellow than milia.

   4. Milia. Tiny (1-mm) white-yellow papules frequently present on the face, chin, and forehead and scalp. Caused by sebaceous retention cysts (Plate 6).

   5. Miliaria crystallina. Obstruction of eccrine/sweat ducts. May appear on the scalp or face as vesicular or papular lesions with or without erythema. Exacerbated by heat and humidity, they resolve quickly when the infant is cooled.

   6. Miliaria rubra (“prickly heat”). One- to two-millimeter papules or papulopustules surrounded by small red areas. Miliaria pustulosis is a variant with more pustules and less erythema.

   7. Miliaria profunda. Deep obstruction of the sweat ducts. White papules and edema that can prevent sweating, leading to hyperthermia.

   8. Acropustulosis of infancy. Recurrent areas of pruritic vesicopustules develop on the palmar surface of the hand and plantar surface of the feet. Usually last 7–14 days (Plate 7).

   9. Seborrheic dermatitis. Patchy redness and scales on the scalp (“cradle cap”), face, skin folds, and behind the ears.

   10. Neonatal acne. Erythematous comedones, papules, and pustules. May be present at birth or develop during early infancy; may take several weeks/months for complete resolution (Plate 8).

   11. Sucking blister. Vesicular or bullous lesions present at birth on fingers, lips, or hands; no associated erythema (distinguishes these from herpetic lesions).

   12. Subcutaneous fat necrosis. Erythematous nodules and plaques occurring during the first few weeks of life and resolving by 2 months of age. Usually on areas of trauma (face, back, arms, legs, and buttocks). Hypercalcemia can occur if these lesions calcify (Plate 9).

   13. Nevus simplex (“stork bite,” “salmon patch,” “angel's kiss”). Pink macules that are dilated superficial capillaries, seen commonly at the nape of the neck, midforehead, and upper eyelids, but also on nose and upper lip, that usually fade within a year. The most common vascular malformation.

   14. Mongolian spot (dermal melanocytosis). A blue-black macular discoloration at the base of the spine and on the buttocks but can occur elsewhere. More common in blacks (>90%) and Asians (81%); usually fades over several years. Extensive spots have been described in infants with GM1 gangliosidosis (Plate 10).

   15. Harlequin sign (color change, not Harlequin fetus). Secondary to vasomotor instability, there is a sharp demarcation of blanching (one side of the body is red and the other is pale) that usually only lasts a few minutes.

   16. Infantile hemangioma. A common vascular tumor (3–5% of all infants). More common in girls and preterm infants.

   17. Benign petechiae. Nonblanching erythematous macules present on the lower body after a breech delivery or upper body with a vertex presentation.

4. Rashes caused by infections. These typically require intervention. The most common pathogens are S. aureus, Streptococcus spp., Candida albicans, and herpes simplex.

   1. Bacterial infections causing rashes

      1. Staphylococcus aureus

         1. Impetigo (nonbullous and bullous). Usually present with intact vesicles then intact pustules rupture, erode, and dry out to form crusts often secondary to an infected umbilical wound.

         2. Staphylococcal scalded skin syndrome (SSSS). Usually presents during the first week of life; starts with marked erythema of the face then spreads caudally and tends to be most severe in flexural areas. Lesions progress to bullae that are easily denuded (Plate 11).

      2. Cutaneous streptococcal infections. Uncommon, group A streptococcal infections can have an erysipelas-like eruption. Skin infections caused by group B are very rare. Vesiculopustular lesions, abscesses, and cellulitis (most common) have been reported.

      3. Syphilis. Usually macular or maculopapular eruptions (in congenital syphilis, infants present with vesicles, bullae, and erosions). Hemorrhagic bullae on the palms and soles are characteristic of syphilis.

      4. Listeria monocytogenes. Small (2- to 3-mm) cutaneous pinkish-gray granulomas are characteristic.

   2. Viral infections causing rashes

      1. Herpes simplex virus (HSV). (Plate 12) See also Chapter 96.

         1. Neonatal HSV. Transmitted perinatally and occurs in 3 forms. Erythematous papules or vesicles progress into pustular clusters with intense erythema.

         2. Congenital HSV. Acquired in utero and presents with vesicles that are apparent the first day of life. Typically associated with low birthweight, chorioretinitis, and microcephaly.

      2. Varicella-zoster. See also Chapter 147.

         1. Congenital/fetal varicella syndrome. Acquired in utero in the first/second trimester. Infant is born with cicatricial scars.

         2. Congenital/perinatal/early neonatal varicella. Acquired in utero late in third trimester or in the first few days postpartum. Infant presents with centripetal rash similar to postnatal rash in the first 10-12 days of life.

         3. Postnatally acquired chickenpox. No transplacental infection. Infant presents with symptoms on days 12–28 of life. Typical chicken pox rash with centripetal spreading first on trunk, then face and scalp. All stages appear (red macules, clear vesicles, crusting). (Image 13)

      3. CMV. Most common congenital infection. The majority of cases are asymptomatic. If symptomatic, they can have multi-organ disease with petechiae and purpura and jaundice.

      4. Rubella. Can have petechiae, but if severe, may have bluish red papules (“blueberry muffin”) that appear on the head and trunk and extremities.

   3. Fungal infections causing rashes

      1. Candida albicans. The most common fungal infection that causes problems in neonates is discussed here. Other species (Candida parapsilosis, Candida lusitaniae, Candida glabrata, etc.) are less common.

         1. Candida diaper dermatitis/oral candidiasis (thrush). Most common presentation of Candida infections in a normal infant. The diaper rash is usually erythematous with satellite pustules. Oral candidiasis can present with fussiness and refusal to take oral feedings, and is characterized by white patches in the oral cavity.

         2. Congenital candidiasis. Acquired in utero, infants can either have cutaneous or systemic disease. Congenital cutaneous candidiasis presents with an extensive rash within 12 hours of birth. The rash consists of erythematous macules, diffuse papules and pustules. Unlike erythema toxicum, this rash frequently involves the palms and soles. In term infants there is desquamation of these lesions, which may mimic SSSS. Premature infants may present with a rash with variable presentation (pustules, vesicles, or a burn-like dermatitis with peeling) (Plate 14). In congenital systemic candidiasis, majority of infants do not have a rash but can present with pneumonia, meningitis, or other presentations. This is a very invasive infection with a high mortality rate, especially in very low birthweight infants.

      2. Aspergillus. This has been reported to cause cutaneous fungal infections but is rare.

         1. Primary cutaneous aspergillosis (PCA). Characterized by lack of involvement of organs except the skin at the time of diagnosis. Preterm infants are at risk for PCA because of vulnerability of their skin and immature host defenses. Risk factors include prematurity, neutropenia, prior use of antibiotics, and glucocorticoid administration. A plaque with an eschar is characteristic of PCA. Disruption of the skin occurs due to contaminated hand splints, skin maceration caused by oximeter sensor, or adhesive tape.

         2. Secondary aspergillosis. Characterized by involvement of organs and a maculopapular eruption caused by thrombosis of small vessels.

   4. Parasitic infections that can cause a rash

      1. Scabies. An infestation with the mite Sarcoptes scabiei that has been reported in infants as young as 2 weeks. Infants tend to have widespread lesions often on the face and scalp (usually not seen in older patients who present with intertriginous locations). They can have papules, nodules, vesicles, and pustules.

      2. Toxoplasma gondii infections. Present with a generalized maculopapular rash.

5. Rashes that cause scaling. Usually benign and self-limited. Infectious and dietary etiologies need to be ruled out because they need immediate treatment. Genetic and immune etiologies can be considered later in the differential.

   1. Postmaturity. The majority of term and postmature infants normally shed their skin. The skin appears similar to parchment paper and peels off. This is a normal physiologic finding that requires no medical treatment.

   2. Seborrheic dermatitis. Usually seen on the scalp and flexure areas. It is a red erythematous rash with yellow scaling. Can be seen in the diaper area and is self-limiting.

   3. Fatty acid deficiency. Superficial scaling and desquamation can occur. Can be seen in fatty acid deficiency syndrome (where infants have decreased fat stores) or in fat malabsorption. Fatty acid replacement is necessary.

   4. Ichthyoses. May present as “harlequin fetus” or “collodion baby.” Many types of ichthyoses are present. These are genetic disorders that cause severe thick, scaly skin sometimes with restricted movement. The infant can have a shiny, tight membrane covering at birth that can peel off. The skin is prone to cracking and secondary infection. Some of the ichthyoses are associated with sensorineural hearing defects; others are associated with neurological problems including seizures (Plate 15).

   5. Infantile eczema. Scaling is present. Eczema is rarely seen in the newborn but is often seen in infants.

   6. Atopic dermatitis. Red scaly and itchy rash; rarely seen in the newborn.

   7. Staphylococcal scalded skin syndrome. See Infantile hemangioma.

   8. Psoriasis. Seen as a diaper rash with dissemination (scaly patches beyond the diaper rash) or can present as erythroderma progressing to pustular psoriasis.

   9. Candida infections. These can also cause redness and scaling (see earlier).

   10. Syphilis infections. See Infantile hemangioma.

   11. Ectodermal dysplasias. There are over 150 subtypes but the most common form is X-linked recessive hypohidrotic ectodermal dysplasia.

   12. Immunodeficiencies. Infants with immunodeficiencies (acquired immune deficiency syndrome [AIDS], severe combined immunodeficiency, diffuse cutaneous mastocytosis, Wiskott- Aldrich syndrome, Langerhans cell histiocytosis) can have a red scaly rash.

   13. Neonatal lupus. An immune-mediated disorder that is caused by maternally transferred autoantibodies (SSA/Ro, SSB/La, and/or U1RNP). Major manifestations are skin and cardiac (primary cause of congenital heart block). Skin findings are transient and usually start at a few weeks of age but can be present at birth. Two variants are papulosquamous and annular. Fifteen to twenty-five percent of cases have cutaneous manifestations. Typical rash is 0.5- to 3-cm annular erythematous papules with a central scale. Liver and hematologic involvement occurs in about 10% of cases (Plate 16).

6. Rashes that cause blisters and bullae. Infectious and dietary causes need to be ruled out because they require immediate treatment. Then less common causes can be evaluated.

   1. Epidermolysis bullosa. A group of inherited diseases that cause blistering. Trauma induces the blisters. Congenital localized absence of skin is often noted at birth (Plate 17).

   2. Zinc deficiency dermatosis. Presents as a blistering rash or more commonly an eczematous rash on the angle of the mouth, chin, or cheeks in a U-shaped distribution, and on the diaper area. It can be red and scaly, and can have a dark color at the periphery.

   3. Congenital herpes infection. See above.

   4. Staphylococcal scalded skin syndrome (SSSS). See above.

   5. Incontinentia pigmenti. A rare X-linked dominant genetic disorder that is more common in females. It has 4 stages: stage 1 occurs at birth to 2 weeks— vesiculobullous (erythematous vesicles/bullae that occur linearly on the trunk, extremities, and scalp); stage 2 occurs 2–3 weeks after birth—verrucous; stages 3 and 4 occur later. Infants can also have neurologic, dental, and ophthalmologic abnormalities. Note: Stage 1 lesions can be confused with herpes simplex infection (Plate 18).

   6. Less common causes. Genetic disorders, epidermolytic hyperkeratosis, toxic epidermal necrolysis, and bullous mastocytosis.

7. Birthmarks. The majority of birthmarks are benign and require no treatment. However, if there are many lesions present, this may signify an associated syndrome, or if very large they can be at risk for melanoma and have to be closely followed. Vascular lesions need to be evaluated as they may interfere with vital organs.

   1. Pigmented lesions

      1. Café-au-lait spots (macule). Benign lesions that are oval or irregular with a light brown color. If they are >5 mm in diameter and there are more than 6, suspect an associated syndrome (think neurofibromatosis, Albright syndrome, Turner and Noonan syndrome, tuberous sclerosis, ataxia-telangiectasia).

      2. Mongolian blue spot. See Infantile hemangioma.

      3. Congenital melanocytic nevus. Small (<1.5 cm) and intermediate (<20 cm) in size are common with a small risk of malignant melanoma. Large congenital nevi (>20 cm) and giant congenital nevi (>40 cm) have a higher risk of malignant transformation (5–15% risk). Giant congenital nevi also have a risk of neurocutaneous melanosis (Plate 19).

      4. Nevus of Ota/Ito. A blue or grayish area involving the orbital and zygomatic area common in Asians, and carries the risk of glaucoma. Unlike Mongolian spots, these do not fade.

   2. Diffuse hyperpigmentation. Not normal and can be secondary to Addison disease, biliary atresia, hepatic atresia, sprue, melanism, lentiginosis, porphyria, Hartnup disease, or idiopathic.

   3. Hypopigmentation/hypopigmented lesions

      1. Diffuse or localized loss of pigment. This can be secondary to phenylketonuria, Addison disease, trauma, postinflammation, or genetic.

      2. Ash-leaf macules. Small area of hypopigmentation, oval shaped and similar to the leaf of an ash tree; a marker for tuberous sclerosis.

      3. Hypomelanosis of Ito. Syndrome (primarily neurologic) with hypopigmented macule or linear/whorled pattern of hypopigmentation.

      4. Partial albinism (piebaldism). An autosomal dominant disorder of off-white macules on forehead, scalp, trunk, and extremities. This type of depigmentation can also be seen secondary to Addison disease, tuberous sclerosis, vitiligo, and Klein-Waardenburg syndrome.

      5. Albinism. Genetic disorders that cause abnormal melanin synthesis.

   4. Vascular lesions

      1. Port wine stain (nevus flammeus). This usually presents at birth on the face or extremities. It is a permanent capillary angioma. Rarely it is associated with Sturge-Weber syndrome, Klippel-Trenaunay syndrome, Parkes-Weber syndrome, or RASA-1 mutations (Plate 20).

      2. Nevus simplex/salmon patch. See Infantile hemangioma.

      3. Hemangiomas. Most common benign tumors of vascular endothelium of infancy and are more frequent in premature infants. Females are more likely to be affected, and they occur most commonly on the head and neck, followed by the trunk and limb.

         1. Superficial (“strawberry”) hemangioma. A bright red tumor (of dilated mass of capillaries) that protrudes above the skin and can appear anywhere on the body. Usually does not require treatment unless it interferes with vital functions.

         2. Deep (cavernous) hemangioma. More deep into the skin and bluish red; usually benign unless it interferes with vital organs.

         3. Mixed hemangioma. This has both deep and superficial components.

         4. Benign neonatal hemangiomatosis. Multiple congenital cutaneous hemangiomas that appear at birth or shortly after with spontaneous regression of lesions within the first 2 years of life.

         5. Diffuse neonatal hemangiomatosis. Multiple cutaneous hemangiomas plus hemangiomas of at least 3 separate organ systems. This disorder has a grave prognosis if not recognized and treated.

8. Rashes that cause petechiae/purpura. These may relate to birth trauma (considered “normal”) or if generalized and recurring can signify a serious infection or hematologic etiology that needs immediate evaluation and treatment.

   1. Birth trauma. Petechiae on the lower body after a breech delivery or on the upper body with a vertex presentation can be normal. They do not recur.

   2. Autoimmune disorders. Neonatal lupus with maternal transmission of autoantibodies.

   3. Thrombocytopenia. Usually generalized scattered petechiae, particularly in response to minor trauma.

   4. Neonatal alloimmune thrombocytopenia (NAIT). An isoimmune reaction that can cause thrombocytopenia and severe bleeding, including intracranial bleeding.

   5. Maternal idiopathic thrombocytopenic purpura. Approximately 80% of cases of thrombocytopenia are caused by autoimmune form.

   6. Coagulation factor deficiencies. Petechiae and purpura are more common in thrombocytopenia, with large ecchymoses, muscle hemorrhages, and bleeding from heelsticks, venipuncture, and other sites more common with coagulation defects.

   7. Sepsis/infection related. Usually caused by gram-negative bacterial sepsis (Escherichia coli, Pseudomonas). Listeriosis and aspergillosis also involved. Coxsackievirus can cause “blueberry muffin” lesions.

   8. Disseminated intravascular coagulation. Main causes are sepsis, birth asphyxia, necrotizing enterocolitis (NEC), and respiratory distress syndrome (RDS). One can see petechiae on the skin and bleeding can occur from venipuncture sites and the GI tract.

   9. Purpura fulminans. Symmetrical and well-defined lakes of confluent ecchymosis without petechiae, with sudden onset and development of hemorrhagic bullae and sudden death. Characteristic of meningococcal sepsis or other life-threatening infection.

   10. TORCH infections. Toxoplasmosis, other infections (syphilis), rubella, cytomegalovirus, and herpes can all cause “blueberry muffin” lesions (widespread papules and purpura) (Plate 21).

   11. Maternal drugs. Salicylates, steroids.

   12. Congenital leukemia cutis. A rare disorder. Cutaneous leukemic infiltrates occur in 25–30% of infants with congenital leukemia. Infants can present with petechiae, purpura, firm blue violaceous lesions, purple nodules, hepatosplenomegaly, lethargy, pallor, and fever. Chloromas (nodular infiltrations of the skin that are solid collections of myeloblasts) look like red-purple papules and nodules (blueberry muffin rash) (Plate 21). Congenital leukemia can be associated with Down, Edward, and Patau syndromes. Can be confused with benign hemangiomatosis.

1. History. Obtain thorough history from the mother and family, and a detailed history from the obstetric department. Is there a family history of skin disorders? Many skin disorders are genetic (ichthyoses, immunodeficiency, albinism). Asking about recent infections (varicella), early infections during the pregnancy (congenital infections), sexual history (syphilis), unusual travel destinations, and pet history (T. gondii) may provide some clues to the diagnosis. Does the mother have herpes? Did she have any unusual food products or undercooked food (Listeria)? What medications did the mother take?

2. Physical examination. Check vital signs. Is the infant febrile or does the infant appear sick, suggesting an infection? Do not just examine the presenting rash, but check the entire body to see if there are signs of the rash anywhere else. The distribution of the rash may be characteristic. Frequent follow-up examinations may document any progression or resolution of the lesions. Examine the eye to check for chorioretinitis (TORCH infections). Hepatosplenomegaly may be seen in congenital TORCH infections.

3. Laboratory studies

   1. Sepsis evaluation of labs if systemic infection suspected. Aspirate appropriate lesion fluid for bacterial culture, viral culture and polymerase chain reaction (PCR), and fungal cultures.

   2. If active bleeding suspected, send complete blood count with differential, platelet and reticulocyte count, TORCH titers, and sepsis workup.

   3. Potassium hydroxide prep if Candida/fungal infection suspected (reveals pseudohyphae).

   4. Wright stain of lesion fluid can show polymorphonuclear neutrophils with bullous impetigo and transient neonatal pustulosis and eosinophils with erythema toxicum. Wright stain of milia shows keratinocytes.

   5. Mineral oil prep can show mites and ova with scabies.

   6. PCR or direct fluorescent antibody (DFA) of cutaneous lesion if herpes suspected.

   7. Skin biopsy is sometimes indicated, especially if the lesion is atypical.

   8. Gram stain, culture of the purulent material of cellulitis/abscess. Gram stain of staph pustulosis will show gram-positive cocci and neutrophils.

   9. Maternal and neonatal autoantibodies for lupus.

   10. Coagulation studies. Platelet count, fibrinogen, prothrombin time (PT), and partial thromboplastin time (PTT).

   11. Molecular cytogenetic analysis of DNA of skin biopsy if congenital leukemia suspected.

4. Imaging and other studies (rarely needed)

   1. Computed tomography (CT) scan or contrast-enhanced magnetic resonance image (MRI) of the head to rule out calcifications if Sturge-Weber syndrome is suspected.

   2. Ultrasound, echocardiography, and/or CT scans may be needed to manage hemangiomas.

   3. Imaging studies may be necessary for larger defects of aplasia cutis congenita to rule out underlying bone or soft tissue defects.

As noted previously, the majority of rashes that occur in the neonate do not require treatment. Pediatric dermatology consultation is recommended for any unusual lesions. It is important to note that acyclovir is recommended early in cases of infants with a vesicular skin rash, even if the diagnosis of herpes is not confirmed.

1. Aplasia cutis congenita. Depending on the size and involvement, treatment includes medical (local includes cleaning, ointment, possible antibiotics), surgical (none if small; larger may require excision with closure, skin grafting, and other treatment), or both.

2. Skin and soft tissue infections (abscesses and/or cellulitis, omphalitis). Incision and drainage of abscess (if indicated) with local wound care. Send fluid for culture and sensitivity. An incision and drainage of the abscess may be adequate if the infant is not clinically ill. If the infant is clinically ill, a complete blood count (CBC) with differential and blood culture is usually done. Antibiotics (usually nafcillin) are started to cover for skin flora, unless methicillin-resistant S. aureus (MRSA) is suspected (use vancomycin). Lumbar puncture is done if the infant has any signs suspicious for meningitis. Infants with cellulitis, especially at IV sites, are usually treated with antibiotics based on culture or empirically with oxacillin or nafcillin and gentamicin. Omphalitis is treated with a full sepsis workup (including a lumbar puncture [LP]), and antibiotics (ampicillin and gentamicin) are started to cover both gram-positive and -negative organisms until culture and sensitivities come back.

3. Benign skin disorders. No treatment is necessary.

4. Rashes that are caused by infections. See specific infectious disease chapters for more details where appropriate.

   1. Staphylococcus aureus. Systemic antibiotics.

   2. Streptococcus. Cutaneous infection is usually group A, treated by penicillin.

   3. Syphilis. See Chapter 138.

   4. Listeria monocytogenes. Ampicillin and an aminoglycoside such as gentamicin (cephalosporins are not active).

   5. Herpes simplex. Acyclovir 60 mg/kg/d IV divided twice daily for 14 days for SEM (skin, eyes, and/or mouth) disease only if there is no central nervous system (CNS) or disseminated disease.

   6. Varicella. Acyclovir and VariZIG (see doses in Chapter 148) may decrease the severity of the course and improve outcome. Treatment must begin early in the course of the illness.

   7. Cytomegalovirus (CMV). Ganciclovir (Cytovene) in adults; limited data in pediatrics. Parenteral ganciclovir and oral valganciclovir are being used but are limited to use in those with symptomatic congenital CMV disease with CNS disease. See Chapter 90.

   8. Rubella. Supportive management. See Chapter 128.

   9. Candida albicans. Systemic antifungal medications such as amphotericin B to treat disseminated infection. Topical antifungals are used to treat isolated skin lesions. Thrush is treated by oral nystatin, 0.5 mL inside each cheek twice daily, for 10 days.

   10. Aspergillus. Amphotericin B deoxycholate in high doses.

   11. Scabies. Apply 5% permethrin cream topically. (Note: Permethrin is off label in neonates and may not be safe in very low birthweight infants.)

   12. Toxoplasmosis. See Chapter 142.

5. Rashes that cause scaling

   1. Postmaturity. No treatment is necessary.

   2. Seborrheic dermatitis. Usually resolves by 1 year; supportive care.

   3. Fatty acid deficiency. Replacement of fatty acids through IV lipid solutions or diet is necessary.

   4. Ichthyoses. Aggressive supportive care, close monitoring of fluid and electrolytes, and meticulous skin care with the goal of preventing infections. Collodion babies frequently have temperature instability and excessive fluid loss.

   5. Infantile eczema. Avoid any irritants; use protective creams such as zinc oxide in the diaper area. Short-term topical steroids may be needed.

   6. Atopic dermatitis. Emollients and mild topical steroids are used.

   7. Staphylococcal scalded skin syndrome. Antibiotics (IV penicillinase resistant, antistaphylococcal [eg, Cloxacillin]), supportive care, and attention to fluid and electrolyte management.

   8. Psoriasis. Topical steroids and sometimes wet dressings are used.

   9. Candida. See above.

   10. Syphilis. See Chapter 138.

   11. Ectodermal dysplasias. Supportive care with artificial tears.

   12. Immunodeficiencies. See specific entity.

   13. Neonatal lupus erythematosus. Thorough cardiac examination, liver function tests, and CBC. Apply protective sunscreen and avoid direct sunlight for 4 to 6 months. Topical steroids may be necessary.

6. Rashes that cause blisters and bullae

   1. Epidermolysis bullosa. Meticulous skin care, infection prevention, and special attention to nutrition and feedings because of the problems with dysphagia from scarring.

   2. Zinc deficiency dermatosis. Zinc supplementation.

   3. Congenital herpes. See Chapter 96.

   4. Staphylococcal scalded skin syndrome. See Section V.D.1.

   5. Incontinentia pigmenti. No specific treatment for the cutaneous lesions.

7. Birthmarks

   1. Hyperpigmented lesions. Most of these lesions require no treatment. If the lesions are large (>3 cm), removal is recommended. Giant hairy nevi are removed to prevent cancer development.

   2. Hypopigmented lesions. Piebaldism is treated with cosmetic camouflage. Those with albinism should follow sun restriction guidelines and sunblock.

   3. Vascular lesions. Many hemangiomas can be closely watched and left to regress. If treatment is necessary, steroid therapy, embolization, excision, occlusion, laser therapy, α-interferon, and radiotherapy are options. Port wine stains can be treated with laser therapy. Need to differentiate benign neonatal hemangiomatosis from diffuse neonatal hemangiomatosis as treatment is different.

8. Rashes that cause petechiae and purpura. These rashes usually require a thorough workup and treatment if there is a bleeding disorder. Infections are treated with antibiotics or antiviral agents. Platelet transfusion and replacement of coagulation factors may be necessary.

   1. Birth trauma. No treatment is necessary.

   2. Autoimmune disorders such as maternal or neonatal lupus. Cardiology examination to look for heart block is indicated.

   3. Thrombocytopenia, neonatal isoimmune thrombocytopenia (NAIT)/maternal idiopathic thrombocytopenic purpura (ITTP). See Chapter 139.

   4. Coagulation factor deficiencies. See Chapter 87.

   5. Sepsis/TORCH infections. See Chapters 130 and 141.

   6. Disseminated intravascular coagulation (DIC). See Chapter 87.

   7. Purpura fulminans. Treat the underlying infection.

   8. Congenital leukemia. The optimum treatment plan is unclear. Treatment consists of intensive combination therapy plus supportive care.