Psoriasis can first manifest itself as a recalcitrant diaper
rash, and should be considered if conventional diaper rash remedies
are not effective. Other stigmata of early psoriasis include seborrhea, nail
pitting, and intergluteal erythema. A family history of psoriasis
can also suggest this diagnosis.
Age Any age, typically seen first
in the diaper area of children younger than 2 years.
Genetics Possible autosomal dominant
inheritance with incomplete penetrance. Associated with HLA B13,
HLA B17, HLA Bw16, HLA B37, and HLA Cw6.
In normal skin, the cells mature, shed, and are replaced every
3 to 4 weeks. In psoriasis, there is shortening of the cell cycle
to 3 to 4 days. This leads to increased epidermal cell turnover
with decreased shedding and, hence, the accumulation of dead cells
as layers of silvery-white scale.
Type Scattered erythematous papules,
may coalesce into a well-delineated erythematous plaque.
Color Dark-red plaques, may have
a silvery mica-like scale.
Size Pinpoint to several centimeters.
Distribution Anogenital area, may
also involve intergluteal cleft, umbilicus, behind or inside the
ears, scalp, extremities (Fig. 3-2).
Psoriasis in the diaper area Well-delineated
bright-red erythematous plaques in the diaper area and on the extremities
of a child.
Nails May have pinpoint pits indicative
The well-delineated bright-red plaque and silvery mica-like scale
is characteristic of psoriasis. Removal of the scale may result
in punctate bleeding (Auspitz sign).
Dermatopathology Epidermal thickening
with edema of dermal papillae, thinning of suprapapillary area.
Chronic course with remissions and exacerbations, very unpredictable.
Some children progress to mild disease with intermittent exacerbations.
Other children have a more severe course with recurrent flares,
and 5% develop an associated arthritis.
Psoriasis in the diaper area can be both refractory and/or
1. Emollients such as petrolatum, moisturizers, or
diaper creams can help minimize flares and reduce itching.
2. Allowing the diaper area to be exposed to air for short periods
can also help reduce the chafing that can exacerbate psoriasis (Koebner
3. Sunlight exposure is thought to help psoriasis, but sunburns
should be avoided.
4. Topical tar preparations are anti-inflammatory and can be
used in the bath water (Balnetar or Polytar) or in creams (MG217
or Elta tar creams). Prolonged use of these agents is not recommended.
5. More severe cases may require short courses of mild topical
steroids (hydrocortisone 2.5%, desonide 0.05% cream
or ointment). It is important to use steroid in the diaper area
sparingly since the occlusive diaper increases the steroid potency
as well as the risk of steroid side effects such as skin thinning
Commonly overlooked, Candida diaper dermatitis should be suspected
whenever a diaper rash fails to respond to conventional treatment,
especially following a course of systemic antibiotic therapy. The
infection is propagated by the chronic occlusive state of diapers
worn during infancy.
Synonym Monilial diaper dermatitis.
Age Any diaper wearing stage (approximately
infancy to 3 years of age).
Etiology C. albicans organisms
are harbored in the lower intestinal tract of the infant. Upon defecation, infected
feces introduce yeast into area. The moist occlusive diaper environment
favors candidal overgrowth and leads to a rash.
C. albicans in mouth or GI tract of infant can proliferate in
moist diaper environment. Predisposing factors such as systemic
antibiotics can also contribute to Candida overgrowth.
Candidal overgrowth is seen most frequently following systemic
antibiotic therapy. Lesions appear first in the perianal area and
then spread to perineum and inguinal creases. The diaper rash may
occur in conjunction with oral thrush.
Type Erythema with fragile satellite
pustules (Fig. 3-3). Rash involving perineum is sharply demarcated
with elevated rim and variable scaling along the border. Pinpoint
satellite vesicopustules often present.
Primary candidal infection in
the diaper area Beefy red plaque in the diaper area surrounded
by characteristic satellite pustules.
Color Beefy red erythematous base.
Distribution Genitocrural area,
buttocks, lower abdomen, and inner aspects of the thighs, does not
Diagnosis Observation of beefy
red rash with vesicopustular satellite lesions is diagnostic. Scrapings
and cultures can be confirmatory.
Differential Diagnosis Primary
candidal infection in the diaper area has characteristic satellite
papules and pustules that are virtually diagnostic. It may be confused
with other recalcitrant rashes in the diaper area such as psoriasis,
AE, or histiocytosis X. Most commonly, it can be seen in conjunction
with other diaper rashes as a secondary infection.
Microscopic examination of skin scrapings with potassium hydroxide,
Gram stain or periodic acid-Schiff, demonstrate budding yeasts and
hyphae or pseudohyphae. Lesions may be cultured on Sabouraud’s
or Nickerson’s medium. White mucoid colonies grow within
48 to 72 hours.
Rash progresses until Candida is treated. Area may become eroded
1. Topical nystatin (Mycostatin) or clotrimazole (Lotrimin)
creams tid to area will clear rash. Care should be taken to avoid
anticandidal preparations that are mixed with cortisones (Lotrisone, Vytone)
since these may be too strong and result in unwanted steroid side
effects in the occluded diaper area against baby skin.
2. Oral nystatin suspension (nystatin swish and swallow, 1 to
3 mL po qid) can be applied to the affected mouth areas to treat
oral thrush or gastrointestinal candidal overgrowth. This will also reduce
chance of candidal recurrence in the diaper area.
AE is a hereditary or acquired clinical syndrome caused by zinc
deficiency. It is characterized by an acral, vesiculobullous eczematous
dermatitis characteristically distributed on the face, hands, feet,
and anogenital area.
Enteropathica Is Suspected, an Alkaline Phosphatase Level Can Be
Helpful; This Zinc-Dependent Enzyme Is Often Low in Such Patients.
Hereditary In infants given bovine
milk: days to few weeks after birth. In breast-fed infants: soon
Acquired Older children and adults
with illness that depletes zinc supply.
Etiology Hereditary or acquired
deficiency in zinc.
Hereditary Autosomal recessively
inherited disorder in zinc absorption thought to be secondary to
abnormal zinc-binding ligands. Zinc in human milk appears to be
more bioavailable to infants than zinc from bovine milk.
Acquired Long-term zinc deficiency
can be seen in patients with parenteral nutrition lacking zinc,
bowel bypass syndrome, Crohn’s disease, HIV, cystic fibrosis,
alcoholism, vegetarian diets, essential fatty acid deficiencies,
and other syndromes.
Low serum zinc levels in infancy or childhood leads to AE. The
basic defect is a GI malabsorption of zinc. Hereditary AE may be
secondary to an abnormal zinc-binding ligand. Acquired AE can be
seen with any systemic disease with GI malabsorption of zinc. The
major function of zinc is to be incorporated into enzymes, and there
are more than 200 zinc metalloenzymes in the body.
Review of systems may include diarrhea, weight loss, listlessness,
and behavioral changes (irritability, crying, and restlessness).
Type of Lesion Eczematous or psoriasiform
plaques may progress to vesiculobullae or erosions that crust and become
dry (Fig. 3-4A and B).
A. Refractory erythematous, scaly macerated plaque in the diaper
area. B. Erythematous and eroded plaques
in the perioral region.
Distribution Perioral and symmetrically
located on buttocks, extensor surfaces (elbows and knees), and acral areas
(fingers and toes).
Diarrhea, cachexia, alopecia, nail dystrophy, glossitis, stomatitis,
hypogeusia (decreased sense of taste), photophobia, drooling, and
growth retardation. Hypogonadism in males (more evident in adolescence).
Low serum zinc (<50 μg/dL) or alkaline phosphatase
levels. “Zebra striped” light banding of hair can
be seen with polarized light microscopy. However, zinc levels may
fluctuate with stress or infection.
The differential diagnosis includes other bullous diseases (such
as linear IgA chronic bullous disease of childhood and bullous impetigo),
psoriasis, histiocytosis X, and candidal infection. Refractory diaper
dermatitis associated with periorificial and acral findings should
lead the clinician to suspect AE.
If unrecognized, AE has a relentlessly progressive course leading
to infection and disability. Once recognized and treated, AE manifestations
are quickly corrected and reversed.
1. Topical zinc creams (Desitin, A&D ointment,
zinc oxide paste, and Triple Paste) can begin to improve the rash.
2. Dietary supplement of zinc gluconate, acetate, or sulfate
(5 mg/kg/d divided bid/tid) is usually curative.
The RDA (recommended daily allowance) of zinc is 15 mg/d.
3. In severe cases, ZnCl2 may be administered intravenously.
Granuloma gluteale infantum is a benign rash in the diaper area
characterized by reddish-purple granulomatous nodules. The disorder
represents a cutaneous response to a foreign body (talc or zirconium),
topical steroids, infection (candida), or inflammation.
Synonyms Kaposi sarcoma-like granuloma
and granuloma intertriginosum infantum.
Age Any diaper wearing age, typically
birth to 3 years.
Etiology Initiating inflammatory
process with maceration and secondary infection leads to a granulomatous
Lesions believed to be a unique benign granulomatous response
to a foreign body, inflammation, maceration, and/or secondary
Lesions appear in the diaper area several months after treatment
of inciting factors. The rash symptoms can range from being asymptomatic
to very painful.
Type Granulomatous papules and
nodules (Fig. 3-5).
Granuloma gluteale infantum Nodular
granulomatous response in the diaper area to topical steroids.
Size 0.5 to 4.0 cm in diameter.
Distribution Groin, buttocks, lower
aspect of abdomen, penis, rarely intertriginous areas of axillae,
The papular and nodular lesions of granuloma gluteale infantum
can appear very worrisome despite their benign nature because similar
lesions can also be seen in sarcomas and lymphomas. Similar lesions
may be found in Kaposi sarcoma, tuberculosis, syphilis, and deep
fungal infections. The diagnosis of granuloma gluteale infantum
is often made clinically and can be confirmed by skin biopsy.
Light and Electron Microscopy Hyperplastic
epidermis with inflammatory cells (mostly neutrophils), a parakeratotic
stratum corneum, and a dense inflammatory infiltrate throughout
the depth of the cutis with hemorrhage, neutrophils, lymphocytes,
histiocytes, plasma cells, eosinophils, newly formed capillaries,
and giant cells. Lesions of granuloma gluteale infantum lack the
masses of patchy accumulations of lymphoma cells seen in lymphomatous
disorders. Granuloma gluteale infantum may be differentiated from
more severe granulomatous processes by its lack of fibrous proliferative
features, spindle cell formations, and mitoses.
Benign. Lesions resolve completely and spontaneously several
months after treatment of the primary inciting inflammatory process
Treatment begins with the identification and elimination of the
primary inciting inflammatory process and/or infection.
1. If topical steroids are being used, discontinuing
them often leads to resolution of the nodules.
2. Conversely, if topical steroids are not being used, a short
2-week trial of topical, intralesional, or impregnated steroid tape
(Cordran tape) may hasten resolution of these nodules. Close follow-up
is necessary because steroids may worsen the condition.
Histiocytosis in Diaper Area
Langerhans cell histiocytosis (LCH) is a rare proliferative disorder
of the Langerhans cell (a histiocyte that migrates throughout the
skin as an antigen-presenting cell). Although the disease is rare,
it frequently presents in infancy as a rash in the diaper area.
LCH should be considered if a diaper rash is particularly recalcitrant
to usual remedies, especially if systemic symptoms are present.
In refractory diaper dermatitis with petechiae or purpura, Langerhans
cell histiocytosis must be excluded.
Synonym Histiocytosis X, Langerhans
cell granulomatosis, type II histiocytosis, LCH
Age Usually in first year of life.
Can be seen up to 3 years of age.
Gender M > F in children.
Incidence Extremely rare, 1 per 5 million children annually.
Genetics Possibly autosomal recessive
with reduced penetrance.
Unclear. Several proposed mechanisms include a disturbance of
intracellular lipid metabolism, a reactive histiocytic response
to infection, or a neoplastic process. Some cases may be hereditary. All
theories somehow implicate the Langerhans cell as a primary component.
Type Begins as erythema and scale
and progresses to purpuric papules, nodules, or vesicles (Fig. 3-6).
Langerhans cell histiocytosis
in the diaper area Refractory diaper rash with scattered erythematous
papules and a petechial component. Skin biopsy revealed Langerhans
cells, which confirmed the diagnosis.
Color Reddish-brown or purple.
Distribution Inguinal and perineal
areas, axilla, behind ears, and scalp, can be on the trunk.
Decreased immunity can lead to increased susceptibility to infections.
Severe forms of the disease can have cell infiltrates of the bone,
lung, liver, and lymph nodes
Dermatopathology Skin biopsy will
reveal characteristic Langerhans cells, which are diagnostic of
a histiocyte proliferative disorder. Langerhans cells can be identified
with positive S-100 and CD1a stains, which will be positive, or
electron microscopy demonstration of Birbeck granules in the cytoplasm
of the histiocytes.
The initial presentation of histiocytosis X is similar to seborrhea,
with an erythematous scaly rash, but histiocytosis X becomes more
extensive cutaneously and can have associated systemic disease.
A skin biopsy is usually needed to confirm the diagnosis.
Langerhans cell histiocytosis constitutes a spectrum of disease.
The highest mortality rate (38%–54%)
is seen in the most severe form: Letterer-Siwe disease (associated
with fever, anemia, thrombocytopenia, lymphadenopathy, hepatosplenomegaly,
and skeletal tumors). Less severe clinical courses are seen with
Hand-Schuller-Christian disease (associated with osteolytic defects, diabetes
mellitus, and exophthalmos). Eosinophilic granuloma (associated
with osteolytic defects and spontaneous fractures) and Hashimoto-Pritzker
disease (aka congenital self-healing reticulohistiocytosis; a benign,
self-limited entity with skin lesions only) represent the mildest
The diagnosis of Langerhans cell histiocytosis is important to
recognize when an ill infant presents with a refractory or recurrent
diaper rash. The diagnosis should be entertained and confirmed by
skin biopsy at an early age. For localized skin disease, topical
corticosteroids, topical antibiotics, PUVA, and topical nitrogen
mustard may be used. For noncutaneous lesions, more supportive and
aggressive therapies may be needed (see Section 20, “Langerhans
Cell Histiocytosis” for more details).