Mild potency topical steroids (eg, desonide and hydrocortisone valerate) to start with; moderate-potency topical steroids (eg, triamcinolone) for more severe disease; calcipotriene twice daily as a steroid-sparing agent or with topical steroids at the start; may need to consider the topical antifungal agent if secondary yeast infection is suspected, especially in intertriginous areas; infantile psoriasis is also often misdiagnosed as candidiasis, and appropriate therapy may be delayed; regular use of emollients to protect the skin. Topical calcineurin inhibitors (ie, tacrolimus and pimecrolimus) are off-label in children under 2 years of age but are safe and often helpful. If refractory to topical therapy, systemic medications (ie, methotrexate, acitretin, and cyclosporine) may be considered; may also refer to a dermatologist and/or a pediatric dermatologist. When topical and other systemic agents fail, biologic agents may be relatively safe and effective (TNF-α inhibitors). Difficult to diagnosis given the clinical overlap with other diseases. Severe disease and joint symptoms are rare. Manageable with topical steroid or nonsteroidal therapies alone. Triggers include trauma, certain drugs and specific infections, such as oral steroids, antimalarials, HIV, and streptococcus. Emphasize that positive family history is indicative of risk for progressive and persistent psoriasis. 7.1. Infantile psoriasis. Inherited and acquired forms have similar clinical features. Is this because of something I did during pregnancy? No, this is a genetic disorder. Will my child always have this problem? Maybe yes maybe no. The underlying gene defect is permanent, but treatment is easy, safe, and effective. 7.2. Acrodermatitis enteropathica. 7.3. Acrodermatitis enteropathica. Passive, transplacental transfer of maternal autoantibodies to the fetus. These antibodies are hypothesized to cause antibody-mediated cytotoxicity and the clinical features. Born to mothers with an underlying autoimmune, rheumatic, or connective tissue disease, most commonly Sjogren syndrome, rheumatoid arthritis, and lupus. ~50% of moms are asymptomatic and not aware of their underlying diagnosis.
CHAPTER
7
RED AND SCALY (PAPULOSQUAMOUS DISORDERS)
Infantile Psoriasis
Synonyms
Napkin psoriasis, napkin psoriasis with dissemination, pustular psoriasis, sebopsoriasis.
Inheritance
Strong familial association but no specific inheritance pattern.
Prenatal Diagnosis
n/a
Incidence
Up to 3 per 1,000 children in European studies, but poorly studied. More common in females than males.
Age at Presentation
First year of life.
Pathogenesis
Immune dysregulation, possibly perpetuated by T-helper 17 cells, causing the proliferation of keratinocytes and infiltration of epidermis with inflammatory cells (T cells and macrophages).
Key Features
Differential Diagnosis
Laboratory Data
Diagnosis is clinical; skin biopsy may be helpful in some cases when the diagnosis is not obvious.
Management
Prognosis
Variable; family history of psoriasis may predict more severe and lifelong course; occasional secondary candida infections; possible association with obesity and metabolic syndrome.
PEARL/WHAT PARENTS ASK
Skin
|
Associated Findings 
Acrodermatitis Enteropathica
Synonym
None.
Inheritance
Autosomal recessive: The SLC39A4 gene on 8q24.3 encoding Zip4, an intestinal zinc transport protein; also an acquired form.
Prenatal Diagnosis
Not available.
Incidence
1:500,000.
Age at Presentation
Infancy, sometimes as early as 3 to 4 months of age.
Pathogenesis
Poor zinc absorption in duodenum and jejunum. However, the specific pathology to explain skin findings is not known. For the acquired form, inadequate zinc intake, malabsorption owing to underlying GI/bowel disease. Excessive loss of urinary zinc in nephrotic syndrome, increased catabolic state, glucagonoma (necrolytic migratory erythema).
Key Features
Differential Diagnosis
Acrodermatitis acidemia, seborrheic dermatitis, other gastrointestinal diseases causing malabsorption, acquired zinc deficiency (low levels of zinc in breast milk), candidiasis, and atopic dermatitis.
Laboratory Data
No definitive diagnostic test; low plasma zinc levels (<50 µg/dL) taken prior to breakfast; alkaline phosphatase, a zinc-dependent enzyme, can be low in the presence of normal zinc levels.
Management
2 to 3 mg/kg zinc gluconate or sulfate orally daily; monitor the serum zinc level and alkaline phosphatase every 3 to 6 months; adjust dose as necessary; lifelong therapy for the inherited form of disease.
Prognosis
Excellent if treated; skin lesions respond within a week and resolve within 2 to 4 weeks, though sometimes may take longer; undiagnosed patients develop growth retardation, anemia, hypogonadism in males, delayed mental development, and delayed puberty.
PEARL/WHAT PARENTS ASK
Skin
|
Associated Findings 
Neonatal Lupus Erythematosus
Synonym(s)
Congenital lupus, neonatal lupus.
Inheritance
n/a
Prenatal Diagnosis
No definitive diagnostic test; maternal serum testing for anti-Ro/SSA, anti-La/SSB, and anti-U1RNP antibodies; frequent prenatal ultrasound monitoring of the fetal heart rate in at-risk mother; ultrasound, echocardiogram, if suspect cardiac involvement.
Incidence
Estimated 1:20,000 live births; cutaneous manifestations affect more females than males (3:1); cardiac manifestations also affect more girls than boys (2:1).
Age at Presentation
Birth to first few weeks of life.
Pathogenesis
Key Features
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