Keep an open mind when considering skin signs and symptoms in children because dermatologic conditions may be a manifestation of systemic disease, according to Dr. Lawrence Eichenfield.
Recognizing the chronic nature of some of these conditions, and employing a collaborative approach when necessary, can substantially streamline management, Dr. Eichenfield reported.
Henoch-Schönlein
Dr. Eichenfield described Henoch-Schönlein purpura, a type of vasculitis that occurs most frequently in children and young adults and is characterized by abdominal cramps, hematuria, and a purpuric rash mainly on the lower legs and buttocks.
In addition to the skin and the gastrointestinal tract, the joints and kidneys are also end-organ targets, noted Dr. Eichenfield, professor of pediatrics and dermatology at the University of California, San Diego, at the women's and pediatric dermatology seminar sponsored by Skin Disease Education Foundation (SDEF).
Arthralgia or arthritis—most commonly in the knees and ankles but also in the wrists, elbows, fingers, and toes—is reported in 65%–85% of the cases, and sometimes precedes the rash. Renal involvement has been estimated to occur in 10%–60% of cases, typically within the first month (and often within the first week) of the appearance of the rash, according to Dr. Eichenfield.
Because of the risk of nephritis, urinalysis is a key consideration in the management of Henoch-Schönlein purpura. A negative urinalysis during week 1 should be followed by repeat tests for up to 6 months, based on the literature, he added.
The clinical course averages about 4 weeks. Treatment can include NSAIDs for the joint symptoms and corticosteroids, although the use of the latter for this indication remains controversial, he noted, referring to data in the literature demonstrating the efficacy of prednisone for reducing abdominal and joint pain and for the treatment, but not prevention, of renal involvement.
Juvenile Dermatomyositis
The cutaneous findings of juvenile dermatomyositis, which affects 3,000–5,000 children in the United States, include Gottron's papules; heliotrope rash; periungual telangiectasias; photosensitivity or scaly erythema of the face, neck, anterior chest, or shawl area; calcinosis cutis; pruritic eruption on exposed surfaces; and scaly scalp or diffuse hair loss.
These symptoms provide a window into autoimmune vasculopathy, Dr. Eichenfield said.
The disease is typically associated with progressive symmetric involvement of the proximal musculature. Gowers' sign, difficulty climbing stairs, arising from chairs, and combing hair—as well as muscle enzyme elevations, arrhythmia, dyspnea, dysphonia, and dysphagia—may be observed and should be assessed in the review of systems.
Neonatal Lupus Erythematosus
Dr. Eichenfield also described neonatal lupus erythematosus, which typically presents within the first 12 weeks of an infant's life with scaling and facial eruptions that may be mistaken for tinea.
Because of the associated cardiac, hepatic, hematologic, and central nervous system considerations, a full work-up for accurate diagnosis is essential.
The work-up should include a head circumference measure; serological testing for renal function, cytopenia, liver enzyme elevations, antinuclear antibody, anti-dsDNA, anti-Sm, anti-RNP, anti-Ro/SS-A, and anti-La/SS-B; electrocardiography; and, potentially, a skin biopsy, he noted. Examples of other systemic conditions that may present with skin signs in children and young adults include minocycline-induced autoimmunity, psoriasis, and pediatric morphea, according to Dr. Eichenfield.
He reported that he did not have any conflicts of interest in regards to his presentation. SDEF and this news organization are owned by Elsevier.
This CT image shows extensive soft-tissue calcifications in dermatomyo-sitis, largely in the subcutan-eous gluteal fat (arrowheads) in this 19-year-old female with severe multisystemic involvement.
Source ©Elsevier
Arthritis or arthralgia occurs in up to 85% of Henoch-Schönlein cases, renal damage in up to 60%.
Source Dr. Eichenfield
Barium enema postevacuation film shows marginal filling defects in the distal ileum in Henoch-Schönlein.
Source ©Elsevier