▶A rare immunobullous disorder in children.
▶Associated with celiac disease (ie, gluten-sensitive enteropathy) in 75% to 95% of affected patients.
▶Typically seen between 2 and 7 years of age.
▶Children diagnosed with celiac disease have circulating IgA antibodies to tissue transglutaminase and endomysium.
▶Characterized by intensely pruritic papulovesicular lesions (Figure 82.1) with a bilateral, symmetric distribution
▶Most often located on the extensor knees, elbows, sacrum, buttocks, posterior neck, scalp, and shoulders
▶Mucous membrane involvement usually absent
Look-alikes
Disorder | Differentiating Features |
•Bullae tend to be larger. •“Cluster of jewels” pattern (annular grouping of bullae) often noted. •Not as symmetric in distribution. •Direct immunofluorescence and immunoblotting studies help confirm diagnosis. | |
•Other features of systemic lupus erythematosus (SLE) usually present. •Concentrated in sun-exposed areas. •Bullae tend to be larger. •Antinuclear antibody (and other serologic studies) will confirm diagnosis of SLE. •Direct immunofluorescence and immunoblotting studies help confirm the diagnosis. | |
•Urticarial plaques present in addition to tense blisters. •Bullae tend to be larger. •Pruritus common with early lesions. •Direct immunofluorescence and immunoblotting studies help confirm the diagnosis. | |
Herpes simplex virus infection | •Most often clustered vesicles and erosions on an erythematous base. •Often occur inside or around the mouth. •Tend to be painful, less often pruritic. •More often focal. |
Arthropod bites/ papular urticaria | •Papules may have a central punctum on close inspection. •Usually concentrated on exposed areas of skin. •Linear groupings of papules may be observed. |
•Mixture of papules, linear burrows, and crusted papules. •Palm and sole lesions very common, as is involvement of the areolae and penis. •Other family members often report lesions or pruritus. •Mineral oil examination of skin scrapings confirms the diagnosis. | |
Pityriasis lichenoides et varioliformis acuta | •Scaly, red papules with necrotic surface changes. •Usually not pruritic. •Associated fever may be present. •Usually responds to treatment with oral erythromycin or tetracycline. |
Epidermolysis bullosa | •Inherited (not acquired) mechanobullous disease. •Usually begins in neonatal period or during early infancy. •Blisters induced by trauma. •Blisters usually larger than those seen in dermatitis herpetiformis (with exception of some simplex forms of epidermolysis bullosa). •Certain subtypes may reveal nail dystrophy, milia, extensive scarring, mitten-hand deformities. •Immunomapping, mutation analysis, or electron microscopy of skin biopsy specimens confirms diagnosis. |
•Acquired autoimmune blistering disease. •Blisters induced by trauma. •Scarring common. •Direct fluorescence and immunoblotting studies will help confirm diagnosis. •Blisters and erosions usually larger than those seen in dermatitis herpetiformis. |
▶The diagnosis is suggested clinically and confirmed by skin biopsy with immunofluorescence study, which reveals IgA at dermal papillary tips in a granular pattern.
▶Circulating serum antibodies to tissue transglutaminase or endomysium may be present (in gluten-sensitive patients).
▶Dapsone at 1 to 2 mg/kg/d is usually very effective (must first confirm normal glucose-6-phosphate dehydrogenase level and follow complete blood cell counts and liver function tests).
▶Sulfapyridine may be an effective alternative for therapy.
▶Gluten-free diet may be effective in certain patients, although challenging for children and parents.
Treating Associated Conditions
▶Patients with gluten sensitivity should be referred to an experienced gastroenterologist for baseline and follow-up care.
▶The prognosis for children with dermatitis herpetiformis is unpredictable.
▶Many recommend indefinite continuation of the gluten-free diet in gluten-sensitive individuals.