DESCRIPTION
A rare, chronic, intense and unremitting itching and burning, vesicular and bullous dermatosis associated with a gluten-sensitive enteropathy.

HISTORY

• Age of onset most often between the second and fifth decades.
• Rare in children.
• Prevalence 11-39 per 100 000.
• Affects males twice as often as females.
• Rare in black and Asian people.
• Increased incidence in association with human leukocyte antigens DRw3, B8, and DQw2.

PHYSICAL FINDINGS

• Most often clustered vesicles or excoriations, erythematous or urticarial papules, symmetrically distributed on elbows, knees, sacrum, and base of scalp. Less often generalized. Oral lesions rare.
• Gastrointestinal involvement usually asympto­matic. Severity of skin disease does not correlate with degree of intestinal involvement. Small bowel biopsy shows villous atrophy. Increased risk of small bowel lymphoma and non-intestinal lymphoma, which is reduced with gluten-free diet.
• Skin biopsy with immunofluorescence recommended for all blistering diseases; shows inflammatory infiltrate of neutrophils and occasional eosinophils in upper dermis.
• Direct immunofluorescence of skin biopsy from adjacent normal perilesional skin shows granular or fibrillar IgA deposits in dermal papillae in 90% of cases.

TREATMENT

• Dermatologists are trained well in bullous dis­orders and the biopsy techniques and tests required for accurate diagnosis.
• The goal of therapy is to arrest blister formation and relieve itching.
• Oral dapsone 100-150 mg every day relieves itching and burning within 48-72 h; maintenance dose varies in the range of 25-200 mg/day. Check glucose-6-phosphate dehydrogenase before starting dapsone.
• A gluten-free diet can control the disease alone or allow decreased requirement for oral medication.