TABLE 67.3 AUTOIMMUNE BULLOUS DISORDERS OF CHILDHOOD Chronic bullous disease of childhood Type of lesions Distribution Bullous pemphigoid Large, tense, clear Large, tense Grouped bullae; annular bullae papulovesicles, plaques with bullae, or active vesicular urticarial borders lesions Scalp, lower Trunk and flexor Back, buttocks, trunk, genitals, surfaces of scalp, extensor buttocks, inner extremities surface of thighs extremities, often symmetric None to severe Mild Intense Occasionally Yes No Pruritus Mucous membrane involvement Duration Months to years Months to years Immunofluorescence + or − + Linear IgA Linear IgG on basement basement membrane (+ membrane (+ circulating IgA) circulating IgG) Treatment Dermatitis herpetiformis Dapsone > Corticosteroids Corticosteroids Months to years + Granular IgA at tips of dermal papilla of uninvolved perilesional skin Dapsone > Sulfapyridine Diagnosis is confirmed by biopsy of a papule or vesicle showing a predominately neutrophilic infiltrate focused at the tip of the dermal papillae DIF reveals granular IgA deposition at the dermal papillae Diagnosis may also be made by circulating serum IgA antibody to tissue transglutaminase in the blood IgA levels should be checked as IgA deficiency may lead to a false-negative test