Linear Iga Bullous Disease And Chronic Bullous Disease Of Childhood

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This is the same disease in different age groups. Clinical:

Linear IgA bullous disease is an acquired autoimmune blistering disease that may be idiopathic or drug induced. In children the disease is referred to as chronic bullous disease of childhood. Cutaneous lesions of linear IgA bullous disease (LABD) are usually nonscarring blisters, often extensive on trunk and extremities. They are characterized by the "cluster of jewels" sign, with vesicles and bullae at edges of polycylic lesions (Fig. 7A). Linear IgA bullous disease resembles dermatitis herpetiformis or bullous pem-phigoid, but is usually not symmetric. Patients with LABD may occasionally have ocular involvement that can be indistinguishable from mucous membrane pemphigoid. Chronic bullous disease of childhood has a predilection for vesicles and blisters in the diaper area.

Histology:

Linear IgA bullous disease and chronic bullous disease of childhood are similar (Figs. 7C-F).

■ Subepidermal bullae with eosinophil or neutrophil predominance (Fig. 7C)

■ Neutrophils lined up along the basement membrane zone—the best clue (Figs. 7D and E)

■ Neutrophilic microabscesses like dermatitis herpetifor-mis can be present (Figs. 7C and F)

■ Effacement of the epidermal architecture

Differential Diagnosis:

■ Bullous lupus erythematosus; bullous pemphigoid; dermatitis herpetiformis

Immunofluorescence:

■ DIF: linear IgA at the basement membrane zone (Fig. 7B)

■ IIF: circulating IgA antibodies that bind to monkey esophagus in 30% to 50% of patients

■ IIF-SSS: most circulating antibodies bind to the epidermal side of the split, but some bind to the dermal side, suggesting complex antigens

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