Unusual clinicopathological and immunological presentation of chronic bullous dermatosis of childhood (linear IgA dermatosis)

Abstract

Linear IgA bullous dermatosis is a rare sulfone-responsive subepidermal blistering disorder of unknown etiology in which smooth linear deposits of IgA are found in the basement membrane zone. Chronic bullous dermatosis of childhood is equivalent to linear IgA disease of adulthood and is characterized by an abrupt onset of large, widespread and tense bullae on a normal or erythematous base. In this case, we describe an unusual presentation of chronic bullous dermatosis in a 14-month-old Saudi girl. Histopathological examination revealed subepidermal cell poor blisters with linear deposition of IgA, IgG, IgM, and C3 along the dermoepidermal junction. The unusual clinical, histopathological and immunofluorescence findings in this patient are discussed, with an account on the differential diagnosis in such cases along with a detailed review of the relevant literature.

Keywords: Chronic bullous dermatosis of childhood; immunofluorescence; linear IgA dermatosis.

Figure 1

(a) Chronic bullous disease of childhood. Note the presence of vesicular and crusted skin lesions on the face. (b) Chronic bullous disease of childhood. Eroded and vesicular skin lesions on the external genitalia

Figure 2

(a) Chronic bullous dermatosis of childhood (linear IgA dermatosis). A “cell poor” subepidermal bullae is seen. (H/E stain ×200). (b) Chronic bullous dermatosis of childhood (linear IgA dermatosis). Direct immunofluorescence study showing strong linear deposits of IgA at the dermo-epidermal/basement membrane zone. (Direct IF ×200)