Linear IgA bullous dermatosis (LABD) is an immune-mediated subepidermal vesiculobullous eruption that occurs in both adults and children. It has been defined on the basis of a unique immunopathology consisting of a linear deposition of IgA along the basement membrane zone in perilesional skin biopsy specimens. The childhood form is also frequently termed “chronic bullous disease of childhood”, characterized by the peculiar clinical findings of annular blisters that develop predominantly in flexural areas, particularly the lower trunk, thigh, and groin. Differential diagnosis from DH and PB can be difficult clinically and requires typical DIF findings. The true incidence of LABD is unknown, but it is considered relatively rare. There appears to be a slight female preponderance, and in childhood occurs at a mean age of 4.5 years. We report on a 3-year-old girl, who presented with annular blisters and erosions affecting the genitalia, simulating an herpes simplex infection. Treatment with systemic antiviral were completely ineffective. Subsequently, scattered and asymmetric blisters with a “crown of jewels” feature appeared in the perioral region, neck, umbilicus, buttocks, thigh and malleolar surface of the legs. Lesions appeared both on erythematous and normal appearing skin. Histopathology showed an aspecific dermo-epidermal blistering, but direct immunofluorescence showed the characteristic positive linear IgA band at the basement membrane zone (BMZ). Complete hemato-chemical and instrumental examinations gave normal results, excluding associated pathologies. Treatment with dapsone 2 mg/Kg daily led to rapid improvement.
Linear IgA bullous dermatosis: a case description
ATZORI, LAURA;FERRELI, CATERINA
2004-01-01
Abstract
Linear IgA bullous dermatosis (LABD) is an immune-mediated subepidermal vesiculobullous eruption that occurs in both adults and children. It has been defined on the basis of a unique immunopathology consisting of a linear deposition of IgA along the basement membrane zone in perilesional skin biopsy specimens. The childhood form is also frequently termed “chronic bullous disease of childhood”, characterized by the peculiar clinical findings of annular blisters that develop predominantly in flexural areas, particularly the lower trunk, thigh, and groin. Differential diagnosis from DH and PB can be difficult clinically and requires typical DIF findings. The true incidence of LABD is unknown, but it is considered relatively rare. There appears to be a slight female preponderance, and in childhood occurs at a mean age of 4.5 years. We report on a 3-year-old girl, who presented with annular blisters and erosions affecting the genitalia, simulating an herpes simplex infection. Treatment with systemic antiviral were completely ineffective. Subsequently, scattered and asymmetric blisters with a “crown of jewels” feature appeared in the perioral region, neck, umbilicus, buttocks, thigh and malleolar surface of the legs. Lesions appeared both on erythematous and normal appearing skin. Histopathology showed an aspecific dermo-epidermal blistering, but direct immunofluorescence showed the characteristic positive linear IgA band at the basement membrane zone (BMZ). Complete hemato-chemical and instrumental examinations gave normal results, excluding associated pathologies. Treatment with dapsone 2 mg/Kg daily led to rapid improvement.
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