linear IgA bullous dermatosis
Pronunciation: LIN-ee-ar eye-jee-AY BUL-us der-mah-TOH-sis
A rare autoimmune blistering disease of childhood characterized by linear IgA deposition at the dermoepidermal junction.
Full Definition
Linear IgA bullous dermatosis is an uncommon autoimmune subepidermal blistering disease that predominantly affects children, with peak incidence in the first decade of life. The condition is characterized by linear deposition of IgA antibodies along the basement membrane zone, leading to tense bullae and vesicles often arranged in annular or polycyclic patterns. Clinical presentation may include the pathognomonic 'string of pearls' sign, where vesicles appear at the periphery of healing lesions. In pediatric dermatology practice, proper documentation includes lesion morphology, distribution pattern, and response to dapsone therapy, which is typically the first-line treatment. The condition often has a self-limited course in children.
Usage
Usage note: May be drug-induced in adults but typically idiopathic in children. Document 'string of pearls' sign if present.
In Context
- "Direct immunofluorescence showed linear IgA deposition confirming linear IgA bullous dermatosis." — dermatopathology report
- "The child's linear IgA bullous dermatosis responded well to dapsone with resolution of new blister formation." — pediatric immunodermatology follow-up