Chronic Bullous Disease or Linear IgA Dermatosis of Childhood -Revisited

Abstract

Linear IgA dermatosis or chronic bullous disease of childhood (CBDC) is a nonhereditary, autoimmune subepidermal bullous disease, characterized by the presence of continuous linear deposits of IgA autoantibodies along the basement membrane zone. Antigens mainly involved in the pathogenesis of CBDC are a 97-kDa and a 120 -kDa protein, which represent fragments of the extracellular domain of collagen XVII (BP180), a transmembrane protein playing a critical role in maintaining the linkage between the intracellular and the extracellular structural elements involved in epidermal adhesion. Humoral and cellular response contributes to the pathogenetic mechanism leading to blister formation. CBDC is characterized by a clinical polymorphism in each patient, with the "cluster of jewels" pattern being the most typical clinical lesion. For the establishment of CBDC diagnosis, combination of histology and immunofluorescence studies are of utmost importance. The majority of patients respond to dapsone, sulfapiridine or systemic steroids in cases of widespread disease. CBDC tends to resolve spontaneously within several months to 5 years after its onset. In this review article, the key features of this rare autoimmune bullous disease are revisited.

Circular Erythematous Plaque at the Area of Median Sternotomy

“…Clinically, considerable variation exists regarding age at disease onset, clinical morphology, and mucosal involvement . Typical cutaneous manifestations include tense arciform bullae in a cluster-of-jewels configuration or, less commonly, grouped papulovesicles . Atypical lesions owing to Koebner phenomenon have also been reported in areas where adhesives were previously applied .…”

“…Drug-induced LAD is usually self-limiting and gradually resolves after discontinuation of the causative agent. Additional therapy, such as dapsone, sulfonamides, corticosteroids, colchicine, tetracyclines, and nicotinamide, may be required …”

“…1,5 Typical cutaneous manifestations include tense arciform bullae in a cluster-ofjewels configuration or, less commonly, grouped papulovesicles. 5,6 Atypical lesions owing to Koebner phenomenon have also been reported in areas where adhesives were previously applied. 7,8 This patient developed a lesion on the median sternotomy scar, which was highly suggestive of koebnerization.…”

“…Additional therapy, such as dapsone, sulfonamides, corticosteroids, colchicine, tetracyclines, and nicotinamide, may be required. 1,6,10 A presumptive clinical diagnosis of annular pustular psoriasis was supported by the annular lesion morphology, formation of pustules, centrifugal expansion, and potential koebnerization; however, pustular psoriasis was unlikely given the DIF positivity and histologic findings. Bullous tinea is a clinical mimicker, and 2 cases have described basement membrane DIF positivity.…”