Acquired Epidermolysis Bullosa and Linear Immunoglobulin A Bullous Dermatosis

Abstract

Acquired epidermolysis bullosa is a rare subepidermal bullous disease characterized by autoantibodies to type VII collagen, the major component of anchoring fibrils.Although the exact pathophysiologic mechanism remains unclear, reduction or perturbation of the anchoring fibrils results subepidermal blister formation and clinical features such as skin fragility, blisters, erosions, scars, milia and nail loss. Acquired epidermolysis bullosa includes various clinical manifestations resembling genetic epidermolysis bullosa, bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid and linear immunoglobulin A bullous dermatosis. Numerous treatment options are available but patients are often refractory to treatment. Linear immunoglobulin A bullous dermatosis is another subepidermal bullous disease characterized by the accumulation of IgA antibodies in lamina densa or sublamina densa region of the basement membrane and neutrophil-rich infiltrates in histopathology. It can be seen both in children and adults. The form seen in children usually begins under the age of 5 and it is called chronic bullous disease of childhood. The classical presentation is annular/polycyclic plaques and papules with blistering on perioral and perineal regions, giving a "cluster of jewels" appearance. The adult form is often seen after the fourth decade and clinical features are similar to those of dermatitis herpetiformis, bullous pemphigoid or cicatricial pemphigoid.

Clinical features of chronic bullous dermatosis of childhood

“…Our study included 74 males and 54 females, and as previously reported in the literature, 10 there was no sex predilection to developing CBDC in children; however, we found a preponderance of CBDC in neonatal males of 19 : 1 over females, which is unexplained 1 …”