Seda Atay

By Gökçen Çelik and Seda Atay

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.

Part of the book: Autoimmune Bullous Diseases

By Havva Hilal Ayvaz, Müzeyyen Gönül and Seda Atay

Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus (SLE). It has characteristical clinic, pathologic, and immunologic findings including antibodies to type VII collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. Clinical presentation combined with histopathology, immunological testing, and concomitant diagnosis of SLE according to the criteria of American College of Rheumatology, are required to distinguish bullous SLE from these bullous diseases. In patients with bullous SLE, SLE disease progression and complications may be worse. Cicatricial pemphigoid is a chronic subepidermal blistering disease which is characterized by erosive lesions of mucous membranes and skin. Pathogenesis of cicatricial pemphigoid is characterized by linear deposition of Immunoglobulin G, A, or complement 3 along the epithelial basement membrane zone. The main target antigens are bullous pemphigoid antigens 180–230, laminin 331–332, type VII collagen, and β-4 integrin subunit. Cicatricial pemphigoid may lead to serious complications such as blindness and airway obstruction. Herein, clinical, histological, immunopathological features, the diagnosis and treatment of bullous SLE and cicatricial pemphigoid diseases are mentioned to raise awareness among the dermatologists about this important but rare heterogeneous bullous disease.

Part of the book: Autoimmune Bullous Diseases