Bullae are elevated, fluid-filled blisters ≥ 10 mm in diameter.
Epidermolysis bullosa acquisita can occur in all ages. The exact etiology of epidermolysis bullosa acquisita is not known, but data suggest it involves autoimmune antibodies directed against type VII collagen, a key component of the anchoring fibrils within the dermal-epithelial junction (1).
Multiple myeloma, amyloidosis, lymphoma, inflammatory bowel disease, and systemic lupus erythematosus have been associated with epidermolysis bullosa acquisita but the nature of the relationship is unclear.
Epidermolysis bullosa acquisita is distinct from the inherited disorder epidermolysis bullosa, which involves various genetic mutations.
General reference
1. Koga H, Prost-Squarcioni C, Iwata H, et al: Epidermolysis bullosa acquisita: The 2019 update. Front Med (Lausanne) 5:362, 2019. doi: 10.3389/fmed.2018.00362
Symptoms and Signs of Epidermolysis Bullosa Acquisita
Initial manifestations of epidermolysis bullosa acquisita are highly variable, sometimes resembling those of bullous pemphigoid. Bullous lesions are most often in areas subject to minor trauma, such as the extensor aspects of the elbows and the dorsal aspects of the hands and feet. Healing usually causes scars, milia (superficial epidermal inclusion cysts), and hyperpigmentation. Some patients have dystrophic nails and scarring alopecia, mucosal involvement, or ocular lesions leading to blindness.
Diagnosis of Epidermolysis Bullosa Acquisita
Skin biopsy and direct immunofluorescence
Diagnosis of epidermolysis bullosa acquisita is confirmed by skin biopsy and direct immunofluorescence. Direct immunofluorescence shows linear deposits of IgG and complement at the basement membrane zone (dermal–epidermal junction).
Treatment of Epidermolysis Bullosa Acquisita
Skin care
Important skin care measures include gentle cleansing of skin, vigilance in preventing skin trauma, and early detection and treatment of skin infections.
Key Points
Epidermolysis bullosa acquisita is a rare acquired disorder that causes bullous lesions.
Confirm the diagnosis using skin biopsy and direct immunofluorescence.
