IgM Epidermolysis Bullosa Acquisita

A recent case report suggests the need for direct immunofluorescence to help diagnose bullous diseases.

A 72-year-old man developed fragile skin, mostly on the back of his hands and on his elbows. Skin biopsy pointed to a subepidermal bullous disease. Direct immunofluorescence demonstrated both immunoglobulin M antibody (IgM) and complement 3 (C3) at the dermal-epidermal junction. No other immunoglobulins (IgG) were present. Special indirect immunofluorescence using conjugated antihuman IgM showed the patient's serum contained IgM antibody reactive with antigens on the dermal side of salt-split normal human skin, thereby establishing the diagnosis of epidermolysis bullosa acquisita.

IgM epidermolysis bullosa acquisita (IgM-EBA) is rare. This is only the second reported case. The clinical appearance in both patients suggested IgG-EBA or porphyr…

Reviewing Author

Mark V. Dahl, MD

Disclosures

Consultant / Advisory board Ulthera, Inc.; AuBio LifeSciences, LLC; Paris Therapeutics

Equity Elorac, Inc.; Makucell, Inc.; Paris Therapeutics

Editorial boards UpToDate

Leadership positions in professional societiesNational Rosacea Society (Chairman, Medical Board)