Article

Epidermolysis bullosa acquisita

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Abstract

A current textbook in dermatology defines epidermolysis bullosa as a heterogeneous group of chronic, hereditary, blistering disorders which range from a mild inconvenience to a disabling illness with significant mortality.1 Classification of this group of mechanobullous dermatoses is difficult because of the clinical overlap of the various clinical types of epidermolysis bullosa, and the lack of adequate clinical or laboratory criteria for their differentiation. The findings by light microscopy are variable; consequently, the various types of this disease are not easily discernible.

With the aid of the electron microscope, more accurate diagnoses have resulted. Since Pearson’s2, 3 detailed classification of these mechanobullous dermatoses by electron microscopy, there have been relatively few reports which have confirmed the ultrastructural findings of epidermolysis bullosa. Because electron microscopy has not yet become readily available to everyone, published classifications based on these findings differ significantly and remain controversial.

We recently had the opportunity to treat and extensively study a patient who had presenting symptoms of the acquired type of epidermolysis bullosa. Of all the clinical types of epidermolysis bullosa, only epidermolysis bullosa acquisita (EBA) is nonhereditary. Roenigk et al4 established that the criteria for the diagnosis of EBA are as follows: the disease occurs primarily in adults who have had no history of a blistering disorder nor a family history of a blistering disorder. The skin manifestations of the disease are characteristic and all other types of blistering diseases must be excluded. As is also found by many others, EBA may be found concomitantly with . . .


 

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