Six patients with an apparently unique, self-limited, primary acantholytic skin disease are described.
The elementary lesions were pruritic, discrete, edematous papules or vesiculopapules which tended to appear first on the trunk, possibly as a Koebnerlike response to nonspecific irritation, such as ultraviolet light. The two youngest patients, aged 46 and 48, developed only localized manifestations which disappeared in a few weeks. The older patients, ranging from 56 to 74 years old, had disseminated eruptions which lasted several months. Although there was a strong histologic resemblance to Darier's disease in three cases, and to Hailey-Hailey's disease in the other three, clinical considerations seem to rule out both of these diagnoses.
It is stressed that cytologic examination is most useful in recognizing the disease.