Papulöse akantholytische Dyskeratose

Abstract

Papular acantholytic dyskeratosis is a very rare skin disease, which shows a suprabasilar cleft with acantholytic and dyskeratotic cells, simulating the light microscopic picture of Darier disease. In this condition, the lesions are acquired without a genetic basis. We observed a 40-year-old woman with about 200 papules on the lower aspects of her legs; histological examination showed changes similar to Darier disease. Transmission electron microscopy showed a reduced number of desmosomes and perinuclear distribution of tonofilaments. The lesions were successfully treated with cryotherapy with spray technique.

Desmosomes in acquired disease

“…The precise pathomechanism of this disease, and the related disorder Stevens-Johnson syndrome, are not known, but the presence of autoantibodies against desmoplakin is considered characteristic of these diseases. Lastly, reduced numbers of desmosomes have been reported in papular acantholytic dyskeratosis (de Almeida Junior, et al, 2001). Thus, a rather extensive number of epidermal disorders involve alterations in desmosome morphology and function, although additional research in this area is needed to clarify precisely how these changes might relate to clinical presentation of these disorders.…”

Effective treatment of papular acantholytic dyskeratosis with oral isotretinoin

“…A 41‐year‐old white woman, seen in our outpatient clinic, presented 5 years ago with lesions on her lower legs, which were diagnosed as PAD, with good response to cryotherapy with liquid nitrogen spray. This case was published elsewhere 4 . Six months ago she presented with a relapse, with the same clinical presentation (fig.…”

“…There is little information regarding PAD therapy. Successful treatment with cryotherapy 4 and topical steroids 1 has been reported. Some other diseases of this group can be treated with oral retinoids or steroids 6–8 .…”

Segmentaler Morbus Darier

“…Als Therapiemöglichkeiten stehen Anwendungen mit topischen Steroiden [7], Vitamin-A-Säure (0,2-1%; [8]), aktiviertem Vitamin D 3 [28], Tacrolimus [27], 5-Fluorouracil [21,32] oder oralen sowie topischen Retinoiden [13,24] zur Verfü-gung. Ferner wurde bereits über Therapieversuche mittels photodynamischer Therapie [31], Dermabrasion [12], Kryotherapie [14], verschiedener Lasersysteme [4,22] und elektrokaustische Therapien [30] [19].…”