Keratin pattern of acanthosis nigricans in syndromelike association with polythelia, polycystic kidneys, and syndactyly.
BACKGROUND
Acanthosis nigricans (AN) comprises a broad spectrum of etiologic subtypes. The underlying pathomechanisms have not yet been completely clarified. We present a patient affected with a syndromelike AN subtype including disturbed epidermopoiesis as evidenced by immunohistologic findings and in situ hybridization.


METHODS
A 54-year-old white man contracted AN during childhood. There were connate malformations consisting of webbed toes II/III on the right side and a supernumerary left mammilla. As an adult he developed psoriasis vulgaris, obesity, and latent diabetes mellitus, polycystic kidney and liver disease. With regard to keratin 6 mRNA, and the protein expression of keratin 6/16, KI-67, and proliferating cell nuclear antigen, the AN lesion showed moderate hyperproliferation. A much higher degree of hyperproliferation was evident in psoriatic areas of the patient's skin. In contrast to psoriatic tissue, basal keratinocytes of the AN showed an unusually high expression of keratin 18 and 19 protein.


CONCLUSIONS
The observation thus deals with a unique, syndromelike constellation of AN characterized by a particular epidermal pattern of moderate hyperproliferation. A further dysregulation of protein expression in the epidermis is indicated by the demonstration of the rare keratins 18 and 19 in basal keratinocytes of the AN lesion.
Bonnekoh
B
B
Department of Dermatology, University of Cologne, Germany.
Wevers
A
A
Spangenberger
H
H
Mahrle
G
G
Krieg
T
T
D000015:Abnormalities, Multiple; D000052:Acanthosis Nigricans / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007633:Keratins / Q000096:biosynthesis* / Q000235:genetics; D008297:Male; D008875:Middle Aged; D009558:Nipples / Q000002:abnormalities*; D007690:Polycystic Kidney Diseases; D012333:RNA, Messenger / Q000032:analysis; D013576:Syndactyly; D013577:Syndrome
D012333:RNA, Messenger; D007633:Keratins