XXXX : clinicopathologic study of 13 cases.

@entity1 @entity3123 @entity788 @entity417 @entity6 @entity203 @entity8286 @entity3 @entity12984 @entity12983
BACKGROUND: The clinical mucocutaneous manifestations of @entity12983 are recognized easily when they occur in the classic pattern of acral or periorificial lesions evolving in recurrent crops, with an annular and migratory distribution, in a @entity1 with @entity6 who has had recent weight loss and @entity417 . Not infrequently, noncharacteristic clinical and histopathologic features are observed and, in these cases, the diagnosis of @entity788 may be delayed. AIM: To review the clinical and histopathologic features of cutaneous manifestations of @entity12983 . METHODS: The clinicopathologic features of 13 @entity1 (eight @entity1 ) with widespread or localized @entity203 as a manifestation of @entity3123 with marked glucagon secretion ( @entity12983 ) were reviewed. RESULTS: The definitive diagnosis of the @entity203 was established at the time of diagnosis of the @entity788 (three @entity1 ) or afterwards (10 @entity1 ). In nine @entity1 , the mucocutaneous manifestations preceded the diagnosis of the @entity788 by 1 month to 3 years (mean, 12 months). In only eight biopsy specimens were the histopathologic features considered to be suggestive or characteristic of @entity12984 . Diffuse @entity8286 , that occasionally arose abruptly from normal epidermis, was observed in 12 biopsy specimens. By the time @entity12984 was diagnosed, the @entity3123 had @entity3 to the liver, regional lymph nodes, or bone in 12 @entity1 . CONCLUSION: Increased awareness of the polymorphic mucocutaneous and nonspecific histopathologic features of @entity12983 is needed to avoid unnecessary delay in the diagnosis of this syndrome.
