Since 1950, the year of Grzybowski's syndrome description by Marian Grzybowski in Warsaw, 30 cases have been described in the literature. This syndrome belongs to the family of multiple generalized keratoacanthomas (KA). It occurs in adults between 50 and 70 years. Clinically, it presents as a rash of hundreds or thousands of small papules 1 to 2mm with well-defined, sometimes keratotic center. The onset is sudden, the evolution is progressive and chronic. It is accompanied by severe and persistent pruritus. There is no family history of KA and histology is compatible with that of KA. Other criteria may be present to varying degrees: the type of facial rash Mask of Zorro, mucosal rash, ectropion, the presence of crater-like lesions. We studied the case of a 58-year-old patient followed in the plastic surgery department of Nîmes' hospital for excision of several skin tumors as part of a Grzybowski's syndrome evolving since 2005. Its handling global and multidisciplinary treatments combining early surgical and complex medical treatments represents a therapeutic challenge.
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