Multiple papules in a localized area. Segmental neurofibromatosis.
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Biomedical subjects
Publications and source records attributed to J C Gretzula.
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We report the case a 69-year-old woman with dermatofibrosarcoma protuberans, present, apparently, since birth. To our knowledge, this case represents the longest reported duration of a dermatofibrosarcoma protuberans.
Primary mucinous carcinoma of the skin is a rare entity. It usually presents as a painless nodule, cyst, or ulcer in the head and neck region (especially the eyelid). These tumors have a low metastatic potential but tend to recur locally. Histologically, groups of light- and dark-staining epithelial cells form cords, nests, and ducts separated by clear areas representing sialomucin. Due to tumor continuity and a low rate of lymphatic or hematologic spread, Mohs micrographic surgery appears to be an effective treatment. Herein, two cases of primary mucinous carcinoma are presented.
In 1980 a syndrome was first described in two adult males, consisting of macrocephaly, pigmented macules on the glans and shaft of the penis, and hamartomatous intestinal polyps. Since then, 10 additional cases have been identified. Herein, we present two new cases and review the cutaneous manifestations as well as additional features in patients with the Ruvalcaba-Myhre-Smith syndrome.
Bloom's syndrome is a rare autosomal recessive disorder that characteristically shows a telangiectatic, sun-sensitive facial rash in conjunction with stunted growth. These patients are prone to respiratory and gastrointestinal infections along with immunologic abnormalities. Chromosomal aberrations include an elevated frequency of sister chromatid exchange in cultured lymphocytes, a phenomenon pathognomonic for this disorder. These patients exhibit a great tendency for neoplasia, particularly acute leukemia and lymphoma. Three cases are reported, including a follow-up of a patient diagnosed 16 years ago.
Syringoid eccrine carcinomas (SECs) are rare adnexal neoplasms that may display aggressive local recurrence and occasional metastases. This report describes three cases of SEC treated by Mohs micrographic surgery.
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