411/719. Metastatic squamous cell carcinoma in linear porokeratosis of Mibelli. We describe the clinical and pathologic observations of a 45-year-old woman with linear porokeratosis who developed squamous cell carcinoma in lesional sites. The squamous cell carcinoma metastasized to a regional lymph node. This is the second case of metastatic squamous cell carcinoma reported in porokeratosis and the first reported in the linear variety. ( info) |
412/719. Focal acantholytic dyskeratosis and epidermolytic hyperkeratosis of the oral mucosa adjacent to squamous cell carcinoma. A 74-year-old woman presented with an ulcerating squamous cell carcinoma of the posterior mandibular gingiva. biopsy of the mucosa directly anterior to the tumor revealed areas of both focal acantholytic dyskeratosis (FAD) and epidermolytic hyperkeratosis (EH). Rare solitary lesions of FAD have been described in the oral mucosa; this is the first report of FAD as an incidental finding. EH has only recently been reported in the mouth, and the concept is unknown in the oral pathology literature. Both FAD and EH may represent nonspecific reaction patterns of skin and oral mucosa to numerous stimuli, including carcinogens or adjacent neoplasms. ( info) |
413/719. Acquired pili torti in two patients treated with synthetic retinoids. A mother and daughter with epidermolytic hyperkeratosis were treated with two different synthetic retinoids at different times. Both patients exhibited the previously unreported side effect of increased curliness of scalp hair. light microscopic examination revealed pili torti during the administration of isotretinoin. Both patients also experienced a better therapeutic response to and more hair loss with etretinate than with isotretinoin. ( info) |
| Two patients are reported in which the development of porokeratosis occurred following chemotherapy for systemic malignancy. immunosuppression associated with malignancy and chemotherapy may exacerbate or initiate the development of porokeratosis in patients predisposed to alterations of cutaneous growth dynamics. ( info) |
| porokeratosis of Mibelli, a disorder of keratinization, has in the past been difficult to treat, especially in its more extensive form. The carbon dioxide laser was used in the treatment of a patient with this disorder. The response was very favorable. Microscopic pathological investigation after treatment revealed no residual evidence of porokeratosis in the areas treated with the carbon dioxide laser. Thus, obliteration of this disease process using the carbon dioxide laser shows potential for future use. ( info) |
416/719. Report of a family with an unusual expression of recessive ichthyosis. review of 42 cases. A family with a history of ichthyosis is described in which the type of scale was thin and fine, resembling that of ichthyosis vulgaris, but the inheritance is of the autosomal recessive type. We have reviewed our previous experience with 42 additional patients whose condition had been diagnosed as recessively inherited ichthyosis and conclude that there is considerable heterogeneity in the cutaneous manifestations. We suggest that it is not possible to classify all patients into categories such as lamellar ichthyosis or congenital ichthyosiform erythroderma, and it may be better to use a more general term such as recessive ichthyosis until specific causes can be established. ( info) |
| External ear canal cholesteatoma (EECC) is a rare otologic entity. Erosion of the inferior canal wall and accumulation of keratin debris are consistent findings. In the past there had been confusion between EECC and keratosis obturans, and they were thought to represent the same disease process. Currently, based on clinical and pathologic findings, it is believed that they are two different entities. In this article we present our experience in treating eight patients with EECC. For limited lesions, local debridement and curettage of necrotic bone is effective management. For more extensive lesions, canalplasty or tympanomastoidectomy is indicated. ( info) |
418/719. Acquired fibrokeratoma of the heel. We describe a patient with a 3-cm sessile tumor of the heel. Despite its large size and unusual location, it had the histologic characteristics of a fibrokeratoma. The essential microscopic feature of fibrokeratoma is its core of collagenous fibers with both interwoven and parallel arrangements. Epidermal hyperplasia and hyperkeratosis are often present, but probably represent secondary changes. Fibrokeratomas, therefore, have some of the clinical and pathologic features of collagenomas. ( info) |
419/719. Variability in the proteus syndrome: report of an affected child with progressive lipomatosis. In 1983 the proteus syndrome was delineated by Wiedemann et al. [12]. We report a 10-month-old girl, a further child affected by the new syndrome. The typical signs are macrodactyly, hemihypertrophy, pigmented nevi, hyperkeratosis, and subcutaneous hamartomatous tumours. Our patient shows an aggressive lipomatosis on the trunk and local relapses after surgical interventions in the regions involved. histology of the adipose tissue showed considerable anisocytosis and increased cell volume. ( info) |
420/719. Multiple epitheliomata cuniculata occurring in a mutilating keratoderma. We report on a young man with a congenital mutilating keratoderma in whom three epitheliomata cuniculata occurred during a two-year period. The clinical findings, associated disease states, and treatment of epithelioma cuniculatum (EC) are reviewed. ( info) |