Ultrastructural evaluation of mid-dermal elastolysis.

Mid-dermal elastolysis is a well-defined clinical and histopathologic entity manifested by fine wrinkling of the skin and a mid-dermal loss of elastic fibers. Ultrastructural and histologic studies were performed in an attempt to better define the cause of the elastolytic process. Biopsy specimens from the lesions of 3 patients with mid-dermal elastolysis were studied at light and electron microscopic levels. Ultrastructural evidence of normal elastic fiber engulfment by activated macrophages was observed; however, some fields also demonstrated envelopment of abnormally degenerated elastic tissue. Although there are many potential causes of this degeneration, photodistribution of the lesions suggests that ultraviolet damage is a primary inciting factor.

Chordoma cutis: a report of nineteen patients with cutaneous involvement of chordoma.

BACKGROUND: Chordoma, an uncommon tumor originating from remnants of the notochord, with cutaneous involvement has rarely been reported. OBJECTIVE: Our purpose was to document clinical manifestations, histopathologic features, immunohistochemical findings, treatment, and course of chordoma with cutaneous involvement. METHODS: Pathologically proven cases of chordoma were reviewed retrospectively for cutaneous involvement. Detailed clinical data and histopathologic changes were studied. Skin biopsy specimens were stained for immunohistochemical phenotyping. RESULTS: Of 207 cases of chordoma, 19 had skin involvement: as local recurrences or metastasis in 12, as direct extension of primary tumor in 6, and as a result of distant metastasis from sacrococcygeal chordoma in 1. Local recurrences were frequent, but distant metastasis to various organs, including skin, occurred. CONCLUSION: We propose the term chordoma cutis to describe this condition. In seven patients, cutaneous lesions were detected when the diagnosis of primary chordoma was made.

Congenital smooth muscle hamartoma of the skin.

A 2-month-old white girl had a congenital, tan-colored, slightly elevated, 3 x 2-cm plaque on the left midback. Clinically, the lesion was suspected to be a solitary mastocytoma. Rubbing the lesion produced a transient erythema and edema that was similar to Darier sign seen in mastocytoma. Analysis of a skin biopsy specimen revealed a smooth muscle hamartoma; special stains did not show evidence of increased numbers of mast cells. This relatively uncommon condition can be confused with a variety of other cutaneous diseases. Light microscopic examination of a skin biopsy specimen establishes the diagnosis. A review of the approximately 50 cases reported in the literature showed that there is no known associated systemic involvement or malignant transformation. The clinical lesions usually become less prominent with time.

Chordoma involving the skin: an immunohistochemical study of 11 cases.

Immunohistochemical properties were studied in 16 lesions from 11 patients with chordoma involving the skin. There were nine men and two women ranging from 21 to 62 years old (mean, 42.6). The initial tumor was sacrococcygeal in 10 cases and nasopharyngeal in 1 case. Three lesions represented a direct extension from the primary tumor to the skin, and 13 lesions were examples of local recurrences in the skin. Immunohistochemically, all lesions showed positivity for keratin, whereas 14 lesions were positive for vimentin and 12 for protein S-100. Epithelial membrane antigen was positive in four instances and carcinoembryonic antigen was negative in all studies. No significant difference was observed between the immunoprofile of cases of direct extension and those of local recurrences. Overall, the remarkable triple positivity for keratin, vimentin, and protein S-100 was observed in 11 lesions from eight different cases (73%). This study confirmed the utility of immunohistochemistry in the differential diagnosis of chordoma from tumors with similar histologic characteristics encountered in the skin.