Perivascular myoma of myopericytoma and myofibromatosis-type arising in a chronic scar.

We describe a case of a cutaneous perivascular myoma with features overlapping between the myofibromatosis and the myopericytoma type. The patient is a 58-year-old woman with a painless plaque-like and multinodular lesion in the pretibial dermis and subcutaneous tissue. She had repeated trauma to this site, first in her early youth that left an area of hyperpigmentation, and then again at age 40. The biopsy showed a biphasic pattern with a myofibromatosis-type component composed of spindle cell myoid nodules and more cellular round cell areas. The myopericytoma-like areas appeared to be infiltrating along vessels. These areas contained aggregates of immature-appearing cells arranged concentrically around vascular lumina in a manner reminiscent of pericytes. Immunohistochemical stains showed focal positivity for smooth muscle actin. Immunohistochemical and ultrastructural studies have showed these pericyte-like cells to be of a myoid origin. The reason for the neoplastic proliferation of perivascular myoid cells is presently unknown. The association of trauma and neoplastic transformation of the skin is rare. We report the first case of a cutaneous perivascular myoma arising in a chronic scar.

Bilateral anterior granulomatous uveitis associated with cutaneous Rosai-Dorfman disease.

BACKGROUND: Rosai-Dorfman disease (RDD) is a benign, rare, histiocytic disorder of unknown etiology. We describe the case of a 61-year-old woman who presented to the ophthalmology clinic with bilateral granulomatous anterior uveitis. METHODS: The uveitis was controlled on topical prednisolone acetate 1%. One month after presentation, she developed generalized macules and papules, and dermatologic and systemic evaluations were performed. RESULTS: On the basis of skin biopsy, purely cutaneous RDD was established. CONCLUSION: Anterior granulomatous uveitis may be the presenting sign of cutaneous RDD. Consultation is necessary for accurate diagnosis.

Cutaneous tumors as the initial presentation of non-T, non-B, nonmyeloid CD4+ CD56+ hematolymphoid malignancy in an adolescent boy.

We describe a 14-year-old Hispanic boy who presented with a 2-month history of enlarging plum-colored cutaneous tumors on his face, trunk, and proximal extremities. Histopathologic examination showed nodular infiltrates of malignant mononuclear cells extending from the superficial dermis to the deep subcutis. Immunohistochemical staining of the biopsy specimen and flow cytometry studies on a bone marrow aspirate revealed a CD4+, CD56+ hematolymphoid tumor that was negative for all other myeloid and lymphoid markers. Based on this information, the patient was diagnosed with the recently described, rare non-T, non-B, nonmyeloid CD4+ CD56+ hematolymphoid malignancy. To our knowledge, this is the youngest patient reported in the literature.