Gibson, Lawrence E.

AbstractFollicular mucinosis (FM) and folliculotropic mycosis fungoides (MF) are rare in children, and data regarding long‐term outcomes are limited. We sought to describe clinical and histopathologic findings of children with FM with and without MF, as well as treatments administered and clinical outcomes. We conducted a retrospective chart review of patients younger than 22 years (at time of diagnosis) with a biopsy demonstrating FM who were seen in the Dermatology Department at the Mayo Clinic from September 1, 1999, to September 1, 2010. Eleven patients (six male, five female) ages 11 to 19 years at the time of diagnosis met the inclusion criteria. Follow‐up data were available for 10 patients, with a mean duration of 4.9 years. The head, neck, and extremities were the most common sites of involvement, and lesions were follicular‐based papules (18%), scaly alopecic patches and plaques (45%), or a combination of the two (36%). Overall, three patients were confirmed to have MF. T‐cell receptor gene rearrangement demonstrated clonality in two cases and was equivocal in one case. Treatments included topical corticosteroids, topical retinoids, oral minocycline, and, in patients with MF, ultraviolet light and topical bexarotene. Lesions resolved completely in seven patients, partially in one, and not at all in two (no follow‐up data on one patient). Of the three patients with MF, two had complete resolution, and one has intermittent flares. To our knowledge, no patients developed other lymphoproliferative disorders. FM in children is rare. A histopathologic diagnosis of FM does not equate to folliculotropic MF in all cases. Most patients responded to treatment with topical steroids, topical retinoids, or phototherapy. In our series of patients, the disease ran a benign course.

BackgroundCutaneous granulomatous inflammation can occur in patients with T‐cell lymphoma and Hodgkin disease. We describe the unusual microscopic pattern of a granuloma annulare (GA)‐like eruption co‐existing with B‐cell chronic lymphocytic leukemia (B‐CLL).MethodsWe reviewed the histopathology and immunophenotype of skin biopsies from two patients with B‐CLL and cutaneous lesions resembling GA.ResultsBoth patients had symptomatic cutaneous lesions clinically resembling GA; one had lesions refractory to standard dermatologic therapy. Histopathology showed GA‐like palisaded histiocytic infiltration, with subtle collections of lymphocytes interspersed among the granulomatous inflammation. Immunohistochemistry showed strong expression of CD20 and CD79a, with aberrant CD5 co‐expression, confirming cutaneous involvement by B‐CLL.ConclusionsCo‐existence of a GA‐like infiltrate and cutaneous B‐CLL raises the possibility that granulomatous inflammation occurs as a secondary response to dermal infiltration by leukemic cells. Because histopathologic findings can be subtle, knowledge of this association is essential to avoid overlooking the diagnosis. Regardless of whether histopathology reflects a reactive or primary phenomenon, documentation of cutaneous involvement by B‐CLL may serve as a rationale for specific treatment of the underlying B‐CLL in patients with skin lesions unresponsive to dermatologic therapy and for whom there is no other justification for leukemia‐targeted therapy.