Abstract
A 71 year old male with chronic lymphocytic leukemia, undergoing active surveillance, presented with a three year history of multiple monomorphic pink indurated papules. The rash started on the distal extremities and slowly progressed to involve his trunk. He denied associated pain or pruritus. H&E revealed a well demarcated granuloma in the superficial dermis composed of epithelioid histiocytes with a peripheral rim of small lymphocytes. Palisading was focally present. The colloidal iron stain highlighted focal mucin within the granuloma, most consistent with granuloma annulare (GA). GMS and Fite stains were negative. A second specimen revealed a band-like infiltrate of small lymphocytes within the upper dermis. A narrow grenz zone was present. At the periphery of the infiltrate, there were epithelioid granulomas which appeared to abut and partially surround the lymphocytic infiltrate. The lymphocyte population in the dermis included a B-cell component, which expressed CD20 and Pax 5. There was weak aberrant expression of CD5 in dermal B-cells. Cyclin D1 was negative. A clonal immunoglobulin gene rearrangement was detected within the specimen by PCR. Taken together, the histopathologic, immunophenotypic, and molecular findings revealed the presence of CLL cells in the skin, associated with a granulomatous inflammatory infiltrate. The skin lesions waxed and waned. He started a clinical trial for CLL within one year after skin biopsy, and the skin lesions resolved. This case demonstrates a rare manifestation of CLL, which has been described in a few case reports. The occurrence of a GA-like eruption in patients with CLL should raise suspicion for CLL involvement of the skin. The atypical lymphocytic infiltrate may not be readily apparent, as represented in the initial biopsy of this case; hence, additional analysis by immunohistochemistry and molecular testing may aid in identification of the leukemic cells in the skin in patients with a history of CLL.
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