Ki-1-positive anaplastic large-cell lymphoma can mimic benign dermatoses

Regressing atypical histiocytosis is a recently described disease characterized by recurrent nodules or ulcers. The cutaneous lesions appear abruptly and then regress only to return in a manner reminiscent of lymphomatoid papulosis. Immunophenotypic analysis has revealed that most cases are a form of anaplastic large-cell Ki-1-positive (CD30+) lymphoma.

We describe two patients with Ki-1-positive anaplastic large-cell lymphoma that had clinical and pathologic features of regressing atypical histiocytosis and mimicked benign dermatoses (pyoderma gangrenosum and morphea), causing a delay in confirming the true diagnosis. A third case that was readily recognized as a lymphoma is also presented.

The clinical and histopathologic findings were recorded. In addition, T-cell receptor gene rearrangement and immunophenotyping were determined in the index case.

The index patient and second patient were diagnosed as having Ki-1-positive anaplastic large-cell lymphoma by immunophenotyping and underwent cyclophosphamide, doxorubicin, prednisone, and vincristine (CHOP) chemotherapy with complete remission. The patient detected by chart review died of her disease without receiving antineoplastic therapy; disseminated lymphoma was diagnosed at autopsy. Studies on paraffin-embedded tissue were consistent with Ki-1-positive anaplastic large-cell lymphoma.

Regressing atypical histiocytosis may clinically resemble some benign dermatoses. Recent evaluation of these cases has shown that many represent a form of Ki-1-positive anaplastic large-cell lymphoma. Multiple skin biopsy specimens with immunophenotyping and gene rearrangement studies are required to arrive at the diagnosis.