Hodak et al.: Anetodermic primary cutaneous B-cell lymphoma: a unique clinicopathological presentation of lymphoma possibly associated with antiphospholipid antibodies

Hodak, E; Feuerman, H; Barzilai, A; David, M; Cerroni, L; Feinmesser, M.
Anetodermic primary cutaneous B-cell lymphoma: a unique clinicopathological presentation of lymphoma possibly associated with antiphospholipid antibodies.
Arch Dermatol. 2010; 146(2): 175-182. [Fulltext] [PubMed]

Abstract:
BACKGROUND: Primary cutaneous B-cell lymphoma manifested by anetoderma has been reported in 7 cases. In all, the secondary anetoderma developed in lesions of marginal-zone lymphoma or posttransplant lymphoproliferative disorder resembling marginal-zone lymphoma. The mechanisms underlying the destruction of elastic tissue in anetoderma are unclear. However, there is growing evidence linking primary anetoderma with a wide range of immunologic abnormalities, the most common being the presence of antiphospholipid antibodies. OBSERVATIONS: We analyzed data from 5 patients (3 male, 2 female) with clinical and histopathological features of anetodermic primary cutaneous B-cell lymphoma. Three had marginal-zone lymphoma and 2 had follicle-center cell lymphoma. In all, secondary anetoderma developed in self-regressing nodules/plaques of the lymphoma. Two patients also had lesions clinically and histopathologically compatible with primary anetoderma. Associated immunologic diseases were systemic lupus erythematosus-like disease and rheumatoid arthritis (1 patient each; not in patients with primary anetoderma). Antiphospholipid antibodies were found in 4 patients. CONCLUSIONS: Anetodermic primary cutaneous B-cell lymphoma is a rare and unique clinicopathological manifestation not only of marginal-zone lymphoma, as previously described, but also of follicle-center cell lymphoma. This type of secondary anetoderma, like primary anetoderma, might be associated with immunologic disorders, particularly antiphospholipid antibodies.


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