Anetoderma arising in cutaneous B-cell lymphoproliferative disease

Anetoderma is circumscribed atrophy of the skin due to a localized deficien cy in elastic tissue. It can follow inflammatory skin diseases of several t ypes, and occasionally is present in the skin around neoplasms. There are a few reports of anetoderma in the lesional skin of cutaneous lymphoma. We r eport on two patients who presented with multiple lesions of anetoderma and who later proved to have low-grade cutaneous B-cell lymphomas. One patient (Patient 1) is a 39-year-old man and the other patient is a 26-year-old wo man who is a renal transplant recipient (Patient 2). Some biopsy specimens from the anetodermic skin of Patient 1 appeared to show an urticarial react ion, although plasma cells were present. A large nodule showed lymphoid fol licles surrounded by plasmacytoid lymphocytes, with loss of elastic tissue in the adjacent dermis. The plasmacytoid cells stained overwhelmingly for l ambda light chain, and staining of the urticarial lesions from this patient also showed a marked majority of lambda positive cells. Immunoglobulin hea vy chain gene (IgH) rearrangements showed a dominant clonal pattern in the nodular lesion. We classified the disease in Patient 1 as marginal zone lym phoma and the disease in Patient 1 as a post-transplant lymphoproliferative disorder. Because of the intimate association of anetoderma and cutaneous B-cell limphoproliferative disorders in these two patients, it seems possib le that anetoderma could result from either local effect of the neoplastic cells or associated inflammatory cells, especially neutrophils as in (Case 1). The infiltrates of Case 1 had many interstitial neutrophils and only a few clonal plasmacytoid lymphocytes, indicating that this presentation of B -cell lymphoma can be a diagnostic pitfall. Given these two cases and simil ar ones in the literature, biopsy of lesional skin in anetoderma should be performed to ensure that lymphomatous infiltrates are not present. Even if plasma cells are sparse, studies to detect clonality an appropriate. Cutane ous B-cell lymphoma can be added to the list of associations of elastolysis and cutaneous lymphoma, which includes granulomatous slack skin (T-cell ly mphoma) and cutis laxa (myeloma).