Sarcoidosis and cutaneous lymphoma: What is the relationship?*

A 30-year-old Caucasian woman with history of chronic active systemic sarcoidosis for 14 years, presented at our dermatology department complaining of recurrent ulcerative papules and nodules that had begun four years ago. Sarcoidosis, involving the lungs, lymph nodes, kidney (interstitial nephritis), skin (pannicu-litis) and eyes (uveitis), was diagnosed by ganglionar and renal biopsy and treated with oral steroid and methotrexate. The papules, located mainly on palms but also on legs and gluteal area, became ulcerated and tend to self-resolve within some weeks, leaving a scar whilst new self-resolving lesions appeared (Figure 1). Biopsy showed a diffuse atypical lymphocytic proliferation involving the papillary dermis, the reticular dermis and the subcutaneous tissue. Immunohistochemistry was CD3+, CD43+ and CD4+ with groups of positive CD30 cells (Figure 2). An extended clinical and radiological study was performed, dismissing the possibility of systemic involvement. Biopsy findings and clinical history were compatible with lymphomatoid papulosis (LyP) type A and she was treated with low dose methotrexate and steroid cream. One year later the lesions became persistent and did not disappear spontaneously (Figure 3). Biopsy was equivalent to the previous. Five months later an infiltrated mass of 7cm on her left groin appeared associated to groin, iliac and retroperitoneal lymphadenopathies. Biopsy of the mass and lymphadenopathies showed a tumoral infiltration of large atypical intensely eosinophilic lymphocytes with irregular nuclei accompanied by an inflammatory infiltrate. The tumoral cells where CD3+, CD4+, CD30+, CD45+ focally, ALK-, and had a high prolifer-ative index (Ki.67 80%). Hence, the diagnosis changed to ALK-primary cutaneous anaplastic large cell lymphoma with lymph node involvement. Chemotherapy with cyclophosphamide, doxorubi-cin, etoposide, vincristine and prednisone (CHOEP) was initiated but it was suspended on the 5th course due to progression. Then, on second line, the patient received the AIEOP LNH-97 protocol, without response, initiating brentuximab with partial remission and performing later a bone marrow transplantation. The relationship between sarcoidosis and lymphoma seems not to be casual and the coexistence of both conditions is not infrequent. 1 In the 1960's Bichel and Brincker suggested a possible association of lymphoma and sar-coidosis, using the term sarcoidosis-lymphoma syndrome. 2 Bonifazi et al conducted a meta-analysis concluding that there is an increased risk of hematological malignancies and a higher overall risk of lym-phoma in patients with systemic chronic active sarcoidosis. 1 In spite of these findings, the relationship between sarcoidosis and cuta-neous lymphoma including mycosis fungoides (MF) has not been studied yet. Several …