Langerhans cell histiocytosis followed by folliculotropic mycosis fungoides

The occurrence of lymphoma and Langerhans cell histiocytosis (LCH) in the same individual, either sequentially or simultaneously is not common [1, 2]. Several cases in which malignant lymphoma occurred prior to LCH are reported [3, 4]; however, a few cases can be found with LCH followed by malignant lymphomas [5]. We report a rare accompaniment of LCH followed by folliculotropic mycosis fungoides (FMF). Due to another diagnosis of the patient described previously in Advances of Dermatology and Allergology [6], we would like to present a continuation of her diagnostic and therapeutic process, because it changes the look on the current course of the illness, forcing clinicians to be vigilant. A 41-year-old woman was admitted to the Department of Dermatology, Venereology and Allergology in 2011 due to diffuse edematous skin lesions and numerous nodules localized on the face. The physical examination revealed also a 2 × 2 cm-sized ulcer covered with necrotic tissue on the skin of the left lower extremity and generalized lymphadenopathy. The diagnostic tests (magnetic resonance imaging (MRI) of the craniofacial area, computed tomography (CT) of the chest, bone marrow biopsy, chest X-ray, immunophenotypic examination of peripheral blood, ultrasonography of lymph nodes) were performed, but they did not reveal any abnormalities. Cervical lymph node biopsy was taken and on the basis of histopathological examination, diagnosis of LCH was made. The neoplastic cells showed the following phenotype: CD1a+, S100–, CD2–, CD3–, CD68–, CD56–, thiaphorin-1–, CD21–, Granzyme B–, CD5–. The final diagnosis of LCH was based on the clinical picture, histopathological examination of the skin and lymph node. The diagnosis was confirmed by immunohistochemical staining, which revealed the presence of aggregates of CD1a+ cells. Prednisone therapy at the initial dose of 1 mg/kg/day was started. During the treatment, rapidly growing facial edema occurred, resulting from compression by enlarged carotid lymph nodes on the blood vessels. In the Department of Oncology and Radiotherapy, the chemotherapy with cladribine was initiated (5 mg/m²/day for 5 days every 4–6 weeks). The patient was given 6 courses of chemotherapy with a significant improvement. Unfortunately, 2 months after completion of treatment, papular lesions localized on the trunk occurred. Their histopathological examination revealed LCH. The chemotherapy with vinblastine 6 mg/m² and prednisone 40...


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