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Mycosis Fungoides: The Multicentric Tumor D’emblee

      A 64-year-old patient in good general condition presented with a 1 1/2-year history of several separate standing ulcerative-necrotic lesions with a diameter between 3 and 8 cm (Supplemental Figures 1 and 2, available online at http://www.mayoclinicproceedings.org), resistant to any kind of oral or topical antibiotic or corticosteroid treatment regimen (Figures 1 and 2).
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      Figure 1Clinical manifestation of the ulcerative lesion with necrotic center localized in the upper eyelid of the right eye. Solitary ulcerated-necrotic lesion on the skin of the upper eyelid was established clinically, with a distinctly limited, rise erythematous shaft, located on erythematous, infiltrated plaques. The visus was not affected.
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      Figure 2Clinical manifestation of the ulcerative-necrotic lesion localized on the left shoulder.
      No subjective complaints or accompanying disease was reported. The paraclinical examinations were within the normal range, except for an elevated erythrocyte sedimentation rate of 58 mm/hr. Differential diagnoses such as leishmaniasis, cutaneous tuberculosis, Wegener granulomatosis, sporotrichosis, granulomatous tinea profunda, and ecthyma were rejected based on the serological, virological, and immunological examinations. Histological and immunohistochemical evaluations confirmed the diagnosis of mycosis fungoides/tumor stage. The patient was directed to the oncology department where he received a chemotherapy regimen with adreamycin, cyclophosphamid, and vincrystin. Within the first therapeutic course, visible clinical improvement, with involution of the tumor formations, was observed. Several months later, however, the patient died from progression of the disease and the additional toxic effects of the chemotherapy.
      Although mycosis fungoides represents the most common T-cell lymphoma, accounting for 50% of all primary cutaneous lymphomas, its tumor form is much less common because the manifestation is uncharacteristic in most reported cases. The d'emblee form is a variation of the tumor stage form, wherein necrotic ulcerative lesions develop spontaneously without evidence of the primary morphological preceding symptoms of centrifugal or gradually evolving plaques.
      • Sahoo A.
      • Biswas M.K.
      Mycosis fungoides: tumour d'emblee.
      • Vora R.
      • Mubashir S.
      • Talavia P.
      • Anjaneyan G.
      Mycosis fungoides: tumour d'emblee.
      This is the main reason the disease is usually diagnosed in advanced stages and patients generally have a poor prognosis.

      Supplemental Online Material

      Supplemental Online Material

      Supplemental material can be found online at http://www.mayoclinicproceedings.org. Supplemental material attached to journal articles has not been edited, and the authors take responsibility for the accuracy of all data.

      References

        • Sahoo A.
        • Biswas M.K.
        Mycosis fungoides: tumour d'emblee.
        Indian J Dermatol Venereol Leprol. 1995; 61: 369-370
        • Vora R.
        • Mubashir S.
        • Talavia P.
        • Anjaneyan G.
        Mycosis fungoides: tumour d'emblee.
        Indian Dermatol Online J. 2012; 3: 122-124