Oral Mycosis Fungoides

Introduction

Oral mycosis fungoides (MF) is an uncommon manifestation of cutaneous T cell lymphoma (CTCL), which is not taken into consideration because of its rarity. Early diagnosis requires a thorough examination of oral cavity in all MF and histopathological evaluation of every suspected lesion. In this study, a patient with oral MF, its course, prognosis and treatment, is discussed.

Clinical presentation

A 41 year-old male was referred to dermatology ward of Razi hospital, Tehran, Iran in April 2011. He complained of a long term history of pruritus and xerosis, all over his body especially on trunk and lower extremities. He was treated with emollient-based topical steroid cream over the years with partial response. Six months prior to this visit, the noted symptoms and signs progressed to erythematous and finely scaling patches on his trunk, lower extremities and forehead, accompanied by an ulcerated tumor on his right shoulder and erosive leukoplakia of hard palate. Biopsies of skin and mucosal lesions were the same and showed diffuse infiltration of mixed small and medium sized cells in dermis with moderate degree of atypia and an interface pattern in the dermoepidermal junction (fig. 1). Immunohistochemical (IHC) studies stained positively for CD3 and CD4 and negatively for CD8, CD20, and CD30. Monoclonal T cell receptor rearrangement was detectable by PCR. The results of skin and mucosal biopsies, IHC and PCR were in favor of MF. A thorough physical examination showed generalized patches involving more than 10% of total skin surface and the noted tumoral lesion on his shoulder and erosive leukoplakia of palate (fig. 2). No enlarged lymph nodes were found. Normal chest and abdominal CT scan, normal bone marrow biops...

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...gh unsatisfactory (5). In our patient, oral manifestation after several months instead of years, posed an aggressive MF, and progression under chemotherapy, caused incomplete courses of CHOP (3 courses instead of 6) which was replaced by TSEB just for palliative therapy. The similarity between mortality rate of oral and visceral involvement in MF (mean survival of less than 1 year), arranges oral MF as an advanced disease with poor prognosis (1).

Because of its rarity, the diagnosis of oral MF can be missed on examination. Early detection of this manifestation has great importance in the efficacy of chemotherapy, in comparison to late identification. Our patient, again emphasized the importance of a complete physical examination with particular attention to oral cavity in MF. To our knowledge, this the first case report of oral involvement in MF, from Iran.