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Cutaneous gamma/delta T‐cell lymphoma treated with retinoid and narrowband ultraviolet B

Cutaneous cd T cell lymphoma (CGD-CTL), a provisional condition in the World Health Organization–European Organization for Research and Treatment of Cancer classification, is composed of a clonal proliferation of mature cd T cells. Only 47 cases have been reported in the English literature since 1990. CGD-CTL has a poor prognosis; Toro et al. classified CGD-TCL by depth of cutaneous involvement, and reported that CGD-TCL with epidermotropic or dermal involvement and subcutaneous involvement had a median survival of 29 and 13 months, respectively. Various treatments have been tried with disappointing results. We report a case of CGD-CTL treated with etretinate and narrowband UVB (NB-UVB). A 62-year-old woman developed erythema on her limbs in April 2003. At a clinic, she was diagnosed with parapsoriasis en plaque and started on topical corticosteroid. Eight months after, a red tumour developed within an erythematous plaque on her lower leg (Fig. 1a). She was referred to our hospital in January 2004. On physical examination, erythematous patches and plaques were found on the patient s limbs (Fig. 1b). Results of a blood cell count and measurement of soluble interleukin-2 receptor levels were normal. Lactate dehydrogenase was high at 290 IU ⁄ L (normal 116–199). Computed tomography, gastrointestinal endoscopy, abdominal ultrasonography, and Ga scintigraphy showed no internal organ involvement. Histopathological examination of a biopsy taken from a lesion on the patient s left arm showed epidermotropism and a dense infiltrate of atypical lymphocytes from the superficial to the deep dermis, extending into the subcutis (Fig. 2a,b). The infiltrate was CD3+ CD4) CD5) CD7) CD8) CD20) CD30) CD56) T-cell receptor (TCR)ab) TCRcd+ (Fig. 2c). Results of tests for perforin and granzyme-B were negative. In situ hybridization for Epstein–Barr virus-encoded RNA was negative. Southern blotting with DNA from the skin lesion showed clonal rearrangement of TCR-Jc and TCR-Cb1. A diagnosis of CGD-CTL was made and electron beam therapy (30 Gy) was performed, resulting in almost complete remission. A new tumour developed on the patient s jaw in September 2004 and was also treated by