Primary cutaneous CD8+ T-cell lymphoma masquerading as acral vascular syndrome.

Acral vascular syndromes (AVS) require thorough testing as, they can reveal varied underlying conditions, including immunological and haematological disorders or neoplasia (1). We report here the first case of primary cutaneous CD8 + T-cell lymphoma (CTCL) presenting with ulcerated infiltrated patches exclusively located on acral areas including fingers, toes, ears and nose. This case which evolved over 4 years, raises challenging questions as to how it should be classified and as to whether it may impact our current diagnostic approach to AVS. A 74-year-old man was hospitalized to investigate ulcerated AVS. He had developed permanent and painful erythematous patches on his fingers and toes 5 months earlier. His condition had worsened in early summer, with onset of ulcerations on distal portions of his fingers and toes, along with extension to his ears and nose without systemic symptoms. The patient had emigrated from Algeria 40 years previously, and had formerly worked in a car factory. Medical records included pulmonary tuberculosis 30 years previously, uncomplicated type 2 diabetes controlled with metformin, uncomplicated chronic hepatitis B and delta, and a 40 pack-year history of cigarette smoking. Physical examination revealed erythematous ulcerated patches on the dorso-lateral aspects of all his fingers and toes, ears and nose (Fig. 1A–C). No livedo or cyanosis were found. Laboratory tests showed normal liver and kidney functions, erythrocyte sedimentation rate, reactive protein C, serum complement levels blood count, and coagulation tests. Serological tests were all negative for rheumatoid factor, antinuclear antibodies, anticardiolipin antibodies, HIV and hepatitis C virus, while chronic co-infection with hepatitis B and Delta viruses without viral replication was confirmed. Brachial arteriography and muscular biopsy were within normal ranges. Tests for cold agglutinins, cryoglo-bulin, and cryofibrinogen were repeatedly negative. Computed tomography of the thorax, abdomen and pelvis revealed only a thyroid multi-nodular goitre, along with biological hyperthyroidism without anti-TSH receptor, thyroglobulin or anti-thyroperoxidase antibodies. The attending hepatologist introduced an antiviral therapy in this setting of chronic B hepatitis (adefovir dipivoxil, 10 mg/day) and the patient stopped smoking, without subsequent improvement. On re-examination, biopsies were performed on infiltrated patches peripheral to the ulcerations of toes and fingers, and corticosteroid regimen was started. On histopathological analysis, the lower dermis was found to be massively infiltrated by medium-to large-sized pleomorphic lymphocytes sparing the epidermis and subcutis. Focal features of angio centrism and angiodestruction were noticed on a small vessel of lower dermis (Fig. S1 1). Atypical cells were diffusely positive …