Destructive facial T-cell lymphoma: the difficulty in diagnosis of pyoderma-like processes.

A 48-year-old woman presented with extensive facial ulceration of 1 year in duration. Based on the combination of the clinical appearance and a “nondiagnostic” biopsy taken elsewhere, the patient was started on oral prednisone at a dose of 40 mg/day, with a working diagnosis of pyoderma gangrenosum. According to the patient, the ulceration worsened over the 2 months whilst on prednisone and pain control was a major issue, controlled for the most part with oral oxycodone. One month prior to our evaluation of the patient, she was started on dapsone at 50 mg/day with no added benefit. Approximately 2 years prior to our evaluation, she developed raised, reddish, skin lesions on the abdomen and legs which recurred, but healed spontaneously each time after a few weeks. Her past medical history was remarkable for the remote use of intravenous drugs (which she had stopped for the past 20 years) and the past and continued use of alcohol on a daily basis. She had recently been tested elsewhere and was found to be positive for hepatitis C, but not human immunodeficiency virus (HIV). Examination revealed several ulcerations of the face and forehead, with an “apple jelly” coloration to the periphery and necrotic center. There was complete erosion of the nasal sidewall with apparent involvement of the septum (Fig. 1). There were also scattered, smaller, nonulcerated, reddish to purplish lesions on the abdomen. Otolaryngologic examination revealed ulceration of the right ala and erosion of the nasal septum, but was otherwise unremarkable. No cervical or submental lymphadenopathy was noted. Figure 1. Facial ulceration at the time of initial evaluation Download figure to PowerPoint Routine laboratory tests showed the patient to be anemic, with a hemoglobin level of 11 g/dL (normal, 12–15.5 g/dL) and a white blood count of 13,300 (normal, 3500–10,500) The total bilirubin was normal at 1.0 mg/dL, but several liver function tests were elevated by 2–4 times the upper limit of normal, including aspartate aminotransferase (AST), alanine aminotransferase (ALT), and γ-glutamyl transpeptidase (G-GT). Hepatitis C tests were abnormal, including antibody to hepatitis B core antigen (anti-HBc) and anti-hepatitis C virus (anti-HCV) antibodies, and hepatitis C-RNA was positive. Anti-hepatitis B surface antigen (anti-HBsAg) and antibody were negative. Human immunodeficiency virus (HIV) was negative. Antinuclear antibodies, antineutrophil cytoplasmic antibodies (ANCA), serologic tests for syphilis, cryoglobulins and cryofibrinogens, renal function, and urinalysis were all either negative or normal. The chest X-ray showed bilateral pulmonary nodules, confirmed by computed tomography (CT) scan of the chest. Serologic tests for several fungal organisms, including Sporothrix and Cryptococcus, were negative. Tuberculin skin testing was negative. Two 4-mm punch biopsies were taken from the right cheek and from the right lower lateral abdomen near the hip. Biopsy of facial tissue cultures for acid-fast bacilli and fungal elements was negative. Bacterial culture revealed 1+Staphylococcus aureus and coagulase-negative Staphylococcus. Polymerase chain reaction (PCR) testing for herpes and varicella viruses was negative. Histologic examination of the skin biopsies revealed identical findings from both sites. There was parakeratosis, serum crust, and mild irregular epidermal acanthosis. Focal interfacial dermatitis was present and characterized by basal vacuolization and melanophages in the superficial dermis. Epidermotropism of atypical lymphocytes singly and in cell clusters was seen in the epidermis. In the papillary and reticular dermis, extending to the subcutaneous fat, there was a dense, diffuse, perivascular, interstitial, and periappendageal infiltrate composed of atypical lymphocytes, lymphocytes, histiocytes, plasma cells, and neutrophils. The atypical lymphocytes infiltrated the hair follicles with little associated spongiosis (pilotropism) (Fig. 2). The atypical lymphocytes also surrounded and infiltrated the eccrine glands and blood vessels. Immunoperoxidase studies were performed on paraffin tissue and showed that the atypical lymphocytes were immunoreactive with CD3 (Fig. 3) and βF1, but not with CD8, CD56, T-cell-restricted intracellular antigen-1 (TIA-1), or granzyme B. There was a reactive population of B lymphocytes immunoreactive with CD20. In situ hybridization for Epstein–Barr virus (EBV), performed on paraffin sections, was negative. Lung biopsy of several nodules was accomplished via video-assisted thoracoscopy and revealed a peripheral T-cell lymphoma. Gene rearrangement studies of skin and lung nodules showed similar T-cell clones and were positive for T-cell rearrangement by PCR (TCR PCR, T γ-chain positive). Figure 2. Histopathology of a skin lesion. (a) Dense atypical lymphocytic infiltrate demonstrating pilotropism and epidermal involvement with little associated spongiosis (hematoxylin and eosin stain, × 200). (b) Higher power view better demonstrating the dense atypical lymphocytic infiltrate (hematoxylin and eosin stain, × 400) Download figure to PowerPoint Figure 3. Strong CD3 immunoreactivity of the infiltrate (CD3 stain, × 200) Download figure to PowerPoint The final diagnosis was peripheral T-cell lymphoma with pulmonary and skin involvement. There was no evidence for EBV infection. The patient returned home to receive treatment with four cycles of hyper-CVAD (cyclophosphamide, vincristine, adriamycin, and dexamethasone), followed by autologous stem cell transplantation. The facial erosions and ulcerations, as well as the systemic nodules, responded well to therapy, with only limited recurrence of the right lower lateral abdominal lesions, which were subsequently treated with radiation (Fig. 4). The patient's clinical status 18 months after treatment is complete remission from lymphoma, but she has developed fulminant liver failure secondary to cirrhosis associated with hepatitis C. Figure 4. Facial lesions after completion of chemotherapy Download figure to PowerPoint