Chronic unilateral facial ulcer revealing Wegener's granulomatosis in a patient with rheumatoid arthritis.

Wegener's granulomatosis (WG) is an autoimmune multisystem small-vessel vasculitis (1). The incidence of cutaneous manifestations of WG (nodules, ulcers, purpura, petechiae, haemorrhagic pustules, erythematous papules and plaques) was reported to range from 28 to 64% (2, 3). We report here the case of a 66-year-old man who presented with chronic unilateral facial ulcer and was proven to have biopsy-confirmed WG. It is noteworthy that the patient had pre-existing rheumatoid arthritis (RA), cutaneous candidiasis and onychomycosis. A 66-year-old Chinese man presented with an 8-month history of non-painful ulcer on the left face. He had a 10-year history of arthritis of the extremities, resulting in symmetrical deformities of multiple small joints of the hands and feet. He was diagnosed with RA by typical clinical manifestations, elevated serum rheumatoid factor (RF) and typical X-ray findings in the department of rheumatology 6 years ago. He had intermittently administered low-dose prednisone since then. A productive cough with bloody sputa developed 2 years before presentation. He had a 30-year history of cigarette smoking. He had no history of tuberculosis or tuberculosis exposure. A purified protein-derivative skin test was negative. Physical examination revealed a 7 × 9 × 11.5 cm deltoid-shaped sharply marginated ulcer on his left face, with erythematous elevated borders, puruloid exudates, bloody crusts and irregular shaped hypertrophic scars in the centre (Fig. 1A). White patches were observed involving the proximal regions of 4 toenails (Fig. stacking of the second and third toes on top of the great toe were also seen (Fig. S1H). Laboratory tests showed a total white blood cell (WBC) count of 20.45×10 9 /l (3.97–9.15), leukocytosis (88.1%), elevated platelet 579×10 9 /l (85–303), and reduced haemoglobin 89 g/l (131–172). Erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), RF and anti-streptolysin O (ASO) were 84 mm/h, 140 mg/dl (0–8), 2470 IU/ml (0–30) and 649 IU/ml (0–116), respectively. Serum γ globulin was 31.4% (11.6–24.4). Serological tests for cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear ANCA were negative. Serum antibodies against HIV and syphilis were negative. Serum 1,3-β-D-glucan was negative. Direct smears of thrush, sputum and pus in the ulcer showed spores and pseudohyphae, and cultures revealed Candida albi-cans. Deep scrapings of the white patches of the toenails and potassium hydroxide preparation from the materials showed numerous hyphae. Cultures on Sabouraud glucose agar revealed colonies of Trichophyton rubrum. Gram stains, bacterial cultures, acid-fast bacilli smears and mycobacterial cultures of pus and sputum were negative. The …