Secondary cryofibrinogenaemia due to bronchial adenocarcinoma presenting as cutaneous necrosis

Abstract

Conflict of interest: there are no conflicts of interest A 59‐year‐old man was admitted with a 1‐week history of painful red swollen legs and haemorrhagic blisters. He was otherwise well, except for a past medical history of pulmonary fibrosis. He was a reformed smoker of 20 cigarettes daily for 40 years, having stopped in early 2005. General examination was unremarkable except for finger clubbing; there was a family history of idiopathic finger clubbing. Cutaneous examination revealed necrotic ulcers covered by black eschar with pink to purple irregular edges (Fig. 1). His feet were warm with palpable peripheral pulses. Routine laboratory investigations were normal except for mild neutrophilia and raised inflammatory markers. Coagulation screen demonstrated a prothrombin time of 16.7 s [normal range (NR) 11.5–14], partial thromboplastin time 29.4 s (NR 25.5–38.5) and fibrinogen 6.3 g/L (NR 1.5–4.5). Protein electrophoresis was consistent with an acute‐phase reaction. Urinary protein level was normal, and Bence–Jones protein was not present. Hepatitis B surface antigen, hepatitis C antibodies, rheumatoid factor, antinuclear antibody, antineutrophil cytoplasmic antibodies, cold agglutinins and cryoglobulins were not detected. Anticardiolipin IgG and IgM antibodies were normal. Appearance of cryofibrinogen in the plasma was noted within 24 h with approximately 1016 mg/L of precipitate. Skin biopsy from the area demonstrated fibrin thrombi within small venous channels in both the dermis and subcutis, resulting in epidermal necrosis and blistering with no evidence of a primary vasculitis or perivascular inflammatory infiltrate (Fig. 2). Direct immunofluorescence staining for IgG, IgA and IgM was negative with faint positivity for C3 in some vessels. Chest X‐ray confirmed the appearance of pulmonary fibrosis with possible underlying lymph‐node enlargement. Computed tomography scan of the chest showed a solid peripheral lung lesion in right lower lobe, and extensive right hilar and mediastinal adenopathy with the added complication of pulmonary emboli. Bronchoscopy revealed widening of the right upper lobe bronchus, although no overt endobronchial tumour was seen. Bronchial aspirate showed groups of cytologically malignant epithelial cells with an appearance consistent with a non‐small‐cell carcinoma. Bronchial‐wall biopsy demonstrated the presence of an adenocarcinoma.