Pyodermia gangraenosum and portal vein thrombosis in a 33-year-old woman

History and clinical findings: The diagnosis of pyoderma gangraenosum (PC) was made in a 33-year-old woman with ulcerative (palm-sized) skin changes a nd pain of the lower leg that had developed over two weeks and was accompan ied by fever (39 degrees C). Treatment with prednisolone and azothiaprine w as initiated. As soon as the medication was reduced new skin changes develo ped. Two months after onset of the illness she had to be hospitalized becau se of fever, epigastric pain on pressure and deteriorating general conditio n. Physical examination provided no significant further information. Laboratory results: The differential count demonstrated leucocytosis (15.5 Gpt/l) with a marked monocytosis (25%) as well as anaemia (haemoglobin conc entration 5.2 mmol/l). C-reactive protein was elevated (120.20 mg/l). Throm boplastin time was 60%, D-dimer 1000 mu g/l, thrombin-antithrombin-III comp lex 9.7 mu g/l. Additional investigations: Sonography and computed tomography of the upper abdomen revealed spenomegaly, ascites, thrombosis of the protal, ploenie an d superior mesenteric veins. Bone marrow puncture showed marked increase in blasts (14%) and monocystes(10%). Treatment and course: The findings indicated chronic myelomonocytic leukaem ia with PG and the described venous thromboses. The cutaneous changes compl etely receded on administration of hydroxyurea (1.0 g/d). Other causes of t he skin eruption were excluded. Phenprocoumon (INR between 2 and 3) was giv en in treatment of the thromboses. Conclusion: When PC is diagnosed, intensive search for an underlying cause must be undertaken, because of its frequent association with serious system ic disease. Only early specific treatment will improve the skin condition.