Osteopoikilosis is a rare asymptomatic sclerosing bony dysplasia of benign origin. It is usually found incidentally on radiological examinations. Familial occurrence indicates a genetic milieu with autosomal dominant pattern. Here, we present a case report of a young woman suffering from pelvic pain due to osteopoikilosis (OPK). The same disorder was later found in her son and daughter.
been started to manage those events. Patient said that she had stopped taking warfarin recently. With this history in mind, APS was suspected and assessment of the antiphospholipid antibodies was ordered. The result showed that her Anticardiolipin antibody (aCL) and lupus anticoagulant (LA) were positive but ANA, DsDNA, cANCA, p-ANCA, SS-A, SS-B and CRP were negative. Her ESR was 33 mm/h and ACL IgG isotype titer was 168. She was transferred to the rheumatology ward, where the treatment with warfarin and aspirin was started. A few days later, she developed gastrointestinal bleeding and then anticoagulation was stopped. Her hemorrhagic bullae was turned to an ulcerative form and expanded, so she had to take an anticoagulant again. One week later, patient complained of fl ank pain and became hypotensive with slight abdominal distention and left fl ank pain. Her hemoglobin was assessed again and it was 8.2 g/dl. A hemorrhagic event was suspected and computed tomographic scan of the abdomen revealed a retroperitoneal hematoma. Warfarin was stopped in fear of expansion of hematoma but aspirin was continued. Risk factors for thrombosis such as arterial sclerosis were assessed using color Doppler Ultrasonography but any kind of stenosis in aorta and main branches of abdominal aorta and lower extremity artery were not found. Skin lesions spread. The patient developed deep necrotic wounds extending to the deep layers in both legs. Her three aff ected fi ngers became necrotic. Patient developed massive hematuria. Cystoscopy revealed bladder wall thickening along with numerous vesicles and necrotic lesions. On the 4th week of her stay in the hospital, she complained of abdominal pain. Sonography indicated echo reduction and rectosigmoid wall thickening which was in favor of colitis and it was confi rmed by Computed Tomography.The blood levels of protein C, protein S, factor V and antithrombin III were in the normal range. She underwent laparotomy because of severe abdominal pain and had a rectosigmoid colon resection with colostomy with Hartman pouch. At surgery, it was found that left ovary and fallopian tube were necrotic and unilateral salpingo-oophorectomy was performed. Mod Rheumatol, 2015; 25(3): 490-492A fulminant, often fatal variant of the antiphospholipid syndrome (APS) was fi rst described in 1992 as catastrophic antiphospholipid syndrome (CAPS) [1,2] in which, small vessel occlusive disease aff ects parenchymal organs and leads to multi organ failure. In nearly 60% of patients, a precipitating factor could be identifi ed [3]. The most common triggering factor is an infection but other factors such as trauma and anticoagulation problems (warfarin withdrawal) could also be a trigger of the catastrophic event.Here, we describe a case of a 26-year-old woman with no history of APS, who developed extensive skin necrosis and multi organ failure following withdrawal of warfarin and a viral infection. She attended the emergency department of academic hospital due to painful large skin necrosis in her right thigh, left l...
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