Aged ; Antiphospholipid Syndrome ; pathology ; Face ; pathology ; Granuloma ; pathology ; Humans ; Male

Antiphospholipid Syndrome ; pathology ; physiopathology ; Child ; Female ; Follow-Up Studies ; Humans

A 32-year-old woman had a recurrent shallow ulcer on the flank. A biopsy specimen showed thromboses in the dermal vessels and she was found to have circulating antiphospholipid antibody with no associated systemic disease. A clean ulcer developed on the flank of a patient with primary antiphospholipid syndrome is considered to be a rarely encountered/unusual presentation of this syndrome.
Adult ; Antiphospholipid Syndrome/pathology ; Antiphospholipid Syndrome/complications* ; Case Report ; Female ; Human ; Skin Ulcer/pathology ; Skin Ulcer/complications*

Abortion, Habitual ; complications ; Antibodies, Antiphospholipid ; blood ; Antiphospholipid Syndrome ; complications ; pathology ; therapy ; Erythema ; complications ; Heart Valve Diseases ; complications ; Humans ; Kidney Diseases ; complications ; Skin Diseases ; complications ; Thrombosis ; complications

Ovarian hyperstimulation syndrome is a serious complication of ovulation induction and has a diverse clinical spectrum from edema to thromboembolism. Antiphospholipid antibody syndrome, one of the well known hypercoagulable states, can be also manifested as an arterial or venous thrombosis and recurrent spontaneous abortion. Sometimes a patient with antiphospholipid antibodies might not notice a miscarriage and seek for assisted reproduction treatment, which harbors a chance of developing ovarian hyperstimulation syndrome. If this happens, the ovarian hyperstimulation syndrome can exacerbate the thrombotic complication of underlying antiphospholipid antibody syndrome, resulting in a catastrophic vascular event. The authors experienced a case of middle cerebral artery infarct, which developed during ovarian hyperstimulation syndrome in a 33-yr-old woman with a previous history of fetal loss. An elevated titer of anticardiolipin antibodies was noticed and persisted thereafter. The authors suggest screening tests for the presence of antiphospholipid antibodies before controlled ovarian hyperstimulation.
Adult ; Antibodies, Anticardiolipin/blood ; Antiphospholipid Syndrome/*complications/pathology ; Female ; Humans ; Iatrogenic Disease ; Infarction, Middle Cerebral Artery/*etiology/pathology ; Magnetic Resonance Angiography ; Ovarian Hyperstimulation Syndrome/*complications/pathology ; Ovulation Induction ; Pregnancy ; *Pregnancy Complications/pathology

OBJECTIVE: To describe the neuroradiologic findings of primary antiphospholipid antibody syndrome (PAPS). MATERIALS AND METHODS: During a recent two-year period, abnormally elevated antiphospholipid antibodies were detected in a total of 751 patients. In any cases in which risk factors for stroke were detected-hypertension, diabetes mellitus, hyperlipidemia, smoking, and the presence of SLE or other connective tissue diseases-PAPS was not diagnosed. Neuroradiologic studies were performed in 11 of 32 patients with PAPS. We retrospectively reviewed brain CT (n = 7), MR (n = 8), and cerebral angiography (n = 8) in 11 patients with special attention to the presence of brain parenchymal lesions and cerebral arterial or venous abnormalities. RESULTS: CT or MR findings of PAPS included nonspecific multiple hyper-inten-sity foci in deep white matter on T2-weighted images (5/11), a large infarct in the territory of the middle cerebral artery (4/11), diffuse cortical atrophy (2/11), focal hemorrhage (2/11), and dural sinus thrombosis (1/11). Angiographic findings were normal (5/8) or reflected either occlusion of a large cerebral artery (2/8) or dural sinus thrombosis (1/8). CONCLUSION: Neuroradiologic findings of PAPS are nonspecific but in young or middle-aged adults who show the above mentioned CT or MR findings, and in whom risk factors for stroke are not present, the condition should be suspected.
Adult ; Antiphospholipid Syndrome/*radiography ; Brain/pathology/*radiography ; Cerebral Angiography ; Female ; Human ; Magnetic Resonance Imaging ; Male ; Middle Age ; Risk Factors ; Tomography, X-Ray Computed

INTRODUCTIONWe report angioedema as a rare presentation leading to a diagnosis of systemic lupus erythematosus (SLE).

CLINICAL PICTUREA diagnosis of angioedema was delayed in a patient presenting with limb and facial swelling until she developed acute upper airway compromise. After excluding allergic and hereditary angioedema, acquired angioedema (AAE) was suspected, possibly precipitated by respiratory tract infection. Associated clinical and laboratory features led to a diagnosis of SLE.

TREATMENTManagement proved challenging and included high dose steroids and immunosuppressants.

OUTCOMEThe patient responded to treatment and remains in remission without recurrence of the angioedema.

CONCLUSIONAAE occurs due to the acquired deficiency of inhibitor of C1 component of complement (C1 INH). Lymphoproliferative disorders and anti-C1 INH antibodies are well-described associations. However, one should also consider the possibility of SLE.

Angioedema ; blood ; etiology ; physiopathology ; therapy ; Antiphospholipid Syndrome ; diagnosis ; etiology ; Brain ; pathology ; Complement C1 Inactivator Proteins ; analysis ; deficiency ; Female ; Humans ; Lupus Erythematosus, Systemic ; complications ; diagnosis ; etiology ; Magnetic Resonance Imaging ; Middle Aged ; Respiration, Artificial ; Respiratory Insufficiency ; etiology ; therapy