Ines Kechaou

Hypokalemic rhabdomyolysis: an unusual presentation of Sjogren's syndrome.

Hypokalaemic rhabdomyolysis represents a medical emergency requiring rapid diagnosis and appropriate aetiological treatment. Renal tubular acidosis is a common cause of hypokalemia which can be idiopathic or secondary to systemic disorders such as Sjogrens syndrome. It can remain asymptomatic or manifest with metabolic abnormalities including hypokalemia paralysis, hypocalcaemia and hyperchloremic metabolic acidosis. Rhabdomyolysis presenting with severe hypokalemia as the first manifestation of Sjogrens syndrome is rare. We report a case of a 59-year-old woman who presented to our department with severe weakness of all limbs. Laboratory examination demonstrated hypokalemic rhabdomyolysis caused by distal renal tubular acidosis. Investigations revealed Sjogrens syndrome as the underlying cause of the metabolic disorders.

Orofacial granulomatosis: do not forget leishmaniasis.

A 24-year-old man presented with a 1-year history of increasing swelling in the upper lip with a painful ulcer on the external oral mucosa. On physical examination, the upper lip showed swelling cheilitis, crusting and fissuring lesions. On the external fold of the upper lip, there were ulcerative lesions with necrotic and oedematous labial mucosa (figure 1A,B). The patient did not …

Liver involvement in tuberous sclerosis

Tuberous sclerosis complex (TSC) is a rare inherited disease that affects many organs and tissues, which explains the diversity of its clinical manifestations. This disease is often diagnosed at an early age when cutaneous angiofibromas, epilepsy and mental retardation are associated. The hepatic involvement in this phakomatosis is an uncommon location. In this context, we report the case of a tuberous sclerosis revealed in adulthood by abdominal pain and distension related to renal and hepatic angiomyolipomas associated with typical skin lesions.

Deep vein thrombosis and tuberculosis: a causative link?

Pulmonary tuberculosis is very devastating in developing countries and its thrombogenic potential is a disturbing new entity. We report an 18-year-old woman who presented with a first episode of deep vein thrombosis. Pulmonary, hepatic and splenic tuberculosis was diagnosed while looking for secondary causes. The patient was treated with rifampicin, isoniazid, pyrazinamide and ethambutol along with low-molecular-weight heparin and antivitamin K. Tuberculosis has several mechanisms that induce a hypercoagulable state and can lead to thromboembolic complications.

Hypoparathyroïdie et démence d’installation rapide: penser au syndrome de Fahr

Fahr syndrome is a rare disorder, whose prevalence is lower than 0.5%. Clinical manifestations are variable ranging from mere behavioral disorders to tetany crises and severe neuropsychiatric manifestations. It may be idiopathic or secondary to numerous causes dominated by phosphocalcium metabolism disorders whose main cause is hypoparathyroidism. Positive diagnosis is based on radiology. We report the case of a 78-year old hypertensive female patient with a history of hypothyroidism, hypoparathyroidism after total thyroidectomy performed 30years before. She was treated with Levothyrox® 100 µg 2 capsules/day , One-Alpha® 1 μg 3 capsules/day and calperos® 2 capsules/day resulting in euthyroidia confirmed by clinical examination and laboratory tests and normal phosphocalcic test. Patients outcome was marked, in 2010, by the development of rapidly worsening cognitive impairment and of early onset dementia in a few months. Neurological examination showed extrapyramidal syndrome. Cerebral CT scan showed large basal ganglia calcifications, white and gray linear substance near the junction, in the brain bulb and in the cerebellum. The diagnosis of Fahr syndrome, revealed by dementia occurred after a history of hypoparathyroidism, was then retained.

[Hypercalcemia related to PTH-rP revealing malignant hepatic epithelioid hemangioendothelioma].

Hypercalcemia caused by tumor production of PTH-rp occurs most often in cases of squamous cell carcinoma of the lung, aerodigestive tract cancer, gynecological cancer and lymphoma. We report an exceptional case of PTH-rp related to a hepatic hemangioendothelioma. A 70 years-old male admitted for deterioration of the general state. The laboratory investigations revealed hypercalcemia, related to tumor production of PTH-rp. Imaging revealed tumoral hepatic lesions. Histopathological study and immunohistochemistry showed diffuse response for CD31 marker, CK20 (+) with CK7 (-) and hepatocyt antigen (-). The diagnosis of PTH-rp related to hepatic hemangioendothelioma was make. The patient died with recurrence of fatal hypercalcemia. Management of patients presenting with humoral hypercalcemia includes a vigorous search for tumor lesions. Elevated PTH-rp can be a bad prognostic factor. In front of tumoral liver lesions, a hepatic epithelioid hemangioendothelioma must be considered. Immunohistochemistry is necessary to make diagnosis.

Unicentric Mixed Variant Castleman Disease Associated with Brachiocephalic Vein Thrombosis: A Rare Presentation

Castleman disease (CD) is an uncommon lymphoproliferative disorder occurring mostly in patients presenting with mediastinal lymphadenopathy. Predominant phenotypes are categorized as localized hyaline vascular or multicentric plasma cell variants. Localized form of CD is usually asymptomatic and rarely associated with deep venous thrombosis. We report an exceptional case of mediastinal CD, which presented as retrosternal chest pain, in a 42-year old man. Imaging and pathological examination showed an atypical, mixed and localized form of CD. Thoracic CT scan revealed brachiocephalic vein thrombosis. Investigations didn’t reveal other risk factors for deep venous thrombosis. Deep venous thrombosis is an exceptional complication of localized variants of CD. Therefore, it seems worth looking carefully for this last type of benign lymphopathy when an unusual thrombosis is found.

Phalangeal lytic lesion: do not forget osseous sarcoidosis

Sarcoidosis is a systemic granulomatous disease that most commonly involves the lung and thoracic lymph nodes. However, any organ can be affected. Osseous sarcoidosis has been reported in 3–13% of the cases. The skeletal involvement on radiographs is usually seen late in the course of the disease and is rarely the initial manifestation. We report a case of sarcoidosis revealed by a lytic lesion of the phalanx.