[Digital ischemia revealing multiple myeloma]. / Une ischémie digitale révélant un myélome multiple.

Digital ulcers generally arise in a context of microangiopathy-related focal ischemia. In women, connective tissue diseases are the main etiology, while in men the cause is often diffuse arterial disease, e.g. Leo-Buerger disease, or emboligenic heart disease. A paraneoplastic origin of digital necrosis due to ischemia is rarely reported. A 75-year-old man presented with cyanosis of the fingertips and toes that had begun one month earlier. The physical examination found pulp ulcers on the fingers and toes of both hands and feet. Two weeks later, necrotic damage developed distally, with no other associated symptoms. Blood tests were suggestive of Kahler disease; immunodeficiency disorders tests were negative; the cyroglobulin test was positive. Multiple-drug chemotherapy was followed by clinical improvement. Distal necrotic damage is a frequent inaugural symptom in vascular disease. If the common causal mechanisms (iatrogenic, occupational, toxic, atheromatous, emboligenic heart disease, or systemic disease) have been ruled out, it is important to search for a blood disorder or cancer as the cause of distal necrotic damage.

[Lymphohistiocytic activation syndrome (LHAS)]. / Le syndrome d'activation lympho-histiocytaire (SALH).

Lymphohistiocytic activation syndrome (LHAS) is related to inappropriate stimulation of macrophage cells in bone marrow and lymphoid system. LHAS combines the non-specific clinical signs (fever, poor general condition, hepatosplenomegaly, lymphadenopathy) and suggestive biological elements (bi-or pancytopenia, abnormal liver function, coagulopathy, increased LDH, ferritin and triglycerides). The diagnosis of SALH remains an emergency every clinician should discuss before any febrile cytopenia. The etiology of LHAS is still obscure, but recent advances in the genetic study of familial forms provide some essential elements in understanding.

[Acquired ichthyosis and haematological malignancies: five cases]. / Ichtyose acquise et hémopathie maligne : cinq observations.

BACKGROUND: Acquired ichthyosis is a rare condition that can reveal an unsuspected haematological malignancy, thus allowing early diagnosis and management. If ichthyosis regresses under treatment for the haematological disorder, its recurrence reflects a turning point in the course of the disease and implies worsening of the prognosis. PATIENTS AND METHODS: The patients were examined at a joint dermatology/haematology consultation. The diagnosis of ichthyosis was based on clinical examination alone with no patients undergoing skin biopsy. RESULTS: Our series included three men and two women aged 38 to 65 years consulting for a variety of reasons including asthenia, anaemia and adenopathy. Ichthyosis occurred 2 to 9 months after the initial symptoms of the blood disease. Lesions consisted of diffuse brown scales. The disease was associated with lymphadenopathy and biological inflammatory syndrome. Two patients were presenting non-Hodgkin lymphoma, one had Hodgkin's disease, one had chronic myeloid leukaemia in progression and one had an undifferentiated lymphomatous process. Treatment was based on chemotherapy and emollients. The ichthyosis progressed in step with the underlying malignancy in all cases, with regression being complete in three cases, partial in one case and absent in one case. DISCUSSION: In rare cases, acquired ichthyosis reveals systemic disease, and may be of infectious, endocrine or drug origin; it may also be idiopathic. However, it is most often a paraneoplastic syndrome with cutaneous expression encountered during haematological malignancies. Because of the variety of causative blood dyscrasias, ichthyosis cannot be used to guide their diagnosis, although it remains a reliable monitoring tool. CONCLUSION: Acquired ichthyosis should prompt the clinician to search for a neoplastic condition, primarily a haematological disorder, guided by other associated signs, given that in our study, skin lesions generally appear to precede signs of the blood disease.

[Intracardiac thrombosis in Behçet disease: clinical presentation and outcome of three cases]. / Thrombose intracardiaque au cours de la maladie de Behçet : étude de trois observations.

BACKGROUND: Behçet's disease is recognized as a multisystem disorder that affects mainly young adults in Mediterranean, Middle Eastern, and Far Eastern countries. The diagnosis is very difficult because there is no laboratory test. Clinical features - such as orogenital aphtae, ocular and skin lesions, arthritis, and neurologic, gastrointestinal, vascular, and pulmonary symptoms - are helpful for diagnosis. Various cardiovascular manifestations, such as pancarditis, acute myocardial infarction, conduction system disturbances, and valvular diseases, have been reported but are rare. Intracardiac thrombus formation, as seen in our patients, is exceptional even among cardiovascular cases of Behçet's. OBSERVATIONS: We report three cases of intracardiac thrombosis among 204 patients followed for Behçet's disease within our unit over a period of 7 years. We report outcomes after corticosteroid, cyclophosphamide, and oral anticoagulant therapy. DISCUSSION: Cardiovascular involvement has been reported in 7 % to 29 % of patients with Behçet's syndrome. Intracardiac thrombosis is extremely rare and the right heart is the most common site of involvement. The first symptoms and signs of the disease frequently precede systemic organ manifestations. CONCLUSION: Diagnosis of Behçet's disease might be considered if a patient presents with a mass in the right-sided cardiac chambers, even in the absence of the characteristic clinical features of the condition. This is particularly applicable if the patient is a young male from the Mediterranean basin or the Middle East. We suggest that the treatment could include colchicine, anticoagulant therapy, and corticosteroids and discuss immunosuppressive therapy.

[Thrombosis of the inferior vena cava revealing primary antiphospholipid syndrome: a case report]. / Thrombose de la veine cave inférieure sur syndrome primaire des antiphospholipides : à propos d'un cas.

Thrombosis of the inferior vena cava is a rare clinical expression of primary antiphospholipid syndrome. This case clearly illustrates the clinical manifestations, work-up findings and management principles of thrombosis of the inferior vena cava in primary antiphospholipid syndrome before the stage of complication. The patient was a 24-year-old female with a history of recurrent deep venous thrombosis. She was admitted to the department of internal medicine for dyspnea and pain of the right hypochondria. Physical examination disclosed an edematous ascitic syndrome. The scanner coupled with Doppler ultrasonography showed thrombosis of the inferior vena cava. On these radiological findings, an immunological work-up was requested, which showed the presence of antiphospholipid antibodies.