[Macrophagic activation syndrome and fulminant hepatitis].
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Biomedical subjects
Publications and source records attributed to S Amal.
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INTRODUCTION: Sweet's syndrome was described for the first time in 1964. It is usually described in adults and remains rare in children. We report a case in a 23 month-old infant. OBSERVATION: A 23 month-old boy presented with diffuse papular, edematous annular plaques surrounded by vesicles and bullas. Laboratory examinations revealed neutrophilic polynuclear hyperleukocytosis, anemia and an inflammatory syndrome. The myelogram was rich and the abdominal sonography normal. Oral corticosteroids (2 mg/kg/d) led to spectacular improvement. After 19 days' treatment, the boy developed cervical adenopathies and hepatomegaly. The second myelogram was normal. Evolution under corticosteroids was good. DISCUSSION: Sweet's syndrome is exceptional in infants. The frequent association with a malignant blood disease should prompt appropriate investigations and prolonged surveillance. Systemic corticosteroid therapy is the reference.
INTRODUCTION: Psoriasis is a frequent dermatosis, its prevalence is estimated of between 1 and 3 p. 100. The severe forms may threaten the functional and life prognosis of patients. PATIENTS AND METHODS: We conducted a retrospective study on 160 cases of severe psoriasis collected between 1990 and 2001. We included patients exhibiting severe psoriasis: pustular, erythrodermic or arthropathic psoriasis, the generalized forms that had developed for more than 6 months, without positive response to treatment and the forms with a PASI greater than 50. RESULTS: The patients' ages ranged from 6 to 85 years with a clear male predominance (96 men, 64 women). Nine familial cases were collected. Erythroderma was noted in 87 cases, pustular psoriasis in 31, generalized psoriasis in 31 and psoriatic rheumatism in 15 (4 of which were associated with pustular psoriasis). Local treatment with topical corticosteroids or a Vitamin D derivative was recommended in respectively 41.8 and 13.75 p. 100 of cases. The administration of general treated relied on methotrexate and was required in 68.75 p. 100 of cases. Progression was usually good, relapses were often noted when treatment was stopped. Four patients died. DISCUSSION: Although the diagnosis of psoriasis is often easy, the severe forms are still difficult to treat. Treatment is complicated, sometimes disappointing and often necessitates heavy and aggressive treatments that require strict surveillance.
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OBJECTIVE: We aimed to determine the epidemiological and clinical profile, and to study the evolution of this disease in the Marrakech region. MATERIAL AND METHODS: We retrospectively studied all patients with a diagnosis of erysipelas admitted in the Department of Dermatology from 1990 to 2002, in the Marrakech Mohamed VI hospital. RESULTS: A total of 100 patients were included in the study, 58 male (58%) and 42 female (42%) patients, age range 9-95 years (mean age: 47 years). The lesions were most frequently located on the lower limbs (87% of the cases), with 82 cases occurring in the legs, whereas the face was affected in 10% of the cases. Erysipelas relapsed in 12 patients (12%). All patients had at least one risk factor: portal of entry (80 cases, with 67 cases of toe web intertrigo), obesity (10% of the cases), lymphedema (6% of the cases), diabetes (3% of the cases). The first line treatment was intravenous penicillin G in 76 cases (76%). Satisfactory results were observed in 78% of the cases. COMMENTS: Erysipelas is common in hospital environment. An early penicillin therapy associated to the treatment of the portal of entry leads to satisfactory results.
BACKGROUND: Specific cutaneous manifestations of sarcoidosis can be extremely varied. We report one observation of cutaneous sarcoidosis with an exceptional clinical presentation: icthyosiform erythroderma. CASE REPORT: A 33-year-old woman had a dry icthyosiform erythroderma associated with arthralgia and muscular deficit of the pelvic belt in a context of fever and weight loss. Skin biopsy revealed the presence of an epithelioid giant-cell granuloma. This granuloma was also found in the salivary glands and in the bronchial mucosa. The chest X ray showed diffuse reticularis opacities. The diagnosis of sarcoidosis was retained. The patient was treated with oral prednisone (0.75 mg/kg/day). Total skin clearing was obtained after 15 days. DISCUSSION: This observation points out the heterogeneous nature of clinical manifestations of cutaneous sarcoidosis. The icthyosis erythroderma form is exceptional. Correct diagnosis is difficult and must be based on clinical, histological, radiological and biological criteria of sarcoidosis.