Synovial sarcoma complicating Maffucci syndrome.

Maffucci syndrome is a rare nonhereditary disorder comprising of lymphovascular malformations and multiple enchondromas, which may be associated with several internal malignancies. This report describes a new association of Maffucci syndrome with pedal synovial sarcoma. Our case is also remarkable as lymphangioma circumscriptum is the sole lymphovascular component, which has been rarely reported. The aim of this report is to generate awareness about this rare condition and also highlight the importance of screening for malignancies in this disorder.

Paracetamol-induced Stevens Johnson syndrome and cholestatic hepatitis.

Stevens-Johnson syndrome (SJS) is an uncommon life-threatening skin disease, generally induced by drugs. Extracutaneous manifestations of the syndrome can occur, and may involve the conjunctiva, buccal mucosa, gastrointestinal and genitourinary tracts. Cholestatic hepatitis has been rarely described in SJS. A 29-year-old woman was admitted with generalized cutaneous eruption. A self-medication with paracetamol had been started three days earlier. Clinical signs and skin biopsy were consistent with SJS. Five days later, the patient developed jaundice. Serial liver function tests showed rising transaminases, bilirubin, alkaline phosphatase and γ-glutamyl transferase. Liver biopsy was performed and was consistent with the diagnosis of drug-induced cholestatic hepatitis. Adequate supportive care was provided to the patient. Skin lesions disappeared within two weeks. Jaundice disappeared progressively, and liver tests returned to normal. Herein, we report the first case of SJS associated with cholestatic hepatitis after ingestion of therapeutic doses of paracetamol.

Dermatomyositis and breast cancer: a multicenter Tunisian retrospective study of 13 cases.

AIM: To evaluate the epidemiological, clinical, biological features and prognostic factors in patients presenting an association of dermatomyositis (DM) and breast cancer (BC). METHODS: Medical records of 13 patients with DM and BC among 210 DM collected from January 1982 to march 2009 were retrospectively reviewed. RESULTS: Mean age was 47 ± 18 years. DM preceded BC in 3 patients, was concurrent with BC in 2 cases and followed it in 8 cases. Clinically, ulcerative and bullous lesions predominating on limbs were observed in 2 patients. A severe proximal muscular weakness was observed in 4 cases and one patient has presented an amyopathic dermatomyositis. BC was staged IV in 3 pts, IIIA in 2 cases while 3 had stage IIB, 3 stage IIA and 2 stage I according to TNM classification. Nine out of 13 patients had parallel improvement of DM symptoms after treatment of BC. Five patients died of recurrence or distant metastasis (mortality 41.66%). Median survival was 35 months (3-177) after DM diagnostic. CONCLUSION: In Tunisia, DM is associated with an increased incidence of BC. A paraneoplastic course of DM is noted in 70% of patients. In view of the increased risk of BC in our country, in addition to routine examination and laboratory screening, mammography, chest ultrasound, and gynaecological examination, are indicated in women with DM older than 40 years, particularly in case of previous personal or familial history of breast neoplasm.

Actinic lichen planus of unusual presentation.

Actinic lichen planus (ALP) is a distinct variant of lichen planus mainly involving teenagers with an Asian racial profile. Three clinical types of ALP have been described: annular, pigmented, and dyschromic. We report an ALP with unusual presentation in a 56-year-old woman with no relevant medical history, which was clinically suggestive of actinic keratosis. Histological findings refined the diagnosis by showing typical aspects of lichen planus. This dermatosis, which is frequent in Tunisia because of sun exposure, may cause mainly aesthetic damage and requires adequate photoprotection.

[Norwegian scabies, a rare diagnosis in Tunisia]. / La gale norvegienne, parasitose rare en Tunisie.

UNLABELLED: Norwegian scabies is a particular form of scabies, rarely reported in Tunisia. It habitually occurs in immunocompromised hosts, with prolonged immunosuppressive therapy, congenital immune disease, HIV-1 infection or neoplasm. We report an observation of norwegian scabies, occuring in a woman with prolounged systemic corticosteroid therapy. OBSERVATION: A 65-year-old woman, with a long history of obstructive bronchoneumopathy and bronchiectasis, treated with systemic corticosteroids for 5 years, was referred with a mild pruriginous and scaly erythroderma. This dermatosis developed within 5 months, with fever and impairing of health. Skin parasitological exams showed a massive infestation by sarcoptes. Histologic findings revealed multiple sarcoptes scabiei and eggs in the stratum corneum. The patient was treated with benzyl benzoate during 48 hours. She died rapidly with acute respiratory distress. DISCUSSION: basing on this observation of norwegian scabies occuring in an old immunocompromised woman, we put the accent on this particular form of scabies, often misappreciated, and incite to practice skin and nail parasitological exams in case of mild pruriginous, chronic scaly erythroderma. developing in immunocompromised patients.

Risk factors for erysipelas of the leg in Tunisia: a multicenter case-control study.

BACKGROUND: Risk factors for erysipelas (cellulitis) were rarely evaluated in controlled studies. Regional variations of these risk factors have never be assessed. OBJECTIVE: To assess risk factors for erysipelas of the leg in Tunisia. SUBJECTS AND METHODS: Case-control study in seven hospital centers in Tunisia. Cases were 114 consecutive patients with erysipelas of the leg [sudden onset (<24 h) of a well-demarcated dermo-hypodermatitis with fever or chills]. Two controls were matched to each case for age, sex, and hospital (n = 208). Main outcome measures are local and general suspected risk factors for erysipelas of the leg. RESULTS: In multivariate analysis, disruption of the cutaneous barrier (i.e. traumatic wound, toe-web intertrigo, excoriated leg dermatosis or plantar squamous lesions) and leg edema were independently associated with erysipelas of the leg, with respective odds ratios of 13.6 (95% confidence interval: 6.0-31) and 7.0 (1.3-38). No association was observed with diabetes, alcoholism, or smoking. CONCLUSIONS: We confirmed the major role of local risk factors and the minor role of general risk factors for erysipelas of the leg, in a setting different than the one previously studied. Detecting and treating toe-web intertrigo and traumatic wounds should be considered in the prevention of erysipelas of the leg.

Dermatomyositis and malignancy in Tunisia: a multicenter national retrospective study of 20 cases.

OBJECTIVE AND METHODS: The aim of our study was to report the epidemiologic, clinical, and biologic profiles of dermatomyositis (DM) associated with malignancy in patients from Tunisia. From January 1982 to January 2000, we collected retrospectively 20 case reports of DM associated with cancer from the different university hospital centers of Tunisia. Initial workup included anamnesis, clinical examination, cancer staging and classification, serum muscle enzymes (creatine phosphokinase, lactate dehydrogenase, aldolase, and transaminases), electromyography, and muscular biopsy. We calculated the median survival and mean value of all the variables. Comparisons of statistical tests were done with the Kruskal-Wallis test. RESULTS: Among the 130 DM cases of our study, 20 were associated with cancer (15.38%). The mean age of our patients was 49.6 years and the sex ratio (female/male) was equal to 3. Cancers were mainly those of the breast (35%) and nasopharynx (25%). DM followed a paraneoplastic course in 90% of the cases. The profile of seric muscular enzymes showed a significant statistical difference (P =.05) between a group of patients with severe muscular weakness and a group with moderate muscle weakness only for creatine kinase. The median survival was 36.5 months after diagnosis of DM and 48.6 months after that of cancer. The 5-year actuarial survival was 38% as related to cancer and 16% as related to DM. Mortality was 45%, in 90% as a result of cancer. CONCLUSIONS: In our study, nasopharyngeal carcinoma represents the second cancer associated with DM, after breast neoplasm, demonstrating the frequency of these 2 cancers in our country. Despite our reduced number of study samples, our study also suggests a relationship between severe muscle weakness and high seric muscle enzymes.