[Hemophagocytic syndrome and metastatic melanoma: 3 cases]. / Syndrome d'activation macrophagique et mélanome métastatique : 3 cas.

BACKGROUND: Macrophage activation syndrome was initially described during viral infections in immunocompromised patients. Since the original report, many diseases have been found to be associated with macrophage activation syndrome. Lymphoproliferative disorders have been more frequently reported to be associated with macrophage activation syndrome than solid tumors. We herein report three cases of macrophage activation syndrome in patients with metastatic malignant melanoma. CASE-REPORTS: Two young 32 and 40 year-old men with a liver metastatic malignant melanoma and a 62 year-old woman with a polymetastatic malignant melanoma presented a sudden deterioration of general health with hyperthermia and biological abnormalities: liver cytolysis, leucocytosis, thrombocytopenia, hypertriglyceridaemia. A fatal clinical outcome occurred rapidly despite corticotherapy and/or chemotherapy. For the first two patients the macrophage activation syndrome diagnosis was delayed because of the similarities of macrophage activation syndrome and metastatic malignant melanoma symptoms. DISCUSSION: The diagnosis of macrophage activation syndrome in patients with metastatic malignant melanoma may be difficult because of the similarities between clinical features of macrophage activation syndrome and those of metastatic malignant melanoma. Hypertriglyceridaemia is present in 60 p. 100 of macrophage activation syndrome and should lead to process a bone marrow aspirate. The search for a triggering infection should be systematically carry out because it is implicated in more than half of macrophage activation syndrome whatever the associated disease may be: neoplasia, autoimmune disease. The pathogenesis of macrophage activation syndromes occurring in patients with metastatic cancer remains unexplained. Treatment of macrophage activation syndrome is not unanimously established and usually consists in the treatment of the associated condition as well as a corticosteroid and/or an immunosuppressive treatment regimens. Prognosis of macrophage activation syndrome is usually poor especially when it is associated with a neoplasia since a fatal outcome occurs in 40 to 60 p. 100 of cases.

[Evaluation of the diagnosis of pigmented tumors of the skin and factors leading to a decision to excise. Dermatologists of the Postgraduate Association of Haute-Normandie]. / Evaluation du diagnostic des tumeurs pigmentées de la peau et des éléments conduisant à une décision d'exérèse. Dermatologues de l'Association de FMC de Haute-Normandie.

INTRODUCTION: The necessity of excising melanomas characterized by a slight thickness at an early stage, leads dermatologists to remove pigmented lesions which do not correspond to melanomas. The aims of this study were: a) to prospectively assess the accuracy of melanoma diagnosis, b) to quantify the number of excisions performed according to the degree of melanoma suspicion, c) to determine the specific clinical sign or signs of relevant diagnostic value. PATIENTS AND METHODS: This study was conducted prospectively from January 1996 to August 1997 by dermatologists in private practice and dermatologists from a University Hospital staff. When it was decided to excise a pigmented lesion, a form was filled out choosing the most appropriate clinical diagnosis, the degree of melanoma suspicion, and clinical signs which lead to surgery. Based on histological findings as the reference, the sensitivity, specificity, accuracy of melanoma diagnosis and the kappa test that evaluates the concordance between clinical and histological diagnosis, were performed. The diagnostic value of clinical signs was assessed by variance analysis. RESULTS: Of the 353 excised lesions, 38 (10.7 p. 100) were identified as melanoma on histologic examination. The sensitivity, the specificity and diagnostic accuracy were: 79 p. 100, 94 p. 100 and 53 p. 100 respectively. The kappa test concordance between clinical and histological diagnosis was 0.66. Two hundred and two lesions (57 p. 100) were excised even though the clinical suspicion of melanoma was poorly considered. Only one of these 202 lesions was identified histologically as a true melanoma. Thirty seven (24.5 p. 100) of the 151 remaining excised lesions with an "average" or "strong" suspicion were true melanomas. The clinical signs considered, alone or associated, had a poor predictive positive value (< 38 p. 100). An analytical approach performed with a logistic model permitted the identification of two associated signs suggesting a best diagnostic value. DISCUSSION: This is the only study, to our knowledge, reported in the literature which prospectively assesses the sensitivity, specificity and concordance between clinical and histological diagnosis of melanoma. Results were considered from average to good. The originality of this study was to assess the number of pigmented lesions excised according to the degree of melanoma suspicion, suggesting the possibility of reducing the number of nevi removed when the melanoma risk was considered clinically poor. Finally, this study emphasizes the limits of clinical semiology and the need for future diagnostic methods in the assessment of melanoma.

Indomethacin treatment of eighteen patients with Sweet's syndrome.

BACKGROUND: The standard treatment for Sweet's syndrome (acute febrile neutrophilic dermatosis) is oral corticosteroids. Despite a good initial response, the disease is characterized by frequent relapses. OBJECTIVE: Our purpose was to test the therapeutic effect of the nonsteroidal antiinflammatory drug indomethacin on Sweet's syndrome. METHODS: All patients with Sweet's syndrome observed during a 4-year period were given indomethacin, 150 mg/day for the first week and 100 mg/day for two additional weeks. The therapeutic response was assessed on days 4, 7, 14, 30, and 180. RESULTS: Seventeen of 18 patients had a good initial response; fever and arthralgias were markedly attenuated within 48 hours and eruptions cleared between 7 and 14 days. The remaining patient's cutaneous lesions continued to develop and were successfully treated with prednisone (1 mg/kg/day). The only side effect of indomethacin treatment was epigastric pain in two patients. No patient had a relapse after discontinuation of indomethacin (mean followup, 20.1 months). CONCLUSION: Indomethacin is a safe and effective treatment for Sweet's syndrome.

[Generalized pseudoxanthoma elasticum combined with vitamin K dependent clotting factors deficiency]. / Pseudoxanthome élastique généralisé associé à un déficit en facteurs vitamine-K dépendants.

INTRODUCTION: Pseudoxanthoma elasticum is a connective tissue disease currently classed in 4 forms. Two forms are inherited via dominant autosomal transmission and the other two via recessive autosomal transmission. The generalized form of pseudoxanthoma elasticum is the most uncommon form and corresponds to recessive type II. Clinical manifestations include the typical generalized "peau d'orange" skin associated with hyperlaxity of the skin. Usually, there is no systemic manifestation. CASE REPORT: We report a case of a patient with generalized pseudoxanthoma elasticum associated with deficiency of vitamin-K dependent factors II, VII, IX, and X. A search for other causes of vitamin-K dependent factor deficiency was negative. DISCUSSION: The association of generalized pseudoxanthoma elasticum with deficiency of vitamin-K dependent clotting factors has been reported previously in very rare cases and is probably not fortuitous. It could led to the definition of a sub-group of recessive autosomal pseudoxanthoma elasticum.

[Acute necrotizing cutaneous streptococcal infection following bites or scratch by dog or cat]. / Streptococcies cutanées aiguës nécrosantes après morsure et griffure de chat et de chien.

INTRODUCTION: Domestic animal bites or scratches are quite frequent. Among banal bacteria isolated from infected bites or scratches, group A streptococcus seems to be frequently associated with severe infections. CASE REPORTS: Three cases of acute necrotizing cutaneous streptococcal infections, following cat or dog bite or scratch are reported. Twice, group A streptococcus was isolated from cutaneous swabs. In the third case, previous antibiotic therapy had sterilised bacteriological samples. Diagnosis was ascertained on the basis of clinical presentation and significant antistreptococcal antibodies elevation. Skin necrosis around the inoculation area was observed in the 3 cases. Cicatrisation required an average of two months under appropriate treatment. DISCUSSION: An evolution towards cutaneous necrosis localized to the initially injured area is common to these three cases. This peculiar evolution is worth to be known in order to choose an effective anti streptococcal antibiotherapy whenever domestic animals bites and scratches are to be treated.

[Type B Haemophilus influenzae cellulitis in an immunocompetent adult]. / Dermohypodermite bactérienne à haemophilus influenzae type B chez un adulte immunocompétent.

INTRODUCTION: Only a few cases of Haemophilus influenzae type B cellulitis have been reported in adult patients. They generally involve immunocompromised patients, and are located in the cervico-facial areas. CASE-REPORT: We report a 43 year-old immunocompetent patient who presented a cellulitis of the leg. No cutaneous or upper respiratory pathways entry portal was found. Abcedation of cutaneous lesions allowed the isolation of numerous colonies of Haemophilus influenzae type B from a subcutaneous fluid aspirate. COMMENTS: The unusual site of the infection, the age and the immunocompetent status of the patient, the lack of initial infectious localisation and the slow healing of lesions despite appropriated antibiotic therapy, are of particular interest.