A Bayesian Neural Network approach to estimating the Energy Equivalent Speed.

To reduce the number and the gravity of accidents, it is necessary to analyse and reconstruct them. Accident modelling requires the modelling of the impact which in turn requires the estimation of the deformation energy. There are several tools available to evaluate the deformation energy absorbed by a vehicle during an impact. However, there is a growing demand for more precise and more powerful tools. In this work, we express the deformation energy absorbed by a vehicle during a crash as a function of the Energy Equivalent Speed (EES). The latter is a difficult parameter to estimate because the structural response of the vehicle during an impact depends on parameters concerning the vehicle, but also parameters concerning the impact. The objective of our work is to design a model to estimate the EES by using an original approach combining Bayesian and Neural Network approaches. Both of these tools are complementary and offer significant advantages, such as the guarantee of finding the optimal model and the implementation of error bars on the computed output. In this paper, we present the procedure for implementing this Bayesian Neural Network approach and the results obtained for the modelling of the EES: our model is able to estimate the EES of the car with a mean error of 1.34 m s(-1). Furthermore, we built a sensitivity analysis to study the relevance of model's inputs.

Determinants of severity for superficial cellutitis (erysipelas) of the leg: a retrospective study.

BACKGROUND: Superficial cellulitis (erysipelas) of the leg is a frequent infectious disease with a favorable outcome, whereas some patients present a serious disease. The determinants of severity for superficial cellulitis (erysipelas) of the leg have not yet been clearly established. In order to determine the characteristics of patients presenting with severe superficial cellulitis of the leg, we analyzed patients with favorable and unfavorable outcome. METHODS: The records of 167 patients referred to Rouen University Hospital for non-superficial cellulitis of the leg were analyzed. Two severity groups of patients were retrospectively defined. Patients in the severe group either died secondary to infection during hospital stay or were hospitalized for a duration at least equal to the 90th percentile (i.e., >21 days of hospitalization). The remaining patients were considered as presenting with non-severe cellulitis. Potential determinants of severity were analyzed by univariate and multivariate analysis based on logistic regression. RESULTS: From univariate analysis, the following general factors were positively associated with severity: advanced age, arterial hypertension, diabetes mellitus, elevated leukocytosis, and elevated neutrophilia. The local factors associated with severity were ulcer of the leg and arteriosclerosis obliterans of the leg. From multivariate analysis, only age (P=0.004), diabetes mellitus (P=0.01), and leukocytosis (P=0.04) appeared to be independently associated with severity. A close to significant association was also found with arteriosclerosis obliterans of the leg (P=0.07). Whereas general complications occurred more frequently in the severe group, no such difference was observed for local complications. CONCLUSIONS: Determinants of severity for superficial cellulitis of the leg include high age and associated medical conditions. Aged patients and patients with diabetes mellitus, elevated leukocytosis, or possibly arteriosclerosis obliterans of the leg should preferably be hospitalized for specific care of associated conditions to avoid the occurrence of general complications.

[Chronic eczematiform eruption in the elderly]. / Eruptions eczématiformes chroniques du sujet âgé

INTRODUCTION: Eczematiform eruptions in the elderly represent a relatively frequent motive for consultation and may lead to repeated hospitalization. Their etiologic diagnosis is often difficult and explains the frequent relapses. The frequent relapses can be explained by the difficulty in determining their etiologic diagnosis. The aims of this study were: 1) to specify the evolving characteristics of these eruptions in elderly patients and 2) determine their etiology. PATIENTS AND METHODS: The inclusion criteria in this retrospective study were: patients aged over 65 at the time of diagnosis, presenting with extensive eczematiform eruption (> 20 p. 100 of body surface) and lasting for more than one month. Eczema on stasis dermatitis of the lower limbs and generalized contact eczema were excluded. Eighty-three patients followed between January 1990 and January 1999 were included. The clinical, biological, histological and evolving characteristics were analyzed. RESULTS: Mean age of patients was 77 +/- 8 years and the male female sex ratio was 2.4. Patients received a mean of 4.0 +/- 2.6 drugs/patient, consisting essentially of cardiovascular and psychotropic agents. The cutaneous eruption had evolved a mean of 12.5 months (1 to 48 months) before diagnosis. Eczema was pruriginous in 92 p. 100 of cases. Frequent relapses were observed in 68 p. 100 of cases. Precise etiologic diagnosis was retained in 48 patients (58 p. 100). This was disseminated contact eczema (n=19), lymphoma cutis (n=10), atopic eczema (n=7), scabies acariasis (n=6) and pemphigoid (n=6). No etiologic diagnosis was retained in the remaining 35 patients (42 p. 100). Comparison of the characteristics in the 2 groups showed excessive consummation of medicinal products (p=0.024), predominant eruption of sun-exposed areas (p=0.004) and a greater frequency of histological images of keratinocyte necrosis (p=0.0072) in patients presenting eczematiform eruptions of unknown etiology. DISCUSSION: These observations suggest the eventual responsibility of medicinal products in the occurrence of certain extensive and chronic eczematiform eruptions in the elderly. However, the delays of imputability of various causal drugs were often longer than those currently admitted for toxidermia, and the withdrawal of potentially imputable agents rarely led to spectacular improvement in the lesions. A case test report is in progress to specify this hypothesis.

[Photodermatosis induced by hydroxychloroquine: 4 cases]. / Toxidermies photo-induites par l'hydroxychloroquine: 4 cas.

BACKGROUND: Hydroxychloroquine is an antimalarial drug often used in dermatology for its photo-protective effects. Four cases of photodermatosis induced by hydroxychloroquine are reported. CASE REPORTS: Four patients, aged from 21 to 68 years, developed a photolocalized eruption from 6 days to 10 weeks after starting hydroxychloroquine. The minimal erythemal dose was decreased in the total spectra and UVA at the onset of the eruption and became normal after stopping hydroxychloroquine in the 2 patients that were controlled. In 3 cases, hydroxychloroquine was the only single drug imputable; chronological imputability was plausible. In the last case, both hydroxychloroquine, carbamazepine and fluvoxamine had a common imputability which was plausible. In the 4 cases, a favourable outcome was observed after stopping hydroxychloroquine, and no recurrence occurred with a mean follow-up of 3.8 years (1-4 years). In one case, a photodistributed eruption occurred during treatment with a related molecule: chloroquine. DISCUSSION: Photodermatosis with hydroxychloroquine have rarely been described in the literature, while quinine from which hydroxychloroquine is derived, is well known for its risk of photosensibilization. The main differential diagnosis of these drug eruptions is an eruption caused by the photodermatosis that initially required treatment with hydroxychloroquine.

[Cutaneous aseptic abscesses, manifestations of neutrophilic diseases]. / Abcès cutanés aseptiques, manifestations des dermatoses neutrophiliques.

BACKGROUND: Neutrophilic skin disease includes several entities: Sweet syndrome, pyoderma gangrenosum, erythema elevatum diutium, Sneddon-Wilkinson sub-keratous pustulosis, and neutrophilic eccrine hidradenitis. We report two cases of aseptic abscesses which correspond to the deepest anatomoclinical form of neutrophilic dermatosis. CASE REPORTS: A 28-year-old man was hospitalized for fever and abdominal pain with bloody diarrhea in relation with Crohn's disease. The patient also presented two skin abscesses on the lower limbs. Bacteriology specimens were negative. The histology specimen of a skin lesion revealed neutrophil infiltration of the hypodermis without granulomatosis. Systemic corticosteroid therapy was given and rapidly led to resolution of the inflammatory bowel disease and the skin lesions. The patient developed inflammatory spondylarthropathy several months later. The second patient was a 36-year-old woman with a history of splenomegaly with asceptic abscesses. She was admitted for abdominal pain with non-bloody diarrhea, fever and multiple joint pain related to spondylarthropathy. She developed several simultaneous abscessed nodules on the legs. Biopsy revealed neutrophil infiltration of the hypodermis. The diagnosis of neutrophilic disease with aseptic cutaneous and visceral abscesses was retained. Nonsteroidal antiinflammatory drugs and dapsone were given leading to regression of the skin lesions and the abdominal and joint pain. DISCUSSION: Aseptic skin abscesses result from a deep localization of neutrophilic disease. They suggest the presence of inflammatory bowel disease, spondylarthropathy or other aseptic visceral localizations.

[Histological and clinical forms of the eosinophilic cellulitis]. / Formes anatomocliniques du syndrome de Wells.

BACKGROUND: Wells' syndrome is characterized by clinical features of cellulitis and a histological picture of eosinophilic infiltrate of the dermis with some "flame" figures. PATIENTS AND METHODS: The clinical and histological features of nine patients with Wells' syndrome seen from 1988 to 1998 were retrospectively reviewed. RESULTS: The clinical features of the nine patients (five men and four women) were urticaria (n=1), cellulitis (n=2), annular plaques (n=3), vesiculo-bullous lesions (n=2) and edema of the face with nodules of the conjunctiva (n=1). Histological examination of skin biopsies showed an eosinophilic infiltrate of the dermis associated with some "flame" figures in all cases. The infiltrate was located in the superficial or deep dermis in accordance with the different clinical features. One patient developed a non Hodgkin lymphoma and presented successively: a Wells' syndrome, a leucocytoklastic vasculitis and a Sweet's syndrome. Numerous treatment were used: topical corticosteroids, H1-antihistamines, dapsone and systemic corticosteroids. Two patients relapsed after treatment withdrawal. DISCUSSION: This study demonstrated a wide polymorphism of the clinical and histological features of Wells' syndrome. The clinical features seem to depend on the location of the dermal infiltrate, suggesting the existence of a spectrum of eosinophilic dermatoses, like in neutrophilic dermatoses. The successive occurrence of vasculitis, Wells' syndrome and Sweet'syndrome in a patient suggests an overlap between these diseases. Systemic corticosteroids are the most effective treatment, but may lead to a corticosteroid dependence.

[Detection of clonal T-cell receptor gamma gene rearrangement with the use of PCR-DGGE for diagnosis of erythroderma]. / Réarrangement des gènes du récepteur des lymphocytes T cutanés par la technique de PCR-DGGE pour le diagnostic étiologique des érythrodermies.

BACKGROUND: It is often difficult to establish the etiological diagnosis of erythroderma because clinical findings and immunohistology cannot always distinguish between lymphomatous erythroderma and inflammatory erythroderma. The purpose of this work was to assess the contribution of PCR-DGGE for detecting clonal T-cell receptor gamma gene rearrangement to the etiological diagnosis of erythroderma. PATIENTS AND METHODS: The following inclusion criteria were used: patient with erythroderma; skin biopsy for histologic study, immunophenotyping and molecular biology; minimal follow-up of 12 months after initial diagnosis. Thirty patients were included from May 1, 1995 to November 30, 1998. Histology slides were reread by one of the authors blinded to other data who classed them in three categories: probable lymphoma, probable inflammatory disease, uncertain diagnosis. Molecular data were also analyzed in the same blinded manner. Immunohistology diagnosis was compared with the molecular data and the final diagnosis retained from clinical, histological and molecular findings as well as the disease course to last follow-up (November 1, 1999) after a mean 12 +/- 18 months follow-up. RESULTS: Eight biopsies were classed as probable lymphomas; a T-cell clonal rearrangement of the TCR genes was detected in 7/8 cases. The one sample with no detectable T clone was a drug-induced Sézary pseudolymphoma. The histologial classification identified 16 cases of probable inflammatory disease; no clonal rearrangement of the TCR genes was found in these cases. One of these patients had fungoid mycosis treated with caryolysin for three months and developed treatment intolerance at the time of the skin biopsy. For six biopsies the histological diagnosis was "uncertain"; a clonal rearrangement of the TCR genes was found in 2/3 of the fungoid mycosis cases and in none of the three cases of toxic dermal reactions. DISCUSSION: This study demonstrated the contribution of genotypic analysis with PCR-DGGE to the diagnosis of erythroderma. Monoclonal TCR gene rearrangement was detected in 9/11 (82 p. 100) of the patients with lymphoma and in 0/19 of the patients with an inflammatory dermatosis. The etiological diagnosis of erythroderma is an excellent indication for molecular stud of skin biopsies with PCR-DGGE.

[Hydroa vacciniforme: dietary fish oil]. / Hydroa vacciniforme: traitement par huiles de poisson (Maxepa(R)).

BACKGROUND: Hydroa vaccinniforme is a highly uncommon photodermatosis acquired in childhood. The clinical course is dominated by the risk of varioliform scars. Numerous treatments have been proposed with variable efficacy. One recent open study suggested dietary fish oil could be useful. We report a case of hydroa vacciniforme treated successfully with dietary fish oil (Maxepa(R)). CASE REPORT: A 15-year-old girl consulted in May 1988 for vesiculobullous lesions typical of hydroa vacciniforme in photo-exposed areas. Anti-malaria drugs and photoprotection had been used for several years without success. Maxepa(R) was introduced in June 1998 and was followed by regression of the lesions within a few weeks despite the summer season. The treatment was interrupted at the patient's request due to fetid breath. Reintroduction of Maxepa(R) in April 1999 after an episode of recurrent lesions, again led to total resolution of the lesions within three weeks. DISCUSSION: Hydroa vacciniforme is an exceptional photodermatosis of uncertain etiology. It may possibly be related to an abnormal sensitivity to ultraviolet A. Fish oil rich in 3-omega polyunsaturated fatty acids would reduce the local inflammation triggered by sun exposure. Recent studies have demonstrated that dietary fish oil can increase the level of 3-omega polyunsaturated fatty acids in the epidermis and reduce the level of prostaglandins in the skin. Our case would appear to confirm the contribution of dietary fish oil to treatment despite the poor tolerance due to fetid breath.

[Mouth ulcers in patients receiving tacrolimus]. / Ulcérations buccales chez un malade recevant du tacrolimus.

INTRODUCTION: The buccal side effects of immunodepressors are well defined with cyclosporine and certain antimitotic agents. We report a case of buccal ulcerations in a patient treated with a new immunosuppressive macrolide: tacrolimus (Prograf). OBSERVATION: A 53 year-old woman presenting a severe cardio-myopathy, underwent heart transplantation in May 1997. Tacrolimus was introduced in October 1997 after 3 episodes of acute reject. Eight months after tacrolimus, painful apthoid buccal ulcerations appeared. Biopsy of the buccal mucosa and other biological examinations revealed no particular etiology. Since tacrolimus could not be stopped, treatment with thalidomide was initiated. It was suspended on two occasions due to adverse events. The buccal ulcerations relapsed rapidly. The intrinsic imputability of tacrolimus in the occurrence of these lesions was noted "l2" ("plausible"). DISCUSSION: Several arguments suggest that these buccal ulcerations may result from the toxicity of tacrolimus: 1) absence of past history of apthae; 2) anatomo-clinical aspect of the lesion differing from that of common apthae, but similar to the ulcerations observed with nicorandil; 3) delay in occurrence of analogous ulcerations compared with that observed with methotrexate or nicorandil; 4) absence of another etiology; 5) relapse of ulcerations on two occasions after suspension of thalidomide, whilst tacrolimus was continued.

[Medium-term renal involvement in adult patients with Henoch-Schönlein purpura]. / Purpura rhumatoïde de l'adulte: evaluation de l'atteinte rénale a moyen terme.

INTRODUCTION: The aim of this study was to estimate the frequency of medium-term renal involvement, in a series of adult patients with Henoch-Schönlein purpura treated in a dermatology department. PATIENTS AND METHODS: Seventeen patients with Henoch-Schönlein purpura followed from 1991 to 1997 in the department of dermatology were included in the study. Renal tests included: research of microscopic hematuria, proteinuria, plasma creatinine levels and calculated creatinine clearance which were measured during initial hospitalization and at the date of the study in May 1998. RESULTS: 10 men and 7 women (mean-age 51 years) were followed up for 39 months (6 to 79 months). Initial renal involvement was observed in 11 patients (65 p. 100). A microscopic hematuria was also observed in 9 patients (53 p. 100), a proteinuria in 6 (35 p. 100), an association of hematuria and proteinuria in 4 (23.5 p. 100). No patient had renal failure. In May 1998, only 2 patients (11.7 p. 100) had renal involvement, that consisted of proteinuria: 3.3 and 3. 9 g/d respectively, with no renal failure. DISCUSSION: The 65 p. 100 frequency of renal involvement discovered during the acute phase of HSP in our series was similar to other series mentioned in the literature, which mainly consisted of hematuria and/or proteinuria. The frequency of long-term renal involvement depends on the origin of patient recruitment. Nephrological studies have reported high levels of renal involvement, that probably overestimated the true frequency. In the present study, as well as in two other studies from a non-nephrological recruitment, the frequency of long-term renal involvement was estimated between 11 p. 100 and 16 p. 100 and was primarily persistent proteinuria. CONCLUSION: The frequency of renal involvement in the adult patient with HSP during the acute phase of the disease and after a medium-term follow-up was approximately 50 p. 100 and between 11 p. 100 to 16 p. 100 respectively.

Sensitivity and specificity of clinical, histologic, and immunologic features in the diagnosis of paraneoplastic pemphigus.

BACKGROUND: Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease characterized by the production of autoantibodies mainly directed against proteins of the plakin family. An overlapping distribution of autoantibody specificities has been recently reported between PNP, pemphigus vulgaris (PV), and pemphigus foliaceus (PF), which suggests a relationship between the different types of pemphigus. OBJECTIVE: Our purpose was to evaluate the sensitivity and the specificity of clinical, histologic, and immunologic features in the diagnosis of PNP. METHODS: The clinical, histologic, and immunologic features of 22 PNP patients were retrospectively reviewed and compared with those of 81 PV and PF patients without neoplasia and of 8 PV and 4 PF patients with various neoplasms. RESULTS: One clinical and 2 biologic features had both high sensitivity (82%-86%) and high specificity (83%-100%) whatever the control group considered: (1) association with a lymphoproliferative disorder, (2) indirect immunofluorescence (IIF) labeling of rat bladder, and (3) recognition of the envoplakin and/or periplakin bands in immunoblotting. Two clinicopathologic and two biologic features had high specificity (87%-100%) but poor sensitivity (27%-59%): (1) clinical presentation associating erosive oral lesions with erythema multiforme-like, bullous pemphigoid-like, or lichen planus-like cutaneous lesions; (2) histologic picture of suprabasal acantholysis with keratinocyte necrosis, interface changes, or lichenoid infiltrate; (3) presence of both anti-epithelial cell surface and anti-basement membrane zone antibodies by IIF; and (4) recognition of the desmoplakin I and/or BPAG1 bands in immunoblotting. Interestingly, 45% of patients with PNP presented initially with isolated oral erosions that were undistinguishable from those seen in PV patients, and 27% had histologic findings of only suprabasal acantholysis, which was in accordance with the frequent detection of anti-desmoglein 3 antibodies in PNP sera. CONCLUSION: The association with a lymphoproliferative disorder, the IIF labeling of rat bladder, and the immunoblotting recognition of envoplakin and/or periplakin are both sensitive and specific features in the diagnosis of PNP.

[Inpatient and outpatient follow-up of grade I malignant melanoma]. / Etude comparative du suivi des malades atteints de mélanome de stade Ien pratique libérale et hospitalière.

BACKGROUND: In 1995, the French consensus conference on management of patients with grade I malignant melanoma recommended clinical examination for patient monitoring. To date, only one survey has been conducted to evaluate these recommendations and their consequences, providing no means of assessing follow-up practices. The aim of this study was to assess follow-up practices in patients with grade I malignant melanoma followed in an outpatient private practice setting and in a hospital setting with regular appointments. PATIENTS AND METHODS: This retrospective study was conducted in collaboration with private practice and hospital dermatologists, all members of an association of continuing medical education. Medical records of 584 patients with grade I malignant melanoma who had undergone surgery between January 1, 1991 and December 31, 1995 were reviewed. Three hundred twenty-nine patients were followed in an exclusively outpatient setting by their private dermatologist and 265 were followed in a hospital setting. Follow-up data were: age, sex, date of surgical excision of the melanoma, Breslow thickness, date of each follow-up visit, presence of possible metastases and mode of diagnosis. RESULTS: Patient features were different in the two groups: mainly greater Breslow thickness and more frequent metastatic course in patients followed in a hospital setting. Among all patients, 65 (11 p. 100) developed metastases. Diagnosis of metastasis was made clinically in 95 p. 100 whatever the mode of monitoring considered. The number of patients lost to follow-up was 11p. 100 among those followed in a hospital setting and 42 p. 100 in those followed in a private practice setting. Patients lost to follow-up had a higher risk of developing metastasis as their average Breslow thickness was 1.7 mm. CONCLUSION: This study shows that patients followed in a hospital setting have a more severe prognosis than patients followed in private practice. It confirms that systematic use of complementary tests is of little interest in detecting metastases since over the period considered, the diagnosis of metastasis was made clinically in most cases. It also discloses difficulties encountered in exclusively outpatient follow-up as a high number of patients were lost to follow-up in this setting. A systematic appointment fixed by the private dermatologist during the follow-up period appears to be needed to ensure good quality follow-up. Such an appointment system should help reduce the number of patients lost to follow-up.

Evolution of chronic recurrent multifocal osteitis toward spondylarthropathy over the long term.

OBJECTIVE: To retrospectively assess, with a sufficiently long followup (mean 11.6 years; median 9 years), the long-term outcome of chronic recurrent multifocal osteitis (CRMO), a multifocal, inflammatory bone disease. METHODS: Patients included were 8 children/adolescents and 7 adults with no family history of rheumatic disease who had been diagnosed as having CRMO between 1979 and 1995. Ten patients had undergone at least 1 bone biopsy of the lesions, with histologic examination and multiple cultures. In 1996, in addition to an in-depth interview, 12 patients underwent an extensive physical examination, laboratory evaluation, HLA-A, B, C, and DR typing, bone radiography and scintigraphy, and computed tomography scan of the sternoclavicular and sacroiliac joints. RESULTS: Remission was observed in 3 patients. The other 12 patients developed various associations of vertebral (n = 10), sacroiliac (n = 6), anterior thoracic (n = 7), peripheral articular (n = 2), enthesopathic (n = 4), or dermatologic (palmoplantar pustulosis in 3 cases and psoriasis in 2) involvements. Spine involvement was the most common and occurred the earliest (median time to appearance after the onset of osteitis 5.63 years). Clinical sacroiliitis was always unilateral. No patients carried the HLA-B27 haplotype. CRMO responded well to nonsteroidal antiinflammatory drugs. Twelve patients met the European Spondylarthropathy Study Group criteria for spondylarthopathy. CONCLUSION: After 10 years, CRMO had usually evolved to spondylarthropathy, but with certain features not usually seen in the latter: predominantly, unilateral sacroiliitis, no familial form, and no link with HLA-B27.

[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.

[Epidermal nevus associated with a type I neurofibromatosis and a nephroblastoma: a new epidermal nevus syndrome?]. / Association d'un naevus épidermique, d'une neurofibromatose de type I et d'un néphroblastome: un nouveau syndrome du naevus épidermique?

We report the case of a 6-year-old boy who showed a large epidermal nevus mixed with a plexiform neurofibroma, which was associated with "café au lait" macules and a nephroblastoma. This association could not be classified in one of the five well defined epidermal nevus syndrome. To our knowledge this is the first time that this type of epidermal nevus syndrome has been described, which raises the question of the relationship between neurofibromatosis 1, nephroblastoma and epidermal nevus.

[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.

Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus.

Tunisian pemphigus is a newly described form of endemic pemphigus whose clinical, histological and epidemiological characteristics have recently been detailed. The objective of this study was to analyse the binding properties of autoantibodies present in sera from patients with endemic Tunisian pemphigus using immunoblotting and indirect immunoelectron microscopy (IEM). Thirty patients with pemphigus foliaceus (PF) and six with pemphigus vulgaris (PV) seen in the dermatology department of Tunis Hospital between 1992 and 1994 were selected for this study. Seven of 30 (23%) and six of 12 (50%) PF sera tested bound to the 160 kDa band of desmoglein 1 when tested on bovine tongue and human epidermal extracts, respectively. Two of six and two of three PV sera tested bound to the 130 kDa desmoglein 3 in these two extracts. Immunoblot and indirect IEM showed that 24 of 30 (80%) PF sera contained IgG1, IgG3 or IgG4 antibodies that bound to a 185-kDa polypeptide localized on the desmosomal plaque. This immunological analysis showed that most endemic Tunisian pemphigus sera correspond to PF sera and are characterized by a high frequency of autoantibodies directed against a recently identified 185-kDa antigen of the desmosomal plaque.

[Eczema-like cutaneous graft versus host disease treated by UV-B therapy in a 2-year-old child]. / GVH cutanée eczématiforme traitée par UV-B thérapie chez un enfant de deux ans.

INTRODUCTION: Chronic graft versus host disease (GVHD) has rarely been reported in children. Optimal treatment should minimize infectious complications and preserve the child's growth. We report a case of cutaneous GVHD in a two year-old boy, who presented an eczema-like eruption and responded well to broad band UV-B therapy. CASE REPORT: A two year-old boy with acute myeloblastic leukemia had a heterologous bone marrow transplantation with a graft issued from an unrelated female donor. Three month later, he developed eczema-like lesions of the trunk, arms and legs associated with diffuse alopecia, despite oral corticosteroids and cyclosporine treatment. Histologic findings were consistent with GVHD. Topical corticosteroids and broad band UV-B therapy were initiated, while oral corticosteroids and cyclosporine doses were tappered off. GVHD lesions cleared, allowing withdrawal of oral corticosteroids and cyclosporine 3 and 12 months respectively after initiation of UV-B therapy. No relapse occurred 24 months after systemic treatment discontinuation and 12 months after broad band UV-B therapy was stopped. CONCLUSION: This observation suggests that broad band UV-B therapy is an effective treatment for eczema-like, cutaneous GVHD.