[Folliculosebaceous cystic hamartoma: anatomo-clinical study]. / Hamartome folliculo-sébacé kystique : étude anatomoclinique.

BACKGROUND: Folliculosebaceous cystic hamartoma (FSCH) is a relatively recently described malformation with follicular and sebaceous components and a particular type of stroma with adipocytes. We conducted an anatomo-clinical study in order to clarify the clinical and histological characteristics of FSCH. MATERIALS AND METHODS: We included all cases of FSCH diagnosed between 1985 and February 2011 at our dermatopathology laboratory. Clinical information was obtained from medical records and requests for histological examination. RESULTS: We studied 25 cases of FSCH in 25 patients of mean age 51 years. The sex ratio was 1.3. The mean disease duration was 9 years. Lesions were described mainly as flesh-colored, occasionally pedunculated nodules and were found primarily on the face (60%). The diagnosis of FSCH had never been mentioned by the clinician. Histological examination revealed in all cases one or more follicular cystic structures surrounded by sebaceous glands in a stroma containing adipocytes. A number of variants were identified, such as the presence of a mucinous stroma, a neuroid component with protein S 100 expression, and rudimentary hair follicles in adjacent dermis. One case involved a proliferating cyst while another was on the scalp in the area of pre-existing radiodermatitis. Only one relapse was noted, 5 years after the initial excision. DISCUSSION: FSCH is a benign, underdiagnosed lesion, localized on the face, particularly on the nose. It is dome-shaped or pedunculated and grows slowly. Differential diagnoses include nevus lipomatosus superficialis and "sebaceous" trichofolliculoma. FSCH can be readily identified by the presence of adipocytes and a fibrous stroma. One case was unique in its appearance of a large pedunculated nodule with a proliferating cyst. Prior to the invidualization of this entity, such cases were interpreted as nevus lipomatosus superficialis or "sebaceous" trichofolliculoma, although their histological appearance was inconsistent with such a diagnosis.

[Annular elastolytic giant cell granuloma]. / Granulome élastolytique annulaire à cellules géantes.

BACKGROUND: Annular elastolytic giant cell granuloma (AEGCG) is a rare form of granulomatous dermatosis. It is characterised histologically by phagocytosis of elastic fibres by multinucleated cells. We report a favourable outcome in a case of AEGCG following PUVA therapy and treatment with synthetic antimalarials. PATIENTS AND METHODS: A 67-year-old retired wine grower presented with highly pruritic annular lesions with raised borders on the shoulders and trunk that had been present for several months. Histological examination of a biopsy sample from the erythematous border was characteristic of AEGCG. Various topical treatments proved ineffective and systemic corticosteroids attenuated the patient's pruritus but had no effect on the skin lesions. PUVA therapy resulted in regression of lesions on the trunk, but the rash spread to the patient's arms and was covered with epidermal microcysts. PUVA therapy was discontinued and treatment with a synthetic antimalarial (hydroxychloroquine 400mg/d) was initiated, resulting in complete regression of the lesions. DISCUSSION: AEGC was isolated in 1979 by Hanke et al. on the basis of five cases seen in females. This is a rare form of dermatosis with some 30 cases being reported in the English literature. The clinical aspect is fairly evocative, with erythematous papular lesions, either alone or in groups, with a raised border and a lighter centre tending towards atrophy. In most cases, the lesions are found predominantly in areas exposed to sunlight. The histological appearance is characteristic, with an image of giant cell elastophagic granuloma from which the name of the disease is taken. This appearance allows the disease to be differentiated from a number of other granulomatous diseases. The aetiology is unknown and treatment is empirical. Spontaneous cure can occur and consistent results have not been obtained with any treatments. In our case, PUVA was partly successful, and the synthetic antimalarials resulted in complete regression of residual lesions.

[Blasch-kolinear psoriasis revealed by infliximab therapy]. / Psoriasis blaschko-linéaire révélé par un traitement par infliximab (Remicade).

BACKGROUND: Blaschko-linear psoriasis is a rare disease about which only a few publications have appeared in the literature. This form of psoriasis poses problems of differential diagnosis with regard to other forms of inflammatory Blaschko-linear dermatoses. Herein, we report an original case, the linear nature of which was revealed by treatment with infliximab. CASE REPORT: A 29-year-old man presented chronic psoriasis present for 17 years and resistant to various forms of systemic therapy. Treatment with infliximab 5mg/kg given on D1, D15 and two-monthly, thereafter resulted in practically complete resolution of all skin lesions after the fourth infusion. The only remaining lesions were psoriatic erythematous-squamous, non-pustular lesions with a Blaschko-linear pattern, limited to one side, on the left arm and left leg. These lesions persisted after 10 courses of infliximab, although no other lesions reappeared. DISCUSSION: This case was original in terms of the revelation of Blaschko-linear lesions during treatment with infliximab, despite the complete disappearance of diffuse psoriatic plaques, thus suggesting the existence in this patient of two cell populations, each having a different response to biotherapy.

[Two cases of subcutaneous panniculitis-like T-cell lymphoma (CD4- CD8+ CD56-)]. / Lymphome T sous-cutané (CD4- CD8+ CD56-) à type de panniculite: deux cas.

BACKGROUND: Subcutaneous T-cell lymphoma is a rare disease and diagnosis is often difficult. We report two cases of subcutaneous T-cell lymphoma simulating panniculitis and initially treated with systemic steroids. PATIENTS AND METHODS: Case No. 1. A 75-year-old woman, otherwise asymptomatic, presented with plaques and nodules of the legs present for 4 months. Histological and immunohistochemical analysis revealed subcutaneous T-cell lymphoma of CD4- CD8+ CD56- phenotype. Improvement was observed with systemic steroids followed by radiotherapy. Case No. 2. A 53-year-old woman presented with a 10-year history of recurrent infiltrated plaques. Lupus panniculitis was evoked but not confirmed. In the context of new panniculitis lesions, histological examination showed a dense lymphocytic infiltrate involving the fat lobules. Lymphocyte immunophenotyping and genotyping led to a diagnosis of subcutaneous T-cell lymphoma of CD4- CD8+ CD56- phenotype. Molecular analysis showed T-cell monoclonality (alpha beta). The lesions improved after few months of systemic corticosteroids. Although new nodular lesions appeared from time to time, further therapy was not deemed necessary. DISCUSSION: A distinction is currently made between two types of subcutaneous T-cell lymphomas and the prognosis and therapeutic consequences differ widely. In our two patients, subcutaneous CD8+ T-cell lymphoma was diagnosed. The clinical course was indolent in both cases. Differential diagnosis with regard to lupus panniculitis can be difficult because of its slow progression. In such cases of low-grade lymphomas, first-line therapy may be limited to systemic corticosteroids.

[Prevalence and management of chronic wounds in 14 geriatric institutions of the Haut-Rhin]. / Enquête de prévalence et des pratiques de prise en charge des plaies chroniques dans 14 établissements gériatriques du Haut-Rhin.

BACKGROUND: We carried out a survey to assess the prevalence of various therapeutic approaches for chronic wounds in 14 primary care and rehabilitation units (SSR) and long-term care units (SLD) in the Haut-Rhin department of France, and we attempted to gauge the interest of doctors and nursing staff in the creation of a Mobile Wound and Healing Unit (EMPC). METHODS: Our anonymous transversal survey was based on the results of two questionnaires, one concerning patients and the other concerning medical and nursing staff. RESULTS: 96 of the 1 163 patients hospitalised at the time of our survey met the inclusion criteria. The global prevalence of sores was 8.3%, while that of bedsores was 6.4% and that of leg ulcers was 1.6%. There were no cases of wounds on diabetic feet. The study population was characterised by a M/F sex-ratio of 0.37, with mean age of 86 years for women and 76 years for men. The mean duration of bedsores was 6 months, compared with 14 months for leg ulcers and a relapse rate of 36% for bedsores and 52.6% for leg ulcers. In more than half of all cases the aetiology of the ulcers was not stated. Bacteriological samples were obtained in 7 cases. Wounds were generally cleansed using soap and physiological saline, with mechanical debridement being used in 4 cases. Hydrocolloids were the dressings used most widely for bedsores, while interfaces were most commonly used in leg ulcers. No topical antibiotics were prescribed. A pain evaluation scale was used in only 18 cases and topical anaesthetics were administered in one case prior to debridement of a leg ulcer. A bedsore risk evaluation scale was completed for 27 of the 75 of the patients presenting bedsores and special preventive mattresses were used for two-thirds of these patients. Twelve of 19 patients with leg ulcers had compression bandaging that was changed daily. Most doctors considered their knowledge of chronic wounds to be good, in contrast with nursing staff, 72% of whom judged their knowledge mediocre or insufficient. All the nursing staff and 11 of the 13 doctors expressed interest in the use of a specialised team for difficult cases. DISCUSSION: The main practices consistent with the recommendations were use of modern dressings, although the latter appeared to be changed too frequently, anecdotal use of antiseptic solutions, abandonment of use of topical antibiotics and nutrition management plans. Two-thirds of patients with leg ulcers wore compression bandages. However, improvements remain to be made concerning the use of topical anaesthetics, manual debridement, use of pain evaluation and bedsore risk scales, and assessment of the aetiology of leg ulcers. CONCLUSION: This survey, conducted prior to the creation of a mobile wound and healing unit based at the Colmar General Hospital, showed that doctors and nursing staff are extremely keen on the idea of specific training and practical advice concerning chronic wound management. It provided a clearer vision of the training requirements of SSR and SLD establishments in terms of chronic wound management.

Emergence of Legionella pneumophila pneumonia in patients receiving tumor necrosis factor-alpha antagonists.

BACKGROUND: Patients treated with tumor necrosis factor-alpha (TNF-alpha) antagonists have an increased risk of infection, but infection due to Legionella pneumophila has rarely been described in patients receiving such therapy. METHODS: A registry involving 486 clinical departments in France was designed by a multidisciplinary group (Recherche Axée sur la Tolérance des Biothérapies [RATIO]) to collect data on opportunistic and severe infections occurring in patients treated with TNF-alpha antagonists. All cases are reported to RATIO in accordance with national health authorities and validated by infectious disease experts. The legionellosis rate among patients treated with TNF-alpha antagonists was compared with the rate in France overall. RESULTS: We report a 1-year consecutive series of 10 cases of L. pneumophila pneumonia in France in 2004, including 6 cases treated with adalimumab, 2 treated with etanercept, and 2 treated with infliximab. The median patient age was 51 years (range, 40-69 years). Eight patients were treated for rheumatoid arthritis, 1 was treated for cutaneous psoriasis, and 1 was treated for pyoderma gangrenosum. The median duration of TNF-alpha antagonist treatment at onset of infection was 38.5 weeks (range, 3-73 weeks). Eight patients were receiving concomitant treatment with corticosteroids, and 6 were receiving treatment with methotrexate. The relative risk of legionellosis when receiving treatment with a TNF-alpha antagonist, compared with the relative risk in France overall, was estimated to be between 16.5 and 21.0. We also report a second episode of confirmed legionellosis following the reintroduction of infliximab therapy. CONCLUSIONS: L. pneumophila pneumonia is a potentially severe but curable infection that might complicate anti-TNF-alpha therapy. In patients receiving anti-TNF-alpha who develop pneumonia, legionellosis should be systematically investigated, and first-line antibiotic therapy should be efficient against L. pneumophila.

[Chromium-induced vasculitis-like purpuric allergic contact dermatitis]. / Dermite de contact purpurique au chrome simulant une vasculite.

INTRODUCTION: Purpuric allergic contact dermatitis is a rare and poorly understood condition. CASE REPORT: A 27-year-old male patient with a personal history of atopic dermatitis since childhood consulted for chronic papular-purpuric rash present for 7 years. Moderate pruritus was seen. Profuse lesions were observed on the palms and soles and on the upper and lower limbs, with sparing of the trunk. These lesions consisted of purpuric papules, in some cases with crusts, forming large plaques. The clinical picture was initially suggestive of vasculitis, but this diagnosis was ruled out by histological examination and laboratory tests. Skin patch tests were evocative of chromium-induced contact dermatitis. Retrospective directed history-taking confirmed the relevance of the latter test since it revealed regular wearing of leather clothing. Lasting cure was achieved following eradication of the allergen. DISCUSSION: Reports of contact purpuric dermatitis are rare. This condition has been described principally for allergens consisting of rubber or dyes used in clothing. Our case was notable on account of the severity of the lesions, mimicking vasculitis, as well as the novelty of the incriminated allergen, chromium, found in leather garments. It underlines the value of routine skin patch tests in the event of chronic non-specific dermatitis. To our knowledge, this is the first reported case of chromium-induced purpuric allergic contact dermatitis.

[Hypersensitivity to fluindione (Previscan). Positive skin patch tests]. / Syndrome d'hypersensibilité à la fluindione (Préviscan). Positivité des tests épicutanés.

INTRODUCTION: Fluindione (Previscan) is an oral anticoagulant belonging to the vitamin K antagonist class and is very widely used in France. While bleeding is a common complication, severe immunoallergic reactions are less frequent. The authors report a case of drug-induced hypersensitivity syndrome. CASE REPORT: A 75 year-old woman was hospitalized for diffuse erythematous papular rash associated with facial oedema. These symptoms appeared 3 weeks after the beginning of treatment with fluindione, allopurinol and perindopril. Laboratory tests showed hyperleukocytosis, mixed hepatitis and moderate renal failure, with the entire picture being evocative of drug-induced hypersensitivity reaction. The eruption was associated with eosinophilia, hepatic cytolysis with cholestasis, and acute renale failure. While allopurinol and perindopril were stopped definitively, fluindione was only suspended temporarily following overdosage. On reintroduction, rapid recurrence of clinical and biologic signs was observed with increased severity. The skin rash resolved completely on withdrawal of the drug. Patch tests performed later were positive for fluindione and negative for allopurinol and perindopril. DISCUSSION: These manifestations were consistent with the diagnosis of drug-induced hypersensitivity syndrome due to fluindione. Very few cases have been described with fluindione despite widespread prescription of the treatment is in France. While there may be no skin involvement, immunoallergic signs such as fever, hepatitis and acute tubular interstitial nephritis have been described with fluindione and these may be related to this syndrome (DRESS - Drug Reaction with Eosinophilia and Systemic Symptoms). Skin patch testing, which is easily performed, can be extremely helpful in determining a causal relationship with medication.

[Assessment of a general practitioner training campaign for early detection of cutaneous melanoma in the Haut-Rhin department of France]. / Evaluation d'une campagne de formation des médecins généralistes et des médecins du travail au dépistage du mélanome dans le Haut-Rhin.

INTRODUCTION: Dermatologists frequently provide training for general practitioners, but such training is not systematic and evaluation is rare. MATERIALS AND METHODS: A training campaign for general practitioners and occupational physicians in melanoma screening was carried out in the Haut-Rhin Department of France in 2004. The 630 general practitioners and 120 occupational physicians in this geographic department received repeated mail together with materials (posters and booklets) for their waiting rooms, and they were invited to attend medical training sessions. The sessions consisted of 10 photographs for initial evaluation, provision of epidemiological information, training in identification of subjects at risk and diagnosis of pigmented lesions, with a further 10 photographs for evaluation at the end. The 20 evaluation photographs were randomly selected for each session from 40 photographs comprising 18 cases of melanomas, 14 of nevi, 4 of basal cell carcinomas and 4 of seborrheic keratoses. At the end of each session, the physicians returned 2 anonymous questionnaires containing their overall assessment of the campaign (Q1) and their answers concerning the 20 evaluation photographs (proposed diagnosis and management strategy) (Q2). The questionnaires were then analyzed. The 40 evaluation photographs were also tested on 47 dermatologists. RESULTS: Three hundred and fifty physicians - 278/630 general practitioners (44%) and 72/120 occupational physicians (60%) - attended 18 medical training sessions. Response levels for questionnaires Q1 and Q2 were 88% and 90% respectively. Almost all of the physicians involved considered the campaign to be warranted. At the end of the training sessions, 96% felt they were better equipped to identify subjects at risk for melanoma and 53% (58% of general practitioners and 39% of occupational physicians, p=0.03) felt they had greatly improved their ability to diagnose pigmented lesions. The median number of correct replies concerning the evaluation photographs increased from 6/10 to 7/10 between the initial evaluation photographs and the final evaluation photographs (p<0.0001), with no difference being seen between the general practitioners and occupational physicians. The median number of correct replies from the 47 dermatologists was 35/40 (8.75/10). The most striking improvement noted during the campaign concerned the ability to identify melanomas. The figure rose from an initial 59% at the start of the session (initial evaluation photographs) (versus 86% for the dermatologists) to 84% by the end of the session (final evaluation photographs) (p<0.0001). The management strategy proposed for photographs of melanoma (irrespective of the proposed diagnosis) was judged "appropriate" in 86% of cases at the start of the session versus 94% at the end of the session (p<0.001). Seborrheic keratosis was correctly identified in 43% of cases by the general practitioners/occupational physicians versus 75% by the dermatologists, and was confused with melanoma in 22% of cases by the general practitioners/occupational physicians versus 5% by the dermatologists. No improvement was seen during the training session in terms of the ability of the general practitioners/occupational physicians to correctly identify seborrheic keratosis. The majority of participants indicated their willingness to participate in the screening program and asked to receive information on melanoma on a regular basis. DISCUSSION: This initial study shows the immediate impact of medical training sessions on general practitioners/occupational physicians to be important in terms of sensitization and moderate but significant in terms of improved diagnosis of pigmented lesions. The longer term impact, as well as the impact of the campaign on physicians not taking part in the medical training sessions, remains unknown. This study militates in favor of the involvement of general practitioners and occupational physicians in mass screening for melanoma in collaboration with dermatologists. The 2004 campaign in the Haut-Rhin Department also included information for the general public. The primary evaluation criterion of the campaign will be change in incidence of thick melanomas recorded by the departmental cancer registry.

[Bullous pemphigoid in young patients: a retrospective study of 74 cases]. / Pemphigoïde du sujet jeune.

INTRODUCTION: Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported. OBJECTIVES: Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations. PATIENTS AND METHODS: Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies. RESULTS: Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy. DISCUSSION: Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.

Immunoelectron microscopy in subepidermal autoimmune bullous diseases: a prospective study of IgG and C3 bound in vivo in 32 patients.

Thirty-two patients suffering from subepidermal autoimmune bullous disease were studied prospectively by clinical examination and immunoelectron microscopy. Clinically, 1 patient had herpes gestationnis, 14 typical bullous pemphigoid (BP), 3 epidermolysis acquisita (EBA), 3 cicatricial pemphigoid (CP), and 11 patients overlapping clinical diseases. These 11 patients shared clinical features of BP, EBA, or CP and a clinical diagnosis could not be done safely. Immunoelectron microscopy revealed diaminobenzidine deposits in 20 patients on the epidermal side of dermo-epidermal junction in the lamina lucida as in BP. In 5 patients, deposits located mostly under the anchoring fibril zone, in the floor of a sublamina densa dermoepidermal separation for 2 of them, were consistent with a diagnosis of EBA. In 6 patients, deposits were located mostly in the lamina densa, in the floor of a dermoepidermal separation occurring in the lamina lucida for 3 of them. This suggests that some of these 6 patients had neither EBA or BP, but another autoimmune bullous disease again, an uncharacterized component of dermoepidermal junction located in the lamina densa. Finally, a correlation exists between the sites of IgG and/or C3 components on epidermal or dermal side of dermoepidermal junction and the presence or absence of characteristic clinical features such as scar, milia formation, or mucosal involvement.

Excess of nevi related to immunodeficiency: a study in HIV-infected patients and renal transplant recipients.

To assess the relationship between immune system and nevi, we studied two models of immunodeficiency caused by different mechanisms, i.e., virus and drug. Our rationale was that if an excess of nevi was found in these two epidemiologic models, it could be concluded that the excess was due to immunodeficiency itself rather than its cause. One hundred ten renal transplant recipients (RTR) were compared with age-, sex-, and phenotype-matched controls. Eighty four HIV-positive patients (HIV+) were compared with similarly matched controls. Nevi < 5 mm (N < 5) or > or = 5 mm (N > or = 5) were counted in three sites representative of regularly, intermittently, and never sun-exposed sites. The number of N < 5 was higher in RTR (p < 0.001) and in HIV+ (p < 0.001) than in respective controls. N > or = 5 were significantly higher only in RTR. These differences tended to be the same for all sites and persisted after adjustment for possible confounding factors. The incidence of atypical nevus was higher in RTR than in controls. Immunodeficiency seems to promote the occurrence of nevi. This supports the concept of immune surveillance of nevi and raises the question of whether sun-induced immune suppression plays a role in the development of nevi. As nevi are risk markers for melanoma, a higher incidence of melanoma could be expected in immunocompromised patients.

Diagnosis of adult linear IgA dermatosis by immunoelectronmicroscopy in 16 patients with linear IgA deposits.

Homogeneous linear IgA deposits at the dermo-epidermal junction (DEJ) shown by direct immunofluorescence are characteristic of what is termed linear IgA bullous dermatosis. However, it is not yet certain that this disease constitutes an entity distinct from other subepidermal blistering diseases, especially when IgG deposits are also present. Sixty-one cases of subepidermal blistering disease in adults were therefore investigated by immunoelectron microscopy (IEM), and the 16 patients observed to have homogeneous linear IgA deposits were compared with the 45 who had no IgA but had IgG and/or C3. In 11 of the 16 patients with IgA (four of whom also had IgG), the deposits were linear and formed a mirror image pattern on each side of the lamina densa from which they were separated by a clear space. In contrast to this monomorphic IEM pattern, clinical and other laboratory findings were very heterogeneous, making exact clinical diagnosis difficult. Of the remaining five patients in this group of sixteen, three (all with both IgA and IgG) had bullous pemphigoid, epidermolysis bullosa acquisita, and cicatricial pemphigoid, respectively, on IEM and clinical investigation. In the remaining two patients (one with both IgA and IgG, and one with IgA only) the deposits were located in the lamina lucida, making precise classification impossible. None of the 45 patients with isolated IgG and/or C3 deposition displayed the mirror image pattern. We conclude that this IEM pattern may constitute a specific diagnostic criterion of linear IgA dermatosis.

Melanoma prevention: evaluation of a health education campaign for primary schools.

OBJECTIVE: To evaluate the effectiveness of an educational campaign aimed toward limiting children's sun exposure. DESIGN: Prospective, multicenter trial with before-after comparison. A school-based campaign was presented as a game during a 4-week period (May 25 to June 1992) with primary school teachers as game hosts. Children were interviewed with a standardized questionnaire in September before (1991) and after (1992) the campaign. Comparisons between the children's answers before and after the campaign were made using paired chi2 tests and analysis of variance. SETTING: Five French primary schools. SUBJECTS: All children in their fourth year of primary school (228 children aged 9 years). MAIN OUTCOME MEASURES: Changes after the campaign in children's answers concerning their knowledge, attitude, and behavior toward the sun during summer holidays. RESULTS: Compared with the precampaign answers, more children after the campaign claimed to protect themselves from the sun with a hat (33.7% vs 23.8%; P = .01) or sunscreen (34.8% vs 25.4%; P = .03), avoided going outside during the sunniest hours (76.8% vs 66.0%; P = .02), reapplied sunscreens (22.1% vs 10.6%; P<.001), considered that a T-shirt and shade provided better protection than sunscreen (82.7% vs 74.8%; P = .05), considered sunlight as a risk factor for skin cancer (74.9% vs 50.7%; P<.001), and spent significantly less time in the sun with their arms (P = .005), trunk, legs, and head uncovered (P<.001). Children with a fair complexion, who were the target of this campaign, showed the best improvement in their responses. CONCLUSION: Health education campaigns can be effective in terms of improving the knowledge, attitude, and behavior of young children.

Genetic susceptibility to toxic epidermal necrolysis.

The pathophysiologic events leading to toxic epidermal necrolysis (TEN) remain unknown. With the idea of an immunologically mediated reaction occurring in predisposed subjects we performed HLA-A, -B and -DR typing in 44 patients surviving TEN. We observed a significant increase of only HLA-B12, previously found associated with ocular complications of Stevens-Johnson syndrome. When patients were stratified according to the drugs involved as causes for their TEN, we found other HLA phenotypes associated with B12, varying with each category of drugs. Sulfonamide-related cases of TEN were linked to A29, B12, and DR7, while oxicam-related cases of TEN were linked to A2 and B12. These results suggest that a genetic background, related to the major histocompatibility complex, may contribute to severe blistering drug reactions.

The culprit drugs in 87 cases of toxic epidermal necrolysis (Lyell's syndrome).

Between 1972 and 1985, 87 patients with toxic epidermal necrolysis (TEN) were admitted to the dermatological intensive care unit at Hôpital Henri Mondor, Créteil, France. The culpable drug was determined by standardized criteria. Only three patients had received no drugs before the onset of TEN. Most patients (71 of 87) were receiving more than one drug. Patients had taken an average of 4.4 +/- 3.4 drugs each. A culpable drug was determined in 67 patients (77%). The mean time from first drug administration to onset of TEN was 13.6 +/- 8.4 days. The culprit drugs included the following: sulfonamides, 18 cases, and especially sulfamethoxazole and trimethoprim, 12; anticonvulsants, seven (barbiturates and carbamazepine only); nonsteroidal anti-inflammatory drugs, 29 (especially the phenylbutazone derivative, 16, and oxicam derivatives, 10); allopurinol, three; chlormezanone, three; and others, seven. Aspirin, antipyretics, and antibiotics are infrequently implicated in this series. The pattern of culprit drugs changed with years. The level of sulfonamide-related TEN remained the same, while incidence of nonsteroidal anti-inflammatory drug-induced TEN increased sharply, the introduction of oxicam derivatives being in part responsible.

Prognostic factors and predictive signs of malignancy in adult dermatomyositis. A study of 32 cases.

Dermatomyositis (DM) is an inflammatory myopathy with a severe prognosis mainly determined by its association with malignancy. We present the results of a study of 32 adults with DM. Our aim was to define predictive signs of cancer and establish prognostic factors according to patient survival. Thirteen (41%) of the 32 patients had DM associated with malignancy. After 7 years of follow-up, the overall mortality rate was 52%. Mortality was higher for patients with malignancy. Cutaneous necrosis and an elevated erythrocyte sedimentation rate appeared as potential markers of associated malignancy. Classic signs of poor prognosis, such as age and association with cancer were found. Interestingly, extensive cutaneous lesions on the trunk and an elevated erythrocyte sedimentation rate were also more frequent among the patients who died. Our results demonstrate that selected groups of patients with DM, in part defined by cutaneous signs, have a poor prognosis.

Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid.

BACKGROUND AND DESIGN: Bullous pemphigoid is usually treated with systemic corticosteroids. Side effects are common in elderly patients, justifying the search for adjuvant therapy. This randomized, multicentric unblind study was designed to assess the efficacy of azathioprine or plasma exchange when added to conventional doses of prednisolone. One hundred patients with active disease entered the study. They were randomly allocated to receive 28 days of treatment with oral prednisolone sodium metasulfobenzoate (1 mg/kg per day) either alone or in combination with oral azathioprine (100 to 150 mg/d) or four large-volume plasma exchanges. After 28 days, the prednisolone doses were progressively decreased according to the same strict regimen in the three groups (in combination with oral azathioprine in group 2). RESULTS: The clinical results were evaluable in 98 of the 100 patients included in the study. There was no appreciable difference in the percentages of complete remission of the disease in the three therapeutic groups at 28 days (71%, 80%, and 71%, respectively) or at 6 months (42%, 39%, and 29%, respectively). Severe complications were more often observed among patients receiving azathioprine. At 6 months, 14 of 98 patients had died, without any differences noted among the three study groups. CONCLUSIONS: We conclude that neither azathioprine nor plasma exchange is effective enough to be used routinely as an adjuvant to corticosteroids in the management of bullous pemphigoid.

Acute generalized exanthematous pustulosis. Analysis of 63 cases.

We retrospectively analyzed 63 observations collected in nine French departments of dermatology of an acute pustular dermatosis, recently named in the French literature acute generalized exanthematous pustulosis (AGEP). Even though 11 of these cases occurred in patients with a history of psoriasis, AGEP appeared distinct from pustular psoriasis based on several slight pathologic differences, drug induction in most cases, and a more acute course of fever and pustulosis, with rapid spontaneous healing. We, therefore, suggest that AGEP is a reaction pattern, perhaps favored by a "psoriatic background." The most frequent causes of AGEP seem to be drug reactions, acute infections with enteroviruses, and hypersensitivity to mercury. With 55 (87%) of 63 cases attributed to drugs in this series, AGEP should be added to the list of cutaneous adverse drug reactions. Among drug-induced skin eruptions, AGEP is remarkable by its short time to onset after the administration of the suspected drug (less than 24 hours in half of our cases) and the great predominance (80%) of antibiotics as causative agents. It is suggested that some cases previously reported as "drug-induced pustular psoriasis" were in fact AGEP.