Search

Introduction: The management of metastatic cutaneous melanoma is changing, marked by innovative therapies. However, their respective use and place in the therapeutic strategy continue to be debated by healthcare professionals., Objective: The French national cancer institute has led a national clinical practice guideline project since 2008. It has carried out a review of these modalities of treatment and established recommendations., Methods: The clinical practice guidelines development process is based on systematic literature review and critical appraisal by experts. The recommendations are thus based on the best available evidence and expert agreement. Prior to publication, the guidelines are reviewed by independent practitioners in cancer care delivery., Results: This article presents the results of bibliographic search, the conclusions of the literature and the recommendations concerning locoregional treatments of brain metastases for patients with metastatic cutaneous melanoma., (Copyright © 2014. Published by Elsevier Masson SAS.)

Background: Recent years have seen the emergence of new molecules for the treatment of patients with metastatic cutaneous melanoma, with significant benefits in terms of survival and the opening of new therapeutic perspectives. In addition, many techniques are currently being developed for locoregional treatment of metastatic sites. Management of metastatic melanoma is thus fast-changing and is marked by innovative therapeutic approaches. However, the availability of these new treatments has prompted debate among healthcare professionals concerning their use and their place in therapeutic strategy., Aims: Since 2008, the French National Cancer Institute (INCa) has been leading a project to define and diffuse national clinical practice guidelines. It has performed a review of these treatment methods, which it aims to circulate, and it is seeking to develop recommendations in order to allow nationwide implementation of innovative approaches while promoting good use thereof., Methods: The clinical practice guidelines development process is based on systematic literature review and critical appraisal by experts within a multidisciplinary working group, with feedback from specialists in cancer care delivery. The recommendations are thus based on the best available evidence and expert agreement. Prior to publication, the guidelines are reviewed by independent practitioners in cancer care delivery., Results: This article presents the national recommendations for first- and second-line systemic treatment and for locoregional treatment of metastatic sites in patients presenting metastatic cutaneous melanoma., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: The data concerning changes in the characteristics of hypertensive leg ulcers (HLU) were taken from open studies in a small patient cohort. The aim of this study was to describe the epidemiological characteristics and to identify prognostic factors for healing in a prospective cohort of 59 patients presenting HLU., Patients and Methods: The cohort comprised patients included in a randomized, double-blind, controlled study published elsewhere; the patients were receiving becaplermin gel (Regranex(®)) or Duoderm Hydrogel™ once daily for eight weeks for the most recent wound. Total follow-up was 12 weeks., Results: The epidemiological analysis was performed for 59 consecutive patients randomized in 17 dermatology departments. Mean patient age was 74.5 ± 9 years and 61% were female. Mean wound duration was 11.1 ± 9 weeks and median wound area was 16 cm(2) (q1; q3: 8; 25.5). Among, 94.9% of patients had hypertension and 39.7% were diabetic. A homolateral peripheral pulse was present in 91.5% of patients. At the end of follow-up, complete wound healing was obtained in 30.5% of the patients. In univariate analysis, neither the foregoing criteria nor the treatment group were significantly associated with healing during the study., Conclusion: This study confirms female predominance, old age, prevalence of diabetes and delay in the diagnosis of HLU. The prognosis for healing does not appear to be dependent on wound duration, wound area or the presence of moderate peripheral arterial disease, doubtless because this condition progresses by episodes of flare-up and under specific conditions., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: The usual treatment for extramammary Paget's disease (EMPD) is surgery, but this approach may have grave functional and physical consequences, as well as high recurrence rates. Topical photodynamic therapy (PDT) offers an optional approach for EMPD; it has a high complete response rate and there is no dose restriction. The aim of this study was to evaluate the efficacy and safety of PDT in the treatment of EMPD., Patients and Methods: This series of patients was seen at a single centre between 1 December 2005 and 31 December 2010. All patients with histologically confirmed EMPD were included. Patients received two courses of PDT 21 days apart: 3 hours after topical application of methyl aminolevulinic acid emulsion, they underwent illumination with red light (570-670 nm) at a dose of 37 J/cm(2) for 10 minutes. In the event of relapse, a further cycle was given at week 6., Results: Eight patients (seven female, one male) of a mean age of 69 years were included. After two series of two illuminations, seven patients were in complete clinical remission at 3 months and one patient was in partial remission. Five patients were still in complete clinical remission at 6 months. All patients had relapsed after a mean 8.4 months (4-14 months). The limiting factor appears to be pain occurring during illumination. Patients reported satisfaction with the disappearance of symptoms and a notable improvement in quality of life., Discussion: The complete clinical response rate to PDT at month 6, after two series of two illuminations, was equivalent to that for surgery. Although the recurrence rate was high, this treatment may be repeated without functional or physical consequences. PDT resulted in disappearance of pain and improved quality of life., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Background: Verrucous carcinoma is a rare, low-grade, slow-growing, well-differentiated squamous cell carcinoma affecting the skin (particularly on the foot) and mucosa. The diagnosis is often delayed. We report a study of twelve cases of verrucous carcinoma of the lower limbs., Patients and Methods: We retrospectively studied a series of 12 patients (8 women, 4 men) who developed verrucous carcinoma of the lower limbs between 1978 and 2005 and we analyzed their follow-up data., Results: The mean age of patients was 78 years (66-97 years). Eleven patients exhibited verrucous carcinoma in a previous lesion comprising varicose ulcer (5 cases), mixed ulcer (3 cases), burn (2 cases) or traumatic lesion (1 case). The mean time from onset of lesions to diagnosis was 28 years. Nine patients showed locoregional extension (8 bone involvement, 3 lymph node involvement). No visceral metastasis was detected. Three patients received medical treatment that proved ineffective. Two received secondary surgical treatment. Nine patients underwent surgery (6 amputations, 3 local excisions). Four patients were lost to follow-up, 4 died, 3 showed no recurrence and 1 had a chronic unhealed wound after surgery., Discussion: Verrucous carcinoma of the lower limbs is a disease of the elderly, affecting both men and women, and occurring mainly on chronic venous ulcerations. The clinical presentation is evocative although histopathological diagnosis is difficult, particularly in the event of superinfection. Repeated and deep biopsies are needed to avoid delay in diagnosis. Extension is chiefly locoregional and visceral involvement is rare. Medical treatment is ineffective and may even be harmful, with surgery the best option. Regular monitoring is necessary because of the risk of relapse, although verrucous carcinoma does not seem to directly affect patient survival.

Background: Large B-cell lymphoma of the leg in elderly subjects, of intermediate prognosis according to the new EORTC classification, may present as nodular or ulcerated forms. There has been relatively little study of the various etiological hypotheses advanced, including venous insufficiency. We report the case of an elderly man with chronic leg ulcer, recently undergoing modification, in which microscopy revealed large B-cell lymphoma (CD20-)., Patients and Methods: A 78 year-old man presented chronic ulcer of the right leg of mixed origin with severe venous insufficiency and arteritis. In the previous 2 months, 2 nodules appeared in the centre of the ulcer. Histological examination of a skin biopsy revealed the presence of large B-cell lymphoma and immunohistochemical analysis showed positive anti-CD79a+, CD20- antibody labeling of cells. Staging studies showed only locoregional invasion. Because of the positive CD20- labeling, ZEM chemotherapy (idarubicine, cyclophosphamide, prednisolone) was given, resulting in disappearance of the nodules after four months and preliminary epidermal healing of the ulcer. Several months later, severe infectious complications necessitated amputation. Examination of the excised sample showed no residual tumor., Discussion: Many causal links have been proposed between large B-cell lymphoma of the leg and aetiologies such as infectious agents, Koebner phenomenon and chronic lymphedema, as well as various other vascular factors. A number of hypotheses were proposed in the present case. It may have been leg ulcer cutaneous B-cell lymphoma, or, more likely, development of lymphoma on a chronic mixed ulcer, with the respective roles of vascular disease, local immunosuppression and antigenic stimulation subject to debate.

Background: Pyogenic granuloma, or botryomycosis, occurring after minor injury or scratching with a septic implement, is a rapidly growing benign inflammatory vascular tumour, often involving the skin or mucous membrane. We report 5 unusual cases of pyogenic granuloma revealing fistula tracking to foreign bodies such as a pace-maker or orthopaedic material, and deep infectious sites with fistula., Methods: This retrospective study included 5 men of average age 72.2 years over a period of 22 years (between 1981 and 2003). Clinical images were recorded and a diagnosis of botryomycosis was confirmed in all patients by histological analysis. Four patients presented suppuration. The time from initial clinical signs to diagnosis, presence of traumatic events, screening for microscopic organisms, response to systemic antibiotic therapy, recurrence and clinical features of botriomycosis were analysed., Results: All patients presented botryomycosis with fistula and suppuration revealing deep bone infection and in one patient, pace maker infection. Complete resolution of the lesions occurred after excision of foreign material and prolonged systemic antibiotic therapy., Conclusion: We report the first series of botryomycosis revealed by fistula and showing deep infectious disease. Pyogenic granuloma is a well-known but rarely reported dermatologic condition.

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.

Introduction: Clinical features and extent of bullous pemphigoid lesions differed widely among patients. The pathogenic role of anti-BPAG2 antibodies has been recently demonstrated. The aim of this study was to analyze the relationship between clinical features of bullous pemphigoid patients and the antigens recognized by their serum., Patients and Methods: One hundred and twelve bullous pemphigoid patients were included in this prospective multicenter study. Inclusion criteria were the following: 1) diagnosis of bullous pemphigoid established on the presence of 3 of the 4 clinical features of bullous pemphigoid, histological picture of bullous pemphigoid and positive direct immunofluorescence; 2) serum available for immunoblotting studies. The clinical and biological findings were prospectively recorded on standard forms. Sera were collected and analyzed using indirect immunofluorescence and immunoblotting on human epidermal extracts., Results: Analysis of patient's clinical features depending on the antigens recognized by their serum showed that patients whose serum contained anti-BPAG1 antibodies had more frequently pruritus, blisters on the lower limbs and a positive indirect immunofluorescence. Patients whose serum contained anti-BPAG2 antibodies had blisters more frequently localized on the head, and a more frequently negative indirect immunofluorescence. Patients whose serum was negative by immunoblotting had less frequently urticarial and/or eczematous lesions, bullae less frequently localized on the lower part of the trunk, abdomen and lower limbs, lower eosinophilia and a more frequently negative indirect immunofluorescence., Conclusion: Patients with circulating anti-BPAG1 antibodies exhibited the most typical, clinical and biological features of bullous pemphigoid.

Introduction: The so-called "Lever scheme" therapeutic regimen has been proposed in the borderline forms of pemphigus to reduce the side effects of systemic corticosteroids., Patients and Methods: A retrospective study was conducted in 8 hospital centers. The criteria for inclusion were the clinical diagnosis of pemphigus, confirmed by histological examination and direct immunofluorescence and first line therapy using the "Lever scheme" protocol, combining 40 mg of prednisone on alternate days and 100 mg/day of azathioprine., Results: Twenty-two patients, seen between January 1990 and December 2000 were included in the study. Eighteen patients (82 p. 100) exhibited complete healing of their cutaneous-buccal lesions after a mean delay of 4.3 months. The lesions of 4 patients did not heal. Three of these patients died: a bed-ridden patient, a patient exhibiting a metastatic bronchial carcinoma and a hypertensive patient who died following a hemorrhagic cerebral vascular accident. Twelve patients (54 p. 100) were weaned off treatment after a mean duration of 2.9 years. Five severe adverse events were observed: one pneumonia, 2 unbalanced diabetes, one hepatitis and one pulmonary embolism., Discussion: This study showed that the healing of the cutaneous-buccal lesions was obtained using the "Lever scheme" in 18 cases out of 22 (82 p. 100). The delay to healing was relatively long in view of the delayed effect of azathioprine. This limits the use of the "Lever scheme" protocol to non-extensive and/or early stage pemphigus. The severe adverse events occurred in low-weight patients in whom the dose related to weight was the highest. Hence the doses of azathioprine and prednisone should be adapted to patients' body weight.

Background: Jessner's lymphocytic infiltration of the skin is a rare and benign disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity was previously noticed by authors and recently demonstrated. We report four new cases with positive photobiological investigation., Case Reports: Four patients (2 males, 2 females), with a mean age 36 years were seen with erythematous papules or discoid plaques on face, arms and upper trunk. The onset of disease occurred after sun exposure in summer. Skin biopsies showed perivascular lymphocytic infiltration in the dermis. All patients relapsed cyclically with incomplete healing during winter. Photobiological investigation elicited skin lesions in a broad spectrum of UV: UVB (2 cases), UVA (1 case), UVA and UVB (1 case). In all patients treatment with oral antimalarials and external photoprotection was effective., Discussion: Our data suggest that photosensitivity history in patients with Jessner's lymphocytic infiltration of the skin should be searched for, and confirmed by provocative phototesting. This relevant event could guide the therapeutic strategy because antimalarials were effective for the Jessner's lymphocytic infiltration cases with photosensitivity.

Background: Increased frequency of methiresistant Staphylococcus aureus (MRSA) in inpatients is a day to day problem., Objective: To determine the origin of MRSA, the causes for contamination, and potential complications in a department of Dermatology., Patients and Methods: A retrospective study of patients hospitalized in a dermatology department with cutaneous MRSA during 1997-1998. We considered age, type and duration of dermatitis, geographic origin of patients on admission, previous hospitalizations, time between arrival in the ward and positive MRSA, and complications requiring systemic antibiotics., Results: Out of 4579 of our patients, 53 (0.011 p. 100) had positive MRSA. They were on average 70.86 years-old (26 to 97). The most common underlying dermatitis was leg ulcers (30) and foot sores (4). Dermatitis had lasted for more than 1 month in 48 patients. Most of the patients (40) had their MRSA on admission to our ward. Twenty six patients admitted from home had MRSA; only 9 had never been in an hospital. Six patients had diabetes mellitus. Thirty two patients healed with local treatment for their dermatitis. Thirteen patients have had some complications (erysipela 1, osteoarthritis 3, septicemia 2, febrile syndrome 9)., Discussion: Most patients with MRSA had leg ulcers or foot sores, confirming liability of chronic wounds to MRSA colonizing. Thirty one out of 40 patients with MRSA at arrival had been previously hospitalized. Hospitalization increases the risk of MRSA contamination. Treatment of MRSA is essentially that of the underlying dermatitis. However, it is still necessary to monitor the lesion for complications.

Introduction: The risk of vitamin C deficiency is underestimated in industrialized countries and is only disclosed in rare cases of severe scurvy., Case Report: We report three cases of scurvy presenting with ecchymotic purpura and hemorrhagic ulcerations of the lower limbs. Vitamin C supplementation led to rapid improvement of the skin lesions., Discussion: Clinical diagnosis of low-grade deficiency can be difficult. Biological diagnosis requires special care in sample taking and transport.

Background: We report a case of primary non-autoimmune hypothyroidism causing pretibial epidermolysis bullosa., Case Report: A 70-year-old man with primary non-autoimmune hypothyroidism developed blisters of different ages on the lateral aspect of both legs. Pathology reported blisters with subepidermal cleavage. Direct immunofluorescence was negative. Electron microscope examination showed a variable cleavage level and diffuse infiltration of a granulous and amorphous microfibrillar substance. After hormone replacement therapy, euthyroidism was associated with a reduction in the number of bullae and finally complete remission. After 12 months follow-up, the patient has not experienced recurrence., Discussion: Recurrence-free clinical improvement after hormone replacement therapy suggests the diagnosis of hypothyroidism pretibial epidermolysis bullosae. Mochizuki et al. described a similar case which rapidly regressed after hormone therapy but where the electron microscope showed a different cleavage level. These bullae appear to result from a mechanical mechanism due to their localization in areas exposed to friction and also to the presence of bullae of different ages. This hypothesis is confirmed by the presence of a variable level of cleavage and a substance dense to electrons at electron microscopy as well as by the skin weakness. Our case confirms the reality of hypothyroidism pretibial epidermolysis bullosa. Thyroid hormones should be assayed in patients presenting pretibial bullae.

Objectives: Analysis of the literature on drug-induced aphthous ulcers and mucosal ulcerations and evidence-based grading., Material and Methods: Four literature sources were analyzed. Three groups of key words were used: 1) oral, buccal, genital, mucosal; 2) ulcer, ulceration, aphthous, aphthosis; 3) induced, drug, adverse-effects, with cross-overs. Four grading patterns were used: presence of aphthous term or synonym, typical clinical description of aphthous ulcer, presentation suggesting diagnosis of aphthous ulcer, criteria defining likelihood of drug causality., Results: We examined 66 of the 220 publications responding to our selection criteria. Typical clinical description of aphthous ulcer and/or clinical presentation suggesting the diagnosis of aphthous ulcer were noted for 8 compounds with likely or palausible patterns of causality. For 21 compounds, we found only aphthous term or synonym without a clinical description or presentation., Discussion: Our review of the literature individualized a group of 8 compounds where the diagnosis of aphthous ulcers was plausible and another group of 21 compounds where the diagnosis of aphthous ulcers requires confirmation. The clinical relevance and limitations of this analysis are discussed., Conclusion: Drug-induced aphthosis is probably a real phenomenon. Causality of the 8 compounds in the first group is simply more fully documented than for the 21 compounds in the second group. A low evidence level may not confirm these hypotheses. Some drugs may have been incorrectly ruled out due to lack of information.

Introduction: The aim of this work was to study the long-term course of patients with penile lichen sclerosus and atrophicus., Patients and Methods: We reviewed the files of 16 patients followed at our university center from 1982 to 1997 for pathologically proven penile lichen sclerosus and atrophicus. Age at disease onset, duration of the disease course, presence of a triggering factor, initial localization, signs and symptoms, treatment given and its efficacy were recorded., Results: Mean age at diagnosis was 52 years. Inaugural prepuce localizations were observed in 6 cases, involving the distal part of the penis and the glan in 3 cases. Concomitant involvement of the glan and the balanopreputial groove were seen in 6 cases. The localization was limited to the glan in one case. Six patients underwent posthectomy and one had dorsal plasty of the prepuce. The 9 other patients were treated medically with topical corticosteroids and/or androgens. One patient had died prior to evaluation. Among the 9 patients given medical treatment, lichen sclerosus persisted in 7, one developed a squamous cell carcinoma of the penis and one was cured. Among the 6 cases of surgical treatment, 5 were cured and 1 had persistent lesions. Considering the initial localization, long-term course showed that 7 of the 8 patients with a prepuce localization (alone or in association with glan involvement) were cured while the 7 patients with a lesion of the balanopreputial groove (alone or in association with glan involvement) or a lesion of the glan alone still had lichen sclerosus and atrophicus. The non parametric Fischer test showed that the chances of cure were better for preputial localizations than for balanopreputial localizations (p = 0.001) and that surgical treatment was more effective than medical treatment., Discussion: This study of a small number of patients highlighted two points: prepuce localizations of lichen sclerosus and atrophicus cure better than balanopreputial localizations, and that medical treatment is partially effective in balanopreputial localizations. One patient developed squamous cell carcinoma of the penis. As the level of the cancer risk remains unknown, it would be important to provide the most effective treatment in all cases of penis lichen sclerosus and atrophicus.

Introduction: Pemphigoid vegetans is a rare disease. It has a clinical resemblance to pemphigus vegetans, but there are histological and immunopathological features of bullous pemphigoid., Case Report: We describe a case in a 57-year-old-man who had developed intertriginous vegetating plaques. Histologic examination of a skin biopsy specimen and direct immunofluorescence microscopy of a biopsy specimen were those of bullous pemphigoid. Immunoblot studies and indirect immunofluorescences of salt-split skin were negative. Direct immunoelectron microscopy was consistent with bullous pemphigoïd., Discussion: Only five cases have been reported. We describe the first case including direct immunomicroscopic findings which suggest that pemphigoid vegetans is a subtype of bullous pemphigoid. The other interest was a remarkable improvement with tetracyclines.

Introduction: Waldenström macroglobulinemia is a rare hematologic disorder with characteristic malignant plasma cell proliferation associated with the secretion of a IgM monoclonal immunoglobulin which is the cause of most of the clinical manifestations. Skin involvement is exceptional. We report a new case of Waldenström macroglobulinemia discovered in a patient who developed specific skin lesions with monoclonal immunoglobulin deposits., Case Report: A 50-year-old woman developed spontaneously painful infiltrated erythematopapular plaques over the extension aspects of the limbs. There were no other clinical manifestations. The patient had a kappa IgM monoclonal gammapathy. The diagnosis of Waldenström macroglobulinemia was proposed after the discovery of voluminous intra-abdominal adenopathies and was confirmed by immunohistochemistry of the biopsies, indirect immunofluorescence evidenced reactivity against the epidermal basal membrane. Chemotherapy led to successful regression of the skin lesions, the tumoral mass and circulating IgM., Discussion: This is the second reported case of Waldenström macroglobulinemia with anti-basal membrane monoclonal immunoglobulin. This IgM would recognize an 82 kD antigen on the dermal side of the dermo-epidermal junction situated in the anchoring fibres of the lamina densa, a zone classically involved in acquired bullous epidermolysis.

Introduction: The aim of this work was to present a case of multicentric histiocytosis with haematologic involvement., Case Report: A 68-year-old man presented with poor general health and a nodular eruption of the skin and larynx. On clinical examination there was an enlarged spleen and laboratory results revealed an inflammatory syndrome, platelet count 60,000 and myelemia with 10 p. 100 immature elements in a white cell count of 14,000. Pathology and ultrastructure examinations led to the diagnosis of multicentric histiocytosis. Bone marrow aspiration was normal. Pancytopenia then developed with bone marrow hypoplasia without infiltration. Corticosteroids then cyclophosphamide were uneffective for either the skin disease or the hematologic disorder. The patient developed severe buccal aphthosis which responded well to thalidomide. This treatment reduced the size and the number of skin nodules. Finally, renal failure of unknown origin was observed., Discussion: Electron microscopy is essential for positive and differential diagnosis of atypical multicentric histiocytosis. Hematological disorders associated with multicentric histiocytosis may either be specific or totally independent.

Introduction: Since the description by Toker in 1972, neuroendocrine carcinoma or Merkel cell carcinoma, is a well identified clinical entity although the appropriate treatment is still debated. Wide surgical exeresis is indicated as first line treatment in all cases but the question concerning protocols for adjuvant radiotherapy or chemotherapy remains open. We analyzed retrospectively our series of 13 patients with neuroendocrine carcinoma looking for an association between radiotherapy and surgery., Patients: There were 7 women and 6 men in our study population (age range 43-88 years). Two cancers of the rectum and one prostate cancer were associated. In 6 cases, the tumor was localized on the limbs, in 5 on the face and in 2 on the buttocks. Mean delay to diagnosis was 2.5 months. At diagnosis, only 1 patient had satellite nodes. The pathology examination evidenced intermediary cell type and architecture. Immunohistochemistry tests were positive for NSE, NF, KII and the ultrastructure confirmed the diagnosis. Nine patients were treated with surgical exeresis with wide 1 cm margins and was completed with radiotherapy of the tumoral bed at homogeneous doses of 45 to 60 Gy. Surgery alone was used in 4 patients., Results: Mean follow-up was 27 months (5-98). Among the 13 operated patients, 11 have survived, 1 died due to the neoplasia and in 1 other from another cause. Local and regional recurrence rate after exercise alone was 50 p. 100 (2/4) with concomitant development of metastasis in both cases. When local or locoregional post-operative radiotherapy was given, local or regional recurrence rate was 33 p. 100 (2/6)., Discussion: Cutaneous neuroendocrine carcinoma or Merkel cell carcinoma is an uncommon skin tumor. It is difficult to determine the management protocol on the basis of data in the literature. Due to risk of locoregional recurrence, we currently propose post-operative radiotherapy of the tumor bed and drainage nodes in all patients.

Introduction: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are autoimmune blistering diseases characterized by loss of cell-cell adhesion and by autoantibodies directed against epidermal cadherins. The ultrastructural localization of PV antigen remains controversial, whereas the location of PF antigen seems to be established. The use of different techniques could explain these various data. To investigate this matter, indirect immunoelectron microscopy (IEM) and Western blot analysis on bovine tongue epithelium were used., Material and Method: Serum samples from patients with PF(3), PV (4) and control samples from healthy patients (2) were analysed in this study. The inclusion criteria were based upon characteristic clinical features, level of epidermal cleavage on histological preparations and presence of circulating anti-epithelial cell surface antibodies. Indirect IME was performed on normal human skin. Peroxidase labelling was used. Serum samples were also analysed by western immunoblotting on bovine tongue epithelium., Results: Indirect IEM examination of PV sera showed immune deposits located both on desmosomal and extra-desmosomal areas, whereas in PF, IgG deposits were strictly localized on desmosomal structures. By Western blot analysis, PV sera recognized a 130 kDa polypeptide and PF sera a 150 kDa polypeptide., Discussion: Indirect IEM on normal human skin using peroxidase labelling was used because of the best antigenic conservation obtained. Our results suggest that PV antigen could exist both on desmosomal junctions and adherens junctions, whereas PF antigen (desmoglein I) is restricted to desmosome.

Introduction: Bullous pemphigoid (BP) is the most frequent autoimmune bullous skin disease and usually affects elderly patients. Despite some conflicting data, vital prognosis is poor in BP, of which mortality rate after one year of treatment varies between 10 and 40 p. 100. To date, the factors influencing this prognosis remain to be determined., Patients and Methods: A cohort of 78 consecutive patients with BP (mean age: 80 +- 4 years) has been prospectively selected on a six-year period (1987-1992) in Limoges. The diagnosis of BP was made on clinical criteria (using a standardized questionnaire), direct immunofluorescence (IF) findings (linear deposits of IgG and/or C3 along the basement membrane zone) and confirmed by direct immunoelectron microscopy and/or Western immunoblotting on epidermal extracts., Results: The follow-up analysis (mean duration of follow-up: year of treatment (22 p. 100 in the first three months) with an actuarial survival curve inclined to be horizontal by the end of this first year of treatment. Therefore, we have studied the clinical or immunologic factors susceptible to influence the vital prognosis of BP according to this main criterion: death (or survival) at the end of the first year of treatment. None of the following factors has been found to be significantly linked to the vital prognosis in BP: age, sex, extent of skin lesions at presentation, presence of mucosal lesions, blood eosinophilia, presence of circulating basement membrane zone autoantibodies by indirect IF. The clinical factors of bad prognosis were an altered general condition and a history of coronaropathy. The presence of circulating autoantibodies against BP180 antigen (but not the one of autoantibodies against BP230) as detected by immunoblotting on epidermal extracts was found to be significantly more frequent (50 vs 22 percent) in BP patients who died within the first year of treatment (p < 0.02)., Discussion: Although modestly discriminating, the presence of circulating autoantibodies against BP180 represents the first vital prognosis factor demonstrated in BP. This result confirms the growing pathophysiologic importance of the anti-BP180 autoantibodies of which the pathogenic role has been recently formally demonstrated in BP using an animal model.

Introduction: The frequency of bacterial dermohypodermal infections would appear to be increasing in western countries, particularly severe necrotizing forms., Patients and Methods: We attempted to determine: (1) the incidence of the two most well-defined forms of bacterial dermohypodermal infections (erysipelas and necrotizing fasciitis) in a retrospective study of hospitalized cases seen over a period of 13 years in the Limoges University Hospital and (2) the role of streptococci as causative agents in bacterial dermohypodermal infections in the adult by collecting bacteriological data in 174 cases of erysipelas and 31 cases of necrotizing fasciitis., Results: This retrospective analysis confirmed that the annual incidence of erysipelas was constantly increasing over the study period (1978-1991) while the annual incidence of necrotizing fasciitis was much lower and more constant. The two incidence curves were not parallel. Bacteriology, detection of streptococci (groups A, B, C or G) in skin specimen was better with direct immunofluorescence (64 p. 100) than with latex agglutination (47 p. 100) or classic culture techniques (28 p. 100) for erysipelas. Streptococci were determined to be the causative agent in 79 p. 100 (137/174) of the cases of erysipelas using at least one of the 3 bacteriological tests and/or serology. Group A streptococci predominated (67 p. 100 of the cases). For necrotizing fasciitis, streptococci was identified with classical culture technique, latex agglutination and/or serology in 12/131 cases (39 p. 100), again with a group A predominance (7 cases). In the other cases, multiple microbial flora (12 cases) were found including Staphylococcus aureus (3 cases) and Serratia liquefaciens (1 case)., Conclusion: These results show that streptococci (group A predominantly and group G to a lesser extent) were the major, though not exclusive, causative agents in bacterial dermohypodermal infections in the adult, including necrotizing forms.

Introduction: The diagnosis of mastocytosis is usually not difficult in cases with typical skin lesions. Intertrigo is exceptional., Case Report: An 83-year-old patient developed inguinal intertrigo bilaterally. The lesions contained maculopapulous elements without Darier's sign. The histology examination revealed a mixed mast cell infiltration predominated by histiocytes. The ultrastructure confirmed the diagnosis of cutaneous mastocytosis. No extension was observed., Discussion: Skin folds are rarely involved in infiltrating diffuse mastocytosis. Two cases have been reported in the literature including one doubtful case. Mastocytosis should thus be included in the possible diagnoses in patients with macupopapulous intertrigo. In addition, the mastocytic and histiocytic polymorphism observed here is rare.

Hypersensitivity to dapsone (Disulone) is a rare side effect of sulfone therapy. The diagnosis is based on variable clinical manifestations and laboratory findings dominated by hyperthermia, skin eruptions and a mixed form of hepatitis. We observed a case of hypersensitivity to dapsone in a 57-year-old woman who was treated with Disulone for cicatricaial pemphigoid. Clinical manifestations and laboratory results returned to normal 3 weeks after drug withdrawal. This case is interesting because the skin eruptions classically described were not observed. In addition this is apparently the first case reported of such a hypersensitivity syndrome occurring during treatment of autoimmune bullous dermatosis.

Introduction: We observed two cases of Pasteurella and discuss the role of bacterial sampling in ulcerations of the lower limb., Case Reports: In the first case, Pasteurella was discovered in an ulceration of the lower limb and was cured with no particularly specific care. In the second case, Pasteurella had been inoculated by scratching an ulceration and could not be cured without specific treatment., Discussion: No specific pathological consequence in chronic carriers could be possible, a situation which has often been reported in the literature. Pasteurella is an unusual specific cause of ulcerations of the lower limb.

Objective: Evaluate the clinical features of skin reactions to gold salts., Introduction: Gold dermatitis is described in the literature as a group of reactions non-specifically associated with these drugs., Patients and Methods: Ten patients (7 males, 3 females, mean age 59.6 years [corrected] with a skin reaction were studied over a 6 year period. All were treated with Allochrysine for rheumatoid arthritis (n = 8), rhizomelic pseudopolyarthritis (n = 1) or arthropathic psoriasis (n = 1)., Results: The delay to the first signs was from 2 weeks to 8 months. Three lichenoid eruptions (including two with buccal lesions and two which followed an autonomous course), 2 pityriasis rosea (one with eosinophilia and one with liver disease), 2 eczematoid dermatoses and one urticaria were observed. Pathology examinations (8/10) were in agreement with clinical diagnosis. Imputability was 14 (1 case), 13 (8 cases) and 12 (1 case)., Discussion: The clinical features observed were variable and in agreement with a particular clinical situation. They were not specific to gold salts and cannot be qualified as "gold dermatitis". Two autonomous and severe lichenoid eruptions were observed. Generalized lesions were associated with biological signs. This situation must be considered as a marker of severity.

A 64 year-old man was admitted for congestive heart failure induced by an acute renal failure due to obstruction. Retroperitoneal periaortic-aneurysmal fibrosis was the etiology of the obstruction. Type IV Ehlers-Danlos syndrome was diagnosed based upon clinical and histological cutaneous signs.

Subepidermal autoimmune bullous dermatoses are defined by clinical, histological and immunological criteria, notably the presence of anti-basement membrane antibodies detectable in vivo by direct immunofluorescence. We report three cases where anti-basement membrane antibodies were not detectable in vivo by direct immunofluorescence but were detected in high titres by indirect immunofluorescence. This situation is extremely rare in the literature. The first case concerns a 69-year old woman seen for bullous and pruriginous lesions of the lower limbs of 2 months' duration. Histological examination found dermoepidermal bullae with, in their lumen, a serous fluid spotted with numerous polymorphonuclears. A search for anti-basement membrane antibodies was positive in significant titres (1,280; 320; 480) at indirect immunofluorescence on rabbit lip whereas five successive direct immunofluorescence test in perilesional skin and on the thigh medial surface remained negative. The second case is that of a 91-year old woman suffering from generalized pruritus associated with erythematous lesions predominant on the extension surfaces of the forearms and thighs, without any bullous lesion. Pathological examination only showed a superficial dermal lymphocytic infiltrate. Four direct immunofluorescence tests were negative, whereas a search for anti-basement membrane antibodies on rat oesophagus was positive at 1/1,280. The third case resembles the second one. It concerns a 72-year old woman who consulted for generalized pruritus of several months' duration which interfered with sleep and was incompletely relieved by emollients. There was no specific skin lesion. Pathological examination revealed nothing more than a discrete perivascular mononucleate infiltrate. Direct immunofluorescence tests performed on two occasions on the skin of the abdomen and of the medial thigh surface were negative.(ABSTRACT TRUNCATED AT 250 WORDS)