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Mucocutaneous manifestations can be indicative of a variety of gastrointestinal diseases, and the dermatologist needs to know how to recognize them to refer the right patients to the gastroenterologist. Conversely, the gastroenterologist is often confronted with mucocutaneous lesions that raise the question of a possible association with a known digestive disease. Among the extra-intestinal manifestations of inflammatory bowel disease (IBD), mucocutaneous manifestations are the most common. This review will provide a breakdown by classifying them into 4 groups: 1) reactive manifestations, which include neutrophilic dermatoses, aphthous stomatitis, erythema nodosum, and vasculitis; 2) Crohn's disease-specific granulomatous skin lesions, which are histologically characterized by tuberculoid granulomas similar to those found in the gastrointestinal tract; 3) nutritional deficiency manifestations secondary to anorexia, malabsorption, loss, and drug interactions; and 3) a variety of autonomous autoimmune or inflammatory skin diseases. Dermatologists may also be involved in the management of the adverse effects of IBD treatments, especially the so-called "paradoxical" psoriatic eruptions., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Introduction: Sorafenib is a multikinase inhibitor used in the treatment of hepatocellular carcinoma, advanced renal cell carcinoma, and differentiated thyroid carcinoma. Cutaneous adverse events are numerous and occur frequently., Patients and Methods: We present two cases of nodulocystic lesions associated with comedones in patients treated with sorafenib for hepatocellular carcinoma. In the first patient, a 64-year-old man, lesions appeared on the trunk one year after beginning sorafenib. Histopathological examination revealed a non-granulomatous, perivascular and perisudoral polymorphic cellular infiltrate associated with comedones and microcysts. These lesions progressed via inflammatory episodes interrupted by long periods of spontaneous remission without any specific treatment. In the second patient, a 53-year-old woman, a rash appeared on the buttocks three months after starting sorafenib and then spread to the lumbar region and thighs. Histopathological examination was consistent with granulomatous acne lesions. The initial treatment (oral tetracycline and zinc) given for 3 months proved ineffective. Patient follow-up over 3 years showed gradual regression without the appearance of any further lesions., Discussion: In the literature, several reports discuss acneiform rashes in patients treated with targeted therapy. In most cases, these lesions were papulopustular without retentional lesions. There are few reports of nodulocystic eruptions associated with comedones following sorafenib therapy. The mechanisms of emergence of these lesions seem to involve inhibition of the RAF pathway, C-KIT, and the PDGF signaling pathway., (Copyright © 2019 Elsevier Masson SAS. All rights reserved.)

Background: Bacillus cereus is a ubiquitous telluric organism. B. cereus endocarditis is a rare condition seen mostly in prosthetic heart valves and among intravenous drug users. We report a new case of a patient without risk factors and with a good clinical outcome not requiring valve replacement., Case Report: In October 2014, a 50-year-old woman was referred to the dermatology department of Lille University Hospital for lower-limb wounds developing 6 months earlier. She presented fever without clinical signs of infection, except for the lower-limbs wounds. Blood cultures revealed the presence of B. cereus. Transesophageal echocardiography was performed and revealed two foci of aortic valve vegetation with a diameter of 5mm. After bacterial sensitivity testing, rifampicin and levofloxacin treatment was given for six weeks, with complete remission. A skin graft was performed and good improvement was seen., Discussion: Nineteen cases of B. cereus endocarditis have been described previously, only one of which was without risk factors. We described a case of complete remission after a 6-week course of antibiotics. Our case demonstrates that BC should not be considered as a blood culture contamination, and that treatment may be complex due to antibiotic resistance., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Cryptococcal meningitis is a mycosis encountered especially in patients with Acquired Immunodeficiency Syndrome and is fatal in the absence of treatment. Information on epidemiology, diagnosis and susceptibility profile to antifungal drugs, are scarce in Cameroon. Authors evaluated the diagnosis possibilities of the cryptococcal meningitis in Cameroon, and studied the antifungal susceptibility of isolated strains to fluconazole, used as first line treatment of the disease in Cameroon. Between December 2009 and July 2011, 146 cerebrospinal fluids obtained from HIV patients with suspicion of meningitis were analysed. The diagnosis procedure involved macroscopic and cyto-chemical analysis, India ink test, culture on Sabouraud chloramphenicol medium and antigen latex agglutination test. Antifungal susceptibility testing of isolated strains to fluconazole was done by the E-test(®) method. The diagnosis of cryptococcal meningitis gave 28.08% positive cases. Among these patients, 80% were at stages III and IV and 20% at stage I of the HIV infection, according to the WHO previous classification. Cyto-chemical analysis showed current findings in the case of cryptococcal meningitis. India ink test and latex agglutination test exhibited very high sensitivity and specificity (>94%). Fluconazole antifungal susceptibility testing gave MICs lower than 32μg/mL to 92.7% of isolated strains and MICs greater than this value to 7.3% of isolates. These results showed that cryptococcal meningitis remains a real problem among HIV infected patients in Yaoundé. The emergence of fluconazole reduced susceptibility strains is worrying. Nevertheless, efficacy of rapid detection tests is interesting because this will help in rapid diagnosis and treatment of patients., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Background: Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis often associated with systemic diseases, particularly chronic inflammatory bowel diseases. Extracutaneous manifestations (articular, ocular, renal, pulmonary, hepatosplenic, muscular) exist, with lung involvement being the most common., Patient and Methods: We report a case of PG with skin and lung involvements in a patient treated with high-dose corticosteroids in a setting of severe ulcerative colitis (UC). Lung involvement was diagnosed during the pretreatment assessment performed prior to initiation of anti-TNFα therapy. Infliximab resulted in rapid improvement of the lung and skin lesions., Discussion: In the event of simultaneous cutaneous PG lesions and lung lesions suggestive of abscess, visceral involvement should be suspected. First-line treatment consists of oral corticosteroids. In our patient, the occurrence of lesions under corticosteroids in UC militated in favour of anti-TNFα therapy, which proved effective., (Copyright © 2013 Elsevier Masson SAS. All rights reserved.)

Background: Specific cutaneous infiltrates of chronic lymphocytic leukaemia (CLL) are rare. They occur after a mean disease duration of 3 years. CLL skin infiltrates as the primary manifestation of the disease have been reported, but a normal lymphocyte count at diagnosis is rare. We present two cases of CLL initially presenting in the skin, without lymphocytosis., Patients and Methods: A 53-year-old man developed papulonodular lesions of the face and infiltrated plaques of the scalp, and an 85-year-old woman presented erythematous nodules of the face and neck. Histopathology revealed a lymphocytic infiltrate, consisting of small mature B-cells CD20+, CD79+, with an aberrant phenotype CD5+. CD23 was positive in one case. The two patients had no lymphocytosis, but immunophenotyping was characteristic of CLL. In the second case, there was a sub-mental adenopathy, histologic analysis of which was consistent with CLL. The CLL was classified as Binet stage A in the two cases. No disease progression was noted at follow-up., Discussion: The unusual feature of these cases is the lack of lymphocytosis at diagnosis. Thus, the skin lesions resulted in further evaluations for CLL, although the diagnosis was not suggested by the blood count., Conclusion: Skin involvement in CLL does not appear to be a poor prognostic indicator, arguing in favour of recruitment of circulating monoclonal B-cells rather than an additional tumour mass., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Psoriasis and inflammatory bowel diseases (Crohn's disease and ulcerative colitis) are among the immune-mediated inflammatory diseases. This group includes approximately 80 disorders, some of which can at times be associated in a single patient. In psoriasis, Crohn's disease may be observed slightly more frequently, but ulcerative colitis and celiac disease are also an issue. The underlying relations between these disorders comprise: i) genetic data obtained by genome-wide association studies that show the involvement of shared predisposing loci and/or genes, for example, in innate immunity; ii) immunological data: these disorders share inflammation effector mechanisms, particularly the activation pathway of Th17 lymphocytes, which explains the efficacy of anti-TNF antibodies and anti-IL-12/23; and iii) environmental co-factors such as smoking, possibly certain food proteins (gliadin, etc.), and bacterial infections that are probably decisive elements in the genesis of these diseases., (Copyright © 2012 Elsevier Masson SAS. All rights reserved.)

Background: Several cases of skin cancer have been reported after treatment with etanercept although the causal relationship remains uncertain. We report the case of a patient who rapidly developed multiple basal cell carcinomas (BCC) after discontinuation of this treatment., Patients and Methods: A 42-year-old man presented severe plaque psoriasis after receiving topical therapy, less than 100 sessions of PUVA-therapy, retinoids and repeated solar exposure. Severe worsening of the psoriasis led us to use etanercept for seven months with excellent results. However, 11 BCCs gradually appeared within a year starting one month after the end of treatment., Discussion: There is some controversy about the risk of non melanoma skin cancer associated with etanercept treatment. However, even the most recent studies are contradictory and they mostly concern rheumatological indications. In the past four years, a dozen cases of BCC have been reported following treatment for cutaneous psoriasis. As regards our patient, a genetic predisposition is possible but a potentiating effect of solar exposure is strongly suspected. This observation should lead to reinforced screening for BCC and restriction of anti-TNFalpha therapy to patients who have received less than 1000 J of PUVA-therapy, as recommended by the British Society of Rheumatology for psoriatic rheumatism. Levels of natural solar exposure must be also be taken into account.

Background: Leflunomide is prescribed in inflammatory rheumatisms. Cutaneous side effects have rarely been described. We report the case of a patient presenting skin necrosis attributed to this drug., Patients and Methods: A 73-year-old woman had been taking leflunomide for psoriatic arthritis for one year and subsequently, developed three abdominal ulcerations and necrosis of one hallux. No immunological, vascular or neoplastic aetiology was found. Corticotherapy was started, based on a hypothesis of vasculitis, but lesions progressed, leading to amputation of the hallux. Leflunomide was stopped and the ulcerations healed completely within 12 weeks, whereas prolonged local treatment had failed to yield any improvement., Discussion: Skin necrosis due to leflunomide is rare; we found seven cases in the literature. Ulcerations may occur anywhere. Potentially life-threatening glomerulonephritis with mesangial deposits may be associated. Discontinuation of leflunomide followed by wash-out with cholestyramine allows healing. Corticosteroids or cyclophosphamide are sometimes necessary. The ulcerations appear to be result from excessive immunomodulation in the skin or from an inhibiting role of leflunomide on the epidermal growth factor receptor., Conclusion: In the absence of any demonstrated aetiology in patients presenting ulcerations or skin necrosis, a contributory role of leflunomide must be considered, even in cases of prolonged use.

The association of some immune-mediated inflammatory disease in a same patient is known. Nevertheless, this is difficult to appreciate practically for the psoriasis due to the absence of prospective study. The strongest link is with Crohn's disease and the spondyloartropathies. It is sustained by, i) genetic data obtained by genome-wide association study showing some common loci and/or genes involved in innate immunity, ii) immunologic data, these conditions sharing effector cells among which Th17 lymphocytes and inflammatory mediators (TNF-alpha, IL-1,17,23...), that explain the related efficacy of some biologics like anti-TNF-alpha, IL-12/23 antibody and others in progress and iii) environmental triggers among which bacterial infections are probably determining in the genesis of these diseases even if this is still only an hypothesis.

Background: TNFalpha blockers have recently extended the therapeutic arsenal available in dermatology. However, dermatologists must be informed of their potential adverse dermatological effects. While the chief adverse effect of TNFalpha blockers is risk of infection, cutaneous adverse effects have not yet been clearly elucidated and publications on this topic are few and far between. The aim of our study is to report various dermatological problems noted during treatment with TNFalpha blockers., Patients and Methods: This was a retrospective study of patient files. The study population comprised patients receiving TNFalpha blockers and presenting cutaneous reaction, and seen in the dermatology department between August 2001 and December 2004., Results: Eleven patients were included. The following cutaneous reactions were seen: delayed skin rash (1 case), lupus syndrome (1 case), cutaneous vasculitis (2 cases), palmoplantar pustulosis (2 cases), psoriasis vulgaris (1 case), atopic dermatitis (1 case), lichenoid rash (1 case), purpuric capillaritis (1 case) and melanoma (1 case)., Discussion: The cutaneous manifestations seen represented a wide range of different clinical pictures. Dermatologists must be aware of these potential adverse effects. Future improvement of knowledge of the physiopathological mechanisms as well as the institution of prospective cohort studies should provide clearer guidance on the management of such symptoms.

Background: We recently carried out a study concerning consultations by French dermatologists in private practice. We evaluated consultations at the dermatology departments of 3 university teaching hospitals in France., Materials and Methods: This was a 2-month prospective study conducted in 2003 at the dermatology departments of the university teaching hospitals of Amiens, Lille and Rouen. Each consultant completed a questionnaire covering the duration of the study. The following data were recorded: consultation date, function of the consultant, study centre, type of consultation, type of disease, and whether or not the patient was hospitalised after the consultation., Results: 7296 files were examined during the study. 38% of the consultations were performed by part-time hospital consultants, 29% by dermatology interns, 18% by hospital practitioners, 9% by university professors and 6% by clinical heads or assistant heads. The most commonly encountered diseases were allergies (17%), cancer (16%), arteriovenous disease (15%) and infectious disease (11%). Three types of consultation were identified: emergency consultations without an appointment, consultations by appointment for a specific problem and consultations by appointment without a specific problem. The number of resulting hospital admissions ranged from 2 to 10% of consultations, depending on the type of consultation and the role of the consultant in question., Discussion: This study shows that in France, consultations at hospital dermatology departments differ greatly from those of dermatologists in private practice. The main diseases seen (cancer, arteriovenous disease, allergy, infectious dermatosis) accounted for the majority of hospitalisations in these departments. The organisation of hospital consultations is increasingly tending both towards treatment of highly specialised diseases through specifically oriented consultations and also towards the emergency treatment of certain forms of acute dermatosis.

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.

Background: A measles outbreak occurred in Geneva between March and July 2003. An investigation was conducted to determine its magnitude, identify predominant modes of transmission, and assess disease outcome., Methods: In Switzerland, measles notification has been mandatory for physicians since 1999 and laboratories since 1988. All cases were investigated and followed up by interviewing physicians using a standardized questionnaire. Viral genotype was determined for 4 cases using a saliva specimen processed at the Robert Koch Institute, Berlin. Geographic distribution of cases and vaccination coverage were compared., Results: Overall, 54 cases were notified. Fifty-three cases were between 10 and 19 years of age. Ninety-four percent (n = 46) of 49 cases with known vaccination status were not vaccinated and none were fully immunized (2 doses). Measles complications occurred among 19% (n = 7) of the 36 patients with follow up information: pneumonia (n = 4, combined with pericarditis and hepatitis in one case) and bronchitis (n = 3). Three persons were hospitalized. No complication was reported among 3 persons partially vaccinated (1 dose). The virus belonged to genotypes D8 and D5. Five separate clusters of related cases included 74% of all cases. Transmission mainly occurred within families (43% of all cases) and school (28%). Two of the most affected areas had measles vaccination coverage well below the cantonal average., Conclusion: Low vaccination coverage combined with clusters of unvaccinated household contributed to continued virus circulation and represents a threat to the canton of Geneva as new outbreaks of measles may reoccur.

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: Leflunomide (Arava) is an immunomodulator, recently introduced for systemic treatment of rheumatoid arthritis. We report the first case of lichenoid drug reaction due to this drug., Case Report: A sixty-four year-old woman received leflunomide for rheumatoid arthritis. Two months after initiation of treatment, pruritus and lichenoid papules appeared on her hands and subsequently on her arms and her trunk, with a few bullous lesions. A skin biopsy was evocative for the diagnosis of drug induced lichenoid eruption. The treatment was stopped, and a wash out with colestyramine and topical corticotherapy resulted in dramatic improvement. No relapse was observed. Two months later, patch-tests with leflunomide diluted to 30 p. 100 in white petrolatum were negative., Discussion: Side effects of leflunomide are frequent, generally benign for the cutaneous features. In our case, the delay, clinical and histological aspect and improvement on withdrawal of the drug emphasize the imputability of leflunomide. Few cases have been reported with others immunomodulators.

A 41-year-old patient had a large and quick growing tumor of the scalp, known during many years. The histopathologic and immunohistochemical evaluations lead to the diagnosis of a cutaneous carcinosarcoma or sarcomatoid carcinoma. In spite of the surgical treatment, the evolution was rapidly fatal with widespread metastasis. From data of the literature, we discuss the diagnosis and histogenesis of this rare cutaneous tumor.

Background: Granuloma annulare following BCG vaccination or tuberculin skin tests is rarely reported in the literature. We describe three cases occurring in patients under the age of 6 years., Case Reports: In the first two cases, granuloma annulare was initially localized at the vaccinal site, then generalized. In the third case, diagnosis was deep granuloma annulare localized far from the initial vaccination site, with recurrence following tuberculin test. In the three cases, diagnosis was made on the basis of clinical and histological elements., Discussion: The relationship between granuloma annulare and BCG may seem evident when the eruption occurs at the vaccinal site, but remains difficult to prove. Diagnosis essentially relies on the absence of other classical etiologies and a compatible delay. When a vaccination such as BCG appears to be the cause of granuloma annulare, a possible cause would be injection trauma or a cell-mediated-delayed-hypersensitivity reaction to a specific antigen contained in the vaccine, leading to development of skin disorders such as granuloma annulare in predisposed subjects. Reports of granuloma annulare following BCG are uncommon but the incidence may be underestimated. In practice, diagnosis of granuloma annulare following BCG or tuberculin skin test cannot be established until BCG dissemination infection is ruled out, a necessary clinical and paraclinical step required in all cases of eruptions following this vaccination. Granuloma annulare is a benign complication requiring no treatment and does not constitute a contraindication for other usual vaccinations.

Background: Supernumerary nipples are not rare but the developement of a complete supernumerary breast is exceptional., Case Report: A 59-year-old man presented a progressively increasing ancient right- groin-masse. The histopathologic examination of the lesion confirmed the diagnostic of polymastia., Discussion: The interest of this observation results from the very unusual occurence of polymastia (less than 1 p. 100 of supernumerary nipples), especially for a caucasian man. The prevalence appears to be higher in women and oriental people. Diagnostic of supernumerary nipple is difficult because of its atypical appearance and ectopic location. However, this diagnostic is important because ectopic breast tissue is subject to the same pathologic changes that occur in normally positioned breasts and it can be a marker for associated diseases such as urologic malformations or urogenital malignancies.

Background: Malignant proliferation at the site of implantation of a pacemaker generator is uncommon. We report the case of a patient who developed rhabdomyosarcoma., Case Report: A 85-year-old man presented with a voluminous and rapidly evolving tumor localised beneath the right clavicle. This inflammatory and necrotic lesion developed on the area where a titanium pacemaker had been implanted five years earlier. Rhabdomyosarcoma was diagnosis on the basis of immunohistochemistry findings. In spite of a wide surgical excision of this primitive tumour, visceral dissemination appeared, rapidly leading to the patient's death., Discussion: This observation rises the question of the role of the pacemaker implantation in tumor development. The excellent in vivo tolerance and the widespread utilization of titanium as biomaterial is an argument against its carcinogenic action. Inversely, a metal-related chronic inflammatory reaction could favor the neoplastic process in predisposed subjects as has been observed with prosthetic materials used in orthopaedics.

Woringer-Kolopp disease is a localized epidermotropic T cell lymphoma with good prognosis. We describe a 79-year-old man with an erythematous scaly plaque of the foot. Clinical diagnosis of psoriasis, parapsoriasis and fungal infection were proposed. Histopathological and immunohistochemical findings were characteristic of Woringer-Kolopp disease. We observed an immunohistochemical positivity of tumour cells for the anti-CD103 antibody (alphaEB7 integrin) according to the epidermotropism of this localized cutaneous T cell lymphoma.

Background: Solar urticaria is an uncommon disorder sometimes difficult to treat. It is characterized by the occurrence of typical whealing reactions on exposed skin a few minutes after sun exposure. The reactions resolve 1 to 5 hours after sun exposure ceases. We report a case evolving over several years, unresponsive to antihistamines and successfully treated by PUVAtherapy performed after UVA desensitization., Case Report: For 3 years, a 22-year-old man developed erythema and itchy wheals at each solar exposure. The lesions appeared on all exposed areas including those usually exposed (face and hand) even in winter. An antihistamine regimen given for several weeks (cetirizine, loratadine) was ineffective. One trial of PUVA therapy led to an urticarial reaction of the entire body. Phototesting showed the minimal whealing dose for UVA was 0.4 J/cm(2). Phototherapy was therefore started by segmentary UVA irradiation at an initial dose of 0.1 J/cm(2). Exposure was then progressively increased allowing initiation of the PUVAtherapy on the 9(th) day with a dose of 0.5 J/cm(2) without whealing reaction. Slow increment PUVA therapy was able to induce good tolerance to sun exposure., Discussion: Solar urticaria may sometimes have a deleterious effect on normal daily life. Severe cases are characterized by a whealing reaction after minimal sun exposure, even on regularly exposed skin. Antihistamines can provide some symptom relief in many patients, but high doses are required. If antihistamines are ineffective, PUVA therapy is indicated. Pre-PUVA UVA desensitization is often necessary. However, exposure to UVA alone has to be repeated every 24 to 48 hours to maintain the refractory state. The advantage of PUVA therapy is a more long lasting protection allowing weekly maintenance sessions.

Introduction: Venous malformations are usually easy to recognise. We describe one case in which the clinical aspect was suggestive of Maffucci's syndrome., Observation: A 44-year-old male, had numerous angiomatous nodules which could be emptied by pressure since infancy. The progressive increase in volume of these lesions on the arms and forearms led to enormous deformation and major disability. The thoracic area and the right foot were also affected to a lesser degree. Two endobuccal lesions were also found on the clinical examination The cutaneous lesions were tender and occasionally hyperhidrosis was present. The presence of phleboliths on Xray and the presence of venous and capillary cavities with numerous thrombi confirmed the diagnosis of venous malformation. Following the failure of the Ethibloc(R) embolization, eight excisions were made in a two-year-period with a good functional result. Elastic strapping was then applied., Discussion: The diagnosis of venous malformation was based on clinical, radiological and histological findings. The absence of chondroma excluded the diagnosis of Maffucci's syndrome. Spindle cell hemangioendothelioma, "blue rubber bleb nevus" and glomangiomatosis were confirmed by the histological findings. The absence of port-wine stain or bone hypertrophy and the presence of multiple nodules excluded Klippel-Trenaunay syndrome. The presence of multifocal involvement with oral lesions, the severity of the deformation and the relative success of the surgical procedure make this case unusual.

Background: Tufted angioma belongs to the family of vascular skin proliferations. Clustered capillaries is the characteristic histological feature. Classically, tufted angioma is an acquired disease observed in children or young adults with lesions predominating on the neck, the shoulders and the upper trunk., Case Reports: Four children were seen for flat skin lesions which were either initially or secondarily erythematous. The lesions were observed at birth or within the first weeks of life. In all 4 cases, the lesions progressed by infiltration, leading to sclerous plaques within a few weeks. The hip area was involved in case n(o) 2 and the thigh in the other cases. Pathology reported tufted angioma. A repeated biopsy 4 years after the first biopsy demonstrated the progressive nature of the fibrosis in case n(o) 2. Magnetic resonance imaging, performed in 3 of the 4 cases, demonstrated deep penetration of the vascular proliferation. The lesions were in contact with bony structures in case n(o) 2, reached the hypodermis in case n(o) 3 and infiltrated between the adducter muscles in case n(o) 4., Discussion: Among tufted angiomas, there is a clinical variant characterized by very early discovery (congenital or within the first months of life), preferential localization on the lower limbs and progression to sclerosis. Although non-specific, hypersudation and lanuginous hypertrichosis are also highly suggestive signs. The main clinical and histological differential diagnosis is angio-eccrine hamartoma and, to a lesser degree, Kaposi hemangioendothelioma which does not predominate on the lower limbs. This particular clinical presentation of tufted angioma should lead to wide and sufficiently deep biopsy to allow precise diagnosis prior to initiating therapy. The possibility of spontaneous involution would allow abstention with regular surveillance to detect reactivation and progression or functional impairment. Such unfavorable progression has lead certain authors to propose high-dose systemic corticosteroid therapy or alpha-interferon.

Background: Pyostomatitis-pyodermatitis vegetans is an uncommon condition associated with chronic inflammatory bowel disease in 75% of the cases, usually hemorrhagic rectocolitis., Case Report: A 48-year-old man was referred for recent development of pustulous lesions of the lips and buccal mucosa and weight loss. He complained of abdominal pain and intermittent diarrhea which had persisted for more than one year. During the last three months, a pseudotumoral plaque with a pustulous rim had developed over the two distal phalanxes of the right middle finger in association with ungueal lysis and nodular, vegetating and crusted lesions on the lateral aspect of the left arm. Small pustules covered the entire buccal mucosa excepting the tongue and the glans forming a typical snail trace aspect. Bacterial and mycological samples were negative. The histology reports for skin and mucosa were similar: epithelial hyperplasia, intra- and subepithelial granulocyte micro-abscesses and polymorphous infiltration of the superficial derma with numerous neutrophils and eosinophils. There was a discrete interkeratinocytic fluorescence at direct immunofluorescence but indirect immunofluorescence was negative. Anti-desmogleine 1 immunolabeling showed typical normal skin uptake and immunotransfer was negative. Digestive tract endoscopy and histopathology examination of the bowel specimens confirmed the diagnosis of Crohn's disease. Clinical manifestations improved dramatically with prednisone., Discussion: This case of pyostomatitis-pyodermatitis vegetans involved several aspects rarely reported in the literature: a) the cutaneomucosal signs were inaugural; b) the association with Crohn's disease; c) the presence of lesions to the genital mucosa; d) the unusual localization of the inaugural skin manifestations. This clinical entity has now been clearly distinguished from pemphigus vegetans. There was however a long debate on the similar clinical, histological and even immunological expressions. We suggest that pyostomatitis-pyodermatitis vegetans belongs to the spectrum of neutrophilic dermatoses and other authors even propose it is a clinical form of pyoderma gangrenosum.

Background: Paraneoplastic pemphigus is a bullous skin disease with characteristic polymorphous clinical presentation and precise histological and immunological features. We report a case of paraneoplastic pemphigus associated with chronic lymphoid leukemia involving the tracheobronchial epithelium., Case Report: A patient with chronic lymphoid leukemia developed pluriorificial lesions. There were several conjunctival, buccal and genital erosions associated with erosive plaques on the trunk, Nikolski's sign and bullous lesions suggestive of paraneoplastic pemphigus. Histology examination of a bulla showed intraepidermal blistering and suprabasal acantholysis. Direct immunofluorescence evidenced intercellular IgG and C3 deposits. Search for anti-intercellular substance antibodies was positive with fluorescence on specific paraneoplastic pemphigus substrates. At immunotransfer, the serum recognized several bands corresponding to 250, 230, 210 and 190 kD antigens, confirming the diagnosis of paraneoplastic pemphigus. Several days later, the patient's general condition deteriorated with bronchorrhea. Bronchial endoscopy visualized ulceronecrotic plaques. Tracheal biopsy evidenced acantholytic cells and intraepithelial cleavage. General corticosteroid therapy was initiated and led to improvement of the skin lesions but the patient died rapidly from pneumonia. Autopsy confirmed the presence of epithelial cleavage and acantholysis involving the trachea and bronchi., Discussion: This case illustrates the difficulty of diagnosing paraneoplastic pemphigus in the early stages. The pluriorificial lesions were suggestive of a Stevens-Johnson syndrome. Besides the genital, conjunctival and buccal mucosa, other mucosa can be involved. In our case, despite the absence of an immunological element, histology was highly suggestive of specific tracheobronchial localizations. The presence of such lesions, which should be searched for in all cases with bronchopulmonary manifestations, worsens the prognosis.