Biomedical subjects
Publications and source records attributed to B Sassolas.
The amelanotic melanoma is a rare type of malignant melanoma. The purpose of this report is to point out this aspect of melanoma, whose diagnostic delay is frequent. We describe one localisation on the penis, rare in this kind of melanoma and two cases of the extremities, more frequent localization of the amelanotic melanoma. The therapeutic issues and recommendation are similar to the classical pigmented malignant melanoma.
BACKGROUND: Bullous pemphigoid is an autoimmune disease, common in the elderly and generally of symmetrical and systemic localization. We report a case with sparing of the lower limb and acquired lymphedema secondary to lymph node surgery. CASE REPORT: A 74-year-old woman was hospitalized for a bullous eruption. The left lower limb was completely spared and was unaffected by pruritus. Acquired lymphedema was seen in this limb secondary to lymph node surgery. Standard histopathology tests confirmed the diagnosis of bullous pemphigoid with subepidermal blistering, while a direct immunofluorescence antibody test showed linear binding of IgG and C3 throughout the basement membrane. Western blotting revealed anti-BPAg2 antibodies. Skin biopsy on the lymphedema spared by the disease revealed no inflammatory infiltrate in the dermis. However, linear binding of anti-IgG and anti-C3 autoantibodies was observed. DISCUSSION: Other cases of localized bullous pemphigoid appearing on body areas treated by UV or radiotherapy have been reported. Cases of bullous pemphigoid with predilection for areas of lymphedema have also been previously described: the hypothesis has been advanced of reduced lymphatic flow, with increased antigen-antibody contact enabling better binding. Our case is original and, given the protective nature of this lymphedema, suggests two hypotheses. There could be deterioration of local cellular immunity, with decreased activation of T lymphocytes. They could also be impairment of nervous conduction, as suggested by the absence of pruritus, with partial or total inhibition of neurogenic inflammation.
BACKGROUND: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS syndrome) is a severe drug eruption defined as an association of a drug reaction, mostly cutaneous, laboratory abnormalities with the presence of hypereosinophilia and/or atypical lymphocytes, and involvement of one or more organs. We report a case of DRESS syndrome associated with quinine or thiamine (which in France are combined in Hexaquine with serious hepatitis and renal failure and associated viral reactivation of Human Herpes Virus 6 (HHV6). OBSERVATION: A twenty-three year-old woman was hospitalized for asthenia, fever, erythroderma, facial edema and painful cervical lymph nodes. Laboratory results showed inflammatory syndrome, hypogammaglobulinemia and the presence of activated atypical lymphocytes. Broad screening for infectious serology showed HHV6 reactivation with marked increase in specific IgG levels within two weeks and detection of circulating DNA in blood by PCR (polymerase chain reaction). Two severe complications subsequently appeared: cytolytic hepatitis and interstitial nephritis. The outcome was favorable with oral corticosteroid therapy. Repeated history-taking did not initially identify any causal agent, but several weeks later, the patient remembered taking Hexaquine (quinine and thiamine) for essential muscular cramps daily for 27 days before the onset of symptoms. The patient had taken no other medication during the previous weeks. One year later, at the end of an enteroviral infection, a skin rash similar to the first episode was seen but in isolation on this occasion, suggesting minor reactivation of DRESS syndrome. DISCUSSION: To our knowledge, ours is the first reported case of DRESS syndrome associated with this drug. Because of the difficulty of identifying the causative drug, even though taken alone, our case once again illustrates the importance of spending adequate time on history taking. Although the physiopathology of DRESS syndrome has not yet been fully elucidated, it is nevertheless probable that it is a multi-factor phenomenon (involving medication and viruses among others).
INTRODUCTION: We report on a patient who progressively developed polymorphic expressions of neutrophilic dermatosis (Sneddon-Wilkinson subcorneal pustulosis and pyoderma gangrenosum) associated with p-antineutrophil cytoplasmic antibodies (p-ANCA), while receiving propylthiouracil for hyperthyroidism. To our knowledge, such associations have never been published so far. CASE-REPORT: A 40 year-old woman was treated with propylthiouracil for Graves'disease. After 16 months of therapy, she noted flares of pustular lesions surrounded with erythematous halo mainly localized on the trunk. The lesions became chronic, and were not improved by potent topical corticosteroids. When first seen in our department in February 2003, the eruption was typical of Sneddon-Wilkinson subcorneal pustulosis. This diagnosis was confirmed by the histological examination of a skin biopsy of a pustule. One month later, she developed an inflammatory progressively ulcerative lesion on the right ankle, typical of pyoderma gangrenosum. The diagnosis was confirmed by the histological examination of a skin biopsy taken on the evolving border of the lesion and showed polynuclear neutrophilic infiltration without vasculitis. Direct immunofluorescence was negative. The presence of serum anti-myeloperoxydase p-ANCA was known for this patient since October 2002. No IgA monoclonal gammapathy was revealed on extensive biological check-up. Systemic oral corticosteroid therapy (1 mg/kg/day) dramatically improved skin lesions with complete healing within 8 weeks. DISCUSSION: Propylthiouracil is well known to induce the occurrence of ANCA in 20 to 64p. 100 of patients treated for Graves'disease. The mechanisms involved are badly recognized so far. Cutaneous vasculitis, glomerulonephritis and polychondritis may be clinically associated with those antibodies. Rare observations of neutrophilic dermatosis, mostly Sweet's syndrome, have been described in patients with propylthiouracil-induced ANCA. One case-report described a 44 year-old woman who developed pyoderma gangrenosum associated with propylthiouracil-induced p-ANCA. These manifestations usually appear within 2 years, as our patient. The data in the literature, allows us to report the polymorphic expressions of neutrophilic dermatosis in this patient with p-ANCA which could be related to propylthiouracil. Such association of Sneddon-Wilkinson subcorneal pustulosis and pyoderma gangrenosum with p-ANCA has never been described in this endocrinologic context so far. Furthermore we propose that neutrophilic dermatosis should be inscribed in the list of side effects induced by propylthiouracil therapy.
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: The aim of our study was to understand the motivations of outpatients who come to dermatological emergencies in a university hospital. PATIENTS AND METHOD: This 6-week prospective study included outpatients who came to the dermatology emergency unit. This consultation is proposed each morning (from 8 to 9), from Mondays to Fridays. A questionnaire was distributed to outpatients. They answered questions on the functioning of this consultation and their own symptoms. The consulting dermatologist answered questions on the referring physician, the really urgent characteristics of the disease and the diagnosis. RESULTS: Patients were satisfied by the functioning of the consultation. Indeed, 59 p. 100 of outpatients thought that the timetable was convenient and 70 p. 100 that the delay before getting a consultation was rapid. 75 p. 100 felt they needed treatment rapidly. Nonetheless, 45 p. 100 did not think they had a serious disease. More than half of the outpatients were referred by their general practitioner; the others came spontaneously, or were referred by other departments or general emergencies. The most frequent diagnoses were cutaneous infections (27.6 p. 100), eczema (21 p. 100), then benign tumors, psoriasis, physical dermatoses, viral eruptions... DISCUSSION: A consultation for dermatological emergencies appears to reply to patients' demands. Nonetheless, most of these outpatients do not present with real dermatological emergencies. Criteria for real emergencies needs to be further defined and understood by citizens.
BACKGROUND: Some cases of dermatofibrosarcoma protuberans (DFSP) do not protrude above the skin. OBJECTIVES: To assess the prevalence of these DFSPs and further to describe their presentation and course. METHODS: One hundred and forty-three patients were retrospectively collected. They were asked to complete a standardized questionnaire indicating the history and appearance of the DFSP from the first skin changes identified to the time of diagnosis. RESULTS: Eighty-one DFSPs were described as protuberant ab initio, and 62 as initially nonprotuberant (npDFSP). The latter remained at this stage for a mean period of 7.6 years. Twenty-nine per cent of npDFSPs were 'morphoea-like', 19% were 'atrophoderma-like' and 42% were 'angioma-like'. Age at diagnosis was similar for both initial presentations. npDFSPs were most often misdiagnosed by physicians. CONCLUSIONS: Nearly half the patients first identified their early DFSP-related skin changes as patches. Both this frequency and the long duration at this preprotuberant stage should prompt dermatologists to consider the diagnosis of DFSP earlier, in order to make surgical treatment easier.
INTRODUCTION: The Bardet-Biedl syndrome is a rare autosomal recessive disorder, which associates obesity, pigmentary retinopathy, hexadactyly, hypogenitalism, renal dysfunction and mental retardation. Other abnormalities can be observed in the Bardet-Biedl syndrome, but few cutaneous abnormalities have been described. CASE REPORT: A 41 year-old woman, suffering from a Bardet-Biedl syndrome diagnosed when she was 7 Years old, presented with an atypical pseudo verruca-like, dark red lesion of the interbuttock area that had developed over fifteen Years and had become a handicap. The histological examination revealed a double component: epithelial, papillomatous and acanthosic on the one hand and vascular and lymphatic on the other, suggesting a lymphangioma with epidermal hyperplasia. Magnetic resonance imaging of the sacral area revealed a median subcutaneous lesion, extending deeply to the third coccygial vertebra. DISCUSSION: Such a lymphangioma is unusual. Because it occurred during a rare polymalformative syndrome, we suggest that it may represent a new clinical sign that can be observed during the Bardet-Biedl syndrome.
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.
Infections caused by Cunninghamella bertholletiae are rare but severe. Only 32 cases have been reported as yet, but in 26 of these this species was a contributing cause of the death of the patient. This opportunistic mould in the order Mucorales infects immunocompromized patients suffering from haematological malignancies or diabetes mellitus, as well as solid organ transplant patients. The lung is the organ most often involved. Two cases of primary cutaneous infection have been previously reported subsequent to soft-tissue injuries. We report a case of primary cutaneous C. bertholletiae zygomycosis in a 54-year-old, insulin-dependent diabetic man who was treated with tacrolimus and steroids after kidney transplantation. No extracutaneous involvement was found. In this patient, the infection may have been related to insulin injections. The patient recovered after an early surgical excision of the lesion and daily administration of itraconazole for 2 months. This case emphasizes the importance of an early diagnosis of cutaneous zygomycosis, which often presents as necrotic-looking lesions. Prompt institution of antifungal therapy and rapid surgical intervention are necessary to improve the prospects of patients who have contracted these potentially severe infections.
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BACKGROUND: We previously proposed a set of 4 clinical criteria for the diagnosis of bullous pemphigoid (BP) that consisted of age greater than 70 years, absence of atrophic scars, absence of mucosal involvement and absence of predominant bullous lesions on the neck and head. These results have been challenged because direct immunoelectron microscopy (IEM), which was used as the standard diagnostic criterion in our initial study, does not identify the different antigens of the basement membrane zone. OBJECTIVE: To reassess the validity of these clinical criteria for the diagnosis of BP using immunoblot analysis of patient sera as the main diagnostic criterion, in order to precisely identify the antigens recognized by patient sera. METHODS: One hundred and eighty-nine sera from patients with various subepidermal autoimmune blistering diseases (AIBDs) were tested by immunoblotting using dermal and epidermal extracts. IEM was used as a complementary diagnostic procedure in a few patients whose serum recognized BPAG2 exclusively or was negative in immunoblotting. RESULTS: 142 patients (75%) had at least 3 of the 4 clinical diagnostic criteria. Sera from patients who lacked the set of BP clinical criteria were more frequently immunoblot negative (34%) than sera from patients who had the criteria (18%; p = 0.025). BPAG1 was more frequently recognized by sera from patients with the set of BP clinical criteria (78%) than by sera from patients without the criteria (45%; p = 5.10(-4)). In contrast, BPAG2 was recognized by a great number of sera from patients who lacked the criteria of BP (71%), which was in accordance with the presence of numerous patients with cicatricial pemphigoid in this group. Among patients with various subepidermal AIBDs, the diagnosis of BP could be made with a sensitivity of 86%, a specificity of 90% and an excellent prognostic positive value over 95%, if 3 of these clinical criteria were present. CONCLUSION: These results confirm the interest of this set of clinical criteria for the rapid diagnosis of BP.
INTRODUCTION: Interferon alpha has many side effects. Among them the risk of occurrence of seizures is not well known by dermatologists. We report three cases of seizures that occurred in patients treated with interferon alpha in two dermatological diseases: mycosis fungoides and melanoma. OBSERVATIONS: A 68 year-old man, treated for mycosis fungoides, and two men aged 47 and 52 years, treated for melanoma, were under interferon alpha. After 11 months, 3 weeks and 9.5 months, respectively, the three patients had seizures without any past history of epilepsy. Anamnesis and assessment of each patient (brain CT, biological results) suggested the responsibility of interferon alpha. After withdrawal of the treatment, no relapse was observed after 3 months, 6 months and 1 year later, respectively. DISCUSSION: Seizures during treatment with interferon alpha have already been reported. According to the series their prevalence would be of 1 to 4 p. 100. Their pathophysiology is not well known, but apparently interferon alpha lowers the epileptogenic threshold by affecting the central nervous system either directly or through cytokines or neuromediators. The risk of occurrence of seizures must be known by the prescribing physician who must systematically search for past history of epilepsy or risk factors for seizures. This rare but existing side effect raises the problem of information to be supplied to the patient by the prescribing physician.
INTRODUCTION: Erythermalgia is a rare acrosyndrome characterized by reddening of the skin, local increase heat and pain. The disease is frequently resistant to treatment. Recently, Kuhnert et al. presented very favorable results using a combination of lidocaine and mexiletine. We used this treatment in 4 patients suffering from familial erythermalgia. OBSERVATIONS: In a family exhibiting severe familial erythermalgia involving 5 members over 3 generations, we treated 4 patients aged 41, 39, 19 and 15 years. In these patients, the erythermalgia known since early childhood, progressed in the form of multiple flares (6 to 7/day) during the day and at night, lasting several hours and often accompanied by headaches. The impact of the disease on their quality of life was major. Only cold-water baths provided temporary relief, obliging them to live with their "feet in cold water". After they had been informed of the modalities of treatment and in the absence of any contraindication, notably cardiologic, 200 mg (100 mg in the youngest patient) of lidocaine were infused in 4 hours in a single intravenous injection on the first day. Mixelitine was introduced on the second day at the dose of 600 mg in 3 oral intakes (200 mg in the youngest patient). The painful paroxistic symptomatology rapidly improved and the flares had disappeared on the 3dr day, thus permitting the progressive reduction in analgesics and major improvement in quality of life. This beneficial effect persisted with oral mexiletine alone, 2 years after the infusion of lidocaine in the first patient treated (and one year after in the other patients). COMMENTS: Primary familial erythermalgia is highly resistant to treatment. The combined action of lidocain and mexiletine, usually well tolerated (class IB antiarrythmic), blocks the sodium channels. The mechanism of action of their analgesic effect is peripheral or central or even mixed. This benefit warrants confirmation in other forms of erythermalgia.
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BACKGROUND: The identification of a highly potent immunosuppressive/antiproliferative agent with an acceptable toxicity profile has long been a goal for the management of severe plaque psoriasis. OBJECTIVES: To investigate the efficacy and safety of sirolimus (Rapamune) for severe psoriasis when given alone or in association with cyclosporin. METHODS: In a randomized, double-blind, eight parallel group, pilot study in 24 out-patient centres in seven European countries, 150 patients, 18 years and older, with severe chronic plaque psoriasis were given sirolimus 0.5, 1.5 and 3.0 mg m(-2) daily for 8 weeks, either alone or in association with a subtherapeutic dose of cyclosporin (1.25 mg kg(-1) daily). Cyclosporin 5 mg kg(-1) daily was the positive control and cyclosporin 1.25 mg kg(-1) daily the negative control. The primary efficacy variable was the mean percentage reduction in Psoriasis Area and Severity Index (PASI). Safety assessments included monitoring of adverse events, clinical laboratory parameters and sirolimus/cyclosporin blood concentrations. RESULTS: The greatest mean percentage decreases in PASI were seen with cyclosporin 5.0 mg kg(-1) daily (70.5%) and with sirolimus 3.0 mg m(-2) daily + cyclosporin 1.25 mg kg(-1) daily (63.7%). Both groups demonstrated significantly better results than cyclosporin 1.25 mg kg(-1) daily (mean decrease 33.4%). Serum creatinine levels were significantly lower for groups with sirolimus alone and sirolimus plus reduced-dose cyclosporin when compared with cyclosporin 5.0 mg kg(-1) daily. Adverse events associated with sirolimus included thrombocytopenia (5%), hyperlipidaemia (9%), aphthous stomatitis (9%) and acne (13%), whereas adverse events associated with cyclosporin included hot flushes (12%), hyperlipidaemia (9%) and increased serum creatinine (9%). CONCLUSIONS: The concomitant administration of sirolimus with a subtherapeutic dose of cyclosporin in severe psoriasis may permit a reduction in their respective toxicities, notably cyclosporin-induced nephrotoxicity.
BACKGROUND: The diagnosis of photodermatosis is generally facilitated by the typical localization of the eruption. The causal agent can usually be identified by history taking, allowing eviction and cure. It may be difficult to find the causal agent in cases with a sequential course. The occupational and/or recreational environment may provide helpful information. We present two cases of photodermatosis related to the occupational environment. CASE REPORTS: A 50-year-old woman and a 29-year-old man were farm workers. both consulted for photoinduced eczema. The eczema was triggered by episodic manipulation of an antibiotic used widely for preparing animal feed. The standard allergy tests were negative. The photobiology exploration led to the diagnosis of photoallergy to olaquindox, a growth promotor added to animal feed. The course was favorable after eviction or protection against the product, providing a complementary proof of its triggering effect. DISCUSSION: We emphasize the contribution of photobiological explorations in difficult cases with an occupational background. Generally, these patients are unaware of the composition of the products manipulated, such as feed additives.