Distinctive cutaneous and systemic features associated with specific antimyositis antibodies in adults with dermatomyositis: a prospective multicentric study of 117 patients.

BACKGROUND: Identification of myositis-specific autoantibodies (MSAs) for dermatomyositis (DM) could allow the characterization of an antibody-associated clinical phenotype. OBJECTIVE: We sought to define the clinical phenotype of DM and the risk of cancer, interstitial lung disease (ILD) and calcinosis based on MSA. METHODS: A 3.5-year multicentre prospective study of adult DM patients was conducted to determine the clinical phenotype associated with MSAs and the presence of cancer, ILD and calcinosis. RESULTS: MSAs were detected in 47.1% of 117 included patients. Patients with antimelanoma differentiation-associated protein-5 antibodies (13.7%) had significantly more palmar violaceous macules/papules [odds ratio (OR) 9.9], mechanic's hands (OR 8), cutaneous necrosis (OR 3.2), articular involvement (OR 15.2) and a higher risk of ILD (OR 25.3). Patients with antitranscriptional intermediary factor-1 antibodies (11.1%), antinuclear matrix protein-2 antibodies (6.8%) and antiaminoacyl-transfer RNA synthetase (5.1%) had, respectively, significantly more poikiloderma (OR 5.9), calcinosis (OR 9.8) and articular involvement (OR 15.2). Cutaneous necrosis was the only clinical manifestation significantly associated with cancer (OR 3.1). CONCLUSION: Recognition of the adult DM phenotype associated with MSAs would allow more accurate appraisal of the risk of cancer, ILD and calcinosis.

Dermatomyositis: factors predicting relapse.

BACKGROUND: The course of dermatomyositis (DM) can be chronic with relapses, which are associated with major morbidity. OBJECTIVE: The aim of this study was to identify presentation features that predict DM relapses. METHODS: We retrospectively reviewed data of patients with DM recorded from 1990 to 2011, including muscle biopsy results. Characteristics of patients with and without relapses were compared. Hazard ratios (HRs) were estimated using a Cox model. RESULTS: We identified 34 patients, with a mean age of 46 ± 17 years (range, 18-77) and 24 (71%) women. The muscle and skin abnormalities relapsed in 21 (61%) patients. By univariate analysis, two presentation features were significantly associated with a subsequently relapsing course, namely, dysphonia [HR = 3.2 (1.2-8.5)] and greater skin lesion severity defined as a Cutaneous Disease Area Severity Index [CDASI] > 20 [HR = 3.5 (1.2-7.9)]. CONCLUSION: Dysphonia and skin lesion severity at disease onset must be recorded, as they significantly predict a relapsing disease course.

[Dermatology day-care: between official rules and reality]. / Hôpital de jour de dermatologie : entre circulaire frontière et réalité

BACKGROUND: Official rules published in 2006 and 2010 concerning ambulatory care rates in France led to artificial redistribution of this activity from day-care hospitalization to consultations. In our dermatological day-care establishment, we compared the financial costs engendered for patients admitted for day-care hospitalization and those seen at consultations. PATIENTS AND METHODS: From 2011/01/10 to 2011/02/04, for each patient, we prospectively analyzed the following data: day-care hospitalization or consultation, age, sex, diagnosis, laboratory and radiological examination, non-dermatological consultations, time spent with the patient by doctors (interns, senior doctors) and nurses, with timing by a stop-watch. The hospital cost was the total for medical examinations (official nomenclature), non-dermatological consultations, physicians' and nurses' salaries and establishment overheads (216 €). The hospital revenue regarding the consultation group consisted of the sum of reimbursement for medical examination, dermatological and non-dermatological consultations, and regarding the day-care hospitalization group, the dermatology rate (670 €) or chemotherapy sessions (380 €). Results were compared using a Chi(2) test and a Student's t-test (P ≤ 0.05). RESULTS: One hundred and twenty-seven patients were included: 67 in the day-care hospitalization group and 60 in the consultation group. Patients in the day-care hospitalization group were older and had significantly more radiological examinations and non-dermatological consultations, but the number of laboratory examinations and skin biopsies did not differ between the two groups. The mean time spent by doctors was similar in both groups but the time spent by senior doctors without the help of interns was significantly greater and longer than the time for a standard consultation. Nurses spent a mean 72 minutes with each hospitalized patient and 35 minutes with consultation patients (P = 0.007). Hospital costs were identical in both groups at around 415 €. The hospital showed a profit for day-care hospitalization patients (252 €) and a loss (244 €) for consultation patients. DISCUSSION: Half of the patients studied were in day-care hospitalization and half were seen in consultations. The high number of bed-ridden patients with bullous pemphigoid accounts for the fact that day-care patients were older. The reasons for the significantly longer time spent by nurses with day-care hospitalized patients were administration and supervision of chemotherapy, skin care and nursing of bed-ridden patients. However, nurses spent 35 min with each consultation patient, justifying the need to maintain the posts of these staff in such day-care units. The availability of physicians for patients with severe dermatoses and the organization of medical examinations in the same place in the same day underscore the need for medical structures like day-care hospitalization. At present, time spent on intellectual work involving reflection is regrettably not taken into account, which is detrimental to this specialty. The hospital was in profit for day hospitalizations while consultations resulted in losses, in particular because of the absence of social security reimbursement of the establishment's overheads. CONCLUSION: Rules are in need of modification in order to allow the treatment of patients with more complicated conditions.

[Methotrexate-induced cutaneous reactions: two case reports]. / Toxidermies au méthotrexate : deux observations.

BACKGROUND: Methotrexate has been prescribed for many years in the treatment of dermatological diseases. Cutaneous side effects of methotrexate are rare and consist chiefly of direct toxicity resulting from overdose. We report herein two original cases of methotrexate-induced cutaneous adverse reactions. PATIENTS AND METHODS: Case 1: A 70-year-old man was followed for bullous pemphigoid. After two relapses with clobetasol, methotrexate was initiated. One month later, a vesicular and itching rash localized on the face and trunk was observed. The lesions systematically occurred in the week following drug-intake and finally healed after dosage reduction. Case 2: An 81-year-old woman was hospitalized for bullous pemphigoid, first treated by clobetasol. She relapsed 5 months later, and methotrexate was initiated subcutaneously. After the third injection, an edematous and papular eruption was observed on the patient's face. The eruption recurred systematically after drug injection and healed after dosage reduction. DISCUSSION: The chronology of drug-intake, a positive test following renewed intake and improvement of lesions with a lower dose of methotrexate allowed us to conclude on cutaneous adverse reactions to methotrexate. These two original cases underline the atypical clinical presentation of cutaneous adverse reaction. This diagnosis should be systematically discussed in cases of recurrent rash.

[Subacute cutaneous lupus erythematosus and cancer: two cases and literature review]. / Lupus cutané subaigu et cancer: deux cas et revue de la littérature.

BACKGROUND: Subacute cutaneous lupus erythematosus is an uncommon dermatosis characterised by a non-scarring, annular photo-distributed dermatosis associated with anti-Ro/SSA antibodies. It is remarkable as a paraneoplastic syndrome (12 cases in the literature). We report two cases of subacute cutaneous lupus erythematosus occurring in patients treated for metastatic breast adenocarcinoma. CASE REPORTS: Case 1: a 72-year-old woman with breast carcinoma relapsing after surgery, chemotherapy, hormone therapy, and without treatment for 6 months, was admitted for an acute erythematous slightly squamous and photo-distributed eruption. On clinical examination, she was found to be presenting polyadenopathy and pleural effusion. Case 2: a 46-year-old woman with a history of breast carcinoma was admitted for an erythematopapular, annular and photo-distributed eruption occurring after a second breast cancer relapse and five months after initiation of docetaxel. A new line of chemotherapy initially resulted in regression of the lesions, and progression of the breast cancer was associated with cutaneous relapse. DISCUSSION: The diagnosis of subacute cutaneous lupus erythematosus was supported in our two patients by the presence of an annular photo-distributed eruption associated with positive testing for anti-Ro/SSA antibodies. Occurrence of the eruption in both cases with relapse of the neoplasia and its improvement after oncological treatment reinforced the diagnosis of paraneoplastic syndrome in one case, and the use of chemotherapy known to trigger lupus could have suggested a diagnosis of drug-induced subacute cutaneous lupus erythematosus. Thus, the association between lupus and cancer is relevant.

[Adverse cutaneous reaction to celecoxib: 6 cases]. / Toxidermies au célécoxib: 6 cas.

INTRODUCTION: Our objective was to characterize adverse cutaneous reactions to celecoxib, a new non steroidal anti-inflammatory drug. PATIENTS AND METHODS: A retrospective study of 6 consecutive patients. RESULTS: The average delay before the onset of the reaction was 10.2 days for patients taking the medication for the first time and 48 hours for one patient taking the drug for the second time. Two patients had fever. Patients presented with an exanthema and in most cases an edema of the face. Buccal mucosa was involved in two patients, and one patient had minimal blister lesions. In five of the six patients, minor and transitory biological abnormalities were found. The intrinsic imputability of the celecoxib was I3 (C3S2) in all the cases. DISCUSSION: Our cases are similar to those reported by the French drug regulatory agency (Pharmacovigilance). Usually the adverse cutaneous reactions were not too severe, with maculo-papulo exanthema and edema of the face. The reactions due to celecoxib are more frequent than those due to other non steroidal anti-inflammatory drugs (7.5% versus 4.1%), but severe cases are rarely reported. Besides, an allergic history to sulphonamide contraindicates celecoxib. However celecoxib does not have the aromatic amine common to antibacterial sulphonamides, and there is no proof of cross reactions between these two families. Furthermore, this amine is usually associated with drug reaction severity, which could explain why severe cases due to celecoxib are rare.

Thirteen-megahertz ultrasound probe: its role in diagnosing localized scleroderma.

BACKGROUND: Ultrasound imaging has been shown to be useful for the evaluation of systemic and localized scleroderma (LS). However, its specificity and sensitivity have not been studied. OBJECTIVES: To define morphological ultrasound diagnostic criteria in LS and to test their sensitivity and specificity with a 13-MHz ultrasound probe. METHODS: Forty plaques in 26 consecutive patients with LS were examined and compared blindly with 17 control plaques in 16 patients with skin diseases where LS was in the differential diagnosis. Data were also compared with a normal control group. Five patients were re-evaluated 12-18 months after the first examination. RESULTS: Ultrasound examination disclosed a characteristic dense image resembling a flattened 'yo-yo'. Undulations of the dermis, disorganization, loss of thickness and thickened hyperechoic bands in the hypodermis, and the 'yo-yo' image had a high sensitivity and a high specificity for LS. A 92% sensitivity and a 100% specificity for LS were found when at least four of these five signs were present. CONCLUSIONS: Thirteen-megahertz ultrasound is a valuable tool for diagnosing LS. Morphological ultrasound diagnostic criteria had a high specificity and a high sensitivity.

[Sarcoidosis: thalidomide treatment in ten patients]. / Sarcoïdose: traitement par thalidomide chez 10 malades.

BACKGROUND: Acute cutaneous sarcoidosis is generally spontaneously regressive but persistent chronic cutaneous lesions are esthetically prejudicial. There have been several case reports on thalidomide efficacy but long-term outcome is unknown. We report results in 10 cases of cutaneous sarcoidosis treated with thalidomide. PATIENTS AND METHODS: Data from ten patients with sarcoidosis treated with thalidomide between January 1998 and March 1999 were collected from delivery authorizations and analyzed. All ten patients had chronic cutaneous sarcoidosis resistant to conventional therapy. Six patients had an associated visceral localization and disease duration of 2 to 18 years (median 6 years). We considered that regression was complete when erythema and infiltration had totally disappeared, that regression was incomplete when cutaneous signs remained, and that treatment had failed when no effect was observed or when the disease worsened. RESULTS: Disease regression was noted in 7 patients for a daily dose of 1.84 mg/kg for 2.8 months. Skin lesions totally regressed in 3 patients, an incompletely in 4. Treatment failed in 3 patients. Patients were treated for 10 months (2 to 21 months). The daily dose of thalidomide was gradually reduced in 5 of 7 patients for whom treatment was effective. Three of these 5 patients relapsed and thalidomide was again given and was effective again at the same dose and after the same delay. We observed improved kidney function in one patient, improvement in nasal infiltration in one other and complete regression in 3 patients who achieved long lasting reduction in angiotensin convertase level. When treatment failed, the daily dose was 1.15 mg/kg and the treatment had to be stopped for 2 patients. Side effects were minor, excepting 2 cases of neuropathy. DISCUSSION: This open study of 10 patients treated with thalidomide showed the efficacy of a 1.84 mg/kg daily dose in 7 out of 10, but complete regression of the lesions was obtained in only 3 patients. Thalidomiide appears to suspend the disease, with relapse when the drug is discontinued and efficacy at re-introduction. This would argue against a placebo effect. The mode of action could involve immunomodulating and antiinflammatory mechanisms.

Amicrobial pustulosis associated with autoimmune diseases: healing with zinc supplementation.

Amicrobial pustulosis associated with autoimmune diseases is an eruption involving the cutaneous flexures and scalp. We report two young women with this disease, in whom pustulosis healed with zinc supplementation. We suggest that it may be relevant to measure plasma zinc level in patients with such a disease.

Increased fibroblast elastase activity in acquired cutis laxa.

BACKGROUND: Acquired cutis laxa is a rare disease characterized by sagging skin, premature wrinkling and reduced skin elasticity. OBSERVATION: We report a 21-year-old woman, who presented with acquired cutis laxa on the face and the ear lobes. Urticarial papules had preceded for 6 years. There was no systemic involvement. Skin specimens were obtained from lax skin and urticarial papules, and from healthy controls. Histology showed only few perivascular lymphocytes in lax ear skin and a dense inflammatory infiltrate in urticarial skin. In both biopsies elastic fibres were decreased as demonstrated by computerized morphometric analyses. Elastase activities of fibroblasts in culture were evaluated. There was a 2- to 3-fold increase in elastase activity in urticarial skin fibroblasts, contrasting with a normal elastase activity in lax ear skin. CONCLUSION: Our findings suggest that the inflammatory cells could play a significant role in the destruction of elastic fibres.

The treatment of mild pemphigus vulgaris and pemphigus foliaceus with a topical corticosteroid.

Seven patients with mild pemphigus vulgaris (n = 3) or pemphigus foliaceus (n = 4) were treated with a very potent topical corticosteroid alone. Clobetasol propionate 0.05% cream was applied to mucosal lesions and involved skin twice a day for at least 15 days, then progressively tapered. Pemphigus was considered to be controlled if healing of lesions was obtained, with a 75% decrease in the number of new lesions per week without addition of any systemic treatment. In all seven patients, the disease was controlled initially with healing of cutaneous lesions within 15 days, while healing of mucosal lesions took at least 1 month. In four patients, remission was maintained with topical corticosteroid alone for a mean 19-month follow-up. In three patients, relapse occurred after 2-11 months, requiring a systemic treatment.

Oral Crohn disease: clinical characteristics and long-term follow-up of 9 cases.

BACKGROUND: Oral localization of Crohn disease is uncommon and must be differentiated from nonspecific lesions. Its natural course and its long-term prognosis are unknown. OBSERVATIONS: We studied 9 patients (8 male, 1 female; age range, 7-52 years; median age, 16 years) with Crohn disease and specific oral lesions, including deep linear ulcers, pseudopolyps, and/or labial or buccal swelling and induration. The prevalence of such lesions was 0.5%. The median follow-up was 11 years. Oral localization developed before (n = 2), at the same time as (n = 2), or after (n = 5) the onset of the digestive disease. Noticeable associated localizations were observed in the anoperineum (n = 8) and the esophagus (n = 3). The median duration of the oral lesions was 4 years (range, 1-13 years), without necessary parallelism with the digestive localization. Five patients had complete healing after a median delay of 2 years. CONCLUSIONS: Oral localization of Crohn disease is characterized by a marked male predominance, a young age at onset of Crohn disease, and a very protracted course. The high prevalence of associated anal and esophageal involvement suggests that Crohn lesions have a particular trophicity for squamous cell epithelium.

[Acral erythema and HIV infection]. / Erythème acral et infection par le VIH.

BACKGROUND: Acral erythema is a non specific skin condition observed in connective tissue diseases, viral infections and toxic skin reactions. More recently, this symptom has been reported in HIV-infected patients. CASE REPORT: We observed two cases of acral erythema in HIV seropositive patients. Both patients were drug abusers. Erythema was limited to the limbs with no clinical manifestation of connective tissue disease. Both consumed approximately 40 tablets of codeine daily and had hepatitis C without cirrhosis. DISCUSSION: Our 2 cases of acral erythema in HIV-infected patients can be added to 32 cases of digital erythema in HIV patients reported in the literature. The common features in most of these patients was intravenous opiate abuse and hepatitis C without cirrhosis. The degree of immunodeficiency, the nature of opportunistic infections and antiretroviral treatments do not appear to affect the development of skin signs. HIV infection, simultaneous HIV-HCV infection and codeine suggest they might have a pathogenic role in the development of distal vascularization disorders.