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.

[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.

[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.

[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.

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

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

Internal involvement in localized scleroderma.

We studied 76 consecutive patients with localized scleroderma (morphea with or without linear scleroderma) and analyzed the frequency, prognosis, and predictors of internal involvement in a subset of 53 patients systematically investigated for the presence of such involvement. Internal involvement was found by systematic examination in 16 patients. Only 2 of them, including 1 patient who developed systemic scleroderma, had symptomatic and severe visceral disease. The other 14 patients had asymptomatic and minor abnormalities consisting of abnormal lower sphincter pressure, and/or peristaltic failure in the esophagus and slightly impaired carbon monoxide diffusion in the lung. Frequent seroimmunologic abnormalities had no predictive value. Three parameters were found to be associated with internal involvement: male gender (p < 0.05), increasing number of plaque-like lesions (p = 0.02) and hypergamma-globulinemia at 1st examination (p < 0.005). Mild esophageal and pulmonary involvement are not rare in morphea but are usually silent. In our series, after a median follow-up of 48 months, such involvement did not impair the prognosis. The mildness of these visceral abnormalities suggests that they do not justify routine detection in asymptomatic patients. Morphea and systemic scleroderma behave as 2 different diseases.

Dermatomyositis without muscle weakness. Long-term follow-up of 12 patients without systemic corticosteroids.

BACKGROUND AND DESIGN: We retrospectively studied 12 patients with dermatomyositis (DM) with at least one laboratory criterion of muscle involvement but without muscle weakness. We reported their outcome without systemic corticosteroid treatment and searched for distinctive baseline data and associated neoplasia among a group of 29 patients with classic DM. RESULTS: After a mean follow-up of 5.3 years, complete or partial skin improvement occurred in nine patients with DM without muscle weakness, and muscle enzyme levels were normalized in all. When compared with the classic DM group, no statistical differences were found for associated neoplasia or for any baseline data studied except, as expected, for muscle parameters. CONCLUSIONS: The lack of distinctive baseline data between DM without muscle weakness and classic DM suggests that there is a continuum from amyopathic to classic DM. However, DM without muscle weakness is a clinical entity with respect to favorable long-term prognosis. Unless frank muscle weakness develops, treatment with systemic corticosteroids can be avoided.

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.

[Melanoma and dysplastic nevus after 8 years of topical Caryolysine]. / Mélanome et naevus dysplasiques aprés 8 ans de Caryolysine locale.

A 48-year-old woman with poikiloderma atrophicans vasculare was treated with topical mechlorethamine, applied three times weekly, for eight years. After five years treatment she developed nine melanocytic naevi on covered skin. The naevi spread for the next three years and a surgical excision was performed. Pathological examination revealed one level II SSM, one level I SSM and seven junctional naevi with focal dysplasia or melanocytic hyperplasia. The development of melanoma and dysplastic naevi after topical mechlorethamine has not been previously reported. However it seems likely that mechlorethamine was the causative agent. As for the mechlorethamine's imputability in this case, we have used a method combining intrinsic identification of adverse drug responsibility's score, based on the clinical case and on extrinsic identification based on literature evaluation. We suggest that mechlorethamine could act as UV light.

[Eosinophilic pustular folliculitis (Ofuji) (author's transl)]. / Folliculite à éosinophiles (maladie d'Ofuji).

A case, the first French observation of eosinophilic pustular folliculitis (Ofuji) is reported. The long lasting condition of unknown etiology is characterized by slightly indurated erythematous plaques and formation of follicular small papules and pustules. The lesions have a tendency to central healing and peripheral extension and proceed with remissions and exacerbations. Histological features are pustular folliculitis composed chiefly of eosinophiles. Blood eosinophila is often founded. Local corticotherapy and dapsone are sometimes a effective treatment.

[Resistance to activated protein C disclosed by postphlebitic disease. Two cases]. / Résistance à la protéine activée révélée par une maladie post-phlébitique. Deux cas.

INTRODUCTION: Recently, resistance to activated protein C has been discovered. Resistance to activated protein C appears to be the main cause of familial thrombosis. CASE REPORT: Two consecutive patients have been studied. Both have a venous insufficiency associated with eczema in one patient and venous ulceration in the second patient. The two patients had a personal and familial history of venous thrombosis. In both patients, a resistance to activated protein C was found associated with a mutation in the factor V gene in residue 506. DISCUSSION: When a personal or familial history of the venous thrombosis is associated with symptoms of venous insufficiency, resistance to activated protein C must be added to the search for proteins C, S and anti-thrombin III deficiency.

[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.

[Negativity of Borrelia burgdorferi serology in scleroderma en plaques]. / Négativité de la sérologie pour Borrelia burgdorferi dans la sclérodermie en plaques.

The occurrence of morphea has been attributed to Borrelia, burgdorferi infection, but the relationship between localised scleroderma and borreliosis remains controverted. Antibodies directed against B. burgdorferi were looked for in 21 patients (18 female and 3 male, aged from 8 to 63 years) whose disease had been present for 6 weeks to 13 years. One patient had a single morphea, two had monomelic scleroderma and 18 had multiple localised morpheas. The search for antibodies was conducted in these 21 patients and in 200 blood donors from the Paris region by indirect immunofluorescence techniques, using the CDC method where only antibody titers of 1/256 or more are significant. Serology was lower than 1/256 in 20 cases and equal to 1/256 in one case. Among the 200 blood donors, five (2.5%) had antibody titers of 1/256 or more. None of the patients studied had been exposed to tick bite due to their occupation or place of residence. We therefore found no evidence of a link between B. burgdorferi infection and morphea in the Paris region.(ABSTRACT TRUNCATED AT 250 WORDS)

[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.

[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.

[Cosmetic dermopigmentation. The pigment stays ... as do regrets]. / La dermopigmentation cosmétique. Le pigment reste ... et les regrets aussi.

INTRODUCTION: Cosmetic dermopigmentation designates tattooing of the superficial derma with pigments in order to obtain an aesthetic effect. We observed two cases illustrating the inconveniences of this technique. CASE REPORTS: Two women underwent dermopigmentation on the face in a beauty institution to create pseudo-freckles. Definite macular hyperpigmentation occurred with dyschromia in both subjects. COMMENTS: There is a major risk in subjects who undertake dermopigmentation of freckles and/or naevus, a practice which should be discouraged by dermatologists.