[Juvenile dermatomyositis: A series of 22 cases]. / Dermatomyosite de l'enfant. Série descriptive de 22 cas.

PURPOSE: To report on the characteristics of juvenile dermatomyositis (JDM). PATIENTS AND METHODS: This was a retrospective, descriptive, cross-sectional, non-interventional, multicenter study conducted in Alsace between 2000 and 2015. The patients, aged 0 to 16years, had JDM according to both the Bohan and Peter and the EULAR/ACR criteria. RESULTS: A total of 17 girls and 5 boys were included with a median age at disease onset of 7,8years (Q1-Q3: 4.4-12.9). Median duration of JDM and median patient follow-up were 2.8years and 6.2years, respectively. The most common skin symptoms were papules or Gottron's sign (86 %), nail lesions (82 %), erythema of the face (77 %) and eyelids (59 %), photosensitivity (59 %), and calcinosis (27 %). One patient presented papules with a depressed and porcelain-white center ("Degos-like" lesions). One patient had algodystrophy. Two patients were clinically amyopathic. One girl had intestinal vasculitis. Respiratory function tests were abnormal in 27 % of cases. Median treatment duration was 42 months (Q1-Q3: 19-63). Three patients had a monocyclic form, 12 had a polycyclic form, and 7 had chronic disease. CONCLUSION: The frequency of cutaneous and musculoskeletal signs is comparable to that of other large cohorts of JDM. "Degos-like" lesions and algodystrophy have not yet been described in JDM. This study highlights the type and extent of the dermatological manifestations that frequently constitute the presenting complaint in this disease.

[Fatal interstitial lung disease and pneumocystis during dermatomyositis associated with anti-MDA5 antibodies]. / Dermatomyosite à anticorps anti-MDA-5 compliquée d'une pneumopathie interstitielle et d'une pneumocystose d'évolution fatale.

BACKGROUND: Dermatomyositis (DM) in an auto-immune inflammatory myopathy with skin lesions, and, occasionally, organ involvement. Herein, we report a case of DM during anti-MDA5 antibody therapy associated with interstitial lung disease (ILD) and pneumocystosis. PATIENTS AND METHODS: A 64-year-old woman was hospitalized for impairment of her general health and skin lesions. Dermatological examination revealed classic signs of DM associated with hyperkeratotic papules on the palm creases. This led us to suspect DM with anti-MDA5 antibodies, which was subsequently confirmed by immunologic tests. We also noted dysphonia, exertional dyspnea and proximal muscles weakness. Despite early corticosteroid therapy, combined later with azathioprine, the patient's dyspnoea worsened; one month later, sudden pulmonary decompensation resulted in her admission to intensive care. A chest scan showed evidence of ILD and infectious signs, and the bronchoalveolar lavage was positive for Pneumocystisjiroveci. Despite treatment of this opportunist infection with cotrimoxazole and intensified immunosuppression, the patient died in intensive care. DISCUSSION: Anti-MDA5 antibodies are associated with a specific clinical phenotype and a high degree of risk that should alert the dermatologist to the high likelihood of ILD having a poor prognosis. Associated clinical signs are erythematous, hyperkeratotic or ulcerated papules on the palm creases, as well as fingertip or periungual ulcerations or digital necrosis. This situation is associated with a high risk of pneumocystosis. However, no recommendations concerning prophylaxis are currently available.

Mechanics hands in patients with antisynthetase syndrome: 25 cases.

BACKGROUND: Antisynthetase antibodies (ASA) are directed against aminoacyl-tRNA-synthetases, ubiquitous enzymes of which eight types have hitherto been described. They are seen primarily in antisynthetase syndrome (ASS), in which diffuse interstitial lung disease is associated with inflammatory myopathy, joint involvement and cutaneous signs, in particular mechanic's hands. The aim of this study was to determine the prevalence and semiological characteristics of cutaneous involvement in patients presenting ASA. PATIENTS AND METHODS: We carried out a retrospective study of the medical files of patients with ASA diagnosed at the Strasbourg University Hospital between 1994 and 2009. RESULTS: We identified 22 women and 3 men presenting ASS (n=21), dermatomyositis (n=3) or sclerodermatomyositis (n=1). Mean age at the time of diagnosis was 56 years (12-79). The most commonly seen antibodies were anti-Jo1 (n=19), with the other cases of ASA involving anti-PL12 (n=3), anti-PL7 (n=2) and anti-EJ (n=1) antibodies. Five patients died from pulmonary complications. Mechanic's hands (characteristic plaques and papules along the edge of the first fingers on both hands) were found in 10 patients with ASS (7 cases) or dermatomyositis (3 cases), at the time of diagnosis in 7 cases and during a systemic episode in 3 cases. Muscular involvement was seen in all patients: 9 had diffuse interstitial lung disease and 8 had joint involvement. Cutaneous signs regressed totally or partially in all patients under treatment; in 6 patients, worsening was seen during systemic episodes of the disease. One of the 10 patients died through pulmonary complications. DISCUSSION: Mechanic's hands are a key indicator in cases of ASA and its outcome is intimately linked with underlying systemic involvement, particularly pulmonary. The characteristic semiology enables this disorder to be recognised and allows differentiation from psoriasis or irritant contact dermatitis of the hands, and it does not vary according to antibody. Whether or not the disease is life-threatening is unaffected by the presence of this sign.

[What's new in dermatological treatment?] / Quoi de neuf en thérapeutique dermatologique?

The therapeutic revolution in the management of inflammatory dermatoses is under way. The therapeutic arsenal is expanding in the field of psoriasis, including biologics (TNF blockers, anti-IL12/IL23, anti-IL17, and anti-IL23 antibodies), new small molecules (tyrosine kinase inhibitor), and a new biologic for generalized pustular psoriasis (anti-IL36 receptor). New biologics will be soon available in the field of atopic dermatitis in addition to anti-IL4/IL13 antibodies. New targeted treatments of pruritus are also coming (biologics and small molecules). A first randomized placebo-controlled trial has confirmed the interest of JAK inhibitors in alopecia areata. These molecules seem to be also promising in dermatomyositis. Another therapeutic revolution will be technological with the development of new therapeutic agents: small interfering RNA. Recent clinical trials confirmed their efficacy in hereditary amyloidosis.

[Dermatomyositis associated with anti-MDA5 antibodies and pneumocystis pneumonia: Two lethal cases]. / Dermatomyosite associée aux anticorps anti-MDA5 et pneumocystose pulmonaire : deux cas d'évolution fatale.

BACKGROUND: Dermatomyositis associated with anti-MDA-5 autoantibodies is a recently-described clinical entity. Herein we report two lethal cases involving pneumocystis pneumonia. PATIENTS AND METHODS: Case no 1. A 56-year-old male patient developed cutaneous symptoms consistent with dermatomyositis without muscular involvement. Antinuclear antibodies were present and anti-MDA5 auto-antibodies were identified. The scan showed interstitial lung disease without infection. Significant improvement was obtained with corticosteroids. One month later, the patient presented acute respiratory illness (hypoxemia: PaO2 60mmHg, exacerbation of lung disease evidenced by a scan, and diagnosis of pneumocystis pneumonia on bronchoalveolar lavage). He died despite appropriate antibiotic therapy and immunosuppressant therapy. Case no 2. The second case concerned a 52-year-old Vietnamese man who developed more atypical cutaneous symptoms of dermatomyositis without muscular involvement. ANAb responses were positive (1/400) and MDA5 was present. The patient was treated with corticosteroids (40mg/d), hydroxychloroquine, and intravenous immunoglobulin. After significant improvement, the patient developed an acute respiratory illness due to superinfection with pneumocystis and he died despite specific treatment and cyclophosphamide bolus. CONCLUSION: In dermatomyositis, anti-MDA5 antibody screening is essential for the prognosis since the disease carries a risk of complication with severe lung disease. Bronchial fibroscopy with bronchoalveolar lavage should be considered at the time of diagnosis. Our two cases suggest the need for early screening for pneumocystis pneumonia in the event of respiratory distress and possibly for prophylactic treatment at the start of immunosuppressant therapy.

[Amyopathic dermatomyositis (DM) with anti-MDA5 antibodies, associated with bullous pemphigoid, Sjögren syndrome and gastric MALT lymphoma]. / Dermatomyosite amyopathique avec anticorps anti-MDA-5, associée à une pemphigoïde bulleuse, un syndrome de Sjögren et un lymphome de type MALT.

BACKGROUND: The inflammatory myopathies are a heterogeneous group of muscle diseases and comprise polymyositis, dermatomyositis (DM), myopathies associated with cancers, necrotising myositis and inclusion body myositis. DM occasionally exhibits few or no muscular signs: i.e. hypomyopathic/amyopathic DM. Anti-MDA5 dermatomyositis (DM) is a rare form of dermatomyositis that is frequently amyopathic; the prognosis is linked mainly to pulmonary involvement. PATIENTS AND METHODS: A 69-year-old woman treated for mucosa-associated lymphoid tissue (MALT) gastric lymphoma was referred for a bullous eruption. Based on the investigations performed, a diagnosis was made of bullous pemphigoid. At the same time, amyopathic dermatomyositis was discovered together with interstitial lung disease. Systemic steroids were introduced in combination with rituximab. A favourable outcome was achieved. DISCUSSION: Anti-MDA5 dermatomyositis must be considered systematically in all cases of pulmonary involvement associated with cutaneous signs of dermatomyositis, in which no muscular involvement is generally seen. This condition accounts for up to 7% of DM and carries a severe prognosis due to pulmonary involvement.

[What's new in internal medicine?] / Quoi de neuf en médecine interne ?

As it is practiced in France, internal medicine meets the Anglo-Saxon definition of the specialty, ie doctors "equipped to handle the broad and comprehensive spectrum of illnesses that affect adults, and are recognized as experts in diagnosis, in treatment of chronic illness, and in health promotion and disease prevention - they are not limited to one type of medical problem or organ system". This 2017 "What's new in internal medicine" will consist of 2 parts, a first part on significant publications in the field of systemic and autoimmune diseases and a second part on more diverse publications (HIV, cancer, pregnancy, well-being...) important for medicine in general and its different specialties.

[What's new in internal medecine?] / Quoi de neuf en médecine interne ?

Answering the question « what's new in internal medecine in 2016? ¼ is very challenging. We used 3 methods of article selection to reduce the selection bias: 3 authors, a systematic review of the articles discussed in the weekly bibliographic meeting of our unit (Dermatology department, Saint-Louis Hospital, Paris, France) and a selection of the best articles by several internal medecine practitioners in Paris. Eleven « hot topics ¼ were analyzed: i/lowering cholesterol level but not blood blessure has a significant impact on cardiovascular morbi-mortality in cardiovascular intermediate risk patients; ii/the « treat to treat target ¼ is efficient in psoriatic arthritis; iii/ a genotype/ phenotype correlation favors the separation of ileal Crohn's disease, colonic Crohn's disease and ulcerative colitis; iv/ tocilizumab treatment (anti-IL-6 monoclonal antibody ) is very efficient in giant cell arteritis and slightly efficient in systemic sclerosis; v/ combination therapy using methotrexate plus steroids compared with steroids alone becomes the « gold standard ¼ treatment for juvenile dermatomyositis; vi/ dupilumab treatment (antibody blocking IL-4 and IL-13 receptors) is not only efficient in atopic dermatitis but also in asthma; vii/ think of eosinophilic oesophagitis in a patient with atopic dermatitis and dypshagia or food impaction; viii/ genetic A2 protein dysfunction induces NF-kB hyperactivation and an autoinflammatory disorder with features similar to Behcet's disease; ix/ no new biotherapies have shown high efficacy in systemic lupus erythematosus; x/ nanoparticles loaded with autoantigens induce Tregs and Bregs and may be a promising therapeutic option to treat auto-immune disease in the future; xi/ ipilimumab treatment (anti-CTLA4 antibody, immune checkpoint inhibitor) may induce complete remission in acute myeloid leukemia patients relapsing after haematological stem cell transplantation. Year 2016 is full of great discoveries in internal medicine keeping the dermatologist brain fully open minded.

[Severe edematous dermatomyositis]. / Dermatomyosite œdémateuse sévère.

INTRODUCTION: Edematous dermatomyositis is a rare entity with localized or generalized subcutaneous edema and only 21 cases have been reported in the literature. It is considered to be a severe form of dermatomyositis which needs quick therapeutic decision. We report 2 cases with difficult therapeutic decisions. OBSERVATIONS: Two patients aged 23 and 80 years were admitted in hospital for DM with typical cutaneous and muscular involvement without any sign of gravity and which have been treated by steroids: methylprednisolone bolus and prednisone. They both then developed severe edema of the upper limbs as well as worsening of the cutaneous and muscular symptoms with dysphagia. The addition of mycophenolate mofetil and intravenous immunoglobulin has permitted in the case of the first patient the disappearance of the cutaneous symptoms in particular the edema with restitution of the muscular force and withdrawal of the dysphagia and swallowing symptoms. The therapeutic failure for the second patient was due to a refusal of the treatment and a probable paraneoplastic context. CONCLUSION: Subcutaneous edema localized or generalized must not be confused with periorbital erythematous edema, classically observed in DM, nor with DM with mucinosis. Potential marker of gravity, it is often associated to important muscular weakness and dysphagia. In this case, an aggressive treatment associating corticosteroids, immunosuppressive therapy and intravenous immunoglobulin is necessary.

[Extensive digital necrosis during dermatomyositis associated with MDA-5 antibodies]. / Nécroses digitales étendues au cours d'une dermatomyosite avec anticorps anti-MDA-5.

BACKGROUND: Dermatomyositis (DM) is an inflammatory disease associated with auto-antibodies in 50 to 70% of cases. A new antibody, anti MDA-5, has been described in association with a specific type of DM involving severe interstitial lung disease and minimal muscle disease. We report the first case of DM with MDA-5 antibodies and with interstitial lung disease and rapidly extensive digital necrosis. PATIENTS AND METHODS: A 28-year-old male was hospitalized for asthenia, myalgia and subacute dyspnea. Examination demonstrated skin lesions with edema on every digit associated with purpuric and cyanotic lesions, as well as erythematous papules on the helix and the elbows, and Gottron's papules. Systemic corticosteroid therapy was initiated. The immunoprecipitation results indicated the presence of anti-MDA-5 antibodies. Despite corticosteroid therapy, the patient's respiratory status gradually deteriorated towards pulmonary fibrosis and rapidly extensive necrosis appeared on all fingers and toes. Theses effects were resistant to cyclophosphamide and immunoglobulin but were stabilized by cyclosporine. DISCUSSION: Anti-MDA-5 antibodies are specific to DM and constitute a risk factor for severe interstitial lung disease (70% of cases) with a higher risk of mortality (40%). The cutaneous presentation of this DM is specific with palmar papules and mucocutaneous ulceration. Rapidly extensive digital necrosis has not been previously reported. No treatment has demonstrated superiority. CONCLUSION: We report the first case of DM with anti-MDA-5 antibodies involving interstitial lung disease and massive digital necrosis. Because of the pulmonary risk, in the presence of clinical lesions containing anti-MDA-5 DM, screening for these antibodies should be carried out.

[What's new in internal medicine?]. / Quoi de neuf en Médecine interne?

This article focuses on current trends in various autoimmune diseases of interest for the dermatologist. In the antiphospholipid syndrome, many news: better characterization of the severe disease, involvement of the mTOR pathway in the vasculopathy-induced renal disease, and diversification of the therapeutic approaches: use of mTOR inhibitors and several biologics, new various antiplatelet and anticoagulants. In dermatomyositis, new autoantibodies are better characterized with a good correlation with clinical disease; the results of a large study on genetic predisposition to the disease are available. There are also some therapeutic innovations in systemic sclerosis: benefit of rituximab that seems well tolerated, the results of a large controlled European study about aggressive immunoablative chemotherapy followed by autologous stem cells have just been published, intralesional stem cells injections in the fingers of sclerodactylic patients. Finally, news in celiac disease that is constantly increasing and whose mild forms often have cutaneous manifestations, leading to diagnosis.

[Prevalence of cancer in the Afro-Caribbean population presenting dermatomyositis and anti-synthetase syndrome: a preliminary study conducted at Pointe-à-Pitre University Hospital, 2000-2012]. / Prévalence des cancers dans la population afro-caribéenne atteinte de dermatomyosite et de syndrome des anti-synthétases : étude préliminaire au CHU de Pointe-à-Pitre, 2000-2012.

BACKGROUND: An association with cancer is described in 17-32% of cases of dermatomyositis (DM) and in 5-16% of cases of anti-synthetase syndrome (ASS). The literature contains very few studies involving Afro-Caribbean patients with DM or ASS. The aim of our retrospective study was to determine the prevalence of cancer in a series of patients with DM or ASS at the University Hospital of Pointe-à-Pitre between 1st January 2000 and 31st December 2012. The secondary objective was to review the clinical and laboratory features as well as the course of DM/ASS in these patients. PATIENTS AND METHODS: The inclusion criteria were as follows: Afro-Caribbean origin; age >15 years; patient living in Guadeloupe; screening for malignancy. RESULTS: Twenty-two patients were included (15 DM, 7 ASS). Only one case of cancer was diagnosed in the entire study population at a mean follow-up of 6 ± 4 years (prevalence: 6.7%, CI95% [1.7-31.9]). Of the 15 patients presenting DM (sex ratio F/M: 4, mean age: 45 ± 14 years), 6 (40%) had associated connective tissue disease. CONCLUSION: Our study suggests a weak association between DM and cancer in Afro-Caribbean patients. These results may be explained by the features of the disease seen in these patients (female gender, young age at onset, associated connective tissue disease) and the low prevalence in the Caribbean region of cancers typically associated with DM.

[Dermatomyositis and acute interstitial lung disease associated with MDA-5 antibodies: an atypical case]. / Dermatomyosite et pneumopathie interstitielle rapidement évolutive associées aux auto-anticorps anti-MDA-5 : une présentation clinique atypique.

BACKGROUND: A new antibody known as anti-MDA-5 has recently been identified in association with a specific phenotype of dermatomyositis (DM), in which muscular involvement is minimal or absent, but where there is a particularly high risk of onset of aggressive interstitial lung disease. Below, we report a case associated with the presence of anti-MDA-5 antibodies complicated by aggressive interstitial lung disease ; the cutaneous phenotype of this patient is novel due to the constitution of diffuse ichthyosis and of profuse subcutaneous calcinosis. PATIENTS AND METHODS: A 35-year-old man was hospitalised for a skin rash associated with arthralgia, dry cough, asthenia and weight-loss of 8 kg in 5 months. The dermatological examination revealed purple erythema of the upper eyelids and cheeks, diffuse acquired ichthyosis of the trunk and limbs, and striated erythema on the proximal and distal metacarpal and interphalangeal joints of the fingers. A diagnosis of DM was suspected in patients treated with prednisolone, 1.5 mg/kg/d. Immunological tests identified the presence of anti-MDA-5 antibodies. One month later, the cutaneous signs continued to progress and were marked by the development of painful ulcers on the back of the fingers, as well as dyspnoea accompanying the slightest effort. Chest images showed constituted pulmonary fibrosis lesions. At the same time, within several months, diffuse, pseudo-tumoural calcifications developed in the subcutaneous tissue of the trunk and limbs. The patient was treated successively with intravenous gammaglobulins, cyclophosphamide, mycophenolate mofetil, azathioprine and rituximab in combination with oral corticosteroids, but with no improvement in respiratory function or in the skin lesions. Because of the decline in the patient's respiratory status, a lung graft was envisaged but subsequently abandoned because of the patient's excessively precarious state of health. DISCUSSION: Anti-MDA-5 antibody appears to constitute a specific immunological marker for a special dermatological phenotype of adult DM, significantly associated with the presence of: 1) keratotic papules in the skin folds of the palms and fingers ; 2) cutaneous ulcers along the periungueal edges, Gottron's papules on the back of the hands and on the extensor surface of the elbows and knees ; 3) pain and ulceration in the oral mucosa, diffuse alopecia, and appearance of "mechanic's hands" and Gottron's sign on the elbows and knees. To our knowledge, the presence of extensive ichthyosis and profuse subcutaneous calcinosis has not been previously reported in this particular form of DM. The risk of aggressive interstitial lung disease is particularly high and worsens the prognosis, since 40% of patients with anti-MDA-5 die, usually within the first year. CONCLUSION: Herein, we describe a patient presenting amyopathic DM complicated by aggressive interstitial lung disease associated with the presence of anti-MDA-5 antibodies, but with a hitherto unreported cutaneous phenotype combining diffuse ichthyosis and profuse subcutaneous calcinosis.

[Myositis: From classification to diagnosis]. / Myosites : de la classification au diagnostic.

Idiopathic inflammatory myopathies, or IIM, are a group of acquired diseases that affect the muscle to a certain extent, and may also affect other organs. They include dermatomyositis, which can affect the muscle eventualy, with a typical skin rash; inclusion body myositis, with a purely muscular expression resulting in a slow progressive deficit; and the former group of "polymyositis", a misnomer that actually includes other categories of IIM, such as immune-mediated necrotizing myopathies, with a severe muscle involvement often presents from the onset of the disease; antisynthetase syndrome, which combines muscle damage, joint involvement and a potentially life-threatening lung disease; and overlapping myositis, which combines muscle damage with other organs involvement connected to another autoimmune disease. The diagnosis of IIM is based on rigorous clinical examination and interrogation, electromyographic data and immunological testing for myositis specific antibodies. This antibody dosage must be extended or repeated if necessary to classify correctly the muscle disease under investigation, as the available tests may not perform well enough. Muscle biopsy, although very informative, is not anymore systematically recommended when the clinic and the antibodies are typical. However, some forms of IIM are sometimes difficult to classify; in these cases, muscle biopsy plays a crucial role in the precise etiological diagnosis.

[Severe MDA5 dermatomyositis associated with cancer and controlled by JAK inhibitor]. / Dermatomyosite à anticorps anti-MDA5 sévère associée à un cancer et contrôlée par inhibiteur de JAK.

Dermatomyositis is an idiopathic inflammatory myopathy with various clinical and serological profiles, including poor prognosis forms for which aggressive immunosuppressive treatment is warranted. We report the case of a 60-year-old woman referred to our hospital for an anti-melanoma differentiation-associated 5 gene antibody-positive dermatomyositis (MDA5 DM) with rapidly progressive interstitial pneumonia, typical cutaneous features and muscular impairment. Treatment with high-dose methylprednisolone, cyclophosphamide and gamma globulin was performed, but the patient remained corticodependant. Blood detection of positive interferon signature justified the administration of an anti-JAK1/2, leading to the clinical remission and the regression of the interferon signature. After 12 months of follow up, a small cell carcinoma was discovered, raising the question of a paraneoplastic syndrome, for which the most recent datas are quite reassuring for this kind of MDA5 DM. The presentation of this case is of twofold interest: describing one of the first report of successful treatment of intereronopathy MDA5 DM with ruxolitinib and highlighting an association with a cancer, which is not expected for this phenotype of dermatomyositis.

[Retrospective analysis of anti-TIF1gamma, anti-NXP2 and anti-SAE1/2 antibodies carriers at Bordeaux university hospital from November 2014 to February 2017]. / Analyse rétrospective des patients porteurs d'anticorps anti-TIF1gamma, anti-NXP2 et anti-SAE1/2 au CHU de Bordeaux de novembre 2014 à février 2017.

INTRODUCTION: Dermatomyositis are rare autoimmune diseases. The discovery of specific antibodies such as the anti-TIF1γ, anti-SAE1/2 and anti-NXP2 antibodies has been associated with specific clinical phenotypes. The recent development of standardized kits based on immunodot method is a progress in dermatomyositis diagnosis. Here, we report the clinical characteristics of patients carrying these antibodies with or without clinical setting of dermatomyositis. METHODS: This single-center french retrospective study was conducted from November 2014 to February 2017 at Bordeaux university hospital. Patients carrying anti-TIF1γ, anti-SAE1/2 and anti-NXP2 antibodies, detected by immunodot, were included. RESULTS: Among the 58 patients included, only 10 were finally diagnosed with dermatomyositis. Some form of cancer was found in all anti-TIF1γ antibodies positive patients associated with dermatomyositis. Among the 48 anti-TIF1γ, anti-SAE1/2 and anti-NXP2 antibodies positive patients without clinical phenotype of dermatomyositis, 30 had autoimmune or inflammatory condition and 39 patients presented a significant biological autoimmunity. None of them developed dermatomyositis during the follow-up. CONCLUSION: The immunodot kit allowed the diagnosis of 10 dermatomyositis. A high number of autoantibody positive patients without dermatomyositis raises the issue of the immunodot's performances in the context of biological autoimmunity.

[Spontaneous pneumomediastinum: A rare complication of dermatomyositis]. / Pneumomédiastin spontané : une complication rare de la dermatomyosite.

Dermatomyositis is a rare connective tissue disease of unknown origin, including inflammatory myopathy and cutaneous manifestations. Several pulmonary complications associated to dermatomyositis were described; especially interstitial lung disease. Some rare and particular pulmonary complications were reported in the literature such as pneumodiastinum and pneumothorax. We are describing here, a case report about a female patient, who presented with dermatomyositis associated to pneumomediastinum as a severe and lethal complication without pneumothorax. It is a novel observation depicting this severe and rare complication. Brutal dyspnea and cervical subcutaneous crackling are alarming signs that should make practitioners think about this complication.

[Panniculitis in patient undergoing treatment for dermatomyositis with methotrexate]. / Panniculite au cours d'un traitement d'une dermatomyosite par du méthotrexate.

Panniculitis is a rare cutaneous manifestation of dermatomyositis (DM). The appearance of panniculitis during treatment with methotrexate (MTX) is exceptional and has only been described in 3 cases. We report a case of a 50-year-old woman suffering from DM since 1997 who was treated with corticosteroids showing favorable clinical and biological evolution. When a relapse occurred 2 years later, she was treated with higher-dose of corticosteroids in combination with a 7,5 mg weekly dose of methotrexate. The evolution was rapidly favorable. Eighteen months later, the patient had multiple subcutaneous nodules on limbs and buttocks. Anatomopathological examination showed panniculitis. There was no evidence supporting progression in DM. Prednisone dose was increased to 0.5 mg/kg/day, always in combination with MTX, without any clear signs of improvement. MTX treatment was stopped and the cutaneous lesions completely disappeared in 2 months without any relapse. This objective response lasted for 42 months. Our observation is particular given the occurrence of panniculitis in a patient undergoing treatment for dermatomyositis with methotrexate and illustrates the difficulties in the diagnosis. This entity must be known despite its exceptional nature since cutting off MTX treatment generally induces the disappearance of subcutaneous nodules.