Variant Of Dermatomyositis Research Articles

First report of an adult patient with Clinical amyopathic dermatomyositis associated rapid progressive interstitial lung disease triggered by COVID19

ILD is a common manifestation of dermatomyositis (DM) with indolent course but a unique variant of DM called clinically amyopathic DM (CADM) presents with rapid progressive interstitial lung disease (RP-ILD) and is called CADM associated RP-ILD. Here, we report a case of a 52 year old woman with CADM associated RP-ILD occurred 2 weeks after recovery from COVID19 infection but unfortunately died from RP-ILD, almost 5 months later. This is the first report of CADM associated RP-ILD triggered by COVID19 in an adult patient

P225 A case of PL-7 positive amyopathic dermatomyositis associated with autoimmune liver disease

Abstract Background/Aims Amyopathic dermatomyositis (ADM) is a variant of dermatomyositis (DM) characterized by the presence of pathognomonic skin features of dermatomyositis but without clinical muscle weakness. ADM is believed to account for about 20% of cases of DM. Methods We present the case of a 40-year-old lady who presented in May 2020 with a 6-week history of periorbital oedema and a heliotrope rash. She had a past medical history of rectosigmoiditis diagnosed on colonoscopy in 2018 managed with mesalazine 2g once a day. Blood tests revealed a transaminitis (ALT 372 u/l, AST 201 u/l). Full blood count was normal as were inflammatory markers (ESR 4 mm/hr, CRP 2 mg/dl) and renal function (creatinine 63.9 umol/l). CK was normal (72 u/l). A non-invasive liver screen including hepatitis B and C, caeruloplasmin, alpha-fetoprotein and anti-mitochondrial antibodies was negative. A liver ultrasound was reported as normal. An ANA was positive and an ENA screen revealed positive RNP-68, S/RNP and anti-smooth muscle antibodies. Atypical pANCA antibodies were also positive. A myositis antibody screen revealed a positive PL-7 antibody. A skin biopsy of the affected area around the eye demonstrated prominent dermal periadnexal and perivascular chronic inflammation composed mainly of lymphocytes and plasma cells consistent with a connective tissue disease. A liver biopsy confirmed autoimmune liver disease (AILD). Tumour markers (CEA, CA 125 and CA 19-9) were normal and a CT scan of the chest, abdomen and pelvis to rule out occult malignancy was normal. Full lung function tests including transfer factor were normal. A diagnosis of PL-7 positive ADM with associated AILD was made. Results The patient’s ADM and AILD were successfully treated with a reducing course of budesonide (6mg three times daily for 4 months reduced to 3mg twice daily for four months then 3mg daily for 3 months) and mycophenolate mofetil 720mg twice daily. Currently, 16 months after her initial presentation, the patient’s ADM and AILD are in remission and she remains on mycophenolate mofetil 720mg twice daily. Conclusion Anti-PL-7 is one of a number of anti-aminoacyl tRNA synthetase (ARS) antibodies that are associated with DM, polymyositis (PM) and ADM. Other anti-ARS antibodies include Jo-1, PL-12, EJ, OJ, KS, ZO and YRS/HA. Antibodies to PL-7 have been reported to be associated with milder muscle involvement than antibodies to Jo-1. Antibodies to PL-7 have been associated with severe interstitial lung disease (ILD). An association between AILD and inflammatory myositis has been reported in the literature but only in a handful of case reports. To the best of our knowledge however, AILD has not previously been reported in PL-7 positive DM, PM or indeed ADM. Disclosure M.A. Lee: None. V. Scott-Lang: None. K. Raja: None. S. Sahu: None.

Spinulosis revealing dermatomyositis relapse: A new description of Wong’s dermatomyositis

Sir, Dermatomyositis (DM) is a systemic autoimmune disease affecting characteristically the skin and muscles. The typical cutaneous features include Gottron’s papules, a heliotrope rash, and edema of the eyelids. Spinulosis is an unusual sign, not raising suspicion for a DM diagnosis. Herein, we report a case of DM relapsing as Wong’s dermatomyositis (WDM) as an isolated spinulosic eruption. A 42-year-old female presented with a pruritic papular eruption involving both arms and the back persistent for the last two months. An anamnesis included a history of DM diagnosed five years earlier, in complete remission under the administration of corticosteroids for three years. A physical examination revealed erythematous and violaceous scaly plaques resulting from confluent follicular papules, resembling pityriasis rubra pilaris (PRP) on the lateral sides of the arms (Fig. 1) and the upper back (Fig. 2). Muscular testing was subnormal and the general status was conserved. Laboratory findings revealed a high level of blood CPK (3×N), LDH (4×N), and ASAT (3.5×N), while electromyography displayed myogenic changes. A muscular biopsy revealed necrotic muscular cells with perifascicular atrophy and lymphocytic inflammatory infiltrate. A histological skin examination found follicular hyperkeratosis with perifollicular interface dermatitis and interstitial mucin deposits in the dermis (Fig. 3). Based on these findings, the diagnosis of WDM was retained. A malignancy workup was negative. Corticosteroid therapy was initiated with prednisone 1 mg/kg/day in combination with hydroxychloroquine 200 mg twice a day, resulting in a remarkable clinical and biological improvement.WDM is characterized by the presence of PRP-like follicular papules in a patient with dermatomyositis (DM) [1]. Some authors suggest that this exceptional presentation should be considered a true association of DM with PRP rather than a variant of DM [2]. In this rare form, spinulosic lesions are mainly located on the trunk or limbs with occasional palmoplantar keratoderma [2]. Follicular lesions are usually associated with the typical signs of DM but some cases of isolated PRP features related to WDM have been reported [3]. To our knowledge, none has been reported as a relapse of a previous classic DM. Our patient relapsed showing spinulosic lesions absent in the prior episode five years earlier, not displaying at this time any classic cutaneous signs of DM. Such puzzling presentations are very unusual and may be confusing for physicians. In our patient, the diagnosis was retained on the basis of the histopathological findings of interface dermatitis and interstitial mucin deposits in the dermis, increased serum levels of muscle enzymes with a myogenic electromyographic pattern, the typical muscular histological changes, and a medical history of DM diagnosed five years earlier. In WDM, histology usually reveals follicular hyperkeratosis with keratotic plugs filling dilated follicular infundibula, which may raise several differential diagnoses, including PRP, discoid lupus erythematosus (DLE), and, to a lesser degree, follicular psoriasis and keratosis pilaris [4]. Interface dermatitis and mucin deposits, as in our patient, are the hallmarks of distinguishing DM from PRP, follicular psoriasis, and keratosis pilaris. Although these findings may also be observed in DLE [4], in our patient, there were no clinical or histological arguments supporting DLE rather than a diagnosis of DM. Myositis as, well as interstitial lung disease, appears to be comparable to the classic variant [5]. The risk of associated internal malignancy is unknown but could be lower than in the classic form [4]. Treatment and management do not differ from classic dermatomyositis.

FULMINANT ORGANIZING PNEUMONIA SECONDARY TO MDA5-ASSOCIATED AMYOPATHIC DERMATOMYOSITIS: A CASE REPORT AND REVIEW OF THE LITERATURE

SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Fulminant Organizing Pneumonia (OP) is a rare, clinicopathologic diagnosis considered to have good prognosis with early recognition and steroid therapy. There is a paucity of literature on rapidly progressive presentations [1,3,5,6]. We present the case of a 44-year-old woman who presented with fevers and arthralgias found to have Anti-MDA5 amyopathic dermatomyositis who developed fulminant organizing pneumonia and hypoxemic respiratory failure requiring extracorporeal membrane oxygenation (ECMO). CASE PRESENTATION: The patient was a 44-year-old female without significant past medical history who presented with one month of worsening rash, recurring high fevers, hoarseness, dysphagia, and arthralgias. The rash started on the dorsum of the hands, the distal interphalangeal joints (DIP) and proximal interphalangeal joints (PIP). Within five days, the patient had escalating oxygen requirements requiring mechanical ventilation. Fiberoptic bronchoscopy revealed bilateral vocal cord paralysis and hyperemic airways. Cultures from lavage revealed pan-sensitive klebsiella and patient was started on the appropriate antimicrobial therapy. Computed Tomography Angiography (CTA) at this time was negative for pulmonary embolism (PE) but demonstrated bibasilar airspace disease with small areas of cavitation. A high risk video assisted thoracoscopic lung biopsy was performed and pulse steroids were started pending the results. Lung biopsy demonstrated hyaline membranes and organizing pneumonia. Despite treatment, she did not improve, developed worsening hypoxemia, which required transfer for ECMO. Patient was found to have Anti-MDA5 Antibodies in the serum and was diagnosed with amyopathic dermatomyositis. Despite maximal medical therapy, the patient passed away. DISCUSSION: This is the fourth case report to describe a fulminant onset OP in a patient with MDA5-associated amyopathic dermatomyositis [7,10]. Rapidly progressive OP leading to acute respiratory failure and ARDS has been previously described [5]. This variant of dermatomyositis is not even assayed on certain laboratory “dermatomyositis panels” which can hinder diagnostic clarity. There is controversy with regards to the appropriate timing and choice treatment of MDA5 associated dermatomyositis, but recent literature suggests early administration of Rituximab [12]. This introduces a clinical quandary. These patients require urgent diagnoses and prompt treatment to prevent progression of lung injury, but are often too unstable for lung biopsy or require tests which take several days to return. Rapidly progressive OP secondary to this rare variant of amyopathic dermatomyositis is exceedingly rare, with only a few reported cases [7,10]. CONCLUSIONS: In the setting of acute respiratory failure and a diagnosis of organizing pneumonia the use of early immunologic agents should be considered, even without a definitive immunologic/rheumatologic diagnosis. Reference #1: Kobayashi T, Kitaichi M, Tachibana K, et al. A Cryptogenic Case of Fulminant Fibrosing Organizing Pneumonia. Intern Med. 2017;56(10):1185-1191. Reference #2: Wells, A. U. (2001). Cryptogenic Organizing Pneumonia. Seminars in Respiratory and Critical Care Medicine, 22(04), 449–460. Reference #3: Cohen AJ, King TE, Downey GP. Rapidly progressive bronchiolitis obliterans with organizing pneumonia. Am J Respir Crit Care Med. 1994;149(6):1670-5. DISCLOSURES: No relevant relationships by Bhavna Abbi, source=Web Response Advisory Committee Member relationship with Ambu Please note: $1-$1000 Added 03/29/2019 by Olumayowa Abe, source=Web Response, value=Honoraria No relevant relationships by Christian Castaneda, source=Web Response No relevant relationships by Jerry Centeno, source=Web Response No relevant relationships by Erik Perez, source=Web Response No relevant relationships by John Prudenti, source=Web Response

Poikilodermatomyositis with calcinosis cutis.

Among the known variants of dermatomyositis there is a form in which the cutaneous and muscular alterations are accompanied by calcium deposits. One such case was recently observed by us, and the patient was treated with corticotropin (adrenocorticotropic hormone), which brought on temporary improvement of symptoms. REPORT OF A CASE A 17-year-old white Brazilian-born girl was first seen on April 2, 1951, complaining of generalized dermatitis and muscular and articular pains. Four years previously she had noticed a generalized patchy pigment disorder and a nonitching facial rash, which had persisted, with partial remissions. In October, 1947, she noticed muscular and articular pains, with redness and swelling of the larger joints. During the preceding two years painful periarticular nodules had appeared, which subsequently began discharging a chalky substance. Since May, 1949, knee movements had become reduced, and on admission she was unable to walk. Physical examination revealed an emaciated young woman