Periorbital Rash Research Articles

Comparison of clinical features between patients with anti-synthetase syndrome and dermatomyositis: results from the MYONET registry.

To compare clinical characteristics, including the frequency of cutaneous, extramuscular manifestations and malignancy, between adults with anti-synthetase syndrome (ASyS) and DM. Using data regarding adults from the MYONET registry, a cohort of DM patients with anti-Mi2/-TIF1γ/-NXP2/-SAE/-MDA5 autoantibodies, and a cohort of ASyS patients with anti-tRNA synthetase autoantibodies (anti-Jo1/-PL7/-PL12/-OJ/-EJ/-Zo/-KS) were identified. Patients with DM sine dermatitis or with discordant dual autoantibody specificities were excluded. Sub-cohorts of patients with ASyS with or without skin involvement were defined based on presence of DM-type rashes (heliotrope rash, Gottron's papules/sign, violaceous rash, shawl sign, V-sign, erythroderma, and/or periorbital rash). In total 1054 patients were included (DM, n = 405; ASyS, n = 649). In the ASyS cohort, 31% (n = 203) had DM-type skin involvement (ASyS-DMskin). A higher frequency of extramuscular manifestations, including Mechanic's hands, Raynaud's phenomenon, arthritis, interstitial lung disease and cardiac involvement differentiated ASyS-DMskin from DM (all P < 0.001), whereas higher frequency of any of four DM-type rashes-heliotrope rash (n = 248, 61% vs n = 90, 44%), violaceous rash (n = 166, 41% vs n = 57, 9%), V-sign (n = 124, 31% vs n = 28, 4%), and shawl sign (n = 133, 33% vs n = 18, 3%)-differentiated DM from ASyS-DMskin (all P < 0.005). Cancer-associated myositis (CAM) was more frequent in DM (n = 67, 17%) compared with ASyS (n = 21, 3%) and ASyS-DMskin (n = 7, 3%) cohorts (both P < 0.001). DM-type rashes are frequent in patients with ASyS; however, distinct clinical manifestations differentiate these patients from classical DM. Skin involvement in ASyS does not necessitate increased malignancy surveillance. These findings will inform future ASyS classification criteria and patient management.

Ophthalmic manifestations of monkeypox infection

After the global COVID-19 pandemic, there has been an alarming concern with the monkeypox (mpox) outbreak, which has affected more than 110 countries worldwide. Monkeypox virus is a doublestranded DNA virus of the genus Orthopox of the Poxviridae family, which causes this zoonotic disease. Recently, the mpox outbreak was declared by the World Health Organization (WHO) as a public health emergency of international concern (PHEIC). Monkeypox patients can present with ophthalmic manifestation and ophthalmologists have a role to play in managing this rare entity. Apart from causing systemic involvement such as skin lesions, respiratory infection and involvement of body fluids, Monkeypox related ophthalmic disease (MPXROD) causes varied ocular manifestations such as lid and adnexal involvement, periorbital and lid lesion, periorbital rash, conjunctivitis, blepharocounctivitis and keratitis. A detailed literature review shows few reports on MPXROD infections with limited overview on management strategies. The current review article is aimed to provide the ophthalmologist with an overview of the disease with a spotlight on ophthalmic features. We briefly discuss the morphology of the MPX, various modes of transmission, an infectious pathway of the virus, and the host immune response. A brief overview of the systemic manifestations and complications has also been elucidated. We especially highlight the detailed ophthalmic manifestations of mpox, their management, and prevention of vision threatening sequelae.

Periorbital petechiae after emesis in a young woman.

A 27-year-old woman presented to the dermatology clinic with a 1-day history of sudden-onset, painless, nonpruritic, periorbital rash. The patient had several episodes of intense vomiting related to alcohol intake 1 day before onset of the rash. She was previously healthy and had not taken

P222 Clinical features of extra-muscular disease in dermatomyositis and anti-synthetase syndrome patients with skin involvement classified by presence of disease-specific autoantibodies: results from the EuroMyositis registry

Abstract Background/Aims Anti-synthetase syndrome (ASS) represents a distinct entity within myositis spectrum disorders; however, correct classification of patients with anti-tRNA synthetase autoantibodies and skin manifestations akin to dermatomyositis (DM) remains uncertain. Our aim was to compare clinical characteristics, skin involvement, and malignancy, between patients with ASS and DM, classified by disease-specific autoantibodies. Methods Data from 05/2009-03/2016 from 9 countries from the prospective, myositis EuroMyositis registry were downloaded. Those with anti-tRNA synthetase autoantibodies (Jo-1/PL-7/PL-12/OJ/EJ/KS) were classified as ASS, and those with Mi-2/TIF1-γ/NXP2/SAE/MDA5 autoantibodies as DM. Clinical phenotypes including malignancies (except skin malignancies) were tabulated. Characteristics of patients with skin involvement (excluding mechanic’s hands and Raynaud’s phenomenon) were compared using Fisher’s exact test with Bonferroni corrected p-values. Results Of 3,067 patients, 2,028 had autoantibody profiling results (66.1%), of which 783 (38.6%) were positive for at least one of the autoantibodies being considered. Five patients with dual autoantibody specificities were excluded. Of the remaining 778, 320 (41.1%) were classified as DM, and 458 (58.9%) as ASS. Median age at diagnosis was 48.2 years (interquartile range [IQR] 37.5 to 57.8) in the DM cohort and 49 (IQR 38.3 to 62.2) in the ASS cohort. Skin involvement was present in 277 (86.6%) DM patients (DM skin) vs 204 (44.5%) ASS patients (ASS skin) (pcorr&amp;lt;0.01) (Table 1). Whilst relatively high proportions of so-called “DM-specific” rashes were seen in ASS skin, the frequency of heliotrope rash, Gottron’s papules, violaceous rash, periorbital rash, V-sign, shawl sign, and periungual erythema was significantly higher in DM skin (pcorr&amp;lt;0.01 for all). Conversely, mechanic’s hands, Raynaud’s, arthritis, and interstitial lung disease were more frequent in ASS skin (pcorr&amp;lt;0.01 for all). Malignancy was less frequent in ASS skin vs DM skin (pcorr&amp;lt;0.01) and occurred temporally closer to myositis diagnosis in DM skin (median 0.95 months [IQR -6.54. to 19.45]) vs ASS skin (median 12.17 months [IQR -15.85 to 79.31]). Conclusion Patients with ASS have frequent skin involvement but also a distinct clinical phenotype compared to DM. These findings may inform the development of future classification criteria for ASS. Disclosure R.M. Hum: None. J.B. Lilleker: None. J.A. Lamb: None. W.E. Ollier: None. G. Wang: None. L.R. Wedderburn: None. L.P. Diederichsen: None. J. Schmidt: None. P. Oakley: None. O. Benveniste: None. M.G. Danieli: None. K. Danko: None. N.T.P. Thuy: None. M.V.D. Mercado: None. H. Andersson: None. B.D. Paepe: None. J.L.D. Bleecker: None. B. Maurer: None. L.J. McCann: None. N. Pipitone: None. N. McHugh: None. P. New: None. J. Vencovsky: None. I.E. Lundberg: None. H. Chinoy: None.

Periorbital rash and scaly plaques in a 13-year–old boy

Periorbital rash and scaly plaques in a 13-year–old boy

Telehealth Outpatient Monitoring of a SARS-CoV-2 Familial Cluster Infection in Peru: Adapting to a Healthcare Crisis

The coronavirus disease 2019 (COVID-19) epidemic is evolving in Latin America despite implementation of government measures. We report a familial cluster in Lima, Peru, with confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. Two young and two middle-aged adults with a wide range of COVID-19 manifestations experienced successful management under telehealth outpatient monitoring. Telehealth monitoring was scheduled as suggested by Peruvian Ministry of Health Guidelines and was performed by a designated physician who assessed the patients and prescribed treatment. On May 14, 2020, a 25-year-old male, who worked treating COVID-19 patients, reported constitutional symptoms and tested positive for SARS-CoV-2. Clinical improvement was achieved with azithromycin and ivermectin therapy. He had been in contact with his parents (Cases 2 and 3) and his sister (Case 4). Cases 2 and 3 developed moderate pulmonary compromise requiring oxygen supplementation and pharmacological therapy, including corticosteroids and anticoagulation, under home medical assessment and telehealth monitoring. Case 4 developed mild symptoms and periorbital rash, an atypical dermatological finding. To our knowledge this represents the first report of a familial cluster with COVID-19 that was successfully managed under scheduled telehealth outpatient monitoring in Latin America.

Clinical signs and symptoms in a joint model of four disease activity parameters in juvenile dermatomyositis: a prospective, longitudinal, multicenter cohort study

BackgroundIt is currently impossible to predict the prognosis of patients with juvenile dermatomyositis (JDM). The aim of this study was to find clinical features most strongly associated with outcome variables in JDM as a first step towards tailor-made treatment.MethodsIn a large, prospectively followed, multicenter cohort study of 340 patients with JDM, each contributing multiple visits, a Bayesian model of disease activity was developed, using the four continuous outcome variables creatine kinase (CK), childhood myositis assessment score (CMAS), manual muscle testing of 8 muscle groups (MMT8) and the physician’s global assessment of disease activity (PGA). Covariates were clinical signs and symptoms. Correlations among visits of the same patient were resolved by introducing subject-specific random effects.ResultsMyalgia and dysphonia were associated with worse disease activity according to all outcome variables. Periorbital rash, rash on the trunk, rash over large joints, nail fold changes and facial swelling were associated with higher PGA. Notably, periorbital rash was also associated with higher CK and lower CMAS and nail fold changes with lower CMAS. Contractures were associated with lower CMAS and MMT8 and higher PGA. Patients with higher CMAS exhibited a higher MMT8 as well. PGA had the highest probability among the four outcome variables of being abnormal even if the other three outcome variables were normal.ConclusionsThe signs and symptoms associated with disease activity could be used to stratify patients and adapt treatment plans to disease activity. The correlation between CMAS and MMT8 and the unique information captured by PGA implied that PGA should be maintained as an outcome variable, whereas CMAS and MMT8 might be simplified.

A rash with a heavy heart

Cardiac amyloidosis (CA) is relatively rare and frequently misdiagnosed. Other disorders presenting with increased left ventricular (LV) mass can mimic its diagnosis. This case illustrates unique findings of primary light chain (AL) amyloidosis in a patient with remarkable signs of CA. Here, we report a 49-year-old male with prior diagnosis of hypertrophic cardiomyopathy (HCM) based on an echocardiogram performed 1 year earlier, which presented with 8 weeks of periorbital rash. The patient had numbness in the past 3 years. More recently, the patient presented with shortness of breath. Physical examination was remarkable for periorbital purpura, macroglossia and orthostatic hypotension. Cardiac auscultation showed S3 and S4. Electrocardiography showed diffuse low-voltage QRS complexes. Echocardiography revealed severe diastolic impairment; granular ‘sparkling’ pattern of the myocardium with thickened walls, interatrial septum and valves; and pericardial effusion. Diastolic dysfunction and thick walls with low ECG voltage are compelling diagnostic findings. Laboratory workup showed increased free light chain-differential (FLC-diff), N-terminal fragment of brain natriuretic peptide (NT-BNP) and cardiac Troponin T (cTnT). Bone marrow biopsy confirmed AL amyloidosis. A diagnosis of AL amyloidosis with cardiac involvement mimicking HCM was made. The patient died during hospitalization due to sudden cardiac death. This case illustrates the importance of the combination of clinical, serological, and electro- and echocardiographic findings to establish the diagnosis of CA.

Postictal red eye

A 19 year male presented with red eyes and periorbital rash after an episode of generalised tonic clonic seizure. Capillary leak secondary to Valsalva Manuever or vasoactive substance release are the suggested mechanisms.

A Case of Dyspnea and Periorbital Rash

A 75-year-old woman presented with dyspnea on exertion, orthopnea, paroxysmal nocturnal dyspnea, and edema. Macroglossia, generalized petechiae, raccoon eyes sign, and peripheral neuropathy were present, and an echocardiogram showed cardiac dilatation and left ventricular systolic dysfunction. Rectal biopsy and immune fixation electrophoresis confirmed the diagnosis of AL amyloidosis.

A Novel Dermato-Pulmonary Syndrome Associated With MDA-5 Antibodies

Melanoma differentiation-associated protein 5 (MDA-5) is a novel autoantibody frequently characterized by interstitial lung disease and a distinct cutaneous phenotype with palmar papules, ulceration, and rash. Virtually all patients have underlying dermatomyositis, but many lack the characteristic clinical myopathy associated with it. In the setting of amyopathic disease, the absence of clinically available biomarkers or clear pathologic diagnosis can complicate effective prognostic and therapeutic intervention. Until recently the presence of MDA-5 antibody associated dermato-pulmonary syndrome was described only in Asian populations. We present 2 cases of MDA-5-associated dermato-pulmonary syndrome and provide a comprehensive review of available literature.

A dermatomyositis and scleroderma overlap syndrome with a remarkable high titer of anti-exosome antibodies

An overlap syndrome of dermatomyositis and scleroderma is reported. The case corresponded to a 27-year-old female with a clinical picture of 14 months evolution, characterized by proximal muscles weakness, erythematous rash in wrists, knees, ankles, Gottron sign, heliotrope periorbital rash and dysphagia. A muscle biopsy was compatible with dermatomyositis; meanwhile the skin biopsy was compatible with scleroderma. Muscle enzymes were increased. Interestingly, the antinuclear antibody determination in HEp-2 cells was positive with a remarkable titer of 81,920 exhibiting a nucleolar pattern. Anti-Jo1 antibody was negative, but anti-PM/Scl-100 positive. The patient received methylprednisolone and cyclophosphamide pulses, with gradual improvement. Present report constitutes a case of overlap dermatomyositis-scleroderma syndrome, with anti-PM/Scl autoantibodies (anti-exosome). The remarkable of this case was the exceptional high antinucleolar antibody titer.

Newly Recognized Ocular Side Effects of Erlotinib

An 85-year-old man had a bilateral periorbital rash and conjunctivitis leading to lower eyelid ectropion and epiphora within 6 weeks of treatment with erlotinib (Tarceva, Genentech, Inc., San Francisco, CA, and OSI Pharmaceuticals, Melville, NY), a second-line antineoplastic agent. The treatment was discontinued secondary to toxicity, and the periorbital rash completely resolved within 6 weeks of cessation of the drug. To our knowledge, the periorbital rash resulting in bilateral lower eyelid ectropion associated with epiphora is a newly recognized side effect of erlotinib that is completely reversible with discontinuation of the drug. The rash and ectropion should be treated palliatively, and surgical intervention should be avoided unless the patient cannot be removed from treatment.

Granulomatous Perioral Dermatitis in Children

Five children, aged 3 to 11 years, developed a distinctive perioral, perinasal, and periorbital rash, consisting of tiny, closely spaced, flesh-colored "micronodules." Histopathologic examination in all five cases revealed upper dermal and perifollicular granulomas admixed with lymphocytes. There were no associated systemic abnormalities. The lesions resolved after months to years, leaving no scars. We propose that this condition is a form of perioral dermatitis with granulomatous histologic features, which can be distinguished from sarcoidosis and other facial eruptions in childhood both on clinical and histologic grounds.