The overall features were in keeping with an inflammatory myopathy, with features suggestive of dermatomyositis. Skin biopsy of the left lower lid showed evidence of mild hyperkeratosis in the epidermis.
The dermis showed heavy chronic inflammation cell infiltration that consists mainly of lymphocytes and plasma cells, infiltrating the hair follicles. Adjacent mild dermal fibrosis, focal solar elastosis, and pigment incontinence are noted.
At 6 months, the patient showed a dramatic improvement with normalization of muscle power, fading of the skin rashes, and reduction of muscle enzymes. When evaluating a patient with JDM with unilateral periorbital swelling, caution should be taken not to miss an infectious etiology or infiltrative malignancy which is a well-recognized association [ 4 ].
Imaging and biopsy of the periorbital swelling are warranted before starting immunosuppressive therapy. Additionally, electronic microscopy revealed scattered rod-like structures and cytoplasmic bodies which are not commonly seen but previously described in JDM [ 5 , 6 ].
Treatment of cutaneous manifestations of JDM includes photoprotection, topical corticosteroids, topical calcineurin inhibitors, and antimalarials as first line. Second line agents include corticosteroids, methotrexate, and mycophenolate mofetil. In refractory cases drugs such as dapsone, azathioprine, intravenous immunoglobulins, and rituximab can be tried [ 7 ]. In our case, the response to corticosteroids and methotrexate was adequate for both muscle and skin manifestations.
To our knowledge, this is the first case presenting with a unilateral heliotrope rash and successful treatment with standard immunosuppressive agents.
This is an open access article distributed under the Creative Commons Attribution License , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Article of the Year Award: Outstanding research contributions of , as selected by our Chief Editors. Read the winning articles. Overview Dermatomyositis dur-muh-toe-my-uh-SY-tis is an uncommon inflammatory disease marked by muscle weakness and a distinctive skin rash.
Request an Appointment at Mayo Clinic. Share on: Facebook Twitter. Show references Dermatomyositis. Myositis Association of America. Accessed March 20, Dermatomyositis information page. National Institute of Neurological Disorders and Stroke. What is a heliotrope rash? Medically reviewed by Nancy Carteron, M. What are the causes and risk factors? Symptoms Diagnosis and when to see a doctor Treatment Takeaway A heliotrope rash is a reddish purple rash on or around the eyelids.
Share on Pinterest The heliotrope rash can appear uneven and may be combined with a swollen eyelid. Share on Pinterest A heliotrope rash is the first noticeable symptom of dermatomyositis.
Image credit: Elizabeth M. Dugan, Adam M. Huber, Frederick W. Miller, Lisa G. Rider, Diagnosis and when to see a doctor. Share on Pinterest Dermatomyositis pain may be improved with chiropractic therapies or massage.
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Low-dose methotrexate administered weekly is an effective corticosteroid-sparing agent for the treatment of the cutaneous manifestations of dermatomyositis. Treatment of refractory myositis: a randomized crossover study of two new cytotoxic regimens. Arthritis Rheum. Leflunomide as adjuvant treatment of dermatomyositis. Farinde A. Topical corticosteroids: World Health Organization classification of topical corticosteroids. Up-dated August 19 , Adverse cutaneous reactions to hydroxychloroquine are more common in patients with dermatomyositis than in patients with cutaneous lupus erythematosus.
Cutaneous lesions of dermatomyositis are improved by hydroxychloroquine. Treatment of dermatomyositis with methotrexate. The effect of intravenous immunoglobulin IVIG treatment on patients with dermatomyositis: a 4-year follow-up study. Calcinosis cutis occurring in association with autoimmune connective tissue disease: the Mayo Clinic experience with 78 patients, Juvenile dermatomyositis: clinical profile and disease course in 25 patients. Severe gastrointestinal inflammation in adult dermatomyositis: characterization of a novel clinical association.
Am J Med Sci. Investigation into the cause of mortality in 49 cases of idiopathic inflammatory myopathy: a single center study.
Exp Ther Med. Articles from Clinical Medicine Insights. Support Center Support Center. External link. Please review our privacy policy. Polymyositis Definite: all 1 to 4 Probable: any 3 of 1 to 4 Possible: any 2 of 1 to 4. Dermatomyositis Definite: item 5 plus any 3 of 1 to 4 Probable: item 5 plus any 2 of 1 to 4 Possible: item 5 plus and 1 of 1 to 4. Synthetic glucocorticoid analog, used for anti-inflammatory effects and modifies immune response to diverse stimuli.
Gastrointestinal symptoms, adrenal suppression, immunosuppression, avascular necrosis, and osteoporosis. Initial pharmacologic agent considers adjunctive therapy if no improvement is seen in using objective data.
Antimetabolite that interferes with DNA synthesis, repair and cellular replication by inhibiting dihydrofolate reductase. Stomatitis, hepatic fibrosis, cirrhosis, nausea, abdominal pain, neutropenia, thrombocytopenia, pruritus, fever, pneumonitis, and gastrointestinal symptoms. Suppresses cell-mediated hypersensitivities and causes alterations in antibody production. Acts as an immunosuppressive antimetabolite. Lymphoma, nausea, vomiting, hepatotoxicity, leukopenia, oral ulcers, thrombocytopenia.
Screen patients for thiopurine methyltransferase deficiency before therapy usually seen in 0. Nitrogen mustard-type alkylating agent whose cytotoxic action is primarily due to cross-linking DNA and RNA strands and inhibits protein synthesis. Increased risk for malignancy, leukopenia, thrombocytopenia, hemorrhagic cystitis, anorexia, nausea, vomiting, alopecia, sterility, congestive heart failure, and stomatitis. Inhibition of production and release of interlukin-2, a proliferative factor necessary for the induction of cytotoxic T lymphocytes in response to alloantigenic reaction.
Impaired T-cell proliferation, nephrotoxicity, lymphoma, hypertension, hypertrichosis, gingival hyperplasia, hepatotoxicity, paresthesias, fatigue, hyperesthesia, depression, and seizures. Interferes with antigen processing by increasing the pH where acidity is required to assemble chains of MHC class II proteins, thereby diminishing the formation of peptide-MHCs resulting in the downregulation of immune response against autoantigenic peptides.
Myopathy, hematologic toxicity, hepatotoxicity, antimalarial retinopathy, dizziness, ataxia, and weight loss. Adjunctive topical steroid to treat psoriasis rash. Immunomodulatory effects on T cells, macrophages, and B-cell immune function and its regulatory action on membrane-damaging components of the complement system.
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