Presentation
It is important to be aware of this rare presentation to avoid misdiagnosis. [jmedicalcasereports.biomedcentral.com]
The patient then presented dysphagia and dyspnea progressing to orthopnea, so she was admitted to the hospital. [reumatologiaclinica.org]
Mycosis fungoides: classic disease and variant presentations. Howard MS, Smoller BR. Howard MS, et al. Semin Cutan Med Surg. 2000 Jun;19(2):91-9. doi: 10.1016/s1085-5629(00)80005-x. Semin Cutan Med Surg. 2000. PMID: 10892710 Review. MeSH terms [pubmed.ncbi.nlm.nih.gov]
Primary non-Hodgkin's lymphoma of the transverse colon presenting as dermatomyositis: case presentation and literature review.Med Oncol. 2003;20(4):413-24. Miller FW, Rider LG, Plotz PH, Isenberg DA, Oddis CV. [malattierare.regione.veneto.it]
Gottron papules and nailfold telangiectasia are present in this patient with dermatomyositis. [emedicine.medscape.com]
Entire Body System
- Weakness
Later she developed progressive, symmetrical proximal muscle weakness. [jmedicalcasereports.biomedcentral.com]
Four months later melanonychia appeared, as well as heliotrope erythema, periorbital edema, and Gottron's papules on the proximal and distal interphalangeal joints of both hands (Fig. 2), progressive muscle weakness with a symmetric, proximal distribution [reumatologiaclinica.org]
Muscle involvement manifests as proximal muscle weakness. [emedicine.medscape.com]
Serum muscle enzymes, muscle pathology and clinical muscle weakness: correlation in Thai patients with polymyositis/dermatomyositis. J Med Assoc Thai. 2002;85(1):26-32. Callen JP. [malattierare.regione.veneto.it]
- Weight Loss
Extramuscular manifestations of the disease may include the following: General systemic disturbances, fever, arthralgia, malaise, weight loss, Raynaud phenomenon Dysphagia Gastroesophageal reflux Atrioventricular defects, tachyarrhythmias, dilated cardiomyopathies [emedicine.medscape.com]
Respiratoric
- Pneumonia
During the hospital stay she developed pneumonia associated with mechanical ventilation and septic shock leading to her death. [reumatologiaclinica.org]
Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology. 2004;44(6):585-96. Ruetter A, Luger TA. [malattierare.regione.veneto.it]
Intractable skin necrosis and interstitial pneumonia in amyopathic dermatomyositis, successfully treated with cyclosporin A. Intern Med. 2003 Dec. 42(12):1253-8. [QxMD MEDLINE Link]. [emedicine.medscape.com]
- Dyspnea
The patient then presented dysphagia and dyspnea progressing to orthopnea, so she was admitted to the hospital. [reumatologiaclinica.org]
Systemic manifestations may occur; therefore, the review of systems should assess for the presence of arthralgia, arthritis, dyspnea, dysphagia, arrhythmia, and dysphonia. [emedicine.medscape.com]
- Orthopnea
The patient then presented dysphagia and dyspnea progressing to orthopnea, so she was admitted to the hospital. [reumatologiaclinica.org]
Gastrointestinal
- Dysphagia
The patient then presented dysphagia and dyspnea progressing to orthopnea, so she was admitted to the hospital. [reumatologiaclinica.org]
Systemic manifestations may occur; therefore, the review of systems should assess for the presence of arthralgia, arthritis, dyspnea, dysphagia, arrhythmia, and dysphonia. [emedicine.medscape.com]
Table 1 Full blood count, liver function tests, and renal function tests Full size table Due to dysphagia, she was commenced on nasogastric feeds. Physiotherapy was started and early mobilization initiated. [jmedicalcasereports.biomedcentral.com]
Musculoskeletal
- Muscle Weakness
Later she developed progressive, symmetrical proximal muscle weakness. [jmedicalcasereports.biomedcentral.com]
Four months later melanonychia appeared, as well as heliotrope erythema, periorbital edema, and Gottron's papules on the proximal and distal interphalangeal joints of both hands (Fig. 2), progressive muscle weakness with a symmetric, proximal distribution [reumatologiaclinica.org]
Muscle disease commonly manifests as a proximal symmetrical muscle weakness. The degree of weakness may range from mild to moderate to severe; sometimes, quadriparesis is observed. [emedicine.medscape.com]
Serum muscle enzymes, muscle pathology and clinical muscle weakness: correlation in Thai patients with polymyositis/dermatomyositis. J Med Assoc Thai. 2002;85(1):26-32. Callen JP. [malattierare.regione.veneto.it]
Eyes
- Periorbital Edema
Four months later melanonychia appeared, as well as heliotrope erythema, periorbital edema, and Gottron's papules on the proximal and distal interphalangeal joints of both hands (Fig. 2), progressive muscle weakness with a symmetric, proximal distribution [reumatologiaclinica.org]
After that several cases were reported of which the latest was reported by Perales-Martinez et al.; Perales-Martinez et al. described a female patient who initially had poikiloderma and later developed heliotrope erythema, periorbital edema, Gottron’s [jmedicalcasereports.biomedcentral.com]
Periorbital edema as the presenting sign of dermatomyositis. Int J Dermatol. 2003;42(6):466-7. Silva CA, Sultan SM, Isenberg DA. Pregnancy outcome in adult-onset idiopathic inflammatory myopathy. Rheumatology (Oxford). 2003;42(10):1168-72. [malattierare.regione.veneto.it]
Skin
- Erythema
Gottron’s sign, heliotrope rash, periungual abnormalities, and flagellate erythema are common dermatological features. [jmedicalcasereports.biomedcentral.com]
Four months later melanonychia appeared, as well as heliotrope erythema, periorbital edema, and Gottron's papules on the proximal and distal interphalangeal joints of both hands (Fig. 2), progressive muscle weakness with a symmetric, proximal distribution [reumatologiaclinica.org]
[…] mottled, marbled) refers to clinico-morphologically defined, etiologically distinct, congenital or acquired, variegated clinical pictures with circumscritic or diffuse atrophies, small-spot to reticular hyper- and depigmentation, telangiectasias, and erythema [altmeyers.org]
Characteristic but not pathognomonic features include the following: Malar erythema Poikiloderma in a photosensitive distribution Violaceous erythema on the extensor surfaces Periungual and cuticular changes Poikiloderma, which consists of erythema, hypopigmentation [emedicine.medscape.com]
- Papule
She had an early heliotrope rash, shawl sign, and Gottron papules. Electromyography and muscle biopsy were supportive of inflammatory myositis and skin biopsy showed evidence of dermatomyositis. [jmedicalcasereports.biomedcentral.com]
Four months later melanonychia appeared, as well as heliotrope erythema, periorbital edema, and Gottron's papules on the proximal and distal interphalangeal joints of both hands (Fig. 2), progressive muscle weakness with a symmetric, proximal distribution [reumatologiaclinica.org]
Gottron papules are flat-topped, erythematous to violaceous papules and plaques found over bony prominences, particularly the metacarpophalangeal joints, the proximal interphalangeal joints, and/or the distal interphalangeal joints. [emedicine.medscape.com]
- Skin Disease
Muscle disease may occur concurrently, may precede the skin disease, or may follow the skin disease by weeks to years. Muscle involvement manifests as proximal muscle weakness. [emedicine.medscape.com]
Efficacy and safety of intravenous immunoglobulin for immune-mediated skin disease: current view Am J Clin Dermatol. 2004;5(3):153-60. Huang CM, Yang YH, Chiang BL. [malattierare.regione.veneto.it]
In 1906 and 1908 Jacobi described this unusual cutaneous syndrome which he called poikiloderma atrophicans vasculare [19] and much controversy was raised as to whether it is a separate entity or an early or end stage of other skin diseases. [jmedicalcasereports.biomedcentral.com]
- Alopecia
This presented as a generalized skin rash characterized by confluent lesions, which later was replaced by hyperpigmented and hypopigmented macules (Fig. 1), dryness and scaling, as well as alopecia. [reumatologiaclinica.org]
Patients may also complain of a scaly scalp or diffuse hair loss (see the image below). [3] Diffuse alopecia with scaly scalp dermatosis is common in patients with dermatomyositis. [emedicine.medscape.com]
- Eruptions
Patients often notice an eruption on photoexposed surfaces. The disease is often pruritic, and, sometimes, intense pruritus may disturb sleep patterns. [emedicine.medscape.com]
Hydroxyurea-induced dermatomyositis-like eruption. J Am Acad Dermatol. 2003;48(3):439-41. Juarez M, Misischia R, Alarcon GS. [malattierare.regione.veneto.it]
Treatment
Dermatomyositis: Diagnosis and treatment. J Am Acad Dermatol. 2020 Feb. 82 (2):283-296. [QxMD MEDLINE Link]. Ang GC, Werth VP. Combination antimalarials in the treatment of cutaneous dermatomyositis: a retrospective study. [emedicine.medscape.com]
The results of the treatment may be disappointing. [dermnetnz.org]
After that treatment, lasers can improve the redness. Light therapy can improve both brown and red discoloration. [healthline.com]
Treatment of myositis with "lower" dose corticosteroids.Curr Rheumatol Rep. 2001;3(4):309. Fedczyna TO, Lutz J, Pachman LM. [malattierare.regione.veneto.it]
Our patient made a good recovery with steroid treatment and methotrexate. Up to now screening studies for occult malignancies have been negative. [jmedicalcasereports.biomedcentral.com]
Prognosis
The prognosis in children with dermatomyositis is worse in those in whom diagnosis is delayed. Calcinosis caused by dermatomyositis in childhood can be observed in patient who had active dermatomyositis 15 years before time of this photograph. [emedicine.medscape.com]
Variations in histological patterns of interstitial pneumonia between connective tissue disorders and their relationship to prognosis. Histopathology. 2004;44(6):585-96. Ruetter A, Luger TA. [malattierare.regione.veneto.it]
Etiology
Poikiloderma (poikilos = mottled, marbled) refers to clinico-morphologically defined, etiologically distinct, congenital or acquired, variegated clinical pictures with circumscritic or diffuse atrophies, small-spot to reticular hyper- and depigmentation [altmeyers.org]
Epidemiology
Idiopathic inflammatory myopathies: epidemiology, classification, and diagnostic criteria. Rheum Dis Clin North Am. 2002;28(4):723-41. Fagan KA, Badesch DB. Pulmonary hypertension associated with connective tissue disease. [malattierare.regione.veneto.it]
Pathophysiology
Juvenile dermatomyositis: immunogenetics, pathophysiology, and disease expression. Rheum Dis Clin North Am. 2002;28(3):579-602 Roddy E, Courtney PA, Morris A. [malattierare.regione.veneto.it]
Prevention
[…] minus' sign Virchow -triad Use Parentheses water AND (cup OR glass) Add an asterisk (*) at end of a word to include word stems Neuro* will search for Neurology, Neuroscientist, Neurological, and so on Use quotes to search for an exact phrase "primary prevention [read.qxmd.com]
It isn’t curable, but you can minimize your skin discoloration with treatment and prevent further damage by protecting your skin from the sun. [healthline.com]