Sweet's syndrome (SS) has been reported as an association with malignant neoplasms and autoimmune diseases, e.g., Behcet's disease, Sjogren's syndrome, and rheumatoid arthritis. But dermatomyositis (DM), one of the rare autoimmune diseases, was not reported as an associated disease of SS. We describe an interesting case of SS associated with DM. Diagnosis was made by skin biopsy, and subsequent clinical resolution occurred after institution of prednisolone.
Case Report ; Dermatomyositis/pathology ; Dermatomyositis/drug therapy ; Dermatomyositis/complications* ; Human ; Male ; Middle Age ; Prednisolone/therapeutic use ; Sweet's Syndrome/pathology ; Sweet's Syndrome/drug therapy ; Sweet's Syndrome/complications*

Humans ; Dermatomyositis/pathology* ; Interferon-Induced Helicase, IFIH1/genetics* ; Lung Diseases, Interstitial ; Autoantibodies ; Retrospective Studies

A case of dermatomyositis presented as bronchiolitis obliterans organizing pneumonia has been rarely reported. We describe a 46-year-old female patient with dermatomyositis without elevation of creatine kinase presented as bronchiolitis obliterans organizing pneumonia. She was treated with prednisolone and azathioprine. Over a 2-year follow-up she has had no elevation of creatine kinase. The patient remains asymptomatic and has no medication for dermatomyositis and bronchiolitis obliterans organizing pneumonia two years after initial treatment. It has been suggested that the prognosis of dermatomyositis without creatine kinase elevation may be poor. Because the prognosis of bronchiolitis obliterans organizing pneumonia is generally believed to be good, we tentatively suggest that the normal value of creatine kinase in dermatomyositis does not always seem to herald a poor prognosis, an associated malignancy or severe interstitial lung disease.
Azathioprine/administration +ACY- dosage ; Biopsy, Needle ; Bronchiolitis Obliterans Organizing Pneumonia/pathology ; Bronchiolitis Obliterans Organizing Pneumonia/diagnosis+ACo- ; Case Report ; Creatine Kinase/blood+ACo- ; Dermatomyositis/pathology ; Dermatomyositis/enzymology ; Dermatomyositis/drug therapy ; Dermatomyositis/diagnosis+ACo- ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Human ; Middle Age ; Prednisone/administration +ACY- dosage ; Tomography, X-Ray Computed

Child ; Dermatomyositis ; diagnosis ; pathology ; therapy ; Diagnosis, Differential ; Electromyography ; Glucocorticoids ; administration & dosage ; Humans ; Immunosuppressive Agents ; administration & dosage ; Magnetic Resonance Imaging

Dermatomyositis (DM) is an uncommon inflammatory myopathy with characteristic rash accompanying, or more often preceding, muscle weakness. There is a well-recognized association between DM and several cancers, such as ovarian cancer, breast cancer, melanoma, colon cancer, and non-Hodgkin lymphoma. We report the first case of cancer of unknown primary site associated with DM. A 62-yr-old woman presented to us with both shoulder painful swelling and facial edema. She was diagnosed previously as cancer of unknown primary site, histologically confirmed with squamous cell carcinoma in a pelvic mass. For the following days, she complained of erythematous face followed by progressive weakness of the proximal muscles of upper and lower limbs. The laboratory tests showed an increased muscle enzyme and acute phase reactants. The electromyogram showed the typical findings of DM. After the treatment with high dose steroid and methotrexate, the proximal motor weakness improved, and she received palliative radiation therapy.
Carcinoma, Squamous Cell/complications/diagnosis/pathology ; Dermatomyositis/*complications/diagnosis/pathology/therapy ; Female ; Humans ; Middle Aged ; Neoplasms, Unknown Primary/*complications/diagnosis/pathology

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

Inflammatory myopathies are comprised of three major subsets, polymyositis, dermatomyositis and inclusion body myositis. Although their etiology is unclear, each group retains its characteristic clinical, immunopathologic features. In polymyositis, a CD8+ T-cell mediated cytotoxicity against muscle fibers has emerged as the main pathologic event, whereas in dermatomyositis complement-mediated injury by antibody may be the primary pathology. There has been several reports on polymyositis internationally but we could find only a few reports in Korea. We report here a 8-year old female patient admitted with a stuporous mentality. After coughing and fever for 3 days, she got myalgia, abruptly developed gross hematuria and dyspnea. After admission, she showed weak self respiration and exclussively elevated muscle enzyme in blood chemistry. In muscle biopsy, lymphocytic infiltrations were found in the fascicles without endomysial fibrosis and these lymphocytes were composed of T lymphocytes on immunohistochemical stain. She received two infusions of intravenous immunoglobulin(1g/kg/day), and showed dramatic improvement in symptoms and signs.
Biopsy ; Chemistry ; Child ; Cough ; Dermatomyositis ; Dyspnea ; Female ; Fever ; Fibrosis ; Hematuria ; Humans ; Immunoglobulins* ; Korea ; Lymphocytes ; Myalgia ; Myositis ; Myositis, Inclusion Body ; Pathology ; Polymyositis* ; Respiration ; Stupor ; T-Lymphocytes

OBJECTIVETo assess the significance of single fiber electromyography (SFEMG) in diagnosis of inflammatory myopathies and the correlation with other assistant examination findings.

METHODSSFEMG were recorded from the extensor digitorum communis of 34 patients with polymyositis or dermatomyositis and compared with the findings of routine electromyography (EMG), serum creatine kinase (CK) determination, and muscle biopsy.

RESULTSSFEMG recordings in 34 patients were all abnormal. The prominent feature was markedly increased fiber density (FD) with normally or mildly increased jitter. FD ranged from 1.0 to 6.0 (2.34 +/- 0.43). Jitter ranged from 5 to 78 micros (41.6 +/- 10.3 micros). The potential pairs with jitter values greater than 55 micros ranged from 0% to 55% (7.7% +/- 11.8%). Block was detected at one recording site in only one patient. Routine EMG demonstrated myogenic lesions in only 24 patients (70.6%). FD was a little higher in the normal-EMG subgroup or the neurogenic-EMG subgroup than myogenic-EMG subgroup but without statistical significance. Elevated CK levels were found in 75% patients (24/32). FD in the normal CK subgroup was significantly higher than that in the elevated CK subgroup (2.62 +/- 0.40 vs. 2.28 +/- 0.40, P < 0.05). Muscle pathologies were consistent with the diagnosis of myositis in 75% (15/20).

CONCLUSIONSFEMG is of great value in the diagnosis and disease process understanding of inflammatory myopathies for the clinically suspected patients with normal routine EMG, CK levels, and muscle biopsies.

Adolescent ; Adult ; Aged ; Child ; Creatine Kinase ; blood ; Dermatomyositis ; diagnosis ; pathology ; physiopathology ; Electromyography ; methods ; Female ; Humans ; Male ; Middle Aged ; Muscle Fibers, Skeletal ; pathology ; physiology ; Myocardium ; pathology ; Polymyositis ; diagnosis ; pathology ; physiopathology

Dermatomyositis in pregnancy is rare. Pregnancy may be a precipitating factor at the onset or may develop during the course of dermatomyositis, which would exacerbate disease activity. In this study, we report a 22-year-old female patient who developed generalized skin rash and progressive muscle weakness in the twelfth week of pregnancy. She was diagnosed with dermatomyositis and underwent therapeutic abortion, due to the high fetal mortality rate of the disease when developed in the first trimester. Her symptoms improved with treatment of intravenous immunoglobulin and a high dose of corticosteroids.
Adult ; Anti-Inflammatory Agents/therapeutic use ; Dermatomyositis/drug therapy/*etiology/pathology ; Female ; Human ; Immunoglobulins, Intravenous/*administration & dosage ; Prednisolone/therapeutic use ; Pregnancy ; *Pregnancy Complications ; Pregnancy Trimester, First ; Skin/pathology ; Treatment Outcome

This study was performed in order to characterize the types of the infiltrating cells, and the expression profiles of major histocompatibility complex (MHC) class I and membrane attack complex (MAC) in patients with inflammatory myopathies and dysferlinopathy. Immunohistochemical stains were performed using monoclonal antibodies against several inflammatory cell types, MHC class I, and MAC in muscles from inflammatory myopathies and dysferlinopathy. There was significant difference in the types of infiltrating cells between polymyositis (PM), dermatomyositis (DM), and dysferlinopathy, including significantly high CD4+/CD8+ T cell ratio and B/T cell ratio in DM. In dysferlinopathy, CD4+ T cells were the most abundant and the proportions of infiltrating cell types were similar to those of DM. MHC class I was expressed in muscle fibers of PM and DM regardless of the presence of inflammatory infiltrates. MAC was expressed in necrotic fibers and vessels of PM and DM. One patient with early stage DM had a MAC deposits on endomysial capillaries. In dysferlinopathy, MAC deposit was also observed on the sarcolemma of nonnecrotic fibers. The analysis of inflammatory cells, MHC class I expressions and MAC deposits may help to differentiate dysferlinopathy from idiopathic inflammatory myopathy.
Adult ; Aged ; *Dermatomyositis/immunology/pathology ; Female ; Genes, MHC Class I ; Humans ; Male ; *Membrane Proteins/genetics/immunology ; Middle Aged ; Muscle Fibers, Skeletal/cytology/immunology/pathology ; *Muscle Proteins/genetics/immunology ; *Muscular Dystrophies, Limb-Girdle/immunology/pathology ; *Myositis/immunology/pathology ; *Polymyositis/immunology/pathology ; T-Lymphocytes/cytology/immunology/pathology ; Young Adult