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*

Amyopathic dermatomyositis is diagnosed when the biopsy-confirmed cutaneous lesions of dermatomyositis are present for longer than 2 years in the absence of muscle weakness, elevated muscle enzymes, and the history of immunosuppressive drug therapy and ingestion of drugs such as hydroxyurea that can produce dermatomyositis-like cutaneous hypersensitivity changes. We report a 36-year-old woman with a 3-year history of typical skin features of dermatomyositis with no evidences of muscle involvement.
Adult ; Dermatomyositis* ; Drug Therapy ; Eating ; Female ; Humans ; Hydroxyurea ; Hypersensitivity ; Muscle Weakness ; Skin

Antibodies, Monoclonal, Humanized/therapeutic use* ; Autoantibodies ; Dermatomyositis ; Disease Progression ; Humans ; Lung Diseases, Interstitial/drug therapy*

Objective: To elucidate the clinical features of patients with refractory juvenile dermatomyositis (JDM), and to explore the efficacy and safety of tofacitinib in the treatment of refractory JDM. Methods: A total of 75 JDM patients admitted to the Department of Rheumatology and Immunology in Shenzhen Children's Hospital from January 2012 to January 2021 were retrospectively analyzed, and to analyze the clinical manifestations, efficacy and safety of tofacitinib in the treatment of refractory JDM. Patients were divided into refractory group with using of glucocorticoids in combination with two or more anti-rheumatic drugs for treatment, and the presence of disease activity or steroid dependence after a one-year follow-up. The non-refractory group is defined as clinical symptoms disappeared, laboratory indicators were normal, and clinical remission was achieved after initial treatment, and the clinical manifestations and laboratory indexes of the two groups were compared. The Mann-Whitney U test, Fisher's precision probability test was used for intergroup comparison. Binary Logistic multivariate regression analysis was used to identify risk factors for refractory JDM. Results: Among the 75 children with JDM, 41 were males and 34 were females with a age of onset of 5.3 (2.3, 7.8) years. The refractory group consisted of 27 cases with a age of onset of 4.4 (1.5, 6.8) years, while the non-refractory group consisted of 48 cases with a age of onset of 5.9 (2.5, 8.0) years. Compared with 48 cases in the non-refractory group, the proportion of interstitial lesions and calcinosis in the refractory group was higher than that in the non-refractory group (6 cases (22%) vs. 2 cases (4%), 8 cases (30%) vs. 4 cases (8%), both P<0.05). Binary Logistic regression analysis showed that observation group were more likely to be associated with to interstitial lung disease (OR=6.57, 95%CI 1.22-35.31, P=0.028) and calcinosis (OR=4.63, 95%CI 1.24-17.25, P=0.022). Among the 27 patients in the refractory group, 22 cases were treated with tofacitinib, after treatment with tofacitinib, 15 of 19 cases (86%) children with rashes showed improvement, and 6 cases (27%) with myositis evaluation table score less than 48 score both were improved, 3 of 6 cases (27%) had calcinosis were relieved, and 2 cases (9%) had glucocorticoid-dependence children were successfully weaned off. During the tofacitinib treatment, there was no increase in recurrent infection, blood lipids, liver enzymes, and creatinine were all normal in the 22 cases. Conclusions: Children with JDM with calcinosis and interstitial lung disease are more likely to develop refractory JDM. Tofacitinib is safe and effective for refractory JDM.
Child ; Female ; Male ; Humans ; Dermatomyositis/drug therapy* ; Retrospective Studies ; Risk Factors ; Calcinosis ; Glucocorticoids/therapeutic use*

We report a case of a 38-year-old male with extranodal NK/T-cell lymphoma, nasal type, showing unusual clinical and pathological features. The patient was admitted for soft tissue swelling and tenderness in both legs. The patient had been treated intermittently 8 months prior for repeated muco-cutaneous ulcers. A muscle biopsy showed medium-sized atypical lymphoid cells with bizarre nuclei and plump cytoplasm, infiltrating to the skeletal muscle fibers with angiocentricity. The immunoresults were Ki-1+, CD56+, cytoplasmic CD3+, with EBV-in situ hybridization +. The patient rapidly deteriorated and died of sepsis and respiratory failure shortly after initiation of low-dose chemotherapy. A careful review of previous biopsies revealed scarce atypical lymphoid cells around vessels with similar immunoprofiles without the presence of Ki-1 positive cells. This case emphasizes that an extranodal NK/T-cell lymphoma may have a dermatomyositis-like diffuse presentation. Ki-1 co-expression can be an unexpected event in a process of the disease course; however, this should be validated with future studies.
Adult ; Biopsy ; Cytoplasm ; Dermatomyositis* ; Drug Therapy ; Humans ; Leg ; Lymphocytes ; Lymphoma* ; Male ; Muscle Fibers, Skeletal ; Respiratory Insufficiency ; Sepsis ; Ulcer

Dermatomyositis is an idiopathic inflammatory myopathy characterized by progressive symmetric proximal muscle weakness and typical cutaneous lesions. Recently the association of adult dermatomyositis and malignancy has generated much attention. A 56-year-old male presented with skin eruptions, proximal muscle weakness, and a neck mass on the right side. Diagnosis of dermatomyositis was established by clinical investigation, muscle enzyme study, electromyogram, and histological findings of the skin and muscle. A computerized tomography scan and histologic finding of right neck mass showed nasopharyngeal carcinoma. The patient was treated with chemotherapy and radiotherapy, and showed partial remission of nasopharyngeal carcinoma, with some improvement of the skin eruptions. But muscle weakness and myalgia were aggravated. He was then treated with systemic steroids (prednisolone) and azathioprine.
Adult ; Azathioprine ; Dermatomyositis* ; Diagnosis ; Drug Therapy ; Humans ; Male ; Middle Aged ; Muscle Weakness ; Myalgia ; Myositis ; Neck ; Radiotherapy ; Skin ; Steroids

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

Carcinoma ; Dermatomyositis ; drug therapy ; Dexamethasone ; therapeutic use ; Humans ; Male ; Middle Aged ; Nasopharyngeal Neoplasms ; complications ; Paraneoplastic Syndromes ; drug therapy ; Prednisone ; therapeutic use

Hydroxyurea, one of conventional chemotherapies in chronic myelogenous leukemia(CML) and polycythemia vera, inhibits ribonucleotide reductase, which catalyzes the rate-limiting step in the de novo synthesis of deoxynucleotide triphosphates required for DNA synthesis. Various cutaneous side effects due to hydroxyurea have been reported as follows; alopecia, diffuse hyperpigmentation, scaling, poikiloderma, atrophy of the skin and subcutaneous tissues, nail changes with multiple pigmented nail bands or brittleness, erythema and scaling of acral sites simulating chronic dermatomyositis, lichen planus-like lesions, or skin tumors on the UV-light exposed areas.A 72-year-old woman, receiving hydroxyurea for CML during 5 years, revealed hydroxyurea dermopathy such as scaly papules, poikilodermatous patches, and acral ulceration, and unusually developed squamous cell carcinoma. This is a rare case of hydroxyurea dermopathy and the first case of squamous cell carcinoma by hydroxyurea in Korea.
Aged ; Alopecia ; Atrophy ; Carcinoma, Squamous Cell* ; Dermatomyositis ; DNA ; Drug Therapy ; Erythema ; Female ; Humans ; Hydroxyurea ; Hyperpigmentation ; Korea ; Lichens ; Polycythemia Vera ; Ribonucleotide Reductases ; Skin ; Subcutaneous Tissue ; Ulcer*

Cholangiocarcinoma with paraneoplastic dermatomyositis (DM) is extremely rare, and the whole body positron emission tomography-computed tomography (PET-CT) finding of paraneoplastic DM is rarely reported. We report a 66-year-old woman with metastatic cholangiocarcinoma, initially presented with bilateral proximal muscle uptake on PET-CT without clinical muscle symptoms. The initial interpretation of the high muscle uptake was metastasis to the muscles. However, while awaiting for chemotherapy, muscle weakness evolved and rapidly progressed. The level of creatine phosphokinase was significantly elevated. Electromyography revealed moderate myopathy, and a muscle biopsy showed degenerating myofibers with variable sizes. The diagnosis of paraneoplastic dermatomyositis was made. This case highlights that, although rare, paraneoplastic dermatomyositis can be present with cholangiocarcinoma. Also, muscle inflammation can precede the clinical muscle symptoms, and paraneoplastic DM should be considered as a possible differential diagnosis in the assessment of cancer patients who present with abnormal muscle tracer uptake in PET-CT scans.
Aged ; Biopsy ; Cholangiocarcinoma* ; Creatine Kinase ; Dermatomyositis* ; Diagnosis ; Diagnosis, Differential ; Drug Therapy ; Electromyography ; Electrons* ; Female ; Humans ; Inflammation ; Muscle Weakness ; Muscles ; Muscular Diseases ; Neoplasm Metastasis ; Positron-Emission Tomography