[A Case of Cecal Cancer Associated with Dermatomyositis].

We report a relatively rare case of cecal cancer with dermatomyositis. An 81-year-old man was diagnosed with dermatomyositis associated with the symptoms of eruption, limb muscle weakness, and difficulty swallowing. Colonoscopy revealed a type 2 tumor in the cecum. The patient underwent laparoscopic ileocecal resection. Although it was impossible for the patient to stand before the surgery, he could stand 10 days after the surgery and walk without assistance 14 days after the surgery. In addition, the eruption disappeared, and the preoperatively high creatine kinase(CK)value normalized. Dermatomyositis with malignant tumor has been reported to be associated with poor prognosis. Symptoms related to dermatomyositis may be improved by the resection of the associated tumor. Therefore, it is important to treat the malignant tumor when the patient's condition permits.

Calcinosis in juvenile dermatomyositis mimicking cold abscess.

We report a case of dystrophic calcification presenting as soft cystic swelling in a patient with juvenile dermatomyositis. A 15-year-old boy with lumbosacral cystic swelling, which was considered a cold abscess clinically, was evaluated for nonresponse to antitubercular therapy. The cystic swelling had liquefied calcium with a well circumscribed calcified wall on imaging, which was subsequently excised.

C5b-9 deposits on endomysial capillaries in non-dermatomyositis cases.

Deposits of the terminal-membrane-attack-complex (MAC) C5b-9 on perfascicular endomysial capillaries are generally regarded as diagnostic hallmark of dermatomyositis (DM). Although the pathophysiology is not clear, C5b-9 deposits on capillaries seem to be associated with microinfarctions and vascular damage. Here, we report on a series of 19 patients presenting with C5b-9 accumulation on endomysial capillaries in the absence of features for DM. To decipher differences in the capillary C5b-9 accumulation pattern between DM and non-DM cases, we assessed the extent of endomysial capillary C5b-9 deposits related to capillary density and extent of myofiber necrosis by immunohistochemistry in 12 DM and 8 control patients. We found similar numbers of C5b-9-positive myofibers in both DM and non-DM C5b-9(+) cases. The distribution pattern differed as DM cases showed significantly more perifascicular capillary C5b-9 deposits as compared to non-DM cases, which presented stronger endomysial capillary C5b-9 deposits in a diffuse pattern. While total capillary density was not differing, DM patients displayed significantly more C5b-9(+) necrotic fibers as compared to non-DM C5b-9(+). In summary, endomysial capillary C5b-9 deposits are present in a variety of non-DM cases, however with differing distribution pattern. In conclusion, capillary C5b-9(+) deposits should be assessed critically, taking into consideration the distribution pattern.

Paraneoplastic dermatomyositis associated with adenocarcinoma of the lung.

The association of dermatomyositis and lung cancer has been recognized a long time ago. The case of a 57-year old patient with lung adenocarcinoma and dermatomyositis as the first sign of the illness is presented. The occurrence of dermatomyositis should be considered as a potential presentation of paraneoplastic syndromes, particularly in patients at risk for lung cancer.

Laser therapy in the treatment of connective tissue diseases: a review.

BACKGROUND: Connective tissue diseases (CTD), including lupus erythematosus (LE), scleroderma, sarcoidosis, and dermatomyositis, present with clinically unique cutaneous manifestations often resistant to conventional therapy. The use of lasers in the treatment of various dermatologic conditions continues to expand, presenting an opportunity for incorporation of another mechanism of action in the treatment of CTD. OBJECTIVES: To review the use of laser therapy in the treatment of LE, scleroderma, sarcoidosis, and dermatomyositis. MATERIALS AND METHODS: A MEDLINE search was conducted to find articles detailing treatment of CTD with laser therapy. RESULTS: Thirty-nine published articles were identified. The outcomes and results of case reports were reviewed for each CTD when possible. CONCLUSIONS: Laser therapy offers novel and often effective treatment for recalcitrant cutaneous conditions in LE, scleroderma, sarcoidosis, and dermatomyositis. Review of the literature revealed a limited number of reports, many describing outdated technologies and techniques. It is therefore difficult to draw substantial conclusions regarding safety and the known association with photosensitivity. More-recent reports suggest that, with continued evolution of technology and understanding of CTD, lasers will have an expanding role in the treatment of cutaneous manifestations of CTD.

Successful autologous stem cell transplantation in two patients with juvenile dermatomyositis.

Juvenile dermatomyositis (JDM) is a chronic inflammatory disorder of unknown aetiology that affects muscle and skin. We report on two patients with severe progressive JDM who developed contractures and were wheelchair dependent despite therapy including methotrexate (MTX), steroids, immunoglobulins, cyclosporin A, and rituximab. On account of the refractory disease, autologous stem cell transplantation (ASCT) was performed using a CD3/CD19-depleted graft after immunoablative conditioning with fludarabine, cyclophosphamide, and anti-thymocyte globulin. This induced a dramatic improvement and sustained remission of the disease in both patients. We demonstrate that ASCT is a therapeutic option with low toxicity for patients with severe, refractory JDM.

Case report: successful treatment of posttransplant lymphoproliferative disorder and quiescence of dermatomyositis with rituximab and sirolimus.

Posttransplant lymphoproliferative disorder (PTLD) remains one of the most important complications of intensive immunosuppressive therapy. A 65-year-old Caucasian woman received a primary en bloc kidney transplant from a deceased 2-year-old donor. After antithymocyte globulin induction she was treated with a maintenance regimen including cyclosporine and mycophenylate mofetil (MMF). She had a history of recurrent dermatomyositis, suggesting a flawed immune system. After a benign course for 9 months and after an increase in MMF from 2 to 3 g daily, she presented with pneumonia owing to Candida albicans, which was responsive to antibiotics, as was the PTLD. Persistent fever led to a diagnosis of PTLD. The immunosuppressive regimen was converted to sirolimus (SRL) and rituximab, with over 90% necrosis of the neoplasm at 1 month. However, owing to concern at exploration, the allografts were extirpated. This case documented the benefit of the rituximab-SRL combination to treat PTLD while maintaining dermatomyositis in remission.

Successful resection of dermatomyositis associated with thymic carcinoma: report of a case.

We report a case of thymic carcinoma associated with dermatomyositis (DM) in a 53-year-old man. The patient presented with the characteristic features of a skin rash with Gottron's papules, proximal muscle weakness, and increased serum levels of the muscle-associated enzymes. Comprehensive clinical examinations revealed an anterior mediastinal tumor. We resected the tumor and histological examination confirmed squamous cell carcinoma of the thymus. Thereafter, his clinical symptoms improved dramatically and his serum levels of muscle-associated enzymes dropped, indicating that the DM was a paraneoplastic phenomenon. Our search of the literature found only one other case report of DM accompanied by thymic carcinoma, and to our knowledge, this is the fi rst documented case of dramatic improvement of DM after resection of thymic carcinoma. We propose that thymic carcinoma should be added to the list of malignancies that can complicate DM as a paraneoplastic disease.

Gottron's papules-like eruption developing under hydroxyurea therapy.

A 57-year-old white female developed a Gottron's papules (GP)-like eruption, without any of the other clinical or laboratory signs of dermatomyositis (DM). She was under hydroxyurea treatment for chronic myeloid leukemia at the time. Skin biopsy was compatible with seborrheic keratosis. Conditions presenting with GP but unrelated to DM are reviewed, with emphasis on hydroxyurea-induced skin lesions.

[Extraosseous calcifications in dermatomyositis. A rare differential diagnosis of a benign heterotropic ossification]. / Extraossäre Kalzifikationen bei Dermatomyositis. Eine seltene Differenzialdiagnose bei benignen heterotopen Ossifikationen.

Juvenile dermatomyositis is rare but still the most common idiopathic inflammatory myopathy of the childhood. Operative treatment may be indicated, whenever joint motion and patients mobility is limited through the manifestation of calcinosis cutis. Although Vitamin K dependent coagulation pathways have been described, the entire pathophysiological mechanism of its occurrence is currently not clarified. Standard therapy includes the systemic application of steroids and cytotoxins, other options involve aluminum-hydroxid or diltiazem. Only in serious functional obstructions operative resection may be indicated. The case of a 39-year-old female with a 24 year history of dermatomyositis involving most areas of the upper and lower extremities like a coat of mail is presented. Surgical resection of the calcification revealed a good functional recovery of the joint but because of a high recurrency rate the operation is only indicated in special cases.