| bronchiolitis obliterans organizing pneumonia (BOOP) is rarely associated with dermatomyositis and may be resistant to conventional corticosteroid therapy under this circumstance. We present a case of BOOP associated with dermatomyositis that responded to a combination of cyclophosphamide and corticosteroid therapy after corticosteroid treatments, alone, had failed. We believe this case shows it is important to recognize that facial rash in the presence of respiratory distress may represent dermatomyositis with BOOP and aggressive treatment may be necessary for resolution of pulmonary symptoms. ( info) |
2/563. Periorbital edema as the presenting sign of juvenile dermatomyositis. We report a case of juvenile dermatomyositis that presented with periorbital edema. dermatomyositis is an autoimmune disorder with cutaneous manifestations including heliotrope patches, Gottron's papules, periungual telangiectasisas, and subcutaneous calcifications. Periorbital edema may accompany the classic heliotrope rash and, as in this case, may be the only presenting sign of juvenile dermatomyositis. ( info) |
3/563. Hyperimmunoglobulin E-recurrent infection syndrome in a patient with juvenile dermatomyositis. A 13-year-old girl presented with multiple skin abscesses. She was diagnosed as having juvenile dermatomyositis (DM) at the age of 7 years. She had suffered from recurrent skin infections, atypical pruritic dermatitis and pneumonia since the age of 8 years. Bacteriologic and fungal cultures for skin abscesses and oral mucosa were positive S. aureus and C. albicans, respectively. Chemotactic defect in peripheral blood neutrophils was observed. The level of serum IgE was markedly elevated, and anti-S.aureus specific IgE was found. A diagnosis of hyperimmunoglobulin E-recurrent infection syndrome (HIE) was made and she was successfully treated with surgical drainage and antibiotics. To our knowledge, this is the first case report of HIE in a patient with juvenile dermatomyositis. ( info) |
4/563. Epidermal nuclear CIq deposits in a patient with amyopathic dermatomyositis. We report a case of amyopathic dermatomyositis, in which C1q deposits on the epidermal nucleus were immunohistologically found as well as Ig deposits at the dermoepidermal junction (DEJ). Direct immunofluorescence (IF) examination of infiltrated erythematous lesions revealed C1q deposits on the epidermal nucleus and fibrinogen at the DEJ, and examination of hyperkeratotic erythematous lesions showed linear deposits of Ig G and Ig A at the DEJ but not in the nuclei of epidermal cells. The authors discuss the direct in vivo interaction between nucleus and immunoreactants in dermatomyositis. ( info) |
5/563. Amyopathic dermatomyositis associated with transitional cell carcinoma of the bladder. Transitional cell carcinoma of the bladder is the most common tumor of the urinary tract. However, it has only been reported twice in the literature to be associated with the paraneoplastic syndrome dermatomyositis. We report a case of amyopathic dermatomyositis in a patient whose painless gross hematuria was due to transitional cell carcinoma of the bladder as well as review this association. ( info) |
6/563. "Centripetal flagellate erythema": a cutaneous manifestation associated with dermatomyositis. We describe 3 patients with dermatomyositis who presented with flagellate erythema. This cutaneous eruption is characterized by erythematous linear lesions on the trunk and proximal extremities. Histologic examination of this eruption in one of our cases revealed an interface dermatitis. review of the literature and records of 183 patients with connective tissue diseases from our institution has shown that this peculiar eruption has been reported only in dermatomyositis. Because of the location of this eruption, we encourage the use of the term "centripetal flagellate erythema" to distinguish this entity from other linear eruptions seen in patients with connective tissue diseases. ( info) |
| We describe a 32-year-old patient with adult dermatomyositis who developed dyspnea and worsening of pre-existing infarcted skin lesions of the fingers. Chest radiographs showed diffuse hazy reticulonodular infiltration in both lungs, subcutaneous emphysema, pneumomediastinum, and pneumothorax. The pulmonary symptoms and cutaneous lesions gradually improved with a high dose of prednisolone. Although subcutaneous emphysema and pneumomediastinum occur frequently in association with traumatic disruption of cutaneous and mucosal barriers and assisted ventilation, it has rarely been observed in patients with interstitial pneumonitis in connective tissue diseases. Although dermatomyositis and subcutaneous emphysema are all relatively well-known diseases to dermatologists, the occurrence of spontaneous pneumomediastinum and pneumothorax and subsequent subcutaneous emphysema in connective tissue diseases such as dermatomyositis is unfamiliar. We discuss the possible mechanisms of this condition. ( info) |
8/563. dermatomyositis associated with rapidly progressive fatal interstitial pneumonitis and pneumomediastinum. We describe two cases of dermatomyositis (DM), which subsequently developed into rapidly progressive fatal interstitial pneumonitis and pneumomediastinum during steroid therapy. Both cases showed the classical cutaneous manifestations of DM, but the muscular symptoms were absent or mild. Both rapidly progressive interstitial pneumonitis and pneumomediastinum can occur in DM showing less inflammatory changes in the muscles. patients with this form should be treated with extreme caution. ( info) |
| We describe an unusual case of peritoneal papillary serous carcinoma (PPSC) arising in a female patient with dermatomyositis (DM). Despite periodic extensive searches for an underlying malignancy, no malignancy had been detected in this patient during the first 2.5 years after the diagnosis of DM. It was only when the patient presented with pleural effusion and ascites that the underlying intra-abdominal malignancy was detected by laparoscopy. Treatment with four cycles of pre-operative chemotherapy (taxol and cisplatin) resulted in tumor regression with amelioration in the muscular manifestation of the DM, but without parallelic amelioration in the skin manifestations of the DM. Explorative laparotomy confirmed the presence of papillary serous carcinoma in the omentum, surface of the left ovary and the retroperitoneal lymph nodes, and established the diagnosis of PPSC. Following two cycles of postoperative chemotherapy, the patient is alive with no evidence of internal malignancy. However, although muscle strength and enzymes have remained normal, no effect on the skin manifestation of DM has been observed. This case illustrates that, alongside the more frequently occurring ovarian carcinoma, PPSC should also be considered in the differential diagnosis of the underlying malignancy that may occur in the female patient with DM. ( info) |
10/563. Myositis and malignancy: is there a true association? There may be an association between polymyositis/dermatomyositis and malignant disease. Cancer occurs in patients with polymyositis/dermatomyositis with a frequency estimated between 2.5% and 29% (relative risk 1.0 to 6.5). We present two such cases, associated with colorectal carcinoma and non-Hodgkin's lymphoma respectively, together with an overview of existing controlled studies in the area. ( info) |