1/6. Juvenile dermatomyositis: an atypical presentation.Juvenile dermatomyositis (JDM) is a rare disease of unknown etiology that results in inflammation of the connective tissue of striated muscle, subcutaneous tissue, nail beds, and skin. Although JDM is a rare disease, occurring in 3 per 1 million children, it is the most common idiopathic, inflammatory, myopathic disease of childhood. A case example is provided that describes an atypical presentation of JDM. Information about the disease, the usual and unusual clinical manifestations, the diagnostic processes, and therapeutic management plans for JDM are presented. Results of a comparison between this case and the literature are discussed, along with implications for nurse practitioners.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/6. Juvenile dermatomyositis: literature review and report of a case.dermatomyositis is a rare disease of unknown origin, which affects both children and adults. In the juvenile form, dermatomyositis is a multisystem disease, characterized by myositis; an erythematous rash over the bridge of the nose, around the eyes, and on the trunk and limbs; vasculitis; and dilatation of the capillaries in the nail beds and gum margin. Late development of calcinosis is seen in approximately two-thirds of the patients. This case describes a 5-year-old dental patient diagnosed with dermatomyositis. This article includes the clinical manifestations of dermatomyositis in the body and in the oral cavity as well as the considerations that should be taken when treating these patients.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
3/6. Juvenile dermatomyositis presenting with periorbital edema.Juvenile dermatomyositis is a rare disease that affects the skin and muscles. It often presents with a classic heliotrope eyelid rash. We present a case of juvenile dermatomyositis presenting with significant bilateral periorbital edema, with its complete resolution after systemic antiinflammatory therapy.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
4/6. dermatomyositis and pregnancy. Case report and review of the literature.dermatomyositis is a rare disease complicating pregnancy. We treated a patient in whom two pregnancies were complicated by dermatomyositis. In the first of the two gestations, a mild form of the disease arose in the last trimester. In the second gestation, the disease was inactive following treatment with 0.3 mg/kg/day prednisolone. Both the mother and two children have since been doing well.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
5/6. polymyositis/dermatomyositis associated with dermatitis herpetiformis.We describe 2 patients with dermatitis herpetiformis who developed polymyositis/dermatomyositis. On HLA typing, both patients were found to be HLA-B8, DR3 positive. The concurrence of these two relatively rare diseases, both associated with immunologic abnormalities, further supports the role of autoimmunity in their pathogenesis and indicates a possible common genetic basis. It also suggests that myositis may be more common in patients with dermatitis herpetiformis than in the general population.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |
6/6. Differential diagnosis between fibrodysplasia ossificans progressiva and childhood dermatomyositis with calcinosis.Both fibrodysplasia ossificans progressiva (FOP) and childhood dermatomyositis with calcinosis are rare diseases, and present with ossifying or calcifying processes. Six cases of FOP and one case of childhood dermatomyositis with calcinosis are studied. All six FOP patients had the typical digital anomalies and the characteristic ectopic bone formation starting from the trunk. Calcification in the case of childhood dermatomyositis occurred in the limbs. A carefully differentiated diagnosis between these two diseases is needed, because they share common clinical and radiologic features, but require different management. Delay in the diagnosis of FOP is common, although early recognition of FOP prevents a child from accidental or iatrogenic injury, which can precipitate ectopic ossification. Surgical removal of the ectopic bone or release of the contracture in three FOP patients was followed by rapid recurrence. Entrapment neuropathy and a mild myopathic pattern in two FOP patients, who underwent nerve conduction and electromyographic studies, were secondary to ectopic bone formation. One FOP patient received a computed tomography examination which showed basal ganglia calcification. No coexistence of FOP and childhood dermatomyositis with calcinosis was found.- - - - - - - - - - ranking = 0.5keywords = rare disease (Clic here for more details about this article) |