1/7. Eosinophilic fasciitis--progression to linear scleroderma: a case report.Eosinophilic fasciitis is a rare disease in children. Although changes similar to linear scleroderma have been reported, the outcome is usually good. In this report, a 10-year-old boy who developed eosinophilic fasciitis without a good response to steroids is presented. He progressed to linear scleroderma within months. Our case reinforces the hypothesis that eosinophilic fasciitis may be an early manifestation or a variant of localized scleroderma similar to the other cases in the literature.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/7. Necrotising fasciitis in the head and neck region.Necrotising fasciitis is an uncommon entity in present day medicine. Our review of the literature did not reveal any case involving the head and neck region. A case of this rare disease involving the head and neck region is presented.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/7. Cranial fasciitis of childhood: a case report.Cranial fasciitis of childhood is very rare, only 17 cases having been reported in the literature. We report an additional case of this rare disease. The patient was a 5-year-old boy who complained of left exophthalmos and double vision. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a large epidural mass in the left frontal region that had invaded into the underlying anterior skull base. The tumor showed homogeneous, low density with nonhomogeneous contrast enhancement on the CT scans, and low intensity on the T1-weighted and high intensity on the T2-weighted MRI images. A whitish-pink, elastic, hard tumor was revealed in the epidural space in the left anterior cranial fossa, which was totally excised with curettage of the affected anterior skull base. The origin of the tumor was suspected to be the fibrous connective tissue of the sphenofrontal suture. The histological diagnosis was that of cranial fasciitis. There was no evidence of recurrence 1 year postoperatively.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/7. Self-healing juvenile cutaneous mucinosis: cases highlighting subcutaneous/fascial involvement.BACKGROUND: Self-healing juvenile cutaneous mucinosis is a rare disease affecting young people characterized by transient cutaneous lesions and sometimes mild inflammatory symptoms. The deep dermal and subcutaneous features of this disorder have not yet been well described. OBJECTIVE: The purpose of our study was to present 3 cases of self-healing juvenile cutaneous mucinosis in which the histopathologic features caused diagnostic confusion between this disorder and proliferative fasciitis. methods: The study includes clinical and histologic findings of 3 patients, complemented by a literature review. RESULTS: The histologic descriptions of nodular lesions in self-healing juvenile cutaneous mucinosis reveal features of proliferative fasciitis, including a myxoid stroma and gangliocyte-like giant cells. LIMITATIONS: Self-healing juvenile cutaneous mucinosis is a rare condition and has not been frequently reported in medical literature. Our findings are based on the pathologic features of 3 patients. CONCLUSIONS: Our findings further elucidate the histologic features of self-healing juvenile cutaneous mucinosis and expand the differential diagnosis for entities in which gangliocyte-like giant cells are noted.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/7. Human fibroblasts in idiopathic retroperitoneal fibrosis express hla-dr antigens.Idiopathic retroperitoneal fibrosis (IRF) is a rare human disease characterized by non-neoplastic fibroblastic proliferation associated with chronic inflammatory cells; its pathogenesis is obscure. We undertook an immunohistochemical study for the expression of hla-dr antigens and other immune-related markers by retroperitoneal proliferating fibroblasts and inflammatory cells from 2 IRF patients. Patterns of immunoreactivity were compared with those expressed by human nodular fasciitis (NF) and granulation tissue. In IRF, most fibroblasts immunostained strongly for hla-dr antigens, whereas fibroblasts in NF and granulation tissue did, not immunostain at all. The fibroblasts did not immunostain for interleukin 2 receptor, C3b receptor, CD-4, CD-8, or Leu-M1 in any of the tissue studied. Most macrophages and lymphocytes in IRF and NF immunostained Strangly for hla-dr antigens. In IRF, the CD-4 and CD-8 immunostained t-lymphocytes appeared equally distributed. The expression of hla-dr antigens by fibroblasts in IRF indicates that this rare disease may indeed be an immune-associated hypersensitivity disorder.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/7. Eosinophilic fasciitis during pregnancy.The first reported case of eosinophilic fasciitis developing in pregnancy is discussed, and the obstetric management is reported. Eosinophilic fasciitis is a rare disease characterized by pain, swelling, and tenderness over the extremities, followed by induration of the skin. Laboratory findings include peripheral eosinophilia, hypergammaglobulinemia, an elevated erythrocyte sedimentation rate, distinctive histopathologic changes, and scleroderma-like skin induration without rheumatoid serologic markers. diagnosis is made by biopsy of the deep fascia of the affected area. The deep fascia will be infiltrated with plasma cells, lymphocytes, and eosinophils. prednisone is the therapy of choice. carpal tunnel syndrome and rare serious hematologic abnormalities have been associated with eosinophilic fasciitis.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/7. Eosinophilic fasciitis and aplastic anaemia.Aplastic anemia developed in a 66-yr-old farmer with eosinophilic fasciitis. The concurrence of these 2 rare diseases is noteworthy. The case is presented and the relevant literature is briefly reviewed.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |