1/5. Small vessel vasculitis and relapsing panniculitis in tumour necrosis factor receptor associated periodic syndrome (TRAPS).case reports: A 66 year old female patient had relapsing fever and non-suppurative panniculitis suggestive of enigmatic "Weber-Christian disease" (WCD). Antineutrophil cytoplasmic antibodies with specificity for human leucocyte elastase (HLE-ANCA) were detected. A biopsy showed small vessel vasculitis and panniculitis. A 53 year old man had recurrent episodes of abdominal pain, erythematous rash, and myalgia. Fever attacks had stopped a few years ago. A biopsy showed panniculitis and fasciitis. In both patients mutations (R92Q, T50M) of the tumour necrosis factor receptor super family (TNFRSF) 1A gene were disclosed. Mutations of the TNFRSF 1A gene are the cause of tumour necrosis factor receptor associated periodic syndrome (TRAPS). Both patients responded favourably to treatment with the human soluble p75 TNF alpha receptor fusion protein etanercept (2 x 25 mg subcutaneously/week). DISCUSSION: Small vessel vasculitis and panniculitis have not been reported in TRAPS so far. The cases underline the importance of TNF alpha regulation in inflammatory processes including vasculitis. Genetically determined causes of fever may account for some cases of WCD.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
2/5. Cutaneous manifestations of Takayasu's arteritis. A clinicopathologic correlation.Takayasu's arteritis is a chronic, granulomatous, large-vessel arteriopathy of unknown cause. We retrospectively reviewed the medical records of 38 patients with Takayasu's arteritis and identified 21 with cutaneous findings. Seven patients had lesions that were related to their systemic vasculitis. We found a Churg-Strauss granuloma, a pyodermatous leg ulcer, and inflammatory leg nodules in these patients. biopsy specimens from three patients with presumed "erythema nodosum" did not support the clinical diagnosis but did show arteritis. In patients with Takayasu's arteritis, small-vessel inflammation, and other inflammatory lesions may be present, in addition to large-vessel disease. Histopathologic study is necessary to categorize the nature of inflammatory leg nodules of these patients.- - - - - - - - - - ranking = 0.5keywords = vessel (Clic here for more details about this article) |
3/5. Lupus erythematosus panniculitis(profoundus).A 39 year-old man developed on trunk, buttock and thighs scaling, atrophic, erythematous lesions which in some places became infiltrated and ulcerated. The clinical, histopathological and immunlogical picture corresponded with the diagnosis of discoid lupus erythematosus. In sites of infiltrations and necrosis, however, histological features of panniculitis with proliferative and inflammatory changes of deep vessels could be seen. The direct immunofluorescence of uninvolved skin showed deposits of IgM and C3 at the dermal-epidermal junction and within the vessel walls. The patient responded promptly to treatment with chloroquine.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |
4/5. Systemic form of Weber-Christian disease.A case of systemic Weber-Christian disease, involving retroperitoneal adipose tissue with secondary damage to the associated organs and blood vessels is reported. The involved organs showed varying degrees of necrosis, lipogranulomata and fibrosis.- - - - - - - - - - ranking = 0.16666666666667keywords = vessel (Clic here for more details about this article) |
5/5. Tubular cytoplasmic inclusions in a case of childhood dermatomyositis with migratory subcutaneous nodules.A boy with dermatomyositis which began at the age of one year and five months showed multiple migratory subcutaneous nodules, which have seldom been described. Histological and electron microscopic studies of muscles and subcutaneous nodules demonstrated the following interesting findings. 1. light microscopy. The migratory subcutaneous nodules consisted of non-suppurative panniculitis and ischaemic adipo-necrosis as a sequel to vascular lesions. This finding suggests that the nodules may have arisen from the subcutaneous adipose tissue which had been severely damaged by systemic angiopathy. 2. Electron microscopy. Examination of the vessels in muscle and subcutaneous nodules showed tubular cytoplasmic inclusions with a diameter of approximately 250 A in the endoplasmic reticulum of vascular endothelial cells. These observations provide strong support for the concept that the fundamental pathologic process in childhood dermatomyositis is of a vascular nature, and the primary lesion is in the walls of the intramuscular blood vessels.- - - - - - - - - - ranking = 0.33333333333333keywords = vessel (Clic here for more details about this article) |