1/8. Raynaud's phenomenon possibly induced by a compund drug of tegafur and uracil.We describe a 50-year-old woman who noted acral hyperpigmentation, sclerodactyly and Raynaud's phenomenon with 1:160 of antinuclear antibody titer after treatment with a compound drug with tegafur and uracil. Histological findings of the finger and palm included hyperkeratosis, vacuolar degeneration of basal cells, thickened collagen fibers in the dermis, and dilatation of capillary vessels, perivascular mononuclear cell infiltration and melanophages in the upper dermis. IgG, IgA, IgM, C3 and C1q were not deposited in the skin by direct immunofluorescence study. After cessation of the drug, Raynaud's phenomenon and hyperpigmentation disappeared within 1 month and 4 months, respectively, and antinuclear antibody turned negative within 4 months. These observations suggest that tegafur may have caused not only hyperpigmentation in the palms and soles, but also sclerodactyly and Raynaud's phenomenon in the present case.- - - - - - - - - - ranking = 1keywords = dermis (Clic here for more details about this article) |
2/8. Digital ulcers and necroses: novel manifestations of angiocentric lymphoma.We describe a patient with angiocentric lymphoma whose presenting features were multiple areas of digital ulceration and necrosis, including deep ulcers on both great toes. He lacked the lateral halves of both earlobes because of multiple ulcers. skin biopsy revealed a patchy and diffuse infiltrate of lymphoid cells with nuclear atypia in the dermis and subcutaneous tissue. Angiocentric and angiodestructive features of the lymphoid cells, a prominent histiocytic infiltrate and some epithelioid cell granulomas were found. The results of immunohistochemical staining showed a T-cell phenotype, and showed positive staining for apoptosis. He died in July 1999. Peripheral vascular disturbances including Raynaud's phenomenon, digital skin ulcers and necroses are novel clinical symptoms in patients with angiocentric lymphoma, which should be added to the differential diagnosis in patients with peripheral vascular disturbances.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
3/8. hypereosinophilic syndrome presenting as cutaneous necrotizing eosinophilic vasculitis and Raynaud's phenomenon complicated by digital gangrene.Cutaneous necrotizing eosinophilic vasculitis is a recently identified type of vasculitis that is characterized by an eosinophil-predominant necrotizing vasculitis affecting small dermal vessels. Clinically, it presents with pruritic erythematous and purpuric papules and plaques, peripheral eosinophilia and a good response to systemic steroid therapy. This vasculitis can be idiopathic or associated with connective tissue diseases. Although the pathogenic roles of eosinophil-derived granule proteins and interleukins have been documented in diseases associated with eosinophilia, a role of CD40 (a glycoprotein of the tumour necrosis factor receptor superfamily) has rarely been described. We describe two patients with idiopathic hypereosinophilic syndrome (HES) presenting with multiple erythematous patches and plaques on the lower extremities and Raynaud's phenomenon. They satisfied the criteria for the diagnosis of HES by clinical and laboratory investigations. Histopathology of the cutaneous lesions revealed prominent eosinophilic infiltration with local fibrinoid change in vessel walls in the dermis and subcutis. Immunohistochemical detection of CD3, CD4, CD8 and CD40 was performed. Infiltrating eosinophils were strongly stained by anti-CD40 monoclonal antibody. One patient improved with prednisolone, pentoxifylline and nifedipine, without recurrence. The other patient initially improved with steroids, but after self-withdrawal of steroid developed digital ischaemia that evolved to severe necrosis and required amputation. Cutaneous necrotizing eosinophilic vasculitis, Raynaud's phenomenon and digital gangrene may develop as cutaneous manifestations of HES. CD40 may play a part in the pathogenesis of eosinophilic vasculitis in HES.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
4/8. The role of the intercellular matrix in dermal calcinosis of the CRST syndrome. An electron-microscopic study.We performed an electron-microscopic study on calcium deposits in two patients with calcifying scleroderma (CRST syndrome). calcium deposits were detected both intracellularly in the mitochondria of phagocytic cells and extracellularly. Needle-like crystals measuring up to 4,500 A in length and approximately 60 A in width were present in both the Von Kossa-positive regions and the apparently normal dermal areas around the calcification sites. Although the fibrillar matrix's ultrastructure was normal, hollow oxytalan fibrils were detected. Slightly electron-opaque, star-shaped material was observed among the fibrillar component of the matrix (matrix granules), which is the ultrastructural expression of some types of proteoglycans containing keratan sulphate and chondroitin sulphate. These granules cannot be detected in normal dermis. The extrafibrillary calcium deposits on these mucopolysaccharide structures may represent an early event in the complex pathogenesis of calcification in the CRST syndrome.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
5/8. Development of severe limited scleroderma in complicated Raynaud's phenomenon after limb immobilization: report of two cases and study of collagen biosynthesis.We describe 2 patients with Raynaud's phenomenon who developed severe sclerodermatous changes after immobilization. The scleroderma was confined to the immobilized limb. Histologic examination of tissue samples of the affected skin showed mononuclear cell infiltrates and marked collagen deposition in the dermis and subcutaneous adipose tissue. in vitro biosynthetic studies demonstrated a dramatic increase in collagen production by cultures of involved skin from one of the patients. These results suggest that immobilization resulted in activation of dermal fibroblasts (perhaps mediated by inflammatory cells) and in the subsequent development of fibrosis in both of these patients.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
6/8. Acrosclerosis associated with vibration: an electron microscopic study.A case of typical vibration-induced acrosclerosis was studied with the electron microscope. In addition to increased collagenization of the dermis, vascular wall and perivascular spaces, a severe vacuolization and an increased number of lysosomes in the vascular endothelia cells were observed. In many peripheral nerves, axonal degeneration, demyelination and an extensive collagenization of peri- and endoneurium were observed. These changes appeared to be the organic basis for sclerosis and neuropathies of this condition.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
7/8. Silicone granuloma in acral skin in a patient with silicone-gel breast implants and systemic sclerosis.A 68-year-old white woman was referred to us by her rheumatologist for possible participation in a clinical study of photopheresis for scleroderma. In February 1993, she noticed edema of her distal phalanges, Raynaud's phenomenon in both hands, flu-like symptoms, fatigue, intermittent diarrhea, abdominal pain, tearing in both eyes, dyspnea on exertion, dysphagia, and odynophagia. Bilateral silicone-gel breast implants had been placed 12 years before; 2 months before her present evaluation, they were removed and found to be ruptured. physical examination revealed edema, limited to the fingers and hands bilaterally, and slight induration of the skin on the dorsum of both hands and distal forearms. The remainder of the physical examination was normal. According to our study protocol, a skin biopsy specimen from the dorsum of the right hand was taken, but all other laboratory investigations were refused. Histopathologic examination revealed multiple clear spaces of varying sizes in the dermis and multinucleated macrophages containing small refractile particles, characteristic of silicone granuloma (Figs. 1 and 2); however, the specimen showed no evidence of scleroderma. X-ray energy dispersive analysis by scanning electron-microscopy confirmed the presence of elemental silicon in the small refractile particles. The patient did not receive any treatment after her diagnosis and shortly thereafter, she was lost to follow-up.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |
8/8. Raynaud's phenomenon and digital necrosis induced by interferon-alpha.We report two patients treated with interferon-alpha who developed Raynaud's phenomenon followed by multiple digital necrosis. Arteriography of the legs revealed diffuse distal narrowing. Histological examination of the resected tissue showed ischaemic necrosis within the dermis and subcutaneous tissue without vasculitis. Resolution of the symptoms was observed when interferon-alpha was withdrawn. Since other causes of Raynaud's phenomenon and digital necrosis were ruled out, a drug cause is likely. Raynaud's phenomenon should be looked for in patients treated with interferon-alpha. If present, immediate withdrawal of the drug is required.- - - - - - - - - - ranking = 0.5keywords = dermis (Clic here for more details about this article) |