[Pellagroid erythema with tryptophan metabolism disorder].
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Biomedical subjects
Publications and source records attributed to L Schnitzler.
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30 specimens of nail fold biopsies have been studied through light and electron microscopy, and direct immunofluorescence, in 26 cases of connective tissue diseases: Raynaud's phenomenon and acrosclerosis (7 cases), systemic scleroderma (10 cases), chronic and acute lupus erythematosus (4 cases), dermatomyositis (5 cases). Numerous tissular changes have been observed by these three different ways: capillaries dilatations, dermal sclerosis, numerous, large, dermal and epidermal colloid bodies, basal lamina replications, collagen's modifications, tubuloreticular inclusions, crystalloid bodies, lysosomial inclusions, immunoglobulins deposits. Thus proximal nail fold appears to be an elective tissular area for collagen diseases study.
Based on five personal cases (one neo-natal case, one in childhood, and three cases in pregnant women) and sixteen published cases, this study remind us of the essential caracteristics of unilateral nevoid telangiectasis. Prevalence in women is noticeable appearance during pregnancy, with complete regression during post-partum, is quasi constant. If their nosological place is not clear among the essential telangiectasias, they seem to be quite different from Hutchinson's serpiginious angioma (verrucous telangiectasis), from Osler-Rendu's disease, from Brocq's telangiectasias and from generalizated telangiectasias. The true signification of all these diseases is still completely unknown.
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Normal structure of nail fold capillaries as observed by capillaroscopy and fluoroscopy is recalled. A procedure of proximal nail fold biopsy is described. The technique is simple without disadvantage for patients. Tissue from the proximal nail fold is studied in three different ways: light microscopy with histochemistry, electron microscopy and direct immunofluorescence. In the two normal cases studied, usual epidermal and dermal components are found. Some particular point may be emphasized: sweat's droplets draining of distal part of proximal nail fold, numerous and large nervous structures, capillaries basal lamina multiplication.
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