Erythema dyschromicum perstans.
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Biomedical subjects
Publications and source records attributed to I Kikuchi.
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Three patients had prolidase deficiencies. The family pedigree of these three patients suggests that this rare disorder is inherited through an autosomal recessive gene. this genodermatosis is characterized by a number of signs and symptoms referable to the skin, CNS, teeth, ears, nose, throat, eyes, bones, and joints. Among the skin changes, recalcitrant leg ulcers are the most characteristic. At this time, there is no established method of treatment of this rare disorder, but the use of dapsone was helpful in the treatment of one of our patients.
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Extensive necrosis of the skin, muscle and bone was produced after prolonged cooling in a case of myeloma with cryoglobulinemia. Skin grafting was beneficial.
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A case of incontinentia pigmenti achromians associated with mental retardation, epilepsy, short stature and ocular anomalies was reported. A study of this case together with a review of the 38 cases in the literature revealed that this entity has been associated with central nervous system involvements at a high frequency. It is necessary, therefore, to consider incontinentia pigmenti achromians as a neurocutaneous syndrome from the viewpoint of pediatric neurology.
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