Dermatofibrosis lenticularis disseminata mit Osteopoikilie

Abstract

The cases of Dermatofibrosis lenticularis disseminata (DLD) published under this name are specified in tabular form. It appears that only 11 of the 21 cases can be coordinated to the Buschke-Ollendorff-syndrome – namely those with DLD and O. After explaining an own observation the cases hitherto published are discussed, and the pros and cons of their classification in the syndrome are treated. – Special attention is called to the participation of the elastic tissue to the DLD as proved by us.

Buschke — Ollendorff syndrome in infant patients

“…В зависимости от этого G.E. pierard [1] Синдром бушке -Оллендорф (dermatofibrosis lenticularis desseminata) с остеопойкилией (osteopathia condensans disseminata -наследственное аутосомнодоминантное заболевание с неполной пенетрантностью и вариабельной экспрессивностью, характеризующееся сочетанием соединительнотканного невуса с остеопойкилией [1,2,3,4,[6][7][8][9][10][11][12][13][14].…”

“…Они назвали изменения на коже «диссеминированной лентикулярной дерматофибромой» [5]. Термин «синдром бушке -Оллендорф» ввел A. Schimpf в 1979 году [6].…”

Buschke-Ollendorff syndrome-disseminated dermatofibrosis with osteopoikilosis

“…There is no sharp line of demarcation from the normal dermis and no evidence of inflammatory reaction. Subcutaneous fibrosis may also occur and in some lesions elastic fibres may be increased and or show thickening and clumping (Smith & Waisman, i960;Danielsen, Midtgaard & Christensen, 1969;Raque & Wood, 1970;Schimpf, Roth& Kopper, 1970;Schorr, Opitz & Reyes, 1972). CASE REPORT Mrs E.B.…”