Dermatofibrosis Lenticularis Disseminata and Osteopoikilosis

Connective-Tissue Nevus

“…Three cases reported by Windholz,3 and one reported by Svab4 were accepted by Curth as similar to her original case in her review of the subject in 1934. 13 In one of the cases described by Windholz, the patient had only unilateral involvement of his skin, as did our youngest patient. The more usual bilateral involve¬ ment was demonstrated by our two older patients and the other patients in cases re¬ ported by Windholz,3 Svab,4 Danielsen et al,7 and Serowy.8 Smith and Waisman5 de¬ scribed the case of a father and son with osteopoikilosis and extensive bilateral skin lesions which they classified as "connectivetissue nevi."…”

“…13 Although clinically quite distinctive, with pseudoxanthoma elasticum presenting the most obvious area of confusion; the lesions are less distinctive histologically and present a spectrum of connective-tissue alteration principally involving the elastic or collagen tissue, or both. Cases of connective-tissue nevi have been reported under a number of descriptive terms, depending on whether the elastic tissue fibers or the collagen bundles, or both, were altered, and on variation in the clinical distribution or age at onset of the lesions.…”

Keloids and Hypertrophic Scars

“…Keloid formation has also been associated with progeria, osteogenesis imperfecta, scleroderma, 14 and some inherited connective tissue disorders: Ehlers‐Danlos syndrome, 15 pachydermoperiostosis, 16 osteopoikilosis, 17 trichorhinophalangeal syndrome, 18 and the Rubinstein‐Taybi syndrome 19 . In a series of 574 individuals with the Rubinstein‐Taybi Syndrome, only 28 had lesions diagnosed as keloids and all of these keloid patients were white 19 .…”