 |  | Color Atlas of Pediatric DermatologySection 11. Genodermatoses | |
Topics Discussed: genetics and dysmorphology; pseudoxanthoma elasticum.
Sections: Cutis Laxa, Ehlers-Danlos Syndrome, Focal Dermal Hypoplasia (Goltz Syndrome), Focal Dermal Hypoplasia, Incontinentia Pigmenti, Cutaneous Expressions of the Wiskott-Aldrich Syndrome, Cutaneous Expressions of Ataxia Telangiectasia, Bloom's Syndrome, Poikiloderma Congenitale (Rothmund-Thomson Syndrome), Cutaneous Expressions of Cockayne's Syndrome, Congenital Ectodermal Dysplasia, Hay-Well's Syndrome (AEC), Pachyonychia Congenita, Dyskeratosis Congenita, Neurofibromatosis (Von Recklinghausen's Disease), Neurofibromatosis (Lisch Nodule), Mucosal Neuroma Syndrome, Tuberous Sclerosis, Buschke-Ollendorf Syndrome, Basal Cell Nevus Syndrome, Xeroderma Pigmentosum.
Excerpt:"This is a generalized condition in which elastic fibers are degenerative. Clinical signs of the phenomenon can be recognized in the skin and eyes. In the skin, patches of yellowish discoloration and general laxness or redundancy develop on the neck ("chicken skin"), in the axillae, and in other places, such as the fossae of limbs and the inguinal folds, where considerable movement of skin is normal. In the eye, the so-called angioid streaks can be seen. They represent the result of faulty elastic fibers in Bruch's membrane and generally precede the cutaneous changes. These eye changes frequently result in the loss of central vision and sometimes result in almost complete peripheral vision is maintained blindness...."
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