Concurrence of Pseudoxanthoma Elasticum, Elastosis Perforans Serpiginosa and Systemic Sclerosis

Acquired disorders of elastic tissue: part I. increased elastic tissue and solar elastotic syndromes

“…Additional reports of sporadic cases in patients with morphea, renal failure, and diabetes mellitus have also been reported. [67][68][69][70] In addition, systemic elastosis, with blood vessel involvement leading to ruptured aneurysms and fatal outcomes, has been reported. 71 These associations have led some investigators to adopt the following classification system, which has not been universally accepted: type 1-idiopathic EPS without associated disease; type 2-reactive EPS with associated connective tissue disease; and type 3-penicillamine-induced EPS.…”

Pseudoxanthoma elasticum and Elastosis perforans serpiginosa

“…Such conditions as Down's syndrome, osteogenesis imperfecta, EhlersDanlos syndrome, Marfan's syndrome, acrogeria and Rothmund-Thompson syndrome have been reported in cases of PXE. A search of the literature has revealed 7 cases of EPS occurring in patients with PXE [1,4,8,[11][12][13], R eed et al [9] described histochemical differences in the elastic fibres involved in PXE and EPS. In the former, the fibres were PTAH-negative and in the latter, PTAH-positive.…”