Aim of the study. The authors evaluated the connection between clinico-histological skin aspects and visceral and ophthalmological involvement in 40 patients affected with pseudoxanthoma elasticum, who had been followed for 3 years. Design. Comparative evaluation of patients conditions. Environment. The availability of a laboratory of cutaneous histopathology and of high-level ophthalmological consultants is mandatory. Patients. 40 patients (18 males; 22 females) affected with PXE type 1 after Pope (age range 31-66; mean 53.4 yrs). Methods. Skin biopsy and histopathological examination of samples (H and E, Von Kossa, Weigert-Van Gieson); fundus oculi; slit lamp; fluoroangiography of retinal vessel; blood analysis and urinalysis of typical metabolites and HLA typization. Evaluation. Measurement of thickness and semiquantitative evaluation of histological skin lesions/evaluation of subjective ophthalmological conditions and of objective retinal involvement/evaluation of splanchnic and vascular involvement through imaging techniques. Conclusions. The entity of the ophthalmic syndrome appears to be related to the entity of the cutaneous involvement, and frequently tends to precede it. Anyway, examples are given in which an early diagnosis can be posed through careful evaluation of scanty cutaneous objectivity.
|Translated title of the contribution||Splanchnic, ophthalmological involvement and skin morphological aspects of pseudoxanthoma elasticum. Results of a three-year follow-up|
|Number of pages||5|
|Journal||Giornale Italiano di Dermatologia e Venereologia|
|Publication status||Published - 1993|
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