[Pseudoxanthoma elasticum: 11 cases].
BACKGROUND
Pseudoxanthoma elasticum is a congenital dystrophy of the connective tissue. Its clinical expression is cutaneous, ocular and cardiovascular.


METHODS
The aim of our study was to specify the principle characteristics of this disease and to discuss the interest of various supplementary examinations in its diagnosis and control in a series of 11 patients.


RESULTS
The study included 9 women and 2 men (sex ratio: 4.5). The mean age the onset of the symptoms was of 18 years. In 4 cases there was a family history of the disease. All the patients exhibited yellowish, pigskin, and papular lesions on the sides of the neck. The systematic ophthalmologic examination revealed angioid streaks in 7 cases and a pigskin aspect in 4 cases. The systematic cardiovascular and metabolic explorations revealed no abnormalities specific to pseudoxanthoma elasticum. Two cases of asymptomatic nephrocalcinosis were observed.


CONCLUSIONS
In our patients, the disease was probably of autosomal recessive transmission. The predominance of women consulting for the disease would be explained by the esthetic damage. Diagnosis of pseudoxanthoma elasticum is based on clinical, histological and genetic criteria. Supplementary explorations are useful to confirm the diagnosis and also for the search to other, visceral, localizations. Such examinations vary depending on the teams, means and above all the evocative signs.
Service de Dermatologie, CHU Hédi Chaker, Sfax, Tunisie.
M
M
Mseddi
H
H
Turki
B
B
Kammoun
D
D
Sellemi
S
S
Bouassida
J
J
Fki
A
A
Zahaf
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D008297:Male; D009333:Neck / Q000473:pathology; D011561:Pseudoxanthoma Elasticum / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D012737:Sex Factors