[Pseudoxanthoma elasticum: a report of 11 cases].
BACKGROUND
Pseudoxanthoma elasticum (PXE) is an inherited disorder of elastic tissue with many systemic manifestations


METHODS
We performed a retrospective study from all the patients diagnosed with PXE at the department of dermatology of La Rabta hospital of Tunis, between 1986 and 2003.


RESULTS
During the observation period, we identified 11 patients with PXE, 5 males and 6 females with a mean age of 28 years (10-47 years). Family history was found in 5 patients. Exhibit yellowish, pigskin, and popular lesions on the sides of the neck were observed in all cases. Systematic ophthalmologic examination revealed angioïd streaks in 4 patients. No abnormalities were found in cardiovascular and metabolic explorations.


CONCLUSIONS
Diagnosis of PXE is based on clinical, histological and genetic criteria. Ocular and cardiovascular damage make all the gravity of the disease, from where interest of an ophthalmologic and cardiovascular examination systematic.
Zaraa
Inès
I
Service de Dermatologie, Hôpital La Rabta, Tunis, Tunisie.
Cherif
Faika
F
Abdelmoula
Fadoua
F
Mokni
Mourad
M
Ben Tekaya
Naoufel
N
Haouet
Slim
S
Ben Osman
Dhahri Amel
DA
D000328:Adult; D000793:Angioid Streaks / Q000175:diagnosis; D001706:Biopsy; D002114:Calcinosis / Q000473:pathology; D002648:Child; D004547:Elastic Tissue / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D009333:Neck / Q000473:pathology; D011561:Pseudoxanthoma Elasticum / Q000175:diagnosis* / Q000235:genetics; D012189:Retrospective Studies