[Pseudoxanthoma elasticum: a study of 22 cases]. / Le pseudoxanthome elastique: etude de 22 cas.

Pseudoxanthoma elasticum is a heritable connective-tissue disorder affecting the eye, skin, and vascular system. The aim of our study was to specify the epidemiological and clinical characteristics of this disease in a hospital series. A total of 22 patients were included during a period of 19 years. There were 15 women and 7 men. Patient's mean age was of 36,1 years. Parental inbreeding was reported in 10 cases. However, the parents were apparently healty. All patients exhibited cutaneous signs. The association between pseudoxanthoma elasticum and elastosis perforans serpiginosa was reported in two cases. The histological features were characteristic in all cases. The ophthalmologic examination realized on 13 patients revealed angioid streaks in 6 cases and a orange peel aspect in one case. Cardiac evaluation performed in 11 patients did not reveal specific PXE abnormalities. A follow-up was recommended in the majority of the cases. In conclusion, a family history of PXE was frequently reported in our study mainly among brothers and sisters. The disease was probably of autosomal recessive transmission. Cutaneous lesions were often the first signs. A list of laboratory investigations is useful to search for asymptomatic visceral localizations and specify their frequency.

Dyschromatosis universalis hereditaria: two cases.

Dyschromatosis universalis hereditaria is a rare genodermatosis reported initially and mainly in Japan. However, subsequent cases have been reported from other countries. We describe two Tunisian cases of dyschromatosis universalis hereditaria in a 3-year-old and a 3-month-old girl. They presented to our department with asymptomatic progressive mottled pigmentation over the trunk and limbs, which had been noted since birth and had become more noticeable with age. Palms and soles were also affected in the first case. The two patients did not have any systemic or other cutaneous illness. They were born to healthy, second-degree consanguineous parents (case 1) and non consanguineous parents (case 2), following an uneventful pregnancy. No family members had a similar appearance. Physical examination revealed numerous, generalized, hyperpigmented macules interspersed with spotty de-pigmented macules. Hair, nails, teeth, and mucosae were normal. Systemic examination did not reveal abnormalities. Histological exam revealed basal layer hypermelanosis with pigmentary incontinence in some areas. So based on those findings a clinical diagnosis of DUH was made and the patients were followed up in our department for periodic general evaluation of their skin. After a follow up of 12 months, the first child didn't develop other lesions, but palms and soles were also involved in the second case.

Isolated genital annular lichen planus.

Annular lichen planus is a rarely reported variant of lichen planus (LP). Although genital lesions are frequent in patients with LP, isolated genital LP is rarely reported. We present a case of a 29-year- -old circumcised man with an asymptomatic annular lesion of the penis. Histopathological features were consistent with LP. Topical clobetasol was prescribed, with clinical improvement. It is important to consider annular LP among the possible diagnoses of individual annular genital lesions.