ABSTRACT
Focal dermal hypoplasia [Goltz syndrome] is a rare congenital syndrome, found primarily in females. This syndrome is characterized by a wide range of meso-ectodermal defects. We report on 3 cases [one male and 2 females] who had typical manifestations of this syndrome. The skin lesions comprised of linear hypoplasia and vegetating tumors. They had significant skeletal and dental defects, and ocular abnormalities. Histopathologic examination of the skin lesions showed a markedly thinned dermis that was replaced by adipose tissue. About 200 cases of the syndrome have been reported worldwide, but to our knowledge, this is the first report from Iran
Subject(s)
Humans , Male , Female , Focal Dermal Hypoplasia/geneticsABSTRACT
Cutaneous leishmaniasis is pervalent in Iran. There are many unusual clinical presentations in acute cutaneous leishmaniasis [ACL]. Patients with infiltrative leisons over the face and nose referring to our department were studied. Fourteen female patients aged 45-74 had this unusual presentation. Direct smear and leishmanin skin test were positive in all of them, and histologically they showed cutaneous leishmaniasis. Other paraclinical data were within normal limits. All the patients responded well to methylglucamine antimonate treatment and their lesion healed leaving no scars. Erysipeloid type of leishmaniasis is an unusual clinical presentation of cutaneous leishmaniasis. The reason why this special presentation is seen only in women is not clear. However, host defense mechanisms, hormonal changes of the body [they were all elderly women], and fragility and friability of the nose and face skin due to senility may be involved