ARTICLE INFO ABSTRACTHypereosinophilic syndrome (HES) is a multi-organ disease (heart, eye, lungs or nervous tissue). The etiology and pathogenesis of HES is still unknown. It is characterized by 1500 eosinophils/µl of eosinophils in peripheral blood observed during at least six months without any evidence for other known causes of eosinophilia. The skin manifestations of the disease were pruritic, erythematous macules, papules, plaques or nodules and rarely urticaria, angioedema, livedo reticularis, eosinophilic cellulitis, cutaneous necrotizing eosinophilic vasculitis, retiform purpura. Among the skin lesions erythroderma is a rare complication of HES and has only been reported in a few cases (Launay et al., 2002, Granjo et al., 2002. We report here a 78-yrs-old man with pruriginous erythroderma leading to the diagnosis of hypereosinophilic syndrome. In dermatological examination erythrodermia and mild desquamation was noticed entire the body surface with bilateral ectropion.
Goltz Syndrome (Focal Dermal Hypoplasia) is a rare congenital mesoectodermal dysplasia with multisystemic involvement. It was first described by Goltz in 1962 and more than 200 cases reported worldwide (Leite et al., 2005). Skin lesions are characterized by athrophic, hyperpigmented linear or reticulated macules, multiple mucocutaneous papillomas, fat herniations or fat tumors. Besides the skin, other structures frequently involved are the skeletal system, eyes, teeth, hair, nails and central nervous system (Kanitakis et al., 2003). The association between Goltz syndrome and anti-HIV serology was not found in the literature. We report 32 year-old woman with typical cutaneous leisons of Goltz Syndrome associated with false positive anti-HIV serology and secondary infertility.J. Exp. Clin. Med., 2010; 27:123-125 Journal of Experimental and Clinical MedicineDeneysel ve Klinik Tıp Dergisi ARTICLE INFO ABSTRACT© 2010 OMU All rights reserved Case ReportA 32 year old woman was evaluated for facial dysmorphism, poikilodermic skin lesions, multiple verrucous and papillomatous lesions of the perivulvar region, short stature and kyphoscoliosis causing functional and esthetic discomfort. In her history, she had one abortus in the past, since then she could not become pregnant and had been followed up because of the secondary infertility. She was the seventh child of the non relative parents. She has five sisters and a brother all of whom were healthy. She also had three sisters died of unknown aetiology when they were under age of five.On physical examination she had sparse hair, recession of the frontal hair line and patchy areas of cicatricial alopecia with facial asymmetry. The right mandible was hypoplastic and the nasal bridge was narrow with a broad tip and unilateral notching of right ala nasi, she had low set ears and a triangular-shaped face due to the hypoplastic right orbital region, micrognathia and microdentia.She had anophthalmia on the right side. Her right eye was enucleated at another hospital with an unknown reason. The eyelids were irregularly shaped and could not be closed completely. Ophtalmologic examination of the left eye revealed choroid coloboma (Fig. 1).Her teeth were hypoplastic, anodontic, irregularly spaced, inclined to decay and upper incisors were notched. Multiple milimetric papillomas on the gingiva were noticed. Examination of the lower extremity revealed partial syndactyly between the different toes of right and left feet.Cutaneous examination showed multiple atrophic, pigmented linear streaks and poikiloderma on the trunk and extremities along the lines of blaschko and telangiectasias with atrophic depressions observed on the back of the patient (Fig. 2). Soft yellowish, lipomatous nodules projecting through localized areas of skin atrophy were noticed on shoulders -flanks and legs .Histologic examination of the papillomatous inguinal lesion showed acanthosis and papillomatosis overlaid by thickened, partially parakeratotic stratum corneum. Also lipomatous nodule of the infrapatellar...
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