Browsing by Author "Kayhan, TC"

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    Hypohidrotic Ectodermal Dysplasia Associated with Glucose-6-Phosphate Dehydrogenase Deficiency
    Ermertcan, AT; Yasar, A; Kayhan, TC; Gülen, H; Ertan, P
    Hypohidrotic ectodermal dysplasia (HED) is a syndrome characterized by hypodontia, hypotrichosis, and partial or total ecrine sweat gland deficiency. The most prevalent form of HED is inherited as an X linked pattern. Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is an X-linked recessive disease, which leads to hemolytic anemia and jaundice. It is expressed in males, while heterozygous females are usually clinically normal. A 12-year-old boy with the complaints of hair and eyebrow disturbances, teeth disfigurement, decreased sweating, and xerosis presented to the outpatient clinic. Dermatological examination revealed sparse hair and eyebrows, conical- shaped teeth, xerosis, syndactylia, transverse grooves, and discoloration of nails. Laboratory findings indicated anemia. His 3-year-old sister also had sparse hair and eyebrows, xerosis, and syndactylia. We learned that the patient had a previous history of neonatal jaundice and a diagnosis of G-6-PD deficiency. Although it has been shown that loci of ectodermal dysplasia and G-6-PD deficiency genes are near to one another, we did not find any case study reporting on occurrence of these two genetic diseases together. With the aspect of this rare and interesting case, the relationship between HED and G-6-PD deficiency was defined. (Ann Dermatol 23(S1) S8 similar to S10, 2011)
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    Calcinosis cutis on the face
    Kayhan, TC; Temiz, P; Ermertcan, AT
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    Lamellar Ichtyosis: Case Report
    Gündüz, K; Kayhan, TC; Gençoglan, G; Inanir, I; Temiz, P
    Introduction: Ichtyosiform dermatoses are a group of hereditary disorders characterized by excessive scaling on the skin. Lamellar ichtyosis is an autosomal recessive disorder. The clinical findings are seen at birth and become widespread and prominent in time; gray-brown scales are seen all over the body. Emollients and keratolytics containing salicylic acid or glycolic acid are used topically. Symptoms are improved significantly by acitretin usage. Case Report: Here we present a 5-year-old girl with lamellar ichtyosis. She was born in a collodion membrane. Besides the skin scales, ectropion and deformities of the nails and ears were observed. Significant clinical improvement was seen with acitretin (10 mg/day) therapy. Conclusion: Lamellar ichtyosis is a severe form of ichtyosiform dermatoses and topical agents may be insufficient. Systemic acitretin treatment improves the symptoms significantly but long term side effects limit its usage.