ISSN: 1300-0292
İndekslendiği Dizinler: SCIENCE CITATION INDEX EXPANDED
CINAHL, Index Copernicus,
Chemical Abstracts (CA),
Excerpta Medica / EMBASE
Dil: Türkçe, İngilizce
İçerik: Orijinal Araştırma, Derleme, Editöre Mektup, Olgu Sunumu, Tıp Eğitimi, Tıbbi Kitap İncelemeleri
|
|
|
Primary Complete Pachydermoperiostosis: A Sporodic Case
Çiçek DURUSOY*, Ayşe DİLEK**, Hatice LAKADAMYALI***, Hampar AKKAYA****,
Deniz SEÇKİN*****, Ali HARMAN******
* Uz.Dr., Başkent Üniversitesi Tıp Fakültesi Dermatoloji AD, ALANYA
** Uz.Dr., Başkent Üniversitesi Tıp Fakültesi Fizik Tedavi ve Rehabilitasyon AD, ALANYA
*** Uz.Dr., Başkent Üniversitesi Tıp Fakültesi Radyoloji AD, ALANYA
**** Uz.Dr., Başkent Üniversitesi Tıp Fakültesi Patoloji AD, ALANYA
***** Doç.Dr., Başkent Üniversitesi Tıp Fakültesi Dermatoloji AD, ANKARA
******Arş.Gör.Dr., Başkent Üniversitesi Tıp Fakültesi Radyoloji AD, ANKARA
Purpose: Pachydermoperiostosis, also known as Tauraine-Solente-Gole syndrome, is a rare developmental defect characterized by coarse skin, hyperostosis and clubbing. Herein, a case of complete form pachydermoperiostosis is presented in whom no family history was found. Case Report: A 27-year-old man presented with coarsening of the facial features, furrowing of the skin of the face and scalp after 10 years of progressive thickining of the skin. There was no parental consanguinity and no family history of any similar skin condition. Physical examination showed excessive thickining of the skin folds and accentuation of creases on the face, scalp, forehad and cheeks. Edema of the bilateral eyelids and blepharoptosis of the right eyelid were also noted. There was seborrhoea on the skin of the face and scalp. Enlargement of the extremities, hands and feet, clubbing of the fingers and toes and hyperhidrosis were clearly evident. Hyperkeratotic linear lesions on the palms and soles were also present. Serum osteocalcin levels were high. In light of the clinical manifestations, imaging analysis and pathological findings, complete form pachydermoperiostosis was diagnosed.Results: Although pachydermoperiostosis is belived to be an autosomal dorminant disease, rarely, sporadic cases have also been reported indicating the existence of genetic heterogenity in this condition.Keywords: Osteoarthropathy, Primary HypertrophicTurkiye Klinikleri J Med Sci 2003, 23:245-249
|
|
|
|
|
