Scleroderma in a young man.
Scleroderma is a chronic disorder, slowly progressing characterized by diffuse fibrosis of the skin and internal organs. There are functional and structural abnormalities small blood vessels, fibrosis of the skin and internal organs, immune system activation and autoimmunity. Female predominance is most pronounced during mid and late childbearing age, women to men ratio is 7-12 : 1. In this case, we found a diffuse cutaneus scleroderma. Skin biopsy result showed hyperkeratosis, increased dermis thickness with deposition of collagen. The antigenic specificities of antibodies were related to distinct connective tissue disease. The diffuse cutaneus subset was associated with antibodies to topoisomerase I, formerly described as Scl-70 or Scl-86. Anti Scl-70 antibodies were shown in many studies as a marker antibodies for SSC. Colchicine was given in this case, which has the effects of microtubule assembly inhibition and interference with transcellular movement of collagen. During colchicine treatment serum hydroxyproline increased and serum proline decreased, suggesting increased collagen degradation.
Karnen
Farah Prasanti
FP
Department of Internal Medicine, Faculty of Medicine University of Indonesia-Dr Cipto Mangunkusumo Hospital, Jakarta, Indonesia.
Kongko
H R Herry
HR
Kasjmir
Yoga Iwanoff
YI
D000293:Adolescent; D001706:Biopsy; D004264:DNA Topoisomerases, Type I; D003937:Diagnosis, Differential; D004452:Echocardiography; D006801:Humans; D008297:Male; D009687:Nuclear Proteins / Q000276:immunology*; D012595:Scleroderma, Systemic / Q000175:diagnosis*; D012867:Skin / Q000473:pathology
D009687:Nuclear Proteins; C048100:Scl 70 antigen, human; D004264:DNA Topoisomerases, Type I