One Family Report Of Piebaldism Genealogical

Objective : To diagnosis a family with piebaldism and study it's etiological factor, pathogenesy, clinical phenotypes and remedial methods. Methods : The clinical data of the family with piebaldism were collected and approached, the lesion that resourced the patchy hypopigmentation of the proband's mother were carried out histio-pathology examination. Results : A family with eight affected members spanning four generations of fourty-two person is presented, the three of them are male, the five are female. The proband who is a four-month-old boy had congenital depigmented patches on his fore head, forehead, abdomen, both lower extremities, left elbow and hair from which melanocytes are absent since birth. The depigmented patches showed progressive depigmentation following his growth. His mother had depigmented patches on her fore shanks since birth. The hyperpigmented macules of depigmented presented progressive loss of pigmentation and augmentation in the wake of her age. However, the patchs' appearance and proportion was the static course. The other patients has a similar congenital pigment disorder, the white lithodomous hair of three patients were already completely formed at birth, the other patients have not. The histo-pathology of the proband's mother revealed : coats airily depauperated, trochanterelluses flattened, melanocytes among basal cells vanished or decreased, a few of leukomonocytes infiltrated around blood vessel of the dermis. Conclusions : Eight affected members patients of a family are diagnosised of piebaldism.