[Piebaldism. Clinical, pathological and ultrastructural study of three cases (author's transl)].
Comparative study of achromic and normally pigmented skin of three piebald patients from two families is reported here. DOPA-positive cells were not seen in the achromic areas, but the number of ATPase sites appeared to be normal; therefore the assumption of the replacement of the melanocytes by Langerhans cells cannot be supported. Through electron microscope, Langerhans cells looked strictly normal. Melanosomes appeared reduced in size and some alterations were noticed in their shape and contents. Ultrastructurally, a more prominent change consisted in increased undefined clear cells in the achromic skin. These dendritic suprabasal cells were devoid of melanosomes or Langerhans granules and might represent undifferentiated melanocytes. These features were discussed according to previous literature.
D000255:Adenosine Triphosphate / Q000032:analysis; D000328:Adult; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007801:Langerhans Cells / Q000201:enzymology / Q000473:pathology*; D010375:Pedigree; D010859:Pigmentation Disorders / Q000175:diagnosis* / Q000201:enzymology / Q000235:genetics; D012867:Skin / Q000473:pathology*
D000255:Adenosine Triphosphate
Piebaldisme. Etude anatomo-clinique et ultrastructurale de 3 cas.