Ultrastructural studies of xeroderma pigmentosum.
J Am Acad Dermatol
; 9(6): 876-82, 1983 Dec.
Article
in En
| MEDLINE
| ID: mdl-6643786
ABSTRACT
Electron microscopic observations of both sun-exposed and sun-protected skin from a black patient with xeroderma pigmentosum (XP) revealed abnormal ultrastructural changes in the melanin pigmentary system, tonofibrillar-desmosome complexes, endoplasmic reticulum, mitochondria, and nucleoli of all keratinocytes. The difference between the cellular changes in the sun-exposed skin and that protected from sunlight was quantitative rather than qualitative in character. The most salient changes were seen in sun-exposed specimens melanosomes limited by a membrane showed a high degree of polymorphism with a tendency to form large complexes, and many fibroblast-like cells displayed an intense phagocytic activity for melanosomes, the latter finding not previously reported. The present study supports the conclusions of other investigators that XP is a heterogeneous disease. Clarification of the XP entities by continued research may identify the defective gene and lead to methods of correction by the molecular biologist.
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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Skin Diseases
/
Xeroderma Pigmentosum
Type of study:
Prognostic_studies
/
Qualitative_research
Limits:
Humans
/
Male
Language:
En
Journal:
J Am Acad Dermatol
Year:
1983
Type:
Article