Hereditary hypotrichosis (Marie-Unna type) and juvenile macular degeneration (Stargardt's maculopathy)

Summary Hypotrichosis of the Marie‐Unna variety is a distinctive syndrome eponymously named following a publication in 1925 describing a family in which 27 individuals in seven generations were affected by a previously unreported type of hypotrichosis. Its inheritance is determined by an autosomal d...

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Bibliographic Details
Published in:Clinical and experimental dermatology 1992-05, Vol.17 (3), p.189-191
Main Authors: MARREN, P., WILSON, C., DAWBER, R.P.R., WALSHE, M.M.
Format: Article
Language:English
Subjects:
Child
Child, Preschool
Female
Humans
Hypotrichosis - complications
Hypotrichosis - genetics
Macular Degeneration - complications
Macular Degeneration - genetics
Male
Pedigree
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Summary:Summary Hypotrichosis of the Marie‐Unna variety is a distinctive syndrome eponymously named following a publication in 1925 describing a family in which 27 individuals in seven generations were affected by a previously unreported type of hypotrichosis. Its inheritance is determined by an autosomal dominant gene and it usually occurs as an isolated abnormality. Hereditary macular degeneration (Stargardt's maculopathy) is also well recognized and has been reported in one family in association with alopecia areata but never in association with Marie‐Unna hypotrichosis. Inheritance of Stargardt's maculopathy is autosomal recessive. Our patient demonstrates the co‐existence of these two uncommon genetic disorders and it would appear that both defects have been independently inherited.
ISSN:0307-6938
1365-2230
DOI:10.1111/j.1365-2230.1992.tb00203.x