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F27
THE X-LINKED GENE DEFECT OF ADRENOLEUKODYSTROPHY IS INVOLVED
IN ANDROGENETIC ALOPECIA
1König
A., 1Happle
R., 1Tchitcherina
E., 2Schaefer
J.R., 3Sokolowski
P., 3Köhler
W., 1Hoffmann
R. 1Dept.
of Dermatology, Philipp University, Marburg, Germany; 2Dept.
of Internal Medicine, Philipp University, Marburg, Germany;
3Dept.
of Neurology, Sächsisches Krankenhaus Hubertusburg, Wermsdorf,
Germany.
Adrenoleukodystrophy (ALD) is an X-linked recessive trait
characterized by accumulation of very-long-chain fatty acids
in the brain and other organs such as the adrenal glands.
The ALD gene maps to Xq28 and codes for a peroxisomal membrane
protein which is a member of the ATP-binding cassette transporter
superfamily. Affected men suffer from progressive demyelinization
of the nervous system. Various endocrine and neurological
disorders cause a broad spectrum of clinical manifestations.
This study was performed to delineate the hair status and
type of hair loss in men with ALD. 16 patients were examined.
12 men had male-pattern androgenetic alopecia, Hamilton grades
IV-VIII, 10 of these had reached Hamilton stage VII or VIII.
3 patients showed a female pattern of androgenetic alopecia
(Ludwig grade I or II). The remaining scalp hair was unusually
scarce and thin, 10 of 16 patients showed pronounced madarosis.
ALD gives rise to two different types of hair loss. Affected
men show diffuse hair loss involving the entire scalp and
the eyelashes. Secondly they tend to develop androgenetic
alopecia more frequently and earlier and in a severer form.
The X-linked ALD mutation can be taken as a well-defined gene
within the polygenic spectrum of genes responsible for androgenetic
alopecia.
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