[Leg ulcers with hyperpigmented maculae and white atrophy as manifestation of Klinefelter syndrome].
Two patients, men of 38 and 32 years old, had Klinefelter's syndrome in combination with recurrent lower leg ulcers and hyperpigmentations. For the ulcers no venous insufficiency or other underlying cause could be found. Klinefelter's syndrome is characterised by the existence of at least one extra X-chromosome in phenotypical men. Clinical findings are eunochoid body proportions, scanty facial and body hair, gynaecomastia and small testicles. Plasma follicle-stimulating hormone is usually high and testosterone is reduced in about 50% of the patients. Leg ulcers, especially in combination with hyperpigmentation or atrophie blanche of the surrounding skin, can be a symptom of Klinefelter's syndrome and are not necessarily to be attributed to venous insufficiency.
Veraart
J C
JC
Academisch Ziekenhuis, afd. Dermatologie, Maastricht.
Neumann
H A
HA
Veraart
C J
CJ
Engelen
J
J
D000328:Adult; D001284:Atrophy; D006801:Humans; D007621:Karyotyping; D007713:Klinefelter Syndrome / Q000150:complications* / Q000175:diagnosis; D007871:Leg Ulcer / Q000209:etiology* / Q000473:pathology; D008297:Male; D010859:Pigmentation Disorders / Q000209:etiology; D012008:Recurrence
Ulcera crurum, gehyperpigmenteerde maculae en atrophie blanche als uiting van het syndroom van Klinefelter.