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September 1981

Dyskeratosis Congenita and Intracranial Calcifications

Arch Dermatol. 1981;117(9):523. doi:10.1001/archderm.1981.01650090003003

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To the Editor.—  In the December 1979 Archives (115:1437-1439), Mills et al described two brothers with dyskeratosis congenita (DC) who also had massive intracranial calcifications detected on skull roentgenograms. The authors stated that this finding had not been previously described in patients with this syndrome, although only a small number of pertinent negative statements could be found in the literature.

Report of Cases.—  We have recently observed two West Indian brothers, aged 21 and 15 years, with DC. Both of them have the characteristic reticulated cutaneous hyperpigmentation and dystrophy of the nails. Neither of them has leukoplakia, although the younger brother has splotchy hyperpigmentation of the oral cavity. Both have mild anemia (hematocrit readings, 37.1% and 37.7%), leukopenia (leukocyte levels, 3,300 and 3,000/cu mm), and thrombocytopenia (platelet levels, 20,000 and 45,000/cu mm). There has been no evidence of malignant neoplasms to date.Neither brother has had any neurologic disorders, although

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