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Epidermolytic hyperkeratosis: generalized form in children from parents with systematized linear form.
I Clinica Dermatologica, University of Milan, Italy.
Two unrelated families are presented in both of which a child with generalized epidermolytic hyperkeratosis (congenital bullous ichthyosiform erythroderma) had a parent with linear epidermolytic hyperkeratosis (epidermolytic epidermal naevus). Light and electron microscopy of skin biopsies of lesions from the children and parents showed typical epidermolytic hyperkeratosis. Gonadal mosaicism in patients with linear epidermolytic hyperkeratosis may be responsible for transmission of the abnormality to the offspring.
PMID: 2182100 [PubMed - indexed for MEDLINE]
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Cited by 2 PubMed Central articles
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Allelic loss underlies type 2 segmental Hailey-Hailey disease, providing molecular confirmation of a novel genetic concept.
Poblete-Gutiérrez P, Wiederholt T, König A, Jugert FK, Marquardt Y, Rübben A, Merk HF, Happle R, Frank J.
J Clin Invest. 2004 Nov; 114(10):1467-74.
[J Clin Invest. 2004]
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ReviewNeonatal erythroderma: differential diagnosis and management of the "red baby".
Hoeger PH, Harper JI.
Arch Dis Child. 1998 Aug; 79(2):186-91.
[Arch Dis Child. 1998]