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Poikiloderma of Theresa Kindler: Report of a Case with Ultrastrectural Study, and Review of the Literature
Author(s) -
Hovnanian Alain,
BlanchetBardon Claudine,
Prost Yves
Publication year - 1989
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/j.1525-1470.1989.tb01003.x
Subject(s) - poikiloderma , medicine , epidermolysis bullosa , dermatology , dermoepidermal junction , pathology , dermis
Kindler's syndrome occurred in a 13‐year‐old boy with parental consanguinity. The patient had acrai congenital blistering and photosensitiviiy, both regressive, at 12 years of age, together with diffuse progressive poikiloderma, marked cutaneous atrophy, mild hyperkeratosis of the paEms, gingival hypertrophy and fragility, and anal stenosis. The ultrastructural study of butlae induced by rubbing showed clefts in three levels of the dermoepidermal junction zone. A review of 14 cases in the literature consistent with KIndler's syndrome showed this to be a definite clinical entity among the other bullous hereditary poilcilodermas. In two cases, ullrastructural studies showed Intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and the other bullous hereditary poikiiodermas.