Über einen Fall von Thomson-Syndrom und Epidermolysis bullosa dystrophica-artigen Hautveränderungen

Kindler Syndrome: Report of a Case with Ultrastructural Study and Review of the Literature

“…Since the first description, approximately 70 cases of congenital poikiloderma with blisters and keratosis have been reported worldwide; 16 of these, in 13 families, are like our patient, similar to the original observation of Kindler (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17). A review of these cases reveals the following major features: congenital or neonatal blistering, photosensitivity (both conditions improving during childhood), progressive poikiloderma, and cutaneous atrophy (main feature is cigarette-paper appearance).…”

“…In three patients the age of onset of blistering was not mentioned (12)(13)(14). Photosensitivity was absent or not certain only in three patients (6,12,14) and not mentioned in another three (4,5,11). Poikiloderma starting on sunexposed areas and extending to nonexposed areas was present in all patients and represented by pigmentation, atrophic spots, reticular erythema, and telangiectases (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12).…”

“…Poikiloderma starting on sunexposed areas and extending to nonexposed areas was present in all patients and represented by pigmentation, atrophic spots, reticular erythema, and telangiectases (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). Diffuse cutaneous atrophy was reported in all of them (1)(2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12). The skin appeared thin and cigarette-paperiike; these features were most pronounced on the dorsa-of the hands and feet.…”

Poikiloderma of Theresa Kindler: Report of a Case with Ultrastrectural Study, and Review of the Literature

“…Blisters were congenital in eight patients (1)(2)(3)(4)(5)(6) and appeared during the first days of life in three (7-9); the age of onset in infancy was not mentioned in the others (Table 1) (10-12). They were mostly acral (dorsa of hands and feet).…”

“…Photosensitivity was reported as both severe (5,8,11) and less severe (l,6,7,our patient). It was also noted to be absent (4), not certain (10,12), or not mentioned in the other cases (2,3,9). Photosensitivity was manifested as facial erythema after min-imal sun exposure and worsening of blister formation in summer, and/or severe sunburn (5).…”