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Ann Pathol. 2011 Aug;31(4):246-50. doi: 10.1016/j.annpat.2011.05.009. Epub 2011 Jul 13.

[Kindler syndrome: clinical and ultra-structural particularities, a propos of three cases].

[Article in French]

Author information

  • 1Service de dermatologie, hôpital Charles-Nicolle, Tunis, Tunisie. fekih.nadia@planet.tn

Abstract

INTRODUCTION:

Kindler's syndrome is a rare type of genetic skin condition belonging to the class of bullous poikilodermia. We report three new sibling cases of this rare syndrome.

CASES REPORTS:

The condition was seen in three sisters aged 12, 16 and 20 years, born of a first-degree consanguineous marriage with no family history of Kindler's syndrome. The three patients presented spontaneously regressive bullous eruptions, poikilodermia of gradual onset, major cutaneous atrophy on the back of the hands and the feet, photosensitivity and gingival hypertrophy. Electron microscopy examination of poikilodermic skin showed normal anchoring filaments and intraepidermal cleavage.

DISCUSSION:

Diagnosis of Kindler's syndrome is based upon clinical evidence. Kidler's syndrome is a well defined clinical entity. Ultra-structural studies show intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and other bullous hereditary poikilodermas.

Copyright © 2011 Elsevier Masson SAS. All rights reserved.

PMID:
21839347
[PubMed - indexed for MEDLINE]
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