Kindler Syndrome: A Rare Genodermatosis Presenting in 2 Brothers
Journal: Journal of Cellular Immunology and Serum Biology (Vol.2, No. 1)Publication Date: 2016-05-02
Authors : Ryan Fischer;
Page : 29-31
Keywords : Keratosis pilaris; IPL; Intense pulsed light; Keratosis pilaris treatment;
Abstract
We report a case of two Asian brothers, aged 21 and 29, born to consanguineous parents, who presented with cutaneous hyper- and hypopigmentation, skin atrophy, and skin fragility of the face, neck, and distal parts of the extremities. They were the first and second, full-term pregnancies in a family without any history of skin disease. Both patients had a history of recurrent minor trauma-induced blistering over the extensor aspects of the forearms, lower legs, dorsal hands, and feet since infancy. The blisters contained either serous or hemorrhagic fluid and typically ruptured within 4 to 5 days producing erosions that healed with dyspigmentation and atrophy, but no scarring. The tendency for blister formation after minor trauma gradually subsided by the ages of 13 and 15, respectively. Additionally, the brothers had severe skin fragility affecting the face, neck, distal limbs, and gums. They reported increased photosensitivity with sunburn after minimal sun exposure and exacerbation of their disease during the summer months.
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