Kindler syndrome

Case report


M. El-hashme Mohamad, El-hashme A. Mashina, S. Allaghi

Bir Al-Austa Milad Hospital, Tripoli, Libya

JMJ Vol. 2, No. 3 (March 2003): 74-76


Kindler syndrome was first described by Theresa Kindler in 1954. This recessively transmitted syndrome consists of generalized progressive poikeloderma with cutaneous atrophy, photosensitivity, and traumatic acral skin blistering. Since Kindler’s report of her case, more than 70 cases were reported worldwide having most of the features and other association. Our patient is a 46 years old Libyan man presenting with generalized skin atrophy with poikloderma, cigarette paper scars over knees and dorsa of both hands following traumatic bullea during childhood, photosensitivity associated with loss of all his teeth and dysphagia due to oesophageal stenosis. Family history was negative for similar skin problem. Histopathological features of skin biopsy were compatible with the diagnosis of kindler syndrome. Ultrastructural studies were not done. This is the first reported Libyan case of this syndrome.

Keywords: Kindler syndrome, photosensitivity, atrophy