Kindler syndrome: a study of five Egyptian cases with evaluation of severity
Version of Record online: 28 JUN 2008
© 2008 The International Society of Dermatology
International Journal of Dermatology
Volume 47, Issue 7, pages 658–662, July 2008
How to Cite
Nofal, E., Assaf, M. and Elmosalamy, K. (2008), Kindler syndrome: a study of five Egyptian cases with evaluation of severity. International Journal of Dermatology, 47: 658–662. doi: 10.1111/j.1365-4632.2008.03721.x
- Issue online: 28 JUN 2008
- Version of Record online: 28 JUN 2008
Background Kindler syndrome (KS) is a rare genodermatosis characterized by four major features (acral blisters, photosensitivity, poikiloderma, and cutaneous atrophy) and many associated findings. The diagnosis of KS includes clinical features, ultrastructural findings, and, recently, immunostaining and genetic studies. Varying degrees of severity of the syndrome have been described.
Methods Five patients with clinical features consistent with KS were included in this study. All patients were subjected to histopathologic and ultrastructural studies.
Results Cases 1 and 2 presented with severe major features, severe mucosal involvement, and many other associated findings. Case 3 presented with severe major features, but mild and limited mucosal involvement and other associated findings. Cases 4 and 5 showed mild major features and few other findings. Histopathology revealed nonspecific poikiloderma. Marked thickening of the lamina densa and splitting of the lamina lucida were the main ultrastructural findings.
Conclusion KS may be classified into mild, moderate, and severe according to the severity of the major features and mucosal involvement. Because histopathologic and ultrastructural findings are not pathognomonic, clinical features remain the mainstay for the diagnosis of KS, and the need for immunostaining with kindlin antibody and genetic studies may be restricted to early cases with incomplete features.