Kindler Syndrome: Report of Two Cases and Review of the Literature

We evaluated two patients with hereditary bullous poikiloderma. Both had acral bullae, generalized poikiloderma with prominent atrophy, and acral keratoses. One patient, with sporadic disease, had, in addition, urethral and subglottic stenoses, webbing of digits, and poor dentition. The other patient, whose disease was dominantly inherited, had koilonychia. The results of cutaneous histopathology, electron microscopy, and immunofluorescence mapping studies are presented. It is possible that Kindler syndrome and Weary's hereditary acrokeratotic poikiloderma are variants of the same disease.