Poikiloderma of Theresa Kindler: report of a case with ultrastructural study, and review of the literature.
Kindler's syndrome occurred in a 13-year-old boy with parental consanguinity. The patient had acral congenital blistering and photosensitivity, both regressive, at 12 years of age, together with diffuse progressive poikiloderma, marked cutaneous atrophy, mild hyperkeratosis of the palms, gingival hypertrophy and fragility, and anal stenosis. The ultrastructural study of bullae induced by rubbing showed clefts in three levels of the dermoepidermal junction zone. A review of 14 cases in the literature consistent with Kindler's syndrome showed this to be a definite clinical entity among the other bullous hereditary poikilodermas. In two cases, ultrastructural studies showed intraepidermal, junctional, and dermal cleavage. This syndrome must be differentiated from congenital epidermolysis bullosa, Weary's syndrome, and the other bullous hereditary poikilodermas.
Hovnanian
A
A
Unité de Dermatologie, Hôpital Necker-Enfants Malades, Paris, France.
Blanchet-Bardon
C
C
de Prost
Y
Y
D000293:Adolescent; D000367:Age Factors; D001706:Biopsy; D001768:Blister / Q000151:congenital; D003241:Consanguinity; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D008854:Microscopy, Electron; D010787:Photosensitivity Disorders / Q000473:pathology; D011038:Rothmund-Thomson Syndrome / Q000473:pathology*; D012867:Skin / Q000648:ultrastructure*; D012871:Skin Diseases / Q000473:pathology*