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2015 ; 4
(4
): 214-6
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Nailfold capillaroscopic changes in Kindler syndrome
#MMPMID26668784
Dobrev HP
; Vutova NI
Intractable Rare Dis Res
2015[Nov]; 4
(4
): 214-6
PMID26668784
show ga
Kindler syndrome (KS), the fourth major type of hereditary epidermolysis bullosa
(HEB), is a rare, autosomal recessive disorder characterized by skin fragility
and blistering at birth followed by development of marked photosensitivity and
progressive poikilodermatous skin changes in later years. We reported here the
case of a 54-year-old woman, who fulfills the diagnostic criteria of KS type of
HEB, putting accent on the nailfold capillaroscopic changes. Using
videocapillaroscopy we observed pronounced alterations in finger nail capillaries
including reduction in capillary density, features of neoangiogenesis
(architectural derangement, elongated loops, extremely tortuous, bushy or
branching capillaries, thin, branching and interconnected capillaries), enlarged
and giant capillaries. We consider the changes observed as an adaptive mechanism
that compensate the loss of capillaries due to chronic periungual trauma. Further
studies with larger number of patients are needed to confirm the significance of
capillaroscopy findings for patients with HEB.