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Matrix Metalloproteinase in Hailey-Hailey Disease

L Le Saché-de Peufeilhoux, E Raynaud, A Bouchardeau, S Fraitag, C Bodemer
BACKGROUND: Hailey-Hailey disease (HHD) or familial benign chronic pemphigus is a rare autosomal dominant inherited skin disorder, characterized by flaccid vesicles and erosions on the intertriginous areas. Current treatments are not particularly effective. We report 6 cases dramatically improving with doxycycline. CASE REPORTS: 6 patients, aged from 33 to 77 years old, presented with a variable 4 to 40 year history of severe treatment-resistant HHD. All 6 patients were then treated successfully with doxycycline 100 mg per day for at least 3 months...
March 2014: Journal of the European Academy of Dermatology and Venereology: JEADV
T Kobayashi, K Sakuraoka, S Hattori, H Hori, Y Nagai, S Tajima, T Nishikawa
BACKGROUND: The formation of lacunae and acantholysis as well as dyskeratosis are characteristic features of Hailey-Hailey disease (HHD) and Darier's disease (DD). Matrix metalloproteinases (MMPs) and their inhibitors like tissue inhibitors of metalloproteinases (TIMPs) have been thought to play major roles in the tissue metabolism. OBJECTIVE: The aim of this study was to investigate the role of MMP-9 and TIMP-1 in HHD and DD. METHODS: We examined localizations of these two molecules by immunostaining using specific monoclonal antibodies...
1996: Dermatology: International Journal for Clinical and Investigative Dermatology
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