Medizinische Universität Graz - Research portal
Selected Publication:
SHR Neuro Cancer Cardio Lipid Metab Microb
Saggini, A; Cota, C; Cerroni, L.
Incidental Acantholysis in Hailey-Hailey Disease (Microscopic Nikolsky Sign): An Underappreciated Histologic Sign.
Am J Dermatopathol. 2020; 42(5):e61-e64-e61-e64
Doi: 10.1097/DAD.0000000000001534
Web of Science
PubMed
FullText
FullText_MUG
- Co-authors Med Uni Graz
- Cerroni Lorenzo
- Altmetrics:
- Dimensions Citations:
- Plum Analytics:
- Scite (citation analytics):
- Abstract:
- Identification of subtle disease-specific histologic changes may be of significant help in early diagnosis of acantholytic skin diseases. Hailey-Hailey disease (HHD) is an autosomal dominant genodermatosis characterized by vesiculoerosive lesions favoring the intertriginous areas. Histologically, HHD is characterized by full-thickness acantholysis of the spinous layer in association with dyskeratosis of individual keratinocytes; a pemphigus vulgaris-like suprabasal pattern of acantholysis may be observed in the earliest stages of disease. HHD is characterized by highly variable expressivity regarding the age at onset and severity of the disease. Patients may present with late-onset and/or only mild disease. We report the recurrent presence of incidental foci of variably extensive, subclinical acantholysis in multiple bioptic specimens taken from a patient with known HHD for dermatologic conditions other than HHD. Such histologic finding has gone underappreciated in the literature, despite being a likely frequent occurrence in skin biopsies from HHD patients; recognition of this finding might represent a valuable diagnostic clue in selected cases of HHD.
- Find related publications in this database (using NLM MeSH Indexing)
- Acantholysis - diagnosis
- Acantholysis - etiology
- Acantholysis - pathology
- Humans -
- Incidental Findings -
- Pemphigus, Benign Familial - complications
- Pemphigus, Benign Familial - diagnosis
- Pemphigus, Benign Familial - pathology
- Find related publications in this database (Keywords)
- Hailey-Hailey disease
- acantholysis
- pemphigus vulgaris
- Nikolsky sign
- bullous diseases