TY  - CHAP
M1  - Book, Section
TI  - Chapter 51. Acantholytic Disorders of the Skin
A1  - Burge, Susan
A1  - Hovnanian, Alain
A2  - Goldsmith, Lowell A.
A2  - Katz, Stephen I.
A2  - Gilchrest, Barbara A.
A2  - Paller, Amy S.
A2  - Leffell, David J.
A2  - Wolff, Klaus
Y1  - 2012
N1  - 
T2  - Fitzpatrick's Dermatology in General Medicine, 8e
AB  - |PrintAcantholytic  Diseases  at  a  GlanceThe  acantholytic  diseases  are  a  heterogeneous  group  of  diseases  with  overlapping  clinical  and  histological  features.Darier  (or  Darier–White)  disease  (DD)  and  Hailey–Hailey  disease  (HHD)  are  autosomal  dominant  disorders  that  are  caused  by  defective  calcium  pumps—a  sarco/endoplasmic  reticulum  pump  in  DD  and  a  Golgi  apparatus  pump  in  HHD.Typical  DD  presents  with  greasy  keratotic  papules  in  a  seborrheic  distribution  while  HHD  is  characterized  by  painful  oozing  erosions  in  flexures  and  at  sites  of  trauma.Signs  in  the  nails  (DD,  HHD),  flat-topped  warty  papules  on  dorsa  of  hands  and  feet  (DD)  and  palmar  pits  (DD,  HHD)  or  palmar  keratotic  papules  (DD)  help  to  confirm  the  diagnosis.Hypertrophic  malodorous  flexural  disease  is  particularly  disabling  in  DD  and  HHD.Grover  disease  (GD)  is  a  sporadic  papular  condition  of  uncertain  etiology  that  presents  most  often  in  sun-damaged  skin.  Intractable  pruritus  is  common.Histopathological  examination  of  involved  skin  in  DD,  HH,  and  GD  reveals  breakdown  of  intercellular  contacts  between  suprabasal  keratinocytes  (acantholysis)  with  variable  dyskeratosis.Acrokeratosis  verruciformis  of  Hopf  (AKV)  is  autosomal  dominant  and  characterized  by  signs  that  mimic  acral  DD:  flat-topped  warty  papules  on  dorsa  of  hands  and  feet  and  nail  dystrophy.  The  histology  in  AKV  is  not  acantholytic,  but  some  (if  not  most)  cases  may  be  limited  variants  of  DD.Treatment  options  for  these  diseases  include  topical  corticosteroids  (DD,  HHD,  GD)  and  topical  or  oral  retinoids  (DD,  GD,  AKV).  
SN  - 
PB  - The McGraw-Hill Companies
CY  - New York, NY
Y2  - 2024/04/18
UR  - accessmedicine.mhmedical.com/content.aspx?aid=56036310
ER  -