PMID- 9556111
OWN - NLM
STAT- MEDLINE
DCOM- 19980617
LR  - 20190722
IS  - 0011-9059 (Print)
IS  - 0011-9059 (Linking)
VI  - 37
IP  - 3
DP  - 1998 Mar
TI  - Familial pemphigus vulgaris in mother and daughter.
PG  - 211-4
AB  - BACKGROUND: Pemphigus vulgaris is an autoimmune disease in which genetics appears 
      to be of basic importance. Although association with certain human leukocyte 
      antigen (HLA) alleles has been found in some ethnic groups and individuals, no 
      true disease susceptibility genes have been established, and familial cases are 
      very unusual. METHODS: We report a Polish family with pemphigus vulgaris in the 
      mother and daughter. The diagnosis was confirmed by cytologic, histologic, and 
      immunofluorescence studies. RESULTS: The course was severe and the disease 
      long-lasting in the mother, probably due to treatment with small doses of 
      corticosteroids without immunosuppressive drugs. In the daughter, treated with 
      larger doses of corticosteroids and azathioprine, the lesions regressed within 4 
      months, after which maintenance therapy was instituted with 10 mg of prednisone 
      daily. The HLA studies performed in the daughter and her three children after the 
      mother had died showed identical haplotypes in both the patient and the healthy 
      children. The patient has given birth to a healthy child while still having a 
      high titer of intercellular (IC) antibodies. CONCLUSIONS: The familial occurrence 
      of pemphigus in first-degree relatives is suggestive of inherited susceptibility 
      to the disease, transmitted as a dominant trait. The identical haplotypes in the 
      healthy children of the patient favor the role of other, unknown factors required 
      for the development of the disease in predisposed individuals.
FAU - Starzycki, Z
AU  - Starzycki Z
AD  - Department of Dermatology, Collegium Medicum Jagiellonian University, Cracow, 
      Poland.
FAU - Chorzelski, T P
AU  - Chorzelski TP
FAU - Jablonska, S
AU  - Jablonska S
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - England
TA  - Int J Dermatol
JT  - International journal of dermatology
JID - 0243704
RN  - 0 (HLA Antigens)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Diagnosis, Differential
MH  - Female
MH  - HLA Antigens/genetics
MH  - Haplotypes
MH  - Humans
MH  - Pemphigus, Benign Familial/*diagnosis/drug therapy/*genetics/immunology
EDAT- 1998/04/29 00:00
MHDA- 1998/04/29 00:01
CRDT- 1998/04/29 00:00
PHST- 1998/04/29 00:00 [pubmed]
PHST- 1998/04/29 00:01 [medline]
PHST- 1998/04/29 00:00 [entrez]
AID - 10.1046/j.1365-4362.1998.00325.x [doi]
PST - ppublish
SO  - Int J Dermatol. 1998 Mar;37(3):211-4. doi: 10.1046/j.1365-4362.1998.00325.x.