PMID- 18384565
OWN - NLM
STAT- MEDLINE
DCOM- 20080811
LR  - 20151119
IS  - 1468-3083 (Electronic)
IS  - 0926-9959 (Linking)
VI  - 22
IP  - 6
DP  - 2008 Jun
TI  - A study on serum IgE and clinical symptomatology of atopy in patients suffering 
      from the lysosomal storage disorder Fabry disease.
PG  - 692-5
LID - 10.1111/j.1468-3083.2008.02638.x [doi]
AB  - BACKGROUND: Lysosomal storage disorders may impair intracellular lysosomal 
      processing of antigen with consequences for antibody production [e.g. 
      immunoglobulin E (IgE)] and atopic disease status. AIMS: Serum concentration of 
      total IgE as well as clinical symptoms of atopic disorders as an indirect 
      consequence of lysosomal impairment of antigen processing were studied in male 
      and female Fabry disease (FD) patients with and without replacement of the 
      missing lysosomal enzyme, alpha-galactosidase A (alpha-Gal A). METHODS: 
      Observational study in 31 adult FD patients with measurements of total serum IgE 
      concentration. Questionnaire-derived data were obtained for atopic eczema (AE) 
      skin lesions, allergic rhinoconjunctivitis (RCA) and allergic asthma (AA) at 
      present or in the past. RESULTS: Among 12 FD males under enzyme replacement 
      therapy (ERT), 2 showed total IgE concentrations above 100 kU/L. Clinical 
      symptoms for AA were found in 2, RCA and AE in 1, respectively. Among 10 FD 
      females under ERT, 4 showed total IgE concentrations above 100 kU/L. Clinical 
      symptoms for AA were found in 4, RCA in 2 and AE in 2. Among 9 females without 
      ERT, 2 showed total IgE concentrations above 100 kU/L. Clinical symptoms for AA 
      were found in 2, RCA in 2 and AE in none. CONCLUSIONS: FD patients may 
      demonstrate an increased total serum IgE concentration and may show symptoms of 
      atopic disorders (AA, RCA, AE) in a prevalence rate comparable to international 
      experience for individuals without FD. There was no difference between patients 
      with and without ERT. Lack of detection of AE in females without ERT is suggested 
      to be caused by the small sample size. FD patients without any alpha-Gal A 
      activity prior to initiation of ERT should be in the focus of future studies.
FAU - Mohrenschlager, M
AU  - Mohrenschlager M
AD  - Division Environmental Dermatology and Allergy GSF/TUM, Department of Dermatology 
      and Allergy Biederstein, Technical University of Munich, Munich, Germany. 
      m.moehrenschlager@lrz.tum.de
FAU - Ollert, M
AU  - Ollert M
FAU - Ring, J
AU  - Ring J
LA  - eng
PT  - Journal Article
DEP - 20080401
PL  - England
TA  - J Eur Acad Dermatol Venereol
JT  - Journal of the European Academy of Dermatology and Venereology : JEADV
JID - 9216037
RN  - 37341-29-0 (Immunoglobulin E)
SB  - IM
MH  - Adult
MH  - Aged
MH  - Dermatitis, Atopic/*blood/complications
MH  - Fabry Disease/*blood/complications
MH  - Female
MH  - Humans
MH  - Immunoglobulin E/*blood
MH  - Male
MH  - Middle Aged
MH  - Surveys and Questionnaires
EDAT- 2008/04/04 09:00
MHDA- 2008/08/12 09:00
CRDT- 2008/04/04 09:00
PHST- 2008/04/04 09:00 [pubmed]
PHST- 2008/08/12 09:00 [medline]
PHST- 2008/04/04 09:00 [entrez]
AID - JDV2638 [pii]
AID - 10.1111/j.1468-3083.2008.02638.x [doi]
PST - ppublish
SO  - J Eur Acad Dermatol Venereol. 2008 Jun;22(6):692-5. doi: 
      10.1111/j.1468-3083.2008.02638.x. Epub 2008 Apr 1.