PMID- 33548520
OWN - NLM
STAT- MEDLINE
DCOM- 20210524
LR  - 20210524
IS  - 2213-2201 (Electronic)
VI  - 9
IP  - 4
DP  - 2021 Apr
TI  - Inborn Error of Immunity or Atopic Dermatitis: When to be Concerned and How to 
      Investigate.
PG  - 1501-1507
LID - S2213-2198(21)00165-3 [pii]
LID - 10.1016/j.jaip.2021.01.037 [doi]
AB  - Around 20% of all children worldwide suffer from atopic dermatitis. Therefore, 
      eczematous skin lesions and elevated serum immunoglobulin E (IgE) levels are 
      common findings. Inborn errors of immunity (IEI) may be missed in the context of 
      atopic dermatitis, and management and prognosis of these conditions can be 
      substantially different. Children suffering from IEIs such as hyper-IgE 
      syndromes, Wiskott-Aldrich syndrome, immunodysregulation polyendocrinopathy 
      enteropathy X-linked syndrome, Omenn syndrome, the atypical complete DiGeorge 
      syndrome, and skin barrier disorders like Comel-Netherton syndrome and severe 
      dermatitis-multiple allergies and metabolic wasting syndrome may present with 
      additional red flags, which should raise a clinical suspicion for an underlying 
      IEI. These red flags may include eczematous skin lesion manifesting prior to two 
      months of life, disseminated or recurrent viral, bacterial, or fungal infections, 
      mucocutaneous candidiasis, purpura, chronic diarrhea, or abnormalities in 
      development or of connective tissue. A differential blood count, as well as a 
      lymphocyte subset analysis, total immunoglobulin levels, and vaccination titers 
      can help the clinician to decide whether a patient with eczematous skin lesions 
      and elevated serum IgE should be referred to a clinical immunologist for a full 
      immunological work-up and broad genetic analysis.
CI  - Copyright (c) 2021 American Academy of Allergy, Asthma & Immunology. Published by 
      Elsevier Inc. All rights reserved.
FAU - Stadler, Pia-Charlotte
AU  - Stadler PC
AD  - Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, 
      Germany.
FAU - Renner, Ellen D
AU  - Renner ED
AD  - Translational Immunology in Environmental Medicine, Technical University, Munich, 
      Germany; Translational Immunology, Institute of Environmental Medicine, Helmholtz 
      Zentrum Munchen, Munich, Germany.
FAU - Milner, Joshua
AU  - Milner J
AD  - Department of Pediatrics, Columbia University Irving Medical Center, New York, 
      NY.
FAU - Wollenberg, Andreas
AU  - Wollenberg A
AD  - Department of Dermatology and Allergy, University Hospital, LMU Munich, Munich, 
      Germany. Electronic address: wollenberg@lrz.uni-muenchen.de.
LA  - eng
PT  - Journal Article
DEP - 20210203
PL  - United States
TA  - J Allergy Clin Immunol Pract
JT  - The journal of allergy and clinical immunology. In practice
JID - 101597220
RN  - 37341-29-0 (Immunoglobulin E)
SB  - IM
MH  - Child
MH  - *Dermatitis, Atopic/diagnosis
MH  - *Eczema
MH  - Humans
MH  - Immunoglobulin E
MH  - *Job Syndrome
MH  - *Netherton Syndrome
OTO - NOTNLM
OT  - Atopic dermatitis
OT  - HIES
OT  - Hyper-IgE syndrome
OT  - Inborn error of immunity
EDAT- 2021/02/07 06:00
MHDA- 2021/05/25 06:00
CRDT- 2021/02/06 20:10
PHST- 2020/12/28 00:00 [received]
PHST- 2021/01/28 00:00 [revised]
PHST- 2021/01/28 00:00 [accepted]
PHST- 2021/02/07 06:00 [pubmed]
PHST- 2021/05/25 06:00 [medline]
PHST- 2021/02/06 20:10 [entrez]
AID - S2213-2198(21)00165-3 [pii]
AID - 10.1016/j.jaip.2021.01.037 [doi]
PST - ppublish
SO  - J Allergy Clin Immunol Pract. 2021 Apr;9(4):1501-1507. doi: 
      10.1016/j.jaip.2021.01.037. Epub 2021 Feb 3.