Indian Dermatology Online Journal

REVIEW ARTICLE
Year
: 2017  |  Volume : 8  |  Issue : 6  |  Page : 391--405

Approach to a child with primary immunodeficiency made simple


Dhrubajyoti Sharma, Ankur K Jindal, Amit Rawat, Surjit Singh 
 Allergy Immunology Unit, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research, Chandigarh, India

Correspondence Address:
Amit Rawat
Department of Pediatrics, Advanced Pediatrics Centre, PGIMER, Chandigarh - 160 012
India

Primary immunodeficiency disorders (PIDs) are a group of disorders affecting the capability to fight against infection. These include defects in T cells and B cells affecting cell-mediated and humoral immunity, respectively, combined humoral and cell-mediated immunodeficiency, defects in phagocytosis, complement defects, and defects in cytokine or cytokine signalling pathways which are detrimental for immune function. Depending upon the type and severity, age at onset of symptoms can vary from neonatal period to late childhood. Clinically, this group of disorders can involve any organ system of an individual such as respiratory system, gastrointestinal system, skin and mucous membrane, bone and joints, endocrine organs, and nervous system. Common dermatological manifestations include eczema, warts, molluscum contagiosum, mucocutaneous candidiasis, recurrent nonhealing ulcers, skin abscesses, erythroderma, petechiae, and nail changes. The common skin manifestations of various PIDs include eczema (seen in Wiskott–Aldrich syndrome and autosomal dominant hyper IgE syndrome); erythroderma (in Omen syndrome); viral warts or molluscum contagiosum (in autosomal recessive hyper IgE syndrome); chronic mucocutaneous candidiasis (in hyper IgE syndrome, autoimmune polyendocrinopathy candidiasis ectodermal dysplasia syndrome, Th17 cell defects); recurrent nonhealing ulcers (in leucocyte adhesion defect); skin abscesses (in antibody defects, hyper IgE syndrome, and chronic granulomatous disease); petechial or purpuric spots (in Wiskott–Aldrich syndrome).


How to cite this article:
Sharma D, Jindal AK, Rawat A, Singh S. Approach to a child with primary immunodeficiency made simple.Indian Dermatol Online J 2017;8:391-405


How to cite this URL:
Sharma D, Jindal AK, Rawat A, Singh S. Approach to a child with primary immunodeficiency made simple. Indian Dermatol Online J [serial online] 2017 [cited 2018 Sep 25 ];8:391-405
Available from: http://www.idoj.in/article.asp?issn=2229-5178;year=2017;volume=8;issue=6;spage=391;epage=405;aulast=Sharma;type=0