Table VI.

Warning signs of PIDD for dermatologists

Clinical occurrences	PIDD	Laboratory test
Eczema	Wiskott-Aldrich syndrome (WAS)	CBC including platelet number and size (small sized platelets); CMI, AMI
	Hyper IgE syndrome (HIES)	Serum IgE, eosinophilia
		Specific Scorea
	Immunodysregulation, polyendocrinopathy and enteropathy, X-linked (IPEX)	CMI, ANA, RCP
		Coombs, glycemia, and TSH
	Severe combined immunodeficiency (SCID), erythroderma	CMI
Cutaneous lesions by Mycobacteria	Combined immunodeficiencies	CMI
	Hyper-IgM syndromes	AMI
	Mendelian susceptibility to mycobacterial diseases	II
	Chronic granulomatous diseases (CGD)	P
Partial albinism, gray hair	Chediak-Higashi syndrome	Enlarged cytoplasm granules in blood smear
	Griscelli syndrome
Telangiectasias	Ataxia-telangiectasia	AMI; serum alfa-feto protein
Disseminated warts and molluscum	Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome	AMI, CMI lymphoproliferation assay
Cutaneous herpes infections
	Dedicator of cytokinesis 8 (DOCK8) deficiency
	Idiopathic CD4 lymphopenia
Fragile hair, conic teeth	Ectodermal dysplasia	II

Abbreviations: AMI antibody mediated immunity, ANA antinuclear antibodies, CBC complete blood count, CMI cellular mediated immunity, II innate immunity, P phagocytosis, RCP reactive C protein, TSH thyroid stimulating hormone

^aScore for classical Hyper IgE diagnosis [26]