Table 1. Other primary atopic diseases with disrupted epithelial and/or mucosal skin barrier [9, 10, 11].

Disease	OMIM-P	Gene	Inheritance	Phenotype
Filaggrin deficiency	#146700	FLG	AD or AR (LOF)	– Ichthyosis vulgaris – Early-onset persistent atopic eczema – Elevated risk of food allergy and eczema herpeticum
Severe dermatitis – multiple allergies – metabolic wasting syndrome (SAM)	#615508	DSG1; DSP	AD or AR (LOF)	– Life-threatening condition, severe dermatitis, multiple allergies, metabolic wasting – Hypotrichosis, palmoplantar hyperkeratosis, enamel defects, recurrent (skin) infections – In some patients little systemic involvement
Peeling skin syndrome type B	#270300	CDSN	AR (LOF)	– Lifelong patchy peeling of the skin with chronic pruritus – Frequent food allergy, recurrent skin infections – Usually no failure to thrive
CARD14 deficiency	#602723	CARD14	AD (LOF)	– Severe atopy, severe pyogenic and viral skin and respiratory tract infections due to impaired NFkB activation and impaired epidermal secretion of antimicrobial peptides

AD = autosomal dominant; AR = autosomal recessive; LOF = loss of function.