Table 2.

Syndromes Associated With Hidradenitis Suppurativa.

Classification	Syndrome	Clinical presentation
Syndromes with a genetic background	Bazex-Dupre-Christol Syndrome	Follicular atrophoderma, congenital hypertrichosis, basal cell neoformations 19
	Dowling-Degos disease	Reticular hyperpigmentation of flexural surfaces, comedo-like lesions, perioral or facial scars, epidermoid cysts 20
	Down’s Syndrome	HS over 5 times more likely in this population, HS diagnosed earlier in life 21
	Keratitis-Ichthyosis-Deafness Syndrome	Erythrokeratoderma, follicular keratoses, palmoplantar hyperkeratosis, alopecia 22
	Familial Mediterranean Fever	Fever, peritonitis, arthritis, amyloidosis 23
Syndromes with follicular plugging or structural disorders	Bazex-Dupre-Christol Syndrome	Follicular atrophoderma, congenital hypertrichosis, basal cell neoformations 19
	Dowling-Degos disease	Reticular hyperpigmentation of flexural surfaces, comedo-like lesions, perioral or facial scars, epidermoid cysts 20
	Down’s Syndrome	HS over 5 times more likely in this population, HS diagnosed earlier in life 21
	Keratitis-Ichthyosis-Deafness Syndrome	Erythrokeratoderma, follicular keratoses, palmoplantar hyperkeratosis, alopecia 22
	Follicular Occlusion Syndrome	Acne conglobata, pilonidal disease, dissecting cellulitis, HS
Syndromes with a possible autoinflammatory pathogenesis	PASH	Pyoderma Gangrenosum, Acne, Suppurative Hidradenitis
	PASS	Pyoderma Gangrenosum, Acne, HS, Ankylosing Spondylitis
	PAPASH	Pyogenic Arthritis, Pyoderma Gangrenosum, Acne, HS
	SAPHO	Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis
	Familial Mediterranean Fever (FMF)	Fever, peritonitis, arthritis, amyloidosis 23