	»	Diseases of the Hair and Nails
		»	Menkes Disease
		»	Monilethrix
		»	Trichorhinophalangeal Syndrome
		»	Uncombable Hair Syndrome
		»	Tricho-dento-osseous Syndrome
		»	Björnstad Syndrome
		»	Papular Atrichia
		»	Nail–Patella Syndrome
		»	Pachyonychia Congenita
	»	Disorders of Cornification
		»	Ichthyosis Vulgaris (IV)
		»	X-linked Ichthyosis (XLI)
		»	Lamellar Ichthyosis
		»	Congenital Ichthyosiform Erythroderma
		»	Ichthyosis Bullosa of Siemens
		»	Epidermolytic Hyperkeratosis
		»	Harlequin Ichthyosis
		»	Netherton Syndrome
		»	Sjögren–Larsson Syndrome
		»	CHILD Syndrome
		»	Conradi–Hünermann–Happle Syndrome
		»	Chondrodysplasia Punctata
		»	Rhizomelic Chondrodysplasia Punctata
		»	KID Syndrome
		»	Refsum Disease
		»	Darier Disease
	»	Other Conditions
		»	Palmoplantar Keratodermas
		»	Ectodermal Dysplasias
		»	Metabolic and Enzyme Deficiency Diseases
		»	Signs of Spinal Dysraphism
		»	Keratinopathies
»	References

X-linked Ichthyosis (XLI)

XLR, defect in steroid sulfatase (STS, arylsulfatase C)
Presents around infancy with mild erythroderma and large translucent scales → evolves into adherent brown “dirty” scales over extremities, trunk, neck; variable involvement of flexures, sparing of palms/soles/face
Mother (with affected fetus): low/absent estrogen in urine/amniotic fluid → labor fails to progress
Other associations: comma-shaped corneal opacities, cryptorchidism (↑ risk of testicular CA)
Histology: hyperkeratosis or parakeratosis overlying normal or slightly thickened granular layer
Tests: serum lipoprotein electrophoresis (detects accumulation of cholesterol sulfate)