Autosomal recessive hereditary disorder	Congenital alopecia	Developmental hereditary disorder	Dystrophia unguium	Ectodermal dysplasia with hair-nail defect	Genetic disorder of nail	Genetic disorder of skin pigmentation	Hereditary cancer-predisposing syndrome	Hereditary diffuse palmoplantar keratoderma
\|	\|	\|	\|	\|	\|	\|	\|	\|

Pigmentation defects, palmoplantar keratoderma, skin carcinoma syndrome (disorder)
	Pigmentation defects, palmoplantar keratoderma, skin carcinoma syndrome
	A rare genetic skin disease with characteristics of infantile onset of diffuse alopecia, abnormal skin pigmentation (hypo and hyperpigmented macules of the trunk and face and areas of reticular hypo and hyperpigmentation of the extremities), palmoplantar keratoderma and nail dystrophy. Patients develop recurrent spinocellular carcinomas later in life. Brittle teeth resulting in early loss of dentition have also been described.

Id	1230005002
Status	Primitive

Associated morphology	Dysplasia
Finding site	Ectoderm structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Absence
Finding site	Hair structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Dystrophy
Finding site	Nail unit structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Hyperkeratosis
Finding site	Entire skin of palmar area of hand

Associated morphology	Hyperkeratosis
Finding site	Entire skin of sole of foot

Associated morphology	Pigment alteration
Finding site	Skin structure

ICD-10 complex map reference set

Target	Q82.4
Rule	TRUE
Advice	ALWAYS Q82.4 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified