Autosomal recessive hereditary disorder	Congenital anomaly of skin	Congenital palmoplantar keratoderma	Developmental hereditary disorder	Gonadal dysgenesis	Hereditary cancer-predisposing syndrome	Hereditary diffuse palmoplantar keratoderma	Reproductive system hereditary disorder
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Palmoplantar keratoderma, 46,XX sex reversal, predisposition to squamous cell carcinoma syndrome (disorder)
	Palmoplantar keratoderma, 46,XX sex reversal, predisposition to squamous cell carcinoma syndrome
	This syndrome has characteristics of sex reversal in males with a 46, XX (SRY-negative) karyotype, palmoplantar hyperkeratosis and a predisposition to squamous cell carcinoma. To date, five cases (four of whom were brothers) have been described.
	syndroom van palmoplantaire hyperkeratose, XX-geslachtsomkering en predispositie voor plaveiselcelcarcinoom
	palmoplantaire keratodermie-XX-geslachtsomkering-predispositie voor plaveiselcelcarcinoom-syndroom

Id	722202006
Status	Primitive

Associated morphology	Morphologically abnormal structure
Finding site	Gonadal structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Hyperkeratosis
Finding site	Skin structure
Occurrence	Congenital
Pathological process	Pathological developmental process

Associated morphology	Hyperkeratosis
Finding site	Entire skin of sole of foot

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

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q56.0
Rule	TRUE
Advice	ALWAYS Q56.0 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified