Congenital hypertrichosis lanuginosa
Affected patients may have an increase in the hypertrichosis during childhood, or it may actually decrease. Regardless of hair changes, the lanugo characteristic of the hair will be retained even after puberty.
No abnormalities of other organ systems are clearly associated with the condition, but single cases with coinciding pyloric stenosis, precocious teeth, tetralogy of Fallot, growth and developmental delay, and congenital glaucoma have been reported.
Q84.2 – Other congenital malformations of hair
403799003 – Congenital hypertrichosis lanuginosa
Differential Diagnosis & Pitfalls
Excessive body hair may be transient in premature infants or, less commonly, term newborns. The hair of HLC is typically as long as 2 inches in length – much longer than transient lanugo overgrowth.
Syndromes associated with hypertrichosis must be considered:
- Hurler syndrome
- Cornelia de Lange syndrome
- Rubinstein-Taybi syndrome
- Gingival hyperplasia with hypertrichosis syndrome
- Winchester syndrome
- Recessive dystrophic epidermolysis bullosa
- Fetal hydantoin syndrome
- Erythrohepatic porphyria
spina bifida or other central nervous system malformation.