Hypotrichosis simplex of the scalp

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Hypotrichosis simplex of the scalp

ORPHA:90368Disease

Also called Hereditary hypotrichosis simplex of the scalp

What it is

A rare alopecia characterized by scalp-limited progressive hair loss. Most of the patients present with normal hair at birth, progressive hair loss starts during the first decade of life and leads to almost complete loss (sparse, fine and short hairs may remain in some patients) of scalp hair by the third decade. Body, axillary and facial hair (including eyebrows, eye lashes and beard) are not affected. Teeth and nails develop normally.

Key facts

Age of onset
Childhood
Inheritance
Autosomal dominant
Classified as
Disease

Signs and symptoms

These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.

Genes

CDSNDisease-causing germline mutation(s)
KRT74Disease-causing germline mutation(s)

ICD-10 codes

L65.8filed under a broader ICD-10 category — shared with 1 other rare disease

ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.

Related health topics

Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.

Cross-references

MESH C564143MONDO 0019575OMIM 146520OMIM 613981UMLS C1840299

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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