Hypertrichoses L68.8

Polyätiological, congenital or acquired, localised or generalised hair, occurring as an isolated phenomenon or as a partial manifestation in the context of syndromes, untypically strong hair in the respective body part, which does not correspond to the distribution pattern, ethnic provenance and age of the secondary male sexual hair. Hair-biologically there is a transition from vellus hair to terminal hair.

A distinction is made between:

• Generalized hypertrichoses:
  • Congenital generalized hypertrichoses:
    • Hypertrichosis lanuginosa congenita (autosomal dominant)
    • X-linked congenital generalized hypertrichosis (OMIM 307150)
    • Hypertrichosisuniversalis congenita (Ambras syndrome)
    • Hypertrichosis, prepuberal (syn.: hypertrichosis, generalized constitutional)
    • Gingival fibromatosis with hypertrichosis
• Generalized or circumscritical hypertrichoses as a symptom of rare, congenital complex developmental disorders:
  • Coffin-Siris syndrome
  • Cornelia de Lange syndrome
  • Brachmann de Lange syndrome
  • Dysostosis mandibulofacialis
  • Lawrence syndrome
  • Leprechaunism syndrome
  • Pfaundler-Hurler disease
  • Rubinstein-Taybi syndrome (hypertrichosis of the back)
  • Syringomyelia (circumscripte hpertrichosis)
  • Winchester syndrome (circumscripte hypertrichosis)
  • Osteochondrodysplasia with hypertrichosis (Cantu syndrome)
  • Hypertrichosis-skeletal dysplasia-retardation syndrome with hyperuricemia
  • Congenital amaurosis with hypertrichosis (Jalili syndrome)
• Acquired generalized hypertrichoses in diseases/syndromes:
  • Anorexia nervosa
  • Malabsorption syndromes (common in children, face and trunk)
  • Paraneoplastic hypertrichoses
    • POEMS syndrome
    • Hypertrichosis lanuginosa acquisita as obligate paraneoplasia).
• Localized (circumscribed) hypertrichoses:
  • Congenital localized (circumscritical) hypertrichoses:
    • Hypertrichosis dorsalis superior
    • Trichomegaly syndrome (congenital)
    • Trichomegaly (acquired; see below trichomegaly syndrome)
    • Hypertrichosis, nevoid
    • Hypertrichosis dorsolumbalis (often withdysraphia )
    • Klein-Waardenburg syndrome
    • Nevus pigmentosus et pilosus
    • Curly hair nevus
    • Becker nevus
    • Ectopic hair follicles
    • Hamartoma, eccrine, angiomatous.
  • In embryonic damage/disease:
    • Embryofetal alcohol syndrome (facial hypertrichosis).
    • Antiepileptic embryopathy.
• Acquired localized (circumscritic) or generalized hypertrichoses:
  • Hypertrichosis in systemic diseases, such as: Erythropoietic and hepatic porphyrias, hypothyroidism, myxoedema circumscriptum symmetricum praetibiale, acromegaly, POEMS syndrome, scleroderma, systemic, dermatomyositis, AIDS.
  • Constitutional localized (circumskripte) hypertrichosis (e.g., in the area of the ear, cheeks, eyebrows, back). S.a.u. Hypertrichosis dorsalis superior.
  • Prepuberal hypertrichosis (constitutional generalized hypertrichosis).
  • Iatrogenic localized or generalized hypertrichoses:
    • Hypertrichosis, drug-induced
    • Hypertrichosis after repeated local chemical trauma (e.g., after PUVA or dithranol therapy) or physical trauma (constant rubbing trauma, e.g., from carrying heavy bags) and severe craniocerebral trauma (lanugo-like hair on forehead, cheeks, back, and extremities).

• age of manifestation (congenital or acquired)
• Hair type (lanugo, vellus, terminal hair)
• Localization
• Hair pattern (circumscribed or generalized)
• Comorbidities
• Drugs
• associated anomalies
• Estonian provenance (Nordic or southern Tyous)
• Family reunion

1. Trueb RM (2008) Hypertrichosis. dermatologist 59: 325-338
2. Wolf H (2018) Hair diseases. In: G. Plewig et al (Ed.) Braun-Falco`s Dermatology, Venerology and Allergology, Springer Reference Medicine S1361-1368