Hypertrichosis lanuginosa acquisita L68.1

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 10.11.2022

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Synonym(s)

Acquired hypertrichosis lanuginosa; Herzberg-Potjan-Bauer Syndrome; Hypertrichosis lanuginosa acquired; Hypertrichosis paraneoplasia syndrome; Lanugo-Hypertrichose acquired; Lanugo hypertrichosis; Paraneoplastic hypertrichosis lanuginosa acquisita

History
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Herzberg, 1968

Definition
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Very rare, sudden onset, extensive, usually generalized, acquired growth of downy hair as a paraneoplastic syndrome in metastatic carcinoma (association mainly of lung carcinoma, breast carcinoma). Obligate paraneoplasia.

Classification
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Etiopathogenesis
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Pathogenetically, a hypothetical "pilotropic factor" is held responsible in paraneoplasias, which is thought to occur mainly in metastatic carcinomas. The significance of dysproteinemia, protein deficiency and/or hormonal influences (adrenal gland) cannot be conclusively assessed.

The occurrence of hypertrichosis lanuginosa acquisita in association with autoimmune hepatitis has been described several times (Roh MR et al. 2006; Yim SH et al. 2021).

Clinical features
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Rare but very characteristic disorder in which there is a sudden growth of white-silvery, fine downy hair. The hair growth spreads from cranial to caudal.

Particularly noticeable is the hair growth on such skin areas that primarily appear as "hairless" e.g. in the facial area (nose, ears). There is an anagen synchronization of the body hair.

The mostly unpigmented single hair can grow up to 4 cm long.

A particularly severe hypertrichosis can subsequently develop in the axillary and pubic region, where the hairs, which can grow up to 15 cm in length, can eventually almost cover the external genitalia.

Trichomegaly of the eyelashes is also observed.

Associated hypertrophy of the lingual papillae is possible.

Laboratory
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Hypogonadotropinuria; hypercortisoluria.

Differential diagnosis
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Other hypertrichoses (growth of strong terminal hairs) are to be distinguished

Practically important is the differentiation from drug-induced hypertrichosis (cyclosporine, minoxidil, anti-EGF receptors).

Therapy
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Tumour search and therapy as far as possible.

Progression/forecast
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Poor (as a result of the underlying disease), since the disease occurs mainly in advanced tumors. The average survival time after the onset of hypertrichosis is only a few months.

Case report(s)
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The 76-year-old patient noticed a conspicuous growth of lanugo hair on her face for 2 months, especially on both cheeks, on the chin region and on the bridge of the nose. A growth of the eyelashes (trichomegaly), which started parallel to this symptomatology, was conspicuous.
Clinical exploration with imaging and bioptic procedures revealed an adenocarcinoma in the left upper lobe of the lung.

Literature
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  1. Bruce H et al (2009) Cutaneous manifestations of internal malignancy. Cancer J Clin 59: 73-98
  2. Dalcin D et al (2015) Malignant Down: Hypertrichosis Lanuginosa Acquisita Associated With Endometrial Adenocarcinoma. J Cutan Med Surg 19:507-510.
  3. Herzberg JJ, Potjan K, Gebauer D (1968) Hypertrichosis lanuginosa (et terminalis) acquisita as a paraneoplastic syndrome. Arch klin exp Derm 232: 176-186
  4. Herzberg JJ, Potjan K, Gebauer D (1969) Hypertrichosis lanugineuse acquise. Un nouveau syndrome paranéoplastique cutané. Ann Derm Syph 96: 129-134
  5. Hovenden AL (1993) Hypertrichosis lanuginosa acquisita associated with malignancy. Clin Dermatol 11: 99-106
  6. Jernec GBE (1986) Hypertrichosis lanuginosa acquisita. Arch Derm 122: 805-808
  7. Jesús Gómez-Arias P et al (2021) Paraneoplastic hypertrichosis lanuginosa acquisita. Images in Clinical Practice 88: 525-526.
  8. Perez-Losada E et al (2001) Hypertrichosis lanuginosa acquisita preceding extraskeletal Ewing's sarcoma. Clin Exp Dermatol 26: 182-183
  9. Roh MR et al (2006) Hypertrichosis lanuginosa acquisita associated with autoimmune hepatitis. J Dermatol 33:574-576

  10. Russell P et al (2016) Hypertrichosis lanuginosa acquisita: a rare dermatological disorder. Lancet 387(10032):2035.
  11. Schulz C, Moll I (2002) Hypertrichosis lanuginosa of unknown origin in the area of the face. Porphyria cutanea tarda. Dermatologist 53: 827-829
  12. Yim SH et al (2021) Hypertrichosis lanuginosa acquisita Associated with autoimmune hepatitis. Ann Dermatol 33:200-202.

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Last updated on: 10.11.2022