Herzberg-Potjan-Gebauer syndrome

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Classification according to ICD-10
L68.1 Hypertrichosis lanuginosa acquisita
C80 Malignant neoplasm without specifying the location - paraneoplastic syndrome
ICD-10 online (WHO version 2019)

The Herzberg-Potjan-Gebauer syndrome is a rare skin change in the context of a paraneoplastic disease in metastatic carcinomas of the internal organs , in which the lanugo hairs ( armpit hair and pubic hair ) suddenly begin to grow.

Synonyms are: Hypertrichosis lanuginosa acquisita, hypertrichosis-paraneoplasia syndrome, acquired lanugo-hypertrichosis; Lanugo hypertrichosis; English Malignant Down

The name refers to the authors of the first description from 1968 by the German doctors JJ Herzberg, K. Potjan and D. Gebauer.

pathology

The mechanism has not been clarified; a "pilotroper factor" is suspected in paraneoplasia (especially in metastatic carcinomas ).

Clinical manifestations

Clinical criteria are:

  • sudden onset of hypertrichosis , also in otherwise hairless areas with a fur-like expression
  • Excess length of hair up to 15 cm

An accompanying hypertrophy of the tongue papillae can also occur.

Differential diagnostics

A distinction must be made between hypertrichosis lanuginosa congenita and hypertrichosis in porphyria cutanea tarda .

therapy

Treatment is directed against the underlying malignancy .

literature

  • T. Khoschbin, C. Löser, E. Dippel: Paraneoplastic skin diseases. In: The internist. [Electronic publication before printing] July 2019, doi: 10.1007 / s00108-019-0636-1 , PMID 31273399 (review).
  • JA Frank, N. Rojek, RS Foreman: A case of hypertrichosis lanuginosa acquisita as a sign of malignancy. In: European journal of dermatology: EJD. Volume 27, number 1, February 2017, pp. 66-67, doi: 10.1684 / ejd.2016.2901 , PMID 27748263 .
  • M. Simon: Paraneoplastic Syndromes of the Skin. In: Current Dermatology. 42, 2016, p. 50, doi: 10.1055 / s-0041-110028
  • PH Slee, RI van der Waal, JH Schagen van Leeuwen, RA Tupker, R. Timmer, CA Seldenrijk, MA van Steensel: Paraneoplastic hypertrichosis lanuginosa acquisita: uncommon or overlooked? In: The British journal of dermatology. Volume 157, Number 6, December 2007, pp. 1087-1092, doi: 10.1111 / j.1365-2133.2007.08253.x , PMID 17941940 (review).

Individual evidence

  1. ↑ a b c d Encyclopedia Dermatology
  2. ↑ Entry on Herzberg-Potjan-Gebauer-Syndrome in Flexikon , a Wiki of the DocCheck company
  3. ^ Deutsche Apotheker Zeitung
  4. ↑ JJ Herzberg, K. Potjan, D. Gebauer: D .: Hypertrichosis lanuginosa (et terminalis) acquisita as a paraneoplastic syndrome. In: Archives for Clinical and Experimental Dermatology. Volume 232, Number 2, 1968, pp. 176-186, PMID 5671571 .
  5. ↑ JJ Herzberg, K. Potjan, D. Gebauer: Hypertrichose lanugineuse acquise. Un nouveau syndrome paranéoplasique cutané .. In: Annales de dermatologie et de syphiligraphie. Volume 96, Number 2, 1969, pp. 129-134, PMID 5363509 .