Ichthyosis hystrix

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Classification according to ICD-10
Q80.8 Other congenital ichthyosis
ICD-10 online (WHO version 2019)

The ichthyosis hystrix , from ancient Greek ὕστριξ hústrix , German , porcupine ' , is a group of very rare, the ichthyosis belonging congenital skin diseases with the main feature of a massive hyperkeratosis with distinctive, eponymous spine-like, black-brown plate formations.

Synonyms are: Ichthyosis hystrix gravior; Hyperkeratosis monstruosa; Sauriasis

Edward Lambert, an Englishman with ichthyosis hystrix.
Patient with abnormally distributed ichthyosis hystrix Wellcome L0061144

Classification

The disease group can be divided into:

  • Ichthyosis hystrix Lambert , synonyms: Ichthyosis hystrix gravior; porcupine man
  • Ichthyosis hystrix Curth-Macklin
  • Ichthyosis hystrix Rheydt , synonyms: Erythrokeratodermia progressiva Burns; Ichthyosis hystrix gravior, Rheydt type; Keratitis ichthyosis deafness syndrome ; KID
  • Hystrix-like ichthyosis deafness syndrome (HID)
  • Ichthyosis hystrix Baefverstedt (mainly affecting the face)

literature

  • WH Wang, L. Zhang, LF Li, TT Sun: Ichthyosis hystrix Lambert type and Curth-Macklin type are a single entity with affected (KRT1 mutation) or unaffected (KRT10 mutation) palms and soles? In: European journal of dermatology: EJD. Vol. 26, No. 5, October 2016, pp. 493–495, doi: 10.1684 / ejd.2016.2808 , PMID 27212473 .
  • S. Nayak, B. Acharjya, P. Mohanty: Ichthyosis hystrix. In: Indian dermatology online journal. Vol. 4, No. 1, January 2013, pp. 47-49, doi: 10.4103 / 2229-5178.105483 , PMID 23440302 , PMC 3573454 (free full text).
  • WH Wang, LF Li, Q. Zhang, SM Yang, W. Jiang, YY Wang, PC Lei, XR Chen: Ultrastructural features of ichthyosis hystrix strongly resembling Lambert type. In: The British journal of dermatology. Vol. 156, No. 5, May 2007, pp. 1027-1031, doi: 10.1111 / j.1365-2133.2007.07792.x , PMID 17355236 .

Individual evidence

  1. ↑ Encyclopedia Dermatology
  2. ↑ Ichthyosis hystrix gravior. In: Orphanet (Rare Disease Database).
  3. ↑ Ichthyosis hystrix gravior.  In: Online Mendelian Inheritance in Man . (English)
  4. ↑ KID syndrome. In: Orphanet (Rare Disease Database).
  5. ^ Hystrix-like ichthyosis with deafness.  In: Online Mendelian Inheritance in Man . (English)
  6. ↑ DermIS.net
  7. ^ P. Biswas, A. De, S. Sendur, F. Nag, A. Saha, G. Chatterjee: A case of ichthyosis hystrix: unusual manifestation of this rare disease. In: Indian journal of dermatology. Vol. 59, No. 1, January 2014, pp. 82-84, doi: 10.4103 / 0019-5154.123512 , PMID 24470668 , PMC 3884936 (free full text).