Reticular pigmentosa dermatopathyL81.8

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 23.12.2021

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HistoryThis section has been translated automatically.

Hauss and Oberte-Lehn, 1958

DefinitionThis section has been translated automatically.

Rare (described are <100 persons) disease pattern developing in childhood with initially macular pigmentation, later formation of a reticular network. Characteristic is also a non-scarring alopecia, an onychodystrophy and inconstant diffuse palmoplantar keratosis with pressure point associated callus-like hyperkeratoses.

EtiopathogenesisThis section has been translated automatically.

Mutations are described in KRT14 (17q12-q21), a gene coding for keratin 14 (Burger B et al. 2019).

LocalizationThis section has been translated automatically.

Especially on the trunk, neck, shoulders and hips.

HistologyThis section has been translated automatically.

Histologically, pigment incontinence with melanin-laden macrophages in the upper corium can be detected.

TherapyThis section has been translated automatically.

No specific therapy known. Symptomatic with covering externals.

Note(s)This section has been translated automatically.

Dermatopathia pigmentosa reticularis is regarded as an allelic variant of the Naegeli-Franceschetti-Jadassohn syndrome, since mutations in KRT14- are also detectable in this genodermatosis.

LiteratureThis section has been translated automatically.

  1. Bu TS et al (1997) A case of dermatopathia pigmentosa reticularis. J Dermatol 24: 266-269
  2. Burger B et al. (2019) Naegeli-Franceschetti-Jadassohn syndrome and dermatopathia pigmentosa reticularis: Intrafamilial overlap of phenotypes in patients with the same KRT14 frameshift variant.Br J Dermatol. 2019 Apr 9.
  3. Datta A et al (2019) Dermatopathia pigmentosa reticularis. Indian J Dermatol 64:149-151.
  4. Elias L et al. (2020) Dermatopathia pigmentosa reticularis with beard alopecia: first report from Syria. Oxf Med Case Reports 2020:omaa079.
  5. Goh BK et al. (2009) A case of dermatopathia pigmentosa reticularis with wiry scalp hair and digital fibromatosis resulting from a recurrent KRT14 mutation. Clin Exp Dermatol 34:340-343.
  6. Heimer Wl et al (1992) Dermatopathia pigmentosa reticularis: A report of a familiy demonstrating autosomal dominant inheritance. J Am Acad Dermatol 26: 298-301
  7. Hauss H, Oberste-Lehn H (1958) Dermatopathia pigmentosa reticularis. Derm Wschr 133: 1337
  8. Itin PH et al (1998) Genodermatosis with reticulate, patchy and mottled pigmentation of the neck--a clue to rare dermatologic disorders. Dermatology 197: 281-290
  9. Maso MJ et al (1990) Dermatopathia pigmentosa reticularis. Arch Dermatol 126: 935-939
  10. Rycroft RJG, Calnan CD, Allenby CF (1977) Dermatopathia pigmentosa reticularis. Clin Exp Dermatol 4: 498-710

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Last updated on: 23.12.2021