Reticular pigmentosa dermatopathy L81.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 23.12.2021

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History
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Hauss and Oberte-Lehn, 1958

Definition
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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.

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

Localization
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Especially on the trunk, neck, shoulders and hips.

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

Therapy
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No specific therapy known. Symptomatic with covering externals.

Note(s)
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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.

Literature
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  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