Urticaria pigmentosa

Last Updated: 2022-02-25

Author(s): Anzengruber F., Navarini A.

ICD11: 2A21.10

  • Nettleship, 1869
  • Sangster, 1878

Nettleship's disease.

A disease of the haematopoietic stem cell leads to clonal mast cell proliferation and increased accumulation in the skin. In systemic mastocytosis, internal organs can also be affected.

  • The first manifestation can occur within the first weeks of life, but also up to the 2nd year of life. Incidence frequency is biphasic, so urticaria pigmentosa may occur also after 40 yrs.
  • Incidence: Women = men

Unspecific point mutation KIT D816V.

  • Mostly on the trunk localised flat, oval reddish-grey-brownish macules and papules with few or no telangiectasias.
  • When dermographism is triggered, an elevated, urticarial reaction is seen. There is also triggering of generalised urticaria on rapid temperature change. After mechanical triggering, subepidermal blistering may also occur (pos. Dariers sign).
  • Visceral involvement in < 30% of children and about 50% of adults
  • Diarrhoea, vomiting, gastric ulcer, involvement of liver, spleen, CNS, lymph nodes and vessels

  • Clinical manifestation
  • Darier's sign/dermopgraphism
  • Biopsy
  • Laboratory
  • Blood count, liver enzymes, tryptase (normal value: <20µg/l)
  • To exclude systemic involvement
    • Abdominal sonography, iliac crest biopsy, bone marrow biopsy, scintigraphy, gastroscopy, colonoscopy, bone densitometry
    • Ruling out a carcinoid syndrome or a pheochromocytoma

  • > 50% of all children experience spontaneous remission
  • Life expectancy is normal in 95% of patients

  • Low histamine and low salicylate diet
  • Keep off / avoid:

  • Changes in temperature
  • Insect bites
  • NSAIDs
  • Codeine
  • Procaine
  • Polymyxin B
  • X-ray contrast media
  • Mechanical triggering

 

Topical therapy

  • Antipruritic topical agents
    • Polidocanol cream 5%, several times a day
    • Carbamide/Urea-containing lotion ad libitum several times a day
      • Attention cooling strength!
  • Topical antihistamines
    • Dimetinden gel
  • Dermocorticoids
    • Class I: 
      • Hydrocortisone (hydrocortisoni-17 butyras)
        • Emulsion, cream, lotion
    • Class II:
      • Clobetasone (clobetasoni-17 butyras)
        • Crème, ointment

 

  • Radiation therapy
    • UVA1 irradiation
    • PUVA therapy

 

Systemic therapy

  • Antihistamines
    • H1 blockers
      • Levocetirizine per os 5 mg once daily
      • Desloratadine per os 5 mg once daily
      • Fexofenadine per os 180 mg once daily

 

  • For pruritus-induced sleep difficulties
    • Hydroxyzine 25 mg once daily at night
    • In combination with
      • H2 blocker
        • Ranitidine per os 300mg once daily

 

  • Systemic glucocorticoids
    • Prednisolone per os 40-60 mg

 

  • Monoclonal antibody
    • IgE antibody
      • Omalizumab

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  10. Gobello T, Mazzanti C, Sordi D, et al. Medium- versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study. Journal of the American Academy of Dermatology 2003;49:679-84.