Urticaria pigmentosa

Last Updated: 2021-10-15

Author(s): -

Nettleship, 1869; Sangster, 1878

Generalized eruption of cutaneous mastocytosis (childhood type)

Hematopoietic stem cell disease leads to clonal mast cell proliferation and increased accumulation in the skin. Systemic mastocytosis can also affect internal organs.

  • Initial manifestation can occur within the first weeks of life, but also up to the age of 2. The incidence frequency is biphasic, so that urticaria pigmentosa can also occur after 40 years.
  • Women = Men.

Non-specific point mutation KIT D816V

  • Flat, oval, reddish-greyish-brown maculae and papules with few or no telangiectasia (Teleangiectasia macularis eruptiva perstans), mostly located on the trunk.
  • When dermographism is triggered, an eleventh, urticarial reaction occurs. Generalized urticaria (pos. Darier's sign) is also triggered during rapid temperature changes. After mechanical triggering, subepidermal blistering may also occur.

  • Visceral infestation in < 30% of children and approx. 50% of adults.

  • Diarrhoea, vomiting, stomach ulcer, liver, spleen, CNS, lymph nodes and vessels.

Biopsy

  • Clinic
  • Darier sign/Dermographism 

  • Lab
  • Blood count, liver values, tryptase (standard value: <20µg/l).

  • To the exclusion of a system participation 
    • Abdomen sono, iliac crest biopsy, Contrast agent biopsy, scintigraphy, gastroscopy, colonoscopy, bone density measurement 

    • Exclusion of carcinoid syndrome or pheochromocytoma. 

  • Spontaneous remission occurs in >50% of all children.
  • Life expectancy is normal in 95% of patients 

  • Maternity leave/ Avoidance of 
  • Temperature changes 

  • Insect bites 

  • NSAID/ASA
  • Codeine 
  • Procaine
  • Polymyxin B
  • X-ray contrast agent 
  • Mechanical triggering 

 

  • Low histamine and low salicylate diet 

 

  • Topical Therapy 

  • Antipruritic topicals 

  • #5% Polidocanol in cold cream several times a day
  • #Excipial U Lipolotio® cum 2% Menthol (sic!) ad libitum several times a day 
  • Attention Cooling power!

  • Topical antihistamines 
  • Dimetindene
  • #Fenistil® Gel
  • Dermocorticoids 
  • Class I: (weakly effective)

  • Hydrocortisone (hydrocortisoni-17 butyras)

  • #Locoid® Crelo Emulsion
  • #Locoid® Crème
  • #Locoid® Lipo Creme
  • #Locoid® scalp lotion
  • Class II: (moderately effective)

  • Clobetasone (Clobetasoni-17 butyras)
  • #Emovate® Crème
  • #Emovate® Ointment

 

  • Radiation therapy 
  • UVA1 irradiation 

  • PUVA therapy 

 

·         Systemic Therapy

  • Antihistamines 
  • H1 blocker
  • Levocetirizine (#Xyzal®) p.o. 5 mg 1x day 

  • Desloratadine (#Aerius®) p.o. 5 mg 1x day
  • Fexofenadine (#Telfast®) p.o. 180 mg 1x day

 

  • Pruritus-induced sleeping difficulties 

  • Hydroxyzin (#Atarax®) 25 mg 1x daily 

  • In combination with 

  • H2-blocker

  • Ranitidine 

  • Zantic® p.o. 300 mg 1x daily 

 

  • Systemic glucocorticoids 

  • Prednisolone (#Spiricort®) p.o. 40-60 mg

 

  • Monoclonal antibody 

  • IgE antibodies 

  • Omalizumab
  • #Xolair®

 

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  2. Lehner E. II. Beitr&auml;ge zur Klinik und Histologie der Urticaria pigmentosa. Dermatology 1926;46:87-93.
  3. Caplan RM. The Natural Course of Urticaria Pigmentosa. Arch Dermatol 1963;87:146.
  4. Barton J. Treatment of Urticaria Pigmentosa With Corticosteroids. Arch Dermatol 1985;121:1516.
  5. Czarnetzki BM, Rosenbach T, Kolde G, Frosch PJ. Phototherapy of urticaria pigmentosa: Clinical response and changes of cutaneous reactivity, histamine and chemotactic leukotrienes. Archives of Dermatological Research 1985;277:105-13.
  6. Fenske NA. Congenital Bullous Urticaria Pigmentosa. Arch Dermatol 1985;121:115.
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  8. Topar G, Staudacher C, Geisen F, et al. Urticaria Pigmentosa: A Clinical, Hematopathologic, and Serologic Study of 30 Adults. American Journal of Clinical Pathology 1998;109:279-85.
  9. Guler E, Emir S, Kutluk T, Varan A, Buyukpamukcu M. Urticaria Pigmentosa Associated with Wilms Tumor. Pediatric Dermatology 2001;18:313-5.
  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.