Urticaria pigmentosa

• 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

1. Hannay MG. URTICARIA PIGMENTOSA IN ADULTS. *. Br J Dermatol 1925;37:1-13.
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.
7. Azaña JM, Torrelo A, Mediero IG, Zambrano A. Urticaria Pigmentosa: A Review of 67 Pediatric Cases. Pediatric Dermatology 1994;11:102-6.
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.