Urticaria pigmentosa in a child with sporadic bilateral diffuse conjunctival redness: Case report and review of the literature

Ali H. Al-Ghamdi, Ahmed Hassan Al-Ghamdi, Hasan S. Al-Ghamdi, Aziz A. Al-Sohaimi, Mesfer Al-Ghamdi


We present a case of a 2-year-old girl with urticaria pigmentosa. The clinical features indicated disseminated, discrete, non-clustered, reddish-brown macules, papules, and plaques on her body (0.5-1 cm in diameter).  Skin lesions occurred in the second month of life. There was associated sporadic bilateral lid swelling, diffuse conjunctival redness, and watery discharge. Darier's sign was positive. Diagnosis of mastocytosis was confirmed histopathologically. To the best of our knowledge, this information has not been previously reported.



Mast cells, mastocytosis, urticaria pigmentosa

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Akaglu G, Erkin G, Cakir B, Boztepe G, Sahin S, Karaduman A et al. Cutaneous mastocytosis: demographic aspects and clinical features of 55 patients. J Eur Acad Dermatol Venereol. 2006;20:969-73.

Metcalfe DD. The mastocytosis syndrome. In: Fitzpatrick TB, Eisen AZ, Wolff K, Freedberg IM, Austen K, editors. Dermatology in General Medicine. New York: McGraw-Hill; 1993. P. 2017-23.

Azaña JM, Torrelo A, Mediero IG, Zambrano A. Urticaria pigmentosa: a review of 67 pediatric cases. Pediatr Dermatol. 1994;11:102-6.

Soter NA. The skin in mastocytosis. J Invest Dermatol. 1991;96:32S-39S.

Paller AS, Mancini AJ. Hurwitz Clinical Pediatric Dermatology, 4th ed. New York: Elsevier; 2011.

Nettleship E, Tay W. Rare form of urticaria. Br Med J. 1869;2:323-30.

Unna PG. Anatomie und pathogenese der urticaria simplex und pigmentosa. Monatschr Prakt Dermatol. 1887;6:EH1

Braun-Falco O, Plewig G, Wolff HH, Winkelmann RK. Mastocytosis, editors. Dermatology. Berlin: Springer-Verlag; 1991. P. 1107-12.

Ehrlich P. Beitrege zur Kenntnis der Anilinfaerbungen und ihrer Verwendung in der mikroskopischen Technik. Arch Mikr Anat. 1877;13:263-77.

Erlich P. Uber die specifichen granulationen das blutes. Archiv Physiologie. 1879, 571-9.

Seitz CS, Rose C, Brocker EB, Trautmann A. Intertriginous urticaria pigmentosa. Dermatology. 2005;210:77-9. http:// dx.doi.org/10.1159/000081493

Nagayo K, Sakai M, Mizuno N. Juvenile xanthogranuloma with Darier’s sign. J Dermatol. 1983;10:283-5.

Yen A, Sanchez R, Oblender M, Raimer S. Leukemia cutis: Darier’s sign in a neonate with acute lymphoblastic leukemia. J Am Acad Dermatol 1996; 34:375-8.

Garriga MM, Friedman MM, Metcalfe DD. A survey of the number and distribution of mast cells in the skin of patients with mast cell disorders. J Allergy Clin Immunol. 1988;82:425.

Greaves MW. Mastocytoses. In: Champion RH, Burton JL, Ebling FJG, editors. Textbook of Dermatology. Oxford: Blackwell Scientific Publications; 1992. P. 2065-72.

Shaikh A, Benjamin L. Allergic eye disease associated with mastocytosis. Eye. 2003;17:788-90.

Scheck O, Horny HP, Ruck P, Schmelzle R, Kaiserling E. Solitary mastocytoma of the eyelid. A case report with special reference to the immunocytology of human mast cells and a review of the literature. Virchows Arch A Pathol Anat Histopathol.1987;412:31-6.

Jacoby BG, Wesley RE. Painful orbital inflammatory lesions and mastocytosis. Ann Ophthalmol 1987;149(4):146-7.

Lesser RL, Brodie SE, Sugin SL. Mastocytosis-induced nyctalopia. J Neuroophthalmol. 1996;16:115-9.

Tharp MD. Mastocytosis. Bolognia JL, Jorizzo JL, Schaffer JV, editors. Dermatology, Volume two, 3rd edn. Philadelphia: Elsevier; 2012. P. 1993-2000.

Horny H-P, Akin C, Metcalfe DD, Bennett JM, Valent P, Bain BJ. Mastocytosis. In: Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H et al., editors. WHO Classification of Tumours of Haematopoetic and Lymphoid Tissues. Lyon, France: IARC Press; 2008. P. 54-63.

Keyzer JL, DeMonchy JGR, van Doormaal JJ, van Voorst Vader PC. Improved diagnosis of mastocytosis by measurement of urinary histamine metabolites. N Engl J Med. 1983;309:1603-5.


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