Urticaria pigmentosa is a rare chronic skin disease characterized by pigmented macules, papules, or nodules which become edematous if rubbed. There are three varieties depending upon the predominant type of lesion: (1) macular, (2) papulonodular, and (3) maculopapulonodular. The macular variety is the commonest. Usually, the disease has its onset within the first year of life, and it frequently subsides at puberty. However, the disease may not occur until adult life. Initially, the lesions may be urticarial, or rarely bullous. The sites of predilection are the trunk, arms, and thighs, although any cutaneous area may be affected. A diagnostic sign is urtication of the lesions when they are rubbed or otherwise irritated by friction. A tendency toward generalized dermographism is frequently present. The degree of pruritus varies with the individual but is rarely severe. The only sequel is permanent pigmentation where the lesions have been.
The individual lesions are
SCOTT MJ, LEWIS GM. URTICARIA PIGMENTOSA: Report of Case with Solitary Lesion. AMA Arch Derm Syphilol. 1952;66(5):618–620. doi:10.1001/archderm.1952.01530300074009
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