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Papular Dermographism: A Sign of Urticaria Pigmentosa

A 25-year-old white woman presented with the complaint of recurrent, mildly pruritic, red "blotches" of the face, neck, chest, and back, of six months' duration. Aggravating factors included emotional stress, physical exertion, and hot showers. Examination showed mild freckling of the chest and back. Stroking the skin with the thumb nail evoked a normal red line, flare, and a 3-F beaded urticarial response (Fig. 1), which was intensely pruritic. After two minutes, small, urticarial papular lesions appeared within the red flare. The papular urticarial lesions persisted for 1 hour and 10 minutes. Review of systems was noncontributory except for chronic fatigue. Her maternal grandmother has a thalassemia trait. Routine laboratory tests disclosed a Hgb 11.1 g/dl, RBC5.27 x 10«, WBC 6.8 X 10', Hct 32.7%, MCV 62.1, MCH 21.0 mg, MCHC 33.8 g/dl, RDW 13.3, platelets 3.31 x lO^, polys 66, lymphs 30, and monos 4. There was mild hypochromia, poikilocytosis, anisocytosis, and microcytoses with an occasional target cell, microcyte, ovalocyte, and keratocyte. A Technicon SMA 12 system analysis was normal. Biopsy of a brown macule from the back and stained with Toluidine blue showed a significant increase in the number of mast cells surrounding small blood vessels.