Papular dermographism: a sign of urticaria pigmentosa

Cameo

Papular Dermographism: A Sign of Urticaria Pigmentosa JOSEPH A. WITKOWSKI, M . D . AND LAWRENCE CHARLES PARISH, M . D .

From the Departments of Dermatology, University of Pennsylvania School of Medicine, and the Jefferson Medical College of the Thomas Jefferson University, Philadelphia, Pennsylvania

Case Report 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. Discussion

the lesions appear 3-6 hours after stroking the skin. They reach maximum intensity in 6-8 hours and disappear in 24-48 hours. Delayed dermographism is often accompanied by local burning and tenderness.^ Urticarial dermographism is commonly seen in the various clinical forms of urticaria pigmentosa. It varies from the usual triple response in its intensity and duration. Bullous lesions may be seen and wheals may be present for hours."* An increased number of mast cells is not found in symptomatic dermographism." When stroking evokes an early papular response (which persists) and the appearance of satellite wheals within the red flare, a biopsy of the skin with special stains for mast cells should be performed. Acknowledgment

In 1924, Lewis and Grant described a response of the skin to stroking and other stimuli.' This consisted of a red line, red flare, and a wheal. The wheal component, which appears in 1-3 minutes, is smooth and continuous, while conforming to the deforming injury. Burkhart^ described papular dermatographea. His patient developed papular wheales on scratching, which gradually expanded and coalesced to form linear streaks of dermographism. A papular dermographic response is also seen in delayed dermographism. In this condition.

Histologic interpretation was performed by Margaret G. Wood, M.D.

Address for reprints: Joseph A. Witkowski, M.D., 3501 Ryan Avenue, Philadelphia, PA 19136.

References 1. Lewis T, Grant RT, Vascular reaction of the skin to injury. II. Heart 11:209, 1924 2. Burkhart CF: Papular dermatographia. Int J Dermatol 19:562, 1980 3. Baughman RD, jillson OF: Seven specific types of urticaria: with special reference to delayed persistant dermographism. Ann Allergy 21:248, 1963 4. Hughes J, Warin RP: Diffuse cutaneous mastocytosis. Br J Dermatol 75:296, 1963 5. Levine Ml, Winkler L: Clinical aspects of dermographism. J Montefiore Hosp 1:25, 1966

0011-9059/83/1100/0529/$00.85 © International Society of Tropical Dermatology, Inc.

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