Darier’s Sign in Solitary Mastocytoma ( 2 фото )

A previously healthy 10-month-old girl was referred to the pediatric dermatology clinic with a 6-month history of an intermittently itchy and swollen skin lesion on her neck that had gradually increased in size. On physical examination, a thin, well-demarcated, tan plaque was seen on the neck (Panel A). The lesion was rubbed firmly with a tongue depressor for 5 seconds, and within a few minutes it became edematous with a flare of surrounding erythema — a finding known as Darier’s sign (Panel B). A diagnosis of solitary mastocytoma was made. Solitary mastocytoma is a localized form of cutaneous mastocytosis, a condition that is characterized by an excessive number of mast cells in the skin in the absence of systemic mast-cell disease. Solitary mastocytomas typically develop in infancy and gradually resolve during childhood. Darier’s sign, which results from mast-cell degranulation and histamine release when the skin lesion is rubbed, is seen in most solitary mastocytomas and supports the diagnosis (although the finding is typically more subtle than was observed in this patient). Treatment with a topical glucocorticoid ointment and oral diphenhydramine as needed for itch and swelling was given. Follow-up for a solitary mastocytoma is generally not necessary unless there are unexpected changes associated with the lesion and was not recommended for this patient.
Haleigh Dawn Stafford, B.A. , and Raegan D. Hunt, M.D., Ph.D.
Published January 29, 2025
N Engl J Med 2025;392:494
VOL. 392 NO. 5


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