MULTIPLE KERATOACANTHOMAS OF FERGUSON-SMITH

By Gary M. White, MD


Multiple keratoacanthomas of Ferguson-Smith (MKFS) is also known as familial primary self-healing squamous epithelioma of the skin or multiple self-healing squamous epithelioma of Ferguson-Smith (MSSE). Inheritance is AD. Large families with multiple members being affected have been described. Most cases have been from Scotland and indeed J. Ferguson-Smith was a Scottish Dermatologist who first described the condition. Average age of onset is about 25 years but it may begin in childhood, even as early as 3 days old [PD 1999;16;411] or very late in life, e.g., age 81.

A Danish family with 11 affected members has been described [Acta Derm Venereol 2008; 88: 52–56]. The mean age of onset in the family was 52.6 years (range 30–81 years). A total of 44 tumors, chiefly located in sun-exposed areas, were recorded and the average number of tumors per patient was 4 (range 1–23). 61.4% of the tumors were excised while 31.8% regressed spontaneously without treatment.

Clinical

The morphology is like the typical keratoacanthoma (KA) and the lesions tend to heal spontaneously but scars may result. They may number from a few to hundreds. Lesions are more common at sites of trauma. Some patients have had KAs unilaterally [AD 1956;74;52]. Although many regress, some lesions can be aggressive and metastasize.

See multiple KAs for a discussion of the differential diagnosis.

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