Familial Angiofibrohistiocytic Hamartoma Syndrome

A 27-year-old Hispanic man presented with multiple papules and nodules measuring up to 10 cm in diameter. These lesions were widespread (Figure 1), but not on mucosal epithelium. At birth, the patient had had multiple hypopigmented macules that had progressed to papules and nodules over time. Dermatoscopic examination of these papules and nodules showed prominent capillaries. New lesions were constantly developing but were slow-growing. Other family members had similar lesions, including the patient's three brothers, father, paternal aunt, and a 3-year-old niece (daughter of the 2nd eldest brother), although not as extensively as in this patient. The paternal grandparents were not affected (Figure 2). As the patient had been raised in rural Mexico with limited financial resources, access to health care was limited, so the condition had been left undiagnosed and untreated for most of the patient's life.