In Peutz-Jeghers syndrome (discussed more fully in Chapter 7), oral pigmentation is distinctive and is usually pathognomonic.Multiple focal melanotic brown macules are concentrated about the lips while the remaining facial skin is less strikingly involved. The macules appear as freckles or ephelides, usually measuring < 0.5 cm in diameter (Figure 6-10).
Similar lesions may occur on the anterior tongue, buccal mucosa, and mucosal surface of the lips. Ephelides are also seen on the fingers and hands.Lesions on the perioral areas are essentially pathognomonic although in individuals who have diffuse cutaneous ephelides (such as red-haired light-complected individuals), an erroneous diagnosis could be made.
Histologically, these lesions show basilar melanogenesis without melanocytic proliferation.
