Mystery behind labial and oral melanotic macules: Clinical, dermoscopic and pathological aspects of Laugier-Hunziker syndrome

Table 4 Differential diagnosis between Peutz-Jeghers syndrome and Laugier-Hunziker syndrome

	PJS	LHS
Inheritance	Autosomal dominant (STK11 gene)	Sporadic and acquired
Age of onset	Birth to infancy	Adult onset
Shape of mucocutaneous pigmented macules	Freckle-like	Lenticular
Labial pigmentation	Very common	Very common
Oral pigmentation	Common	Very common
Perioral, perirhinal, or periorbital pigmentation	Common	Uncommon
Nail pigmentation	Uncommon	Very common
Acral skin pigmentation	Common	Common
Systemic involvement	Gastrointestinal polyposis	None
Risk of malignancy	Colon, gastric, small intestinal, pancreatic, breast, ovarian, thyroid, lung, and Sertoli cell (in men) cancers	None

PJS: Peutz-Jeghers syndrome; LHS: Laugier-Hunziker syndrome.