An 11-year-old boy presented with a history of recurrent colicky abdominal pain for the preceding three months. There was no history of hematemesis or melena, or significant family history. He had multiple hyper-pigmented macules over the nose, lips, and buccal mucosa (Fig. 1). There was no mucocutaneous lesion elsewhere in the body. The abdominal and other systemic examination was non-contributory. Complete hemogram, routine biochemical panels, and ultrasonography of the abdomen were normal. Stool for occult blood was negative. Upper gastrointestinal endoscopy showed no abnormality; colonoscopy revealed multiple polyps in the colon. Based on the typical muco-cutaneous pigmen-tation and colono-scopy findings, a diagnosis of Peutz Jegher syndrome (PJS) was made. Histopathology of the colonic specimen further confirmed it to be a PJS -type of intestinal polyp.

Fig. 1 (a) Hyperpigmented macules on lips (especially on lower lip), and a few scattered pigmented macules in the peri-nasal area; and (b) pigmented macules on lips and buccal mucosa.

Mucocutaneous pigmentary changes of PJS usually appear during early infancy and scattered over the lips, buccal mucosa, perioral and perianal areas, fingers, feet, and less commonly over the gums and palate. Differentials of the oral pigmentation of the present case were : Laugier-Hunziker syndrome (PJS like muco-cuteneous changes, pigmented nail streaks, no visceral involvement), Addison’s disease (pigmentation of the oral mucosa, skin creases and pressure points, fatigue, postural hypotension), Carney complex (associated with blue nevus and pigmentery schwannomas, myxomas of skin and heart), oral melanocytic nevus (gray-brown or black lesion, located over hard palate and buccal mucosa, common in females and in old age), and resolving oral lichen planus (usually associated with cutaneous lesions of lichen planus). Management of PJS includes removal of the technically feasible gastrointestinal polyps, laser treatment for the lentigens, and regular surveillance for malignancies.