Lalita, a female patient presents with pigmentation of the lips and oral mucosa and intestinal polyps. Her sister also gives the same history. Most probable diagnosis is:
High-Yield Explanation
The polyps of Peutz-Jeghers syndrome are generally considered to be hamaomas Hamaomatous polyps (usually <100) throughout the GIT, most common in jejunum Associated with Hypermelanotic macule in the perioral region, buccal mucosa. Mucocutaneous pigmentation usually occurs during infancy and most commonly noted in perioral and buccal region. Pigment spots usually appear in first few years of life, reach a maximum level in early adolescence and can fade in adulthood. However, pigmentation on the buccal mucosa remains throughout the life. The pigmented macules of PJS have no malignant potential. Screening consists of a baseline colonoscopy and upper endoscopy at age 20 years, followed by annual flexible sigmoidoscopy thereafter.