Laugier-Hunziker syndrome

Sir, Idiopathic lenticular mucocutaneous pigmentation [Laugier-Hunziker syndrome (LHS)] is a benign pigmentary disorder characterized by discrete and confluent, slate to brown-black macules on skin and mucosae. It is a rare disorder which represents an important clinical differential to Peutz-Jeghers syndrome (PJS), which is associated with gastrointestinal hamartomatous polyps and various benign and malignant neoplasms. Absence of these systemic findings suggests the diagnosis of LHS. 19-year-old female presented with multiple, discrete, brown-black oval to irregular shaped macules on lips, buccal mucosae, palate, tongue, and perioral skin, since birth. She also developed multiple light brown freckles on upper part of face including periorbital skin, nose, cheeks, fingertips and nail folds at the age of 10-12 years, which increased insidiously in number for the next 4-5 years during adolescence and since then it remained static. She had normal built and no history suggestive of intestinal involvement (like recurrent abdominal pain, vomiting, gastrointestinal bleed, malena), palpitation or weakness. There was no history of any drug intake or excessive sun exposure. Rest of the skin was normal and there were no blue nevi, soft tissue swellings or hair abnormalities. There was no family history of similar skin lesions or findings suggestive of gastrointestinal involvement seen in PJS.