Pigmented Macules of the Lips

The patient is a 70 year-old architect who presented for another problem.  Incidental hyperpigmented macules were noted on the lower lip.  No family members are similarly affected nor does he have a family hisrory of colon cancer.  He has had unremarkable colonoscopies.  The pigmentation began ~ 20 years ago.

O/E:  Hyperpigmented macules of lower lip.

Clinical Photo:

Diagnosis: Probable Laugier Hunziker syndrome is favored.

References:

1. Laugier Hunziker syndrome. Jabbari A, et. al. Dermatol Online J. 2010;16(11):23.

Abstract: Laugier Hunziker syndrome is a rare disorder that is characterized by adult-onset hyperpigmented macules of the lips, oral cavity, and fingertips. Longitudinal melanonychia is present in the majority of cases. We present a 45-year-old woman with adult-onset hyperpigmented macules of the oral cavity as well as linear melanonychia that involved multiple fingernails. The history, clinical examination, and paucity of laboratory abnormalities or systemic findings support a diagnosis of Laugier Hunziker syndrome.  Free Full Text.

2. Peutz-Jager Syndrome eMedicine.
Peutz-Jeghers syndrome (PJS) is an autosomal dominant inherited disorder characterized by intestinal hamartomatous polyps in association with a distinct pattern of skin and mucosal macular melanin deposition. Patients with PJS have a 15-fold increased risk of developing intestinal cancer compared with that of the general population.