Case Letter

Laugier-Hunziker Syndrome

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Practice Points

  • Laugier-Hunziker syndrome (LHS) comprises benign mucosal pigmentation in the absence of gastrointestinal pathology.
  • Differentiating LHS from Peutz-Jeghers syndrome can prevent unnecessary aggressive cancer screening protocols.
  • The average age of onset of LHS is 52 years and typically occurs in white adults.
  • Pigmentation in LHS is most commonly distributed on the lower lips and oral mucosa.

 

References

To the Editor:

A 55-year-old man presented with hyperpigmented brown macules on the lips, hands, and fingertips of 6 years’ duration. The spots were persistent, asymptomatic, and had not changed in size. The patient denied a history of alopecia or dystrophic nails. He also denied a family history of similar skin findings. He had no personal history of cancer and a colonoscopy performed 5 years prior revealed no notable abnormalities. His medications included amlodipine and hydrocodone-acetaminophen. His mother died of “abdominal bleeding” at 74 years of age and his father died of a brain tumor at 64 years of age. Physical examination demonstrated numerous well-defined, dark brown macules of variable size distributed on the lower and upper mucosal lips (Figure 1A), buccal mucosa, hard palate, and gingiva, as well as the dorsal aspect of the fingers (Figure 1B) and volar aspect of the fingertips (Figure 1C).

Figure 1. Numerous well-defined, dark brown macules of variable size distributed on the lower lip (A), dorsal aspect of both hands (B), and volar aspect of the fingertips (C).

A shave biopsy of a dark brown macule from the lower lip (Figure 2) was performed. Histopathologic examination revealed pigmentation of the basal layer of the epidermis with pigment-laden cells in the dermis immediately deep to the surface epithelium. Immunoperoxidase stains showed a normal number and distribution of melanocytes.

Figure 2. A shave biopsy of a dark brown macule from the lower lip displayed pigment-laden macrophages in the papillary dermis (H&E, original magnification ×40).

A diagnosis of Laugier-Hunziker syndrome (LHS) was made given the age of onset; distribution of pigmentation; and lack of pathologic colonoscopic findings, personal history of cancer, or gastrointestinal tract symptoms.

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