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47 year old woman notices small dark spots on her lips and inner cheeks developing over 4-6 months.
On examination, there are multiple slate blue grey/brown macules on the tongue, lips and inner cheeks. No fingernail involvement. She recently had a complete medical exam with no hormonal abnormalities detected and no history of skin cancer and no history of congenital diseases. No history of GI symptoms or Peutz-Jeghers Syndrome. Otherwise healthy.
What is your diagnosis?
Diagnosis: Laugier-Hunziker Syndrome
Manifestations and Major Findings: Benign, acquired, lentiginous macular pigmentation of labial and buccal mucosa and nails. Usually develops over 3-9 months.
The condition is not associated with systemic abnormalities (e.g. gastrointestinal polyps) as seen in Peutz Jeghers syndrome. Peutz-Jeghers also presents in infancy and childhood and 2/3 of those affected with Peutz Jeghers also have lesions on the hands and feet.
In this case, interestingly, she was without nail involvement. I reassured her that the condition was benign.
References:
An Illustrated Dictionary of Dermatologic Syndromes
Susan Bayliss Mallory MD
Koch SE, LeBoit PE, Odom RB: Laugier-Hunziker syndrome. J Am Acad Dermatol 1987; 16:431-434.
Sterling GB, Libow LF, Grossman ME: Pigmented nail streaks may indicate Laugier-Hunziker syndrome. Cutis 1988; 42:325-326
Dermnetnz.org
Emedicine.medscape.com
Nature.com
Ncbi.nlm.nih.gov
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