Laugier Hunziker syndrome

The patient is a 74 yo man with a long history of oral hyperpigmentation.  He was presented on VGRD in 2012, but we have further history now.  The pigmentation has been present many years. His mother had a similar process by history.

He has a history of colon polyps. His paternal grandmother had colon cancer. His mother had colonic polyps and breast cancer. His father and his father’s brother both had leukemia.

O/E:  There are multiple dark brown irregular lenticular hyperpigmented macules of 2–5 mm diameteron the lower lip ant tongue.  No other hyperpigmentation was noted.

Clinical Image:

Diagnosis: Laugier Hunziker syndrome vs Peutz Jeghers syndrome

Case to be discussed at Hot Spots 2016

References:

1. Laugier–Hunziker Syndrome: A Rare Cause of Oral and Acral Pigmentation

Silonie Sachdeva, Shabina Sachdeva, and Pranav Kapoor

J Cutan Aesthet Surg. 2011 Jan-Apr; 4(1): 58–60.

Abstract: Laugier–Hunziker syndrome (LHS) is an acquired, benign pigmentary skin condition involving oral cavity including lower lip in the form of brown black macules 1–5 mm in size, frequently associated with longitudinal melanonychia. There is no underlying systemic abnormality or malignant predisposition associated with LHS, and therefore the prognosis is good. Important differential diagnoses include Peutz Jeghers syndrome and Addison’s disease among other causes of oral and acral pigmentation.  PubMed Central.

Labial Macules

The patient is a 33 year-old woman who recently noted pigmented macules on her lower lips.  She admits to having an anxiety disorder and being preoccupied with her health (which is otherwise excellent).  Her anxiety dates back to the death of her father from colon cancer at age 52 (when she was 10 years old).  She was seen for an unusual cheilitis in 2010 and her case was presented on VGRD then.

O/E:  There are six lightly pigmented macules on her lower lip.  No other pigmentation on her oral mucosae. No other pigmented macules noted.

Discussion: While these macules could be post-inflammatory changes from her allergic cheilitis, it's hard to ignore her family history and the possibility that they could be a forme fruste of Peutz-Jeghers Syndrome.  Endoscopy seems, to me at least, to be indicated.  With classical PJS the pigmented macules may be present even in infancy or childhood.  Visualizing her G.I. tract is important here, if only to allay this woman's anxiety.

Your thoughts will be appreciated.