N° 3 - Salute per tutti

Figure 5
Figure 6
Figure 7
Figure 8
Laugier-Hunziker syndrome
gernails, which are common sites for LHS, but
also in genital region that is less frequent.
The cause of this benign condition remains
undetermined. No significant systemic pathological
association has been recognized and the
pigmentary lesions carry no risk of malignant
degeneration.
Histologically, there is increased basal keratinocyte
melanin without expansion of the melanocytic
population and superficial pigmentary
incontinence with dermal melanophages.
Mild to moderate acanthosis may also be
noted. 1,2,6
Electron microscopy reveals increased numbers
of mature, isolated or grouped melanosomes of
variable sizes within the cytoplasm of keratinocytes
and melanophages. 1,3
None of the histological features of L a u -
gier–Hunziker syndro m e (acanthosis, basal
hypermelanosis and dermal pigmentary incontinence)
is specific, and thus any one feature is
insufficient to establish the diagnosis.
However, when in association and accompanied
by a typical clinical setting, these histological
features are characteristic. 7
Several conditions may induce abnormal mucocutaneous
and/or nail pigmentation resembling
the clinical picture of LHS. 8
These include adrenal insufficiency, exposure to
systemic agents, especially tetracyclines and
c h e m o t h e r a p y, and Peutz–Jeghers syndro m e
(PJS). Primary adrenal insufficiency was eliminated
on a clinical and biological basis. Drug
ingestion did not appear relevant in the absence
of prior or current drug intake likely to cause
such pigmentation.
Peutz-Jeghers syndrome features mucocutaneous
pigmentation associated with hamartomatous
polyps of the bowel that show malignant potential
and often occurs in patients with a family
history.
The pigmentation generally occurs at birth, in
infancy, or in early childhood. It consists of
brown to black pigmentation of the buccal
mucosa, the fingers, and especially the lower
lip. Cutaneous pigmentation tends to fade after
adolescence, whereas that in the oral cavity persists.
However, our patient had no hamartomatous
polyps of the bowel on endoscopy and no
positive family history.
Pigmentation first appeared when she was
middle-aged and was not associated with any
other abnormalities.
Many patients with LHS want treatment for
Journal of Plastic Dermatology 2008; 4, 3
287