N° 3 - Salute per tutti

Miroslava Kadurina
Borislav Dimitrov
Slava Bojinova
Antoaneta Jordanova
Ivan Litov
Vesel Kantarjiev
Stoyan Tonev
Department of Dermatovenereology
and Allergology – Military Medical Academy,
Sofia, Bulgaria
Laugier-Hunziker syndrome
SU M M A R Y
I ntroduction
Laugier-Hunziker syndrome (LHS) was
initially described in 1970 as acquired, benign
hyperpigmentated macules of the lips and buccal
mucosa frequently associated with longitudinal
melanonychia. 1 Extended mucocutaneous
features have been observed since that
original description, including macular pigmentation
of the genitalia. No underlying systemic
abnormalities are associated with LHS, and
no malignant predisposition exists.
When associated with nonclassic body locations
or atypical features, the name idiopathic
lenticular mucocutaneous hyperpigmentation
has been used. 2
Recognition of the syndrome is important
because the development of new areas of pigmentation
in midlife, especially on mucosal
surfaces, may be worrying. The differential dia-
Laugier-Hunziker syndrome
Laugier-Hunziker syndrome (LHS) was initially described in 1970 as acquired,
benign hyperpigmentated macules of the lips and buccal mucosa frequently associated
with longitudinal melanonychia. Extended mucocutaneous features have been
observed since that original description, including macular pigmentation of the genitalia.
No underlying systemic abnormalities are associated with LHS, and no malignant
predisposition exists. When associated with nonclassic body locations or atypical
features, the name idiopathic lenticular mucocutaneous hyperpigmentation has
been used. We present a 54 - year old woman with longitudinal brown nail hyperpigmentaion
and pigmented macules on the lips and genitalia. Peutz-Jeghers syndrome
was excluded by appropriate investigations and the diagnosis of Laugier –
Hunziker syndrome was made. The etiology of melanosis in LHS is unknown. A lack
of family members with LHS is characteristic in most cases. The pigmented macules
of LHS are not lentigines. They demonstrate mild-to-moderate acanthosis in most
cases.The predominant finding is basal cell hypermelanosis. The melanin deposition
in the basal layer is dense and uniform. Rete ridges may be normal in size, or they
may be elongated. Numerous melanophages are often present in the papillary dermis.
The basement membrane has been found to be intact. Pigment incontinence may
also be present. Laugier-Hunziker syndrome affects usually women with an average
age of 42 and no spontaneous resolution has been described.We describe the first
case in Bulgaria with good prognosis, not associated with systemic symptoms. The
patient is left under observation.
KEY WORDS: Laugier-Hunziker syndrome
gnosis includes that of melanoma and
underlying systemic disorders such as Peutz-
Jeghers syndrome and adrenal insufficiency.
ase report
C
We report a 54 - year old woman who
was re f e r red to our clinic with a 5-month history
of brown pigmentation in her mouth, on the lips
and genitalia. At the same time, the patient had
noticed vertical brown lines running along a
number of fingernails. The patient was systemically
well with no history of preceding skin
lesions, gastrointestinal problems, or anaemia.
She was not on medications and had never
smoked. There was no family history of mucocutaneous
pigmentation or intestinal polyps.
Journal of Plastic Dermatology 2008; 4, 3 285