Laugier-Hunziker sendromu: bir olgu sunumu

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Introduction
Also known as idiopathic lenticular mucocutaneous pigmentation,
Laugier-Hunziker syndrome (LHS)
is an acquired, idiopathic macular
hyperpigmentation of the oral
mucous membranes and lips, which
is frequently associated with melanonychia (1,2). Subjects involved are
otherwise healthy. LHS is a rare disease with approximately 100 cases
reported in the literature (3).
LHS is characterized by the presence of a variable number of asymptomatic, isolated or confluent, slate to
dark brown pigmented macules
involving the oral mucosa and/or lips,
and in 60%, the fingernails (4,5).
Herein, we describe a female patientCilt 48 · Sayý 2 · Gülhane TD Laugier-Hunziker syndrome · 105
lished and liquid nitrogen cryosurgery using an open spray, single
freeze thaw cycle was carried out.
After 2 weeks of treatment a marked
improvement of the pigmented
lesions was achieved (Figure 1b).
Figure 1a. Confluent, hyperpigmented macules on the lateral margins and tip of the
tongue. 1b. Marked regression of the lesions
after cryosurgery
Discussion
Laugier and Hunziker first
described five cases of essential
melanin pigmentation of the mouth
and lips, with longitudinal nail pigmentation (5). The eponymous term
Laugier-Hunziker syndrome was
later coined to designate the entity.
The onset of the disease is usually
between the third to fifth decade of
life. There is no sex preponderance
(6). Neither familial association nor
any associated systemic diseases have
been reported, to date. LHS is mainly
a disease of Caucasians and most of
the reported cases have been from the
European countries (7).
The clinical features consist of
diffuse or isolated pigmented macules, which have different colors
(from gray to bluish-black) with
well-defined or indistinct margins.
The lesions are most commonly
observed intraorally on the lips, the
buccal mucosa, the hard and the soft
palate, but may also be seen on the
gingiva, tongue and the basis of the
mouth. Pigmented macules may also
occur on the neck, thorax, abdomen,
perineal and perianal region, sclerae
and esophagus (8,9). Involvement of
the tongue in LHS is extremely rare
and is usually observed in association
with other locations of the oral cavity
(1,10). As far as we are aware, this is
the second case report of LHS manifesting with only tongue involvement. The incidence of nail pigmentation in LHS is 60%. Baran et al.
proposed three types of nail pigmentation in LHS: (i) isolated longitudinal streaks of varying degrees of pigmentation 1 to 2 mm in width, (ii) 2
to 3 mm double longitudinal streaks,
and (iii) homogenous pigmentation
of the radial or ulnar half of the nail
(11). Rarely, the the pigmentation
may spread from he proximal nail
fold into the surrounding skin (pseudo Hutchinson's sign) (11).
Histological examination reveals
increased melanin deposition in the
basal layer keratinocytes without an
increase in the number of
melanocytes, dermal pigmentary
incontinence and melanonphages in
the upper lamina propia or dermis.
Mild to moderate acanthosis may also
be observed (5,7). The findings of
confluent bluish-black macular
lesions on the tongue as well as
increased melanin pigmentation in
the basal layer of the epithelium, and
the presence of melanin incontinence
and melanophages in the superficial
lamina propria was consistent with a
diagnosis of LHS in our case.
The cause of this rare disease is
yet unknown. Ultrastructural studies
reveal an increase in the number and
size of mature melanosomes located
in the cytoplasm of basal keratinocytes and dermal melanophages
(3). Some authors suggest that functional alteration of the melanocytes in
the form of an increase in the syntheFigure 2. Increased melanin pigmentation in the basal layer of the epithelium, pigmentary incontinence and melanophages in the superficial lamina propria (hematoxylin & eosin stain, X 40)106 · Haziran 2006 · Gülhane TD Sezer ve ark.
sis of melanosomes and their subsequent transport to the basal layer cells
may give rise to hyperpigmented
lesions (12).
The differential diagnosis of LHS
includes Peutz-Jeghers syndrome
(PJS), Addison's disease, smoker's
melanosis, and melanoacanthosis.
PJS is an autosomal dominant disorder characterized by hamartomatous
gastrointestinal polyposis and melanotic macules, particularly of the face
and mouth. Most of the patients have
a positive family history of PJS. The
polyposis results in episodes of colicky abdominal pain in adolescence
or young adulthood. Because of a significantly increased risk of malignancy, comprehensive screening protocols consisting of periodical upper
and lower gastrointestinal endoscopies are mandatory (13). Lesions
in PJS appear around birth or early
childhood, whereas LHS lesions are
progressively acquired in young or
middle-age adults. Characteristically,
in addition to intraoral pigmentation,
the lesions are found around the
mouth, nose and eyes and on the dorsal and ventral surfaces of the hands
and feet in PJS, while the lesions of
LHS are mainly confined to the oral
mucous membranes and the nails.
Appearance of the lesions in later life
without involvement of the face and
hands, and the absence of a family
history and associated symptoms
helped us to exclude the diagnosis of
PJS in our patient.
In Addison's disease, there is darkening in the areas of trauma, recent
scars, points of pressure, areolae, axilla, and perineum. Longitudinal pigmented bands on the nails and diffuse
pigmentation on oral mucosal surfaces are observed. Other clinical
signs of the disease include fatigue,
weakness, loss of weight, hypotension, and gastrointestinal disturbances (14). In our case, lack of related symptoms and normal serum electrolyte levels ruled out this disease.
Smoker's melanosis is seen predominantly on the anterior attached gingiva, which is a rare location in LHS.
Oral melanoacanthosis shows dendritic melanocytes dispersed throughout the epithelium histologically,
which was not detected in our patient
(9).
LHS is a benign disease that follows a chronic course with a progressive increase in lesions over years or
remain stable. However, spontaneous
remission of a case has also been
reported in the literature (12). There
are paucity of reports of treatment
modalities in the literature, probably
because of general lack of symptoms
in LHS. The reported treatment
options include Q-switched Alexandrite laser, Q-switched Nd-Yag laser,
and cryosurgery (15,16). Sheridan et
al. reported a case of LHS with
involvement of the lips successfully
treated with cryosurgery (16). Because of the complaint of cosmetic disfigurement, we treated our patient
with an eight second, single freeze
thaw cycle open spray technique of
cryosurgery. A marked improvement
was obtained after complete healing
in 2 weeks period. To our knowledge,
successful treatment of tongue
involvement of LHS with cryosurgery has not been reported in the literature.
In summary, we consider that
recognition of LHS and differentiation with PJS is important to prevent
unnecessary periodical investigations
including gastrointestinal endoscopies. We emphasize that cryosurgery should be the first line choice in
the treatment of LHS causing cosmetic disfigurement, as a safe, effective and inexpensive method.