Laugier-Hunziker ᇈ࣏ཏ Laugier

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Laugier-Hunziker ᇈ࣏ཏ
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‫ܘ‬ព୻
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઼ϲέ៉̂ጯᗁጯੰ‫ܢ‬నᗁੰϩቲొ
Laugier-Hunziker Syndrome
Meng-Sui Lee1 Hsieh-Ching Chiu2 Li-Fang Wang2
Laugier-Hunziker syndrome (LHS) is a rare acquired benign hyperpigmentation of the oral
mucosa and lips which is often associated with longitudinal melanonychia and spotted macular pigmentation of the acral skin. We herein report two patients exhibiting the typical features of LHS. (Dermatol
Sinica 24: 209-212, 2006)
Key words: Laugier-Hunziker Syndrome, Longitudinal melanonychia
Laugier-Hunzikerᇈ࣏ཏߏ˘࣎ց֍։ّ۞‫়͇ޢ‬ঽĄѩ়ঽ۞পᇈߏ˾ටᕆቯăᆜࣼ̈́
۳ბϩቲ΍னโҒ৵೹ҡѣᓂШ޽ϥโҒ৵Օ඾Ąώ͛ಡӘ‫ܑ࣎׌‬ன‫ݭ׏‬পᇈ۞LaugierHunzikerᇈ࣏ཏ९ּĄ(̚රϩᄫ 24: 209-212, 2006)
INTRODUCTION
Laugier-Hunziker syndrome (LHS) was
first described in 1970.1 It is characterized by
asymptomatic hyperpigmented macules of the
oral mucosa, lips and acral skin and pigmented
longitudinal bands of the nails. The onset of the
condition is usually in early or mid adult life.2
We herein report two typical cases of LHS.
CASE REPORT
Case 1
An 84-year-old Chinese female presented
with a 40-year history of hyperpigmentation on
the oral mucosa, lips, hands, feet, and nails.
There was no family history of mucocutaneous
pigmentation or intestinal polyps. Physical
examination revealed many variously sized
round, oval or irregularly shaped brown macules were on the conjunctivae, lips, tongue,
palate, buccal and gingival mucosa (Fig. 1A),
and were also found on the distal parts of the
hands and feet (Fig. 1B and 1C). Besides, 1-2
mm wide, brown longitudinal streaks were noted on some fingernails and toenails (Fig. 1D).
From the Department of Dermatology, Taipei City Hospital,1 and National Taiwan University Hospital2
Accepted for publication:March 13,2006
Reprint requests: Hsieh-Ching Chiu, M.D., Department of Dermatology, National Taiwan University Hospital, No. 7, Chung-Shan
South Road, Taipei, R.O.C.
TEL: 886-2-23562141 E-mail: [email protected]
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Laugier-Hunziker ᇈ࣏ཏ
No periorificial lesion was noted.
Results of routine laboratory tests were all
within the normal range, as the serum assays for
thyrotropin, T3, T4, adrenotropin, and cortisol.
Over the past 30 years she had had several
examinations of x-ray studies of barium enema
and upper GI series, oesophagogastroduodenoscopies and colonoscopies which disclosed no
polyp. A previous histological examination of
the skin lesions at another hospital showed
acanthosis and basal layer hyperpigmentation.
Case 2
A 72-year-old Chinese female had a 30year history of hyperpigmentation on the oral
mucosa, lips, and right thumb. She also noticed
vertical brown lines running along three fingers
in recent 2 years. There was no family history
of mucocutaneous pigmentation or intestinal
polyps. Dermatological examination revealed
many variously sized and shaped brown macules on the lips, tongue, palate, and buccal and
gingival mucosa (Fig. 2A), as well as the distal
parts of the right thumb (Fig. 2B). Besides, 1-2
mm wide, brown longitudinal streaks were noted on three fingernails (Fig. 2C). No periorificial lesion was noted.
Laboratory investigations, including urine
cortisol and serum thyrotropin, T3, T4, and
adrenotropin were all within the normal range.
Colonoscopy disclosed no hamartomatous polyposis. Incisional biopsy of the lower lip
revealed basal cell layer hyperpigmentation and
pigment incontinence (Fig. 3).
DISCUSSION
In 1970, Laugier and Hunziker reported
five cases of acquired hyperpigmentation of the
mouth and lips. Two of these patients also displayed longitudinal pigmented streaks on the
Fig. 1A
Fig. 1C
Multiple brown macules on the lips, tongue and gums. (Case 1)
Many hyperpigmented macules and patches on the distal
part of the soles. (Case 1)
Fig. 1B
Fig. 1D
Multiple brown macules on the volar aspect of the fingers and dorsal aspect of the toes. (Case 1)
Longitudinal melanonychia of several finger nails. (Case 1)
Dermatol Sinica, Sep 2006
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nails. 1 The cause of this disorder, named
Laugier-Hunziker syndrome (LHS), is unknown
yet. LHS is a rare acquired disorder which is
known to be an entirely benign condition without systemic manifestations. Most reported cases happened to white European. Initially a
female predominance was suggested,3 but it is
now thought that male and female are equally
affected.4 Almost all known cases to date have
been sporadic. However, Makhoul et al. reported the unusual occurrence of three cases of LHS
in the same family.5
LHS is characterized by the presence of a
variable number of asymptomatic pigmented
lenticular mucocutaneous macules. The colors
of the lesions vary from slate to brown-black.
They can be isolated or confluent. The buccal
mucosa and lips are most commonly involved.
The corners of the mouth, the gingivae,6 tongue,
211
fingers,7 and the plantar aspect of the feet are
less frequent locations. Rarely, pigmented macules occur on genitalia, perineal or perianal
areas, sclerae and esophagus.8-10 The characteristic changes in nails are longitudinal streaks of
pigment known as longitudinal melanonychia.
The nail change can be found in approximately
60% of patients.11 The cause of nail pigmentation is unknown, but it is believed to be similar
to that of the lip and mucosal pigmentation.
Histologically, there is increased basal keratinocyte melanin without expansion of the
melanocytic population and superficial pigmentary incontinence with dermal melanophages.
Mild to moderate acanthosis can also be noted.12,
13
Electron microscopy reveals numerous mature,
isolated or grouped melanosomes of variable
sizes within the cytoplasm of keratinocytes and
melanophages in the papillary dermis.3
Fig. 2A
Fig. 2C
Multiple brown macules on the lips and tongue. (Case 2)
Longitudinal melanonychia of several finger nails. (Case 2)
Fig. 2B
Fig. 3
Several brown macules on the right thumb. (Case 2)
Incisional biopsy of the lower lip revealed mild acanthosis, basal layer hyperpigmentation and pigment incontinence in the papillary dermis. (H & E stain, x100)
Dermatol Sinica, Sep 2006
Laugier-Hunziker ᇈ࣏ཏ
Several conditions must be considered in
the differential diagnosis of nail and mucocutaneous pigmentary abnormalities. Such as adrenal insufficiency and nail changes resulting
from administration of systemic agents, especially after chemotherapy. 14 In our cases,
Addison(s disease was eliminated on the clinical and hormonal basis. Drug ingestion did not
appear relevant since the patients denied any
previous or current drug intake. Peutz-Jeghers
syndrome (PJS) must also be considered in the
differential diagnosis. PJS is an autosomal dominant condition, in which the mucocutaneous
lesions appear around birth or at an early age
with mainly a periorificial distribution. Other
pigmented spots can also be found on the dorsal
and volar aspects of the hands and feet. There is
a strong association with hamartomatous polyposis of the gastrointestinal tract and an
increased risk for neoplasia.15 In our cases, the
late onset of the disease, absence of a family
history and GI polyposis make the diagnosis of
PJS very unlikely. On the contrary, in addition to
the aforementioned findings, the distribution of
the lesions, i.e., lesions confined to the oral
mucosa, lips and acral skin with sparing of the
periorificial sites, and the presence of nail pigmentation are also in favor of the diagnosis of
LHS.
As for our knowledge, LHS has not been
reported in Taiwan. The importance of recognizing this acquired benign disorder is to avoid
unnecessary and potentially hazardous investigations. No treatment is required other than
reassurance.
4. Porneuf M, Dandurand M: Pseudo-melanoma
revealing Laugier-Hunziker syndrome. Int J
Dermatol 36: 138-141, 1997.
5. Makhoul EN, Ayoub NM, Helou JF, et al.: Familial
Laugier-Hunziker syndrome. J Am Acad Dermatol
49: S143-S145, 2003.
6. Koch SE, LeBoit PE, Odom RB: LaugierHunziker syndrome. J Am Acad Dermatol 16:
431-434, 1987.
7. Baran R, Barriére H: Longitudinal melanonychia
with spreading pigmentation in Laugier-Hunziker
syndrome: a report of two cases. Br J Dermatol
115: 707-710, 1986.
8. Began D, Mirowski G: Perioral and acral lentigines in an African American man. Arch Dermatol
136: 471-472, 2000.
9. Dupre A, Viraben R: Laugier(s disease.
Dermatologica 181: 183-186, 1990.
10.Yamamoto O, Yoshinaga K, Asahi M, et al.: A
Laugier-Hunziker syndrome associated with
esophageal melanocytosis. Dermatology 199: 162164, 1999.
11. Kemmett D, Ellis J, Spencer MJ, et al.: The
Laugier-Hunziker syndrome: a clinical review of
six cases. Clin Exp Dermatol 15: 111-114, 1990.
12.Ayoub N, Barete S, Bouaziz JD, et al.: Additional
conjunctival and penile pigmentation in LaugierHunziker syndrome: a report of two cases. Int J
Dermatol 43: 571-574, 2004.
13. Siponen M, Salo T: Idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome): a report of a case. Oral Surg Oral Med
Oral Pathol Oral Radiol Endod 96: 288-292, 2003.
14.Lampe AK, Hampton PJ, Woodford-Richens K, et
al.: Laugier-Hunziker syndrome: an important differential diagnosis for Peutz-Jeghers syndrome.
J Med Genet 40: e77, 2003.
15. Fisher D, Field EA, Welsh S: Laugier-Hunziker
Syndrome. Clin Exp Dermatol 29: 312-313, 2004.
REFERENCES
1. Baran R: Longitudinal melanotic streaks as a clue
to Laugier-Hunziker syndrome. Arch Dermatol
115: 1448-1449, 1979.
2. Mignogna MD, Lo Muzio L, Ruoppo E, et al.:
Manifestations of idiopathic lenticular mucocutaneous pigmentation (Laugier-Hunziker syndrome):
a clinical, histopathological and ultrastructural
review of 12 cases. Oral Dis 5: 80-86, 1999.
3. Veraldi S, Cavicchini S, Benelli C, et al.: LaugierHunziker syndrome: a clinical, histopathologic, and
ultrastructural study of four cases and review of the
literature. J Am Acad Dermatol 25: 632-636, 1991.
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