Dermoscopic Findings in Laugier-Hunziker Syndrome

OBSERVATION Dermoscopic Findings in Laugier-Hunziker Syndrome

Gulsum Gencoglan, MD; Bengu Gerceker-Turk, MD; Isil Kilinc-Karaarslan, MD; Taner Akalin, MD; Fezal Ozdemir, MD

Background: Laugier-Hunziker syndrome (LHS) is a cosal lesions on the patient’s lips and vulva revealed a rare, acquired mucocutaneous hyperpigmentation of- parallel pattern. Longitudinal homogeneous pigmenta- ten associated with longitudinal melanonychia. The clini- tion was observed on the toenails. The pigmented mac- cal behavior of mucocutaneous pigmented lesions ranges ules on the palms and the sole showed a parallel furrow from benign to highly malignant. Therefore, in most cases, pattern. A skin biopsy sample taken from the labial le- the clinical diagnosis should be confirmed by further di- sion was compatible with a diagnosis of mucosal mela- agnostic methods. Dermoscopy is a noninvasive tech- nosis. nique that has been used to make more accurate diag- noses of pigmented skin lesions. Nevertheless, to our Conclusions: By means of this case report, the dermo- knowledge, the dermoscopic features of the pigmented scopic features of the pigmented lesions in LHS are de- lesions in LHS have not been described previously. Herein, scribed for the first time, which facilitates diagnosis with we report a case of LHS together with its dermoscopic a noninvasive technique. Future reports highlighting the features. dermoscopic features of this syndrome may simplify the diagnosis of LHS, which is thought to be underdiag- Observations: The clinical examination revealed macular hyperpigmentation on the oral and genital mucosa, nosed. conjunctiva, and palmoplantar region together with longitudinal melanonychia. Dermoscopic examination of mu- Arch Dermatol. 2007;143:631-633

AUGIER-HUNZIKER SYN- ill-defined hyperpigmented conjunctival drome (LHS) is a rare, ac- macule (Figure 1D), 3 small pigmented quired disorder character- macules on the palms and 1 on the sole, and ized by benign macular linear melanonychia on the toenails were hyperpigmentation of the also observed. She had no medical history oralL and genital mucosa, which is associ- of receiving medication for, or family his- ated with longitudinal melanonychia in tory of, pigmentation disorder or intesti- 50% to 60% of cases.1 Dermoscopic ex- nal polyposis. Findings from the labora- amination should be indicated for pa- tory tests were within reference range, and tients with LHS who have several pig- the assessment of her gastrointestinal sys- mented lesions on the mucosa, nails, or tem included radiological examinations and skin. However, to our knowledge, this is a colonoscopy, the findings from which the first description of the dermoscopic fea- were also within reference range. Find- tures of the lesions in LHS. ings from the histological examination of the lesion on her lip revealed hyperpig- REPORT OF A CASE mentation of basal keratinocytes. There were also perivascular melanin-laden A 24-year-old female patient was referred macrophages in the papillary dermis to our dermoscopy unit because she had (Figure 2A). Masson-Fontana staining was Author Affiliations: bluish-brown maculae on her lips positive for melanin (Figure 2B), but stain- Departments of Dermatology Figure 1 (Drs Gencoglan, Gerceker-Turk, ( A) and gingiva and pigmented ing with Prussian blue was negative for he- Kilinc-Karaarslan, and papillae on her tongue (Figure 1B). In ad- mosiderin. Melanocytic proliferation was Ozdemir) and Pathology dition, a longitudinal hyperpigmented mac- not detected immunohistochemically with (Dr Akalin), Ege University, ule on the vulva extending from the labia S100 antibody. According to these find- Bornova, Izmir, Turkey. minora to the labia majora (Figure 1C), an ings, a diagnosis of LHS was made.

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A B C

Figure 3. Dermoscopic examination of mucosal lesions. A, Parallel furrow pattern with multiple brown dots on the lip. B, Parallel furrow pattern with linear and curvilinear streaks on the vulva. C, Homogeneous regular bandlike pigmentations with indistinct borders on the toenail; inset, melanonychia longitudinalis. C D

A B

Figure 1. A 24-year-old female patient diagnosed as having Laugier-Hunziker C D syndrome. Note the bluish-brown pigmentation of the upper lip (A) and the tongue (B); longitudinal brownish pigmentation on the vulva (C); and an ill-defined conjunctival macule (D).

A B

Figure 4. Parallel furrow pattern of pigmented macules, all characterized by linear pigmentation following the sulci of the surface skin markings. A and B, Single dotted-line variation (single dotted lines along the sulci) on the left palm; C, single-line variant (single line along the sulci) on the right palm; and D, single-line variant with homogeneous pigmentation in the background on the sole.

accompanied by regularly distributed multiple brown dots without any other dermoscopic variable suggestive of a ma- lignancy. To our knowledge, the presence of multiple dots has not been reported in the limited number of articles published on dermoscopic features of mucosal melanosis.2,3 The Figure 2. Hyperpigmentation of basal keratinocytes and melanophage parallel pattern with multiple brown dots correlated his- population in the papillary dermis. A, Hematoxylin-eosin, original magnification ϫ100; B, highlighted hyperpigmentation with Masson-Fontana topathologically with melanin pigmentation of basal ke- stain, original magnification ϫ100. ratinocytes and dermal melanophages, and these dots would be an expected finding when a patient has a superficial pig- mentary incontinence. On the vulva, the pigmentation ob- Dermoscopic examination of mucosal lesions on the served on dermoscopy was seen as partially linear and par- patient’s lips and vulva revealed parallel patterns. On the tially curvilinear brown streaks following the cutaneous lips, linear, streaklike brown pigmentation caused by the profile, and this observation was concordant with the char- skin furrows and reliefs was associated with multiple acteristic features of benign genital melanosis as defined brown dots of different sizes distributed regularly through- by Soyer et al.4 out the lesion (Figure 3A). The parallel pattern seen The longitudinal melanonychia in our patient revealed on the vulva was partially linear and partially curvilin- homogeneous brownish regular bands with indistinct bor- ear, with light- to dark-brown streaks following the cu- ders, and the pigmented macules on the palms and the sole taneous profile (Figure 3B). Homogeneous, brownish, revealed a parallel furrow pattern on dermoscopy. regular bandlike pigmentations with indistinct borders By means of this case, the dermoscopic features of LHS were seen on 4 toenails (Figure 3C). The pigmented mac- are described for the first time. Future reports will im- ules on the palms and on the sole showed a parallel fur- prove our perception of this rarely seen entity. row pattern (Figure 4). Accepted for Publication: September 26, 2006. COMMENT Correspondence: Isil Kilinc-Karaarslan, MD, Ege Uni- versity, Department of Dermatology, 35100, Bornova, In the case reported described herein, a parallel pattern was Izmir, Turkey ([email protected]). observed on the patient’s lips and vulva. On the lips, it was Author Contributions: Study concept and design: Gerceker-

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©2007 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Turk, Kilinc-Karaarslan, and Ozdemir. Acquisition of data: REFERENCES Gencoglan, Gerceker-Turk, and Ozdemir. Analysis and interpretation of data: Gerceker-Turk, Akalin, and Oz- 1. Makhoul EN, Ayoub NM, Helou JF, Abadjian GA. Familial Laugier-Hunziker syndrome. demir. Drafting of the manuscript: Gencoglan, Gerceker- J Am Acad Dermatol. 2003;49(suppl):S143-S145. Turk, Kilinc-Karaarslan, Akalin, and Ozdemir. Critical 2. Gaeta GM, Satriano RA, Baroni A. Oral pigmented lesions. Clin Dermatol. 2002;20: revision of the manuscript for important intellectual con- 286-288. 3. Mannone F, De Giorgi V, Cattaneo A, Massi D, De Magnis A, Carli P. Dermoscopic tent: Ozdemir. Administrative, technical, and material sup- features of mucosal melanosis. Dermatol Surg. 2004;30:1118-1123. port: Gerceker-Turk. Study supervision: Ozdemir. 4. Soyer HP, Argenziano G, Ruocco V, Chimenti S. Dermoscopy of pigmented skin Financial Disclosure: None reported. lesions (part II). Eur J Dermatol. 2001;11:483-498.

Correction

Error in Author Order. In the Evidence-Based Derma- tology Study by Helfrich et al, published in the March issue of the ARCHIVES (2007;143:397-402), there was an error in the author order in the byline. The names should have appeared as follows: Yolanda R. Helfrich, MD; Le Yu, MD; Abena Ofori, MD; Ted A. Hamilton, MS; Jen- nifer Lambert, MS; Anya King, MPH; John J. Voorhees, MD; Sewon Kang, MD. The online version of this ar- ticle was corrected on March 15, 2007.

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