C a s e R e p o r t D e r m a t o l o g y Re c e i v e d : y 1 2 ,Ma 2 0 1 3 A c c e p t e d a f t e r r e v i s i o n : l y 1 7 ,Ju 2 0 1 3 .10 : DO I : .10 1 1 5 9 / 0 0 0 3 5 4 6 0 4 P u b l i s h e d o n l i n e : S e p t e m b e r 2 0 , 2 0 1 3 P r u r i t i c A c q u i r e d N e v u s o f O t a a b a a a c b .S .S Q u e n a n V . S t r u e v e n N . Sa x e r E . La f f i t t e G . Ka y a J . K r i s c h e r F . H a f e z i a F.-A. Le Gal a b c D e p a r t m e n t s o f D e r m a t o l o g y a n d O p h t h a l m o l o g y , Geneva University Hospitals , a n d P r i v a t e P r a c t i c e , , Ge n e v a , Switzerland K e y W o r d s quantity of mast cells observed in the lesion Case Report N e v u s o f O t a · Pr u r i t u s · QAd - s u w l t i t c h e d and/or neuronal stimulation of the ophthal­ laser mic and maxillary divisions of the fifth cranial A 32-year-old Kurdish Iraqi woman nerve. ©2 0 1 3 S . r g e r A G ,Ka B a s e l presented to our clinic with brownish mot­ tled macules that had appeared progres­ A b s t r a c t sively, starting 8 days earlier, on the left N e v u s o f O t a i s a u n i l a t e r a l , a s y m p t o m a t i c cheek, eyelid and forehead and partially on cutaneous and mucosal hyperpigmentation Introduction the iris and sclera of the left eye, following of the face that is congenital or may appear the distribution of the maxillary and oph­ during childhood. We present a case of N e v u s o f O t a i s a u n i l a t e r a l c u t a n e o u s thalmic divisions of the left trigeminal symptomatic acquired nevus of Ota in an and mucosal hyperpigmentation, appearing nerve ( fig. 1 , 2 ). She was in good health and adult, associated with intense pruritus, not as bluish or brownish macules that occur on denied taking any medication or using any described in the literature so far. A 32-year­ the areas of the face innervated by the max­ topical skin preparation on the face. She old woman presented with brownish mot­ illary and ophthalmic branches of the tri­ described a burning and very itchy sensa­ tled macules which appeared on her face geminal nerve, and rarely by the mandibu­ tion that occurred with the lesion, which progressively over 8 days, following the dis­ lar branch [1, 2] . B e s i d e s s k i n , i t i n v o l v e s was strictly located on the pigmented area. tribution of the first and second divisions of ocular tissue and sclera in most cases; it also Clinically, there were no erythematous the left trigeminal nerve and partially cover­ increases the risk of glaucoma, and can be changes, and the examination under Wood’s ing the iris and sclera of the left eye. She re­ associated with melanotic tumors, especial­ lamp revealed a slightly higher contrast of ported an intense pruritus in this area. We ly uveal melanoma [3] . N e v u s o f O t a h a s the pigmented zones. Histological analysis performed a biopsy on the left forehead, been classified into type I, II, III and IV (i.e. of the skin biopsy performed on the left which confirmed the diagnosis of nevus of mild, moderate, intense and bilateral, re­ forehead showed a proliferation of non- Ota. Specific stains and immunohistochem­ spectively) [1] . T h e O r i e n t a l s , B l a c k s , A s i a n s atypical pigmented spindle-shaped cells in istry revealed increased numbers of mast and rarely the Caucasians are affected, with the superficial and reticular dermis ( fig. 3 ). cells. Ophthalmological tests showed acute the highest prevalence in women with a sex The immunohistochemical study revealed acquired melanocytosis of the left iris and ratio of 1: 4 . 8 [2, 4] . N e v u s o f O t a i s a c o n ­ positivity for S-100 protein, HMB-45 and sclera. The origin of the nevus is still unclear. genital unilateral melanocytic hyperpig­ melan-A in these cells. A special stain of Several hypotheses suggest a reactivation of mentation which may appear in early child­ Toluidine blue and immunohistochemis­ melanocytes during their migration from hood or in puberty [4] . W e r e p o r t h e r e a try for CD117 (data not shown) showed an the neural crest. The pruritus reported in our case of acquired unilateral nevus of Ota in a increased number of mast cells in the lesion patient may be explained by the increased 32-year-old Kurdish Iraqi woman. (>15 mast cells per high-power field; fig. 4 ). © 2013 S. Karger AG, Basel Sandrine Quenan 1018–8665/13/0000–0000$38.00/0 Clinic of Dermatology Geneva University Hospitals - 10/7/2013 11:43:13 AM E-Mail [email protected] CH–1211 Geneva (Switzerland) www.karger.com/drm E-Mail sandrine.quenan @ hcuge.ch Downloaded by: Université de Genève 129.195.0.205 The proliferation index (Ki-67) in the spin­ dle-shaped pigmented cells remained less than 5%. Immunolabeling and PCR analy­ sis for herpes simplex virus and varicella zoster virus were negative. An MRI showed a discrete nonspecific hypersignal in the cutaneous tissues, with no abnormalities of the orbital region and the brain. An oph­ Color version available online thalmological examination concluded an acute acquired melanocytosis of the left iris and sclera associated with heterogeneity of the iris structure, but no visual defect. An Color version available online ultrasound biomicroscopy scan revealed no disorganization of the ocular structures. Considering the clinical and histopatho­ logical findings, we diagnosed the lesion as an acquired nevus of Ota. Discussion Dermal melanocytosis, which is de­ scribed to be congenital in most cases, can appear during the first year of life and less Fig. 1. Brownish mottled macules on the Fig. 3. Preserved epidermis overlying a der­ often in adolescence [5] . Cases of late-onset left side of the face. mal proliferation of intensely pigmented dermal melanocytosis in adults are rare. spindle-shaped cells. HE. ×20. Also referred to as acquired dermal mela­ nocytosis (ADM), several hypotheses have been discussed to explain its origin. During embryological development, melanocytes migrate from the neural crest to the basal stratum. In dermal melanocytosis, a defect in this process is described. In ADM, the latent dermal melanocytes are thought to be reactivated by unknown triggers such as sex hormones and local trauma, in addi­ Color version available online tion to sun damage and inflammation [6]. These factors activate the pathway to syn­ thesize melanin. The stability of the cells Color version available online is explained by a protective extracellular sheath surrounding dermal melanocytes in adult life [6]. I n 1 8 6 1 , H u l k e [7] w a s t h e f i r s t t o d e - Fig. 2. Brownish mottled macules on the Fig. 4. Special stain of Toluidine blue con- scribe a unilateral hyperpigmentation of iris and sclera. firming an increased number of mast cells the face and sclera. Then, in 1939, Ota [1] (×20). defined it as a clinical entity named nevus of Ota. It is also referred to as oculodermal melanocytosis, nevus fuscoceruleus oph­ talmomaxillaris, or congenital melanosis nasal and oral cavity are not pigmented Chang et al. [14] , t h e f i r s t i n a 4 4 - y e a r - o l d bulbi [8, 9]. I n 1 9 8 4 , H o r i e t a l . [10] w e r e [10] . I n 1 9 8 7 , S u n e t a l . [11] d e s c r i b e d t h e man and the second in a 49-year-old pioneers in describing the acquired bilat­ acquired nevus fuscoceruleus zygomati­ woman. eral nevus of Ota-like macules as ADM. cus, known as acquired unilateral nevus of Different treatment modalities are pro­ This bilateral dermal melanosis appears in Ota, while in our case it involves the oph­ posed to treat ADM. Laser therapy such as the 3rd or 4th decade of life, with preva­ thalmic and the zygomatic divisions.