Letters to the Editor

855

field-directed AFXL-IngMeb treatment. The prospect is a new procedure for promptly treating AK, thus providing a new AK treatment for non-compliant patients. K.E. Karmisholt,* M. Haedersdal Department of Dermatology, Bispebjerg Hospital, University of Copenhagen, Bispebjerg Bakke 23, 2400 Copenhagen NV, Denmark, *Correspondence: K. Karmisholt. E-mail: [email protected]

(a)

(b)

References 1 Gupta AK, Paquet M, Villanueva E, Brintnell W. Interventions for actinic keratoses. Cochrane Database Syst Rev 2012; 12: CD004415. 2 Dodds A, Chia A, Shumack S. Actinic keratosis: rationale and management. Dermatol Ther 2014; 4: 11–31. 3 de Berker D, McGregor JM, Hughes BR. Guidelines for the management of actinic keratoses. Br J Dermatol 2007; 156: 222–230. 4 Dreno B, Amici JM, Basset-Seguin N, Cribier B, Claudel JP, Richard MA. Management of actinic keratosis: a practical report and treatment algorithm from AKTeam expert clinicians. J Eur Acad Dermatol Venereol 2014; 28: 1141–1149. 5 Stockfleth E. The paradigm shift in treating actinic keratosis: a comprehensive strategy. J Drugs Dermatol 2012; 11: 1462–1467. 6 Lebwohl M, Swanson N, Anderson LL, Melgaard A, Xu Z, Berman B. Ingenol mebutate gel for actinic keratosis. N Engl J Med 2012; 366: 1010–1019. 7 Aditya S, Gupta S. Ingenol mebutate: a novel topical drug for actinic keratosis. Indian Dermatol Online J 2013; 4: 246–249. 8 Martin G, Swanson N. Clinical findings using ingenol mebutate gel to treat actinic keratoses. J Am Acad Dermatol 2013; 68(1 Suppl 1): S39–S48. 9 Haak CS, Bhayana B, Farinelli WA, Anderson RR, Haedersdal M. The impact of treatment density and molecular weight for fractional laserassisted drug delivery. J Control Release 2012; 163: 335–341. 10 Togsverd-Bo K, Haak CS, Thaysen-Petersen D, Wulf HC, Anderson RR, Haedersdal M. Intensified photodynamic therapy of actinic keratoses with fractional CO2 laser: a randomized clinical trial. Br J Dermatol 2012; 166: 1262–1269. DOI: 10.1111/jdv.13021

Vulvar white sponge naevus in a girl Editor White sponge naevus (WSN) is an autosomal dominant disorder. It was first described by Hyde in 1909 and the condition was first reported in 1935 by Cannon.1 Oral mucosa is the most frequently affected but extraoral mucosa sites have been reported such as the nasal, oesophageal, laryngeal and anogenital. Herein, we present a new case of genital WSN without oral affection. An 8-year-old girl was referred to our Dermatology Service with a year’s history of a white itchy spot at the genitalia. The patient general health was good, only allergy to nuts, peach skin, flea and mosquitoes bites and atopic dermatitis was present. On clinical examination, there were white and slightly erythematous confluent papules of 2 mm of diameter in the left labia minor of

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Figure 1 (a) White papules in the left labia minor of the vulva (b) Histology of the affected area shows epithelial thickening, parakeratosis and extensive vacuolization of the suprabasal keratinocytes (HE 920)

the vulva (Fig. 1a). The clinical diagnosis was presumed to be a cutaneous lichenoid lesion, so she was treated with topical corticosteroids without improvement, so a biopsy was taken for histologic examination. The histopathological features showed epithelial thickening, parakeratosis, extensive vacuolization of the suprabasal keratinocytes (Fig. 1b). Based on the clinical and histopathological findings, the lesion was consistent with white sponge naevus. A new physical examination of the buccal mucosa was made with no evidence of similar lesions, and no family history of such lesions was found. WSN is a rare hereditary dyskeratotic hyperplasia of mucous membranes, affecting one in 200 000 people.2 It is autosomal dominant with variable penetrance; even tough sporadic cases have also been reported. The disorder may be detected in early childhood as asymptomatic, white or greyish, thickened and spongy plaques that involves mucosa. Buccal mucosa is most commonly affected and extraoral involvement is occasionally reported. Only five cases of WSN in the genital mucosa have been reported, two of them exclusively located in the vulva, without oral involvement and family history, as our patient (Table 1).3–7 The presented case is the youngest reported in the literature, which could be the reason for the clinical appearance of the lesions, more subtle than the previously reported. WSN is attributed to mutations of keratin 4 and/or 13, which are expressed in the spinous cell layer of the oral mucosa. Liu et al., made a mutational analysis that compared familial and sporadic patients with WSN. They concluded that four of the five sporadic cases had no keratin mutations, and other causes might be responsible for their clinical and histological characteristics.8 The differential diagnosis should be made with other conditions presenting as white lesions on the genital mucosa, including lichen sclerosus, candida vaginitis, pachyonychia congenita and neoplastic conditions, among others. Histological findings of WSN are characteristic but not necessarily pathognomonic. Thickening and vacuolization of the spinous layer, with extensive hyperparakeratosis and acanthosis are common features; the basement membrane is intact; eosino-

© 2015 European Academy of Dermatology and Venereology

Letters to the Editor

856

Table 1 Current and previously reported cases of WSN of the Vulva Authors

Case

Age

Sex

Oral mucosa involvement

Family History

Sayag et al. chholz et al. Bu

1

35

Female

No

No

2

18

Female

No

No

Daniele et al.

3

26

Female

Present

Mother (genital involvement), brother (oral involvement)

Nichols et al.

4

34

Female

Present

Father (oral involvement), sister (oral involvement)

Cutlan et al. Garcıa-Malinis et al.

5

28

Female

Present

Father (oral involvement), paternal uncle (oral involvement)

6

8

Female

No

No

philic condensation is noted in the perinuclear region of the cells in the superficial layers of the epithelium, a feature that is characteristic to WSN.9 Among the treatments used for oral WSN, some antimicrobial therapies, such as tetracycline, penicillin and chlorhexidine, have been effective.10 The successful response to them may suggest that microorganism play a role in the development of WSN still unknown. There is no specific treatment of WSN of the vulva, and observation or surgical removal of the extraoral lesions is recommended.4 In conclusion, we present the first case of WSN exclusively located in the vulva in a girl, without a familial background. Even though, this is a rare entity, WSN must be considering in the differential diagnosis of vulvar leucoplakia in young girls.  n-Banzo,2 M.A. Marigil,3 A.J. Garcıa-Malinis,1,* P.J. Ago Y. Gilaberte1 1 Unit of Dermatology, Hospital San Jorge, 2Unit of Pediatrics, Hospital de n del Pirineo, Jaca, 3Department of Pathology, Hospital San Alta Resolucio Jorge, Huesca, Spain *Correspondence: A.J. Garcıa-Malinis. E-mail: ajgarciamalinis@gmail. com All the authors contributed equally to this work.

References 1 Cannon AB. White sponge nevus of the mucosa (nevus spongious albus mucosae). Arch Dermatol Syphilol 1935; (Chicago) 31: 365. 2 Shibuya Y, Zhang J, Yokoo S, Umeda M, Komori T. Constitutional mutation of keratin 13 gene in familial white sponge nevus. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2003; 96: 561–565. 3 Sayag J, Jancovici E, Lacroix J. Exclusive genital white sponge nevus. Ann Dermatol Venereol 1985; 112: 759–760. 4 Buchholz F, Schubert C, Lehmann-Willenbrock E. White sponge nevus of the vulva. Int J Gynaecol Obstet 1985; 23: 505–507. 5 Daniele E, Florena AM, Margiotta V et al. White sponge nevus. Immunohistochemical and ultrastructural study of a familial case with orogenital presentation. Minerva Stomatol 1988; 37: 981–990. 6 Nichols GE, Cooper PH, Underwood PB Jr, Greer KE White sponge nevus Obstet Gynecol 1990; 2: 545–548. 7 Cutlan JE, Saunders N, Olsen SH, Fullen DR White sponge nevus presenting as genital lesions in a 28-year-old female J Cutan Pathol 2010; 37: 386–389. 8 Liu X, Li Q, Gao Y, Song S, Hua H Mutational analysis in familial and sporadic patients with white sponge naevus Br J Dermatol 2011; 165: 448–451. 9 Zhang JM, Yang ZW, Chen RY, Gao P, Zhang YR, Zhang LF Two new mutations in the keratin 4 gene causing oral white sponge nevus in Chinese family Oral Dis 2009; 15: 100–105.

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10 Satriano RA, Errichetti E, Baroni A White sponge nevus treated with chlorhexidine J Dermatol 2012; 39: 742–743. DOI: 10.1111/jdv.13022

Are IPL home devices really foolproof? Editor Hair removal with lasers and intense pulsed light (IPL) devices have become a mainstay in many physicians’ offices and nonmedical settings (e.g. beauty parlours) all over the world. The great demand for photo-epilation techniques and the ability to cut costs has led to the development of home devices.1 Acne vulgaris, rosacea and photo rejuvenation are further advertised indications for home use. Many devices are available in the market, although only a few have undergone controlled clinical trials to document their safety and efficacy.2 The evidence available from prospective uncontrolled clinical trials indicates that the home-use light-based devices currently available are efficacious in short-term hair removal.3 Of note, most IPL home devices in these studies have been used under medical supervision and/or the studies have been sponsored by the industry itself. Skin pigmentation has already been identified as an independent risk factor for side-effects in low-fluence IPL 4 whereas other studies endorse IPL home treatment for all skin types.5 According to the manufacturers, protective eyewear is not required because the light generated is self-contained within the home device; however, this must be regarded as an additional risk factor for ocular damage in handling all cases.2 Other potential adverse events of IPL home treatment include oedema, pain, erythema, hyper- and hypo-pigmentation, scarring, blistering, crusting, burning, or even paradoxical hair growth. Sufficient safety instructions are often missing or incomplete in the manuals, so users are not made aware of possible complications. Additionally, the lack of individual verbal informed consent generally leads to uncritical application and unrealistic expectations of the user. We report the case of a 25-year-old Caucasian female with Fitzpatrick Phototype III. She presented with complications following her initial hair removal session using an IPL home device

© 2015 European Academy of Dermatology and Venereology

Vulvar white sponge naevus in a girl.

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