Case report

A new site of milia en plaque: report of a case and review of the literature Mohammadreza Barzegar, MD, and Nikoo Mozafari, MD

Department of Dermatology, Skin Research Center, Shahid Beheshti University of Medical Sciences, Tehran, Iran Correspondence Nikoo Mozafari, MD Department of Dermatology Skin Research Center Shohada-e Tajrish Hospital, Tajrish Square Tehran Iran E-mail: [email protected]

Introduction Milia en plaque (MEP) is a rare benign epidermal tumor, characterized as numerous milia arising on an erythematous plaque.1 The first description was given by Balzer and Fouguet in 1903, and named MEP by Hubler in 1978.2 Less than 50 cases of this rare condition have been reported to date. Milia en plaque has been rarely reported in children. Periocular and periauricular areas are the most common sites of involvement,3 and to the best of our knowledge, involvement of nasal ala has not been reported to date. Here we add one more case with a new site of involvement to the reports already published and provide a survey of the clinical aspects and treatment options of patients with milia en plaque.

epithelium. Cysts were surrounded by a mild infiltrate of lymphocytes (Fig. 2). The diagnosis of milia en plaque was made based on clinical and pathological findings of the lesion. After giving reassurance to the parents that the lesion is benign, they refused treatment, although they were informed that the lesion usually persists unchanged without treatment. Discussion Milia are small white benign superficial keratinous cysts.1 Histologically they resemble small epidermal cysts con-

Case report A 4-year-old girl presented with a 2-month history of two adjacent clusters of multiple milia on her right nasal ala area. Cutaneous examination revealed an erythematous well-defined plaque measuring 1 9 0.5 cm containing numerous tiny yellowish papules (Fig. 1). There was no history of trauma, burn, or blistering disease on the affected region. She did not wear glasses and denied use of cosmetic or oily cream or topical steroid. A history of photosensitivity was absent. In general, examination of the patient showed she was otherwise healthy. Punch biopsy of the lesion was performed for histopathological evaluation. Histological section of specimen revealed various small subepidermal cysts filled with concentric lamellar keratin, lined by a stratified squamous ª 2013 The International Society of Dermatology

Figure 1 Grouped milia over a slight erythematous plaque on the right nasal ala International Journal of Dermatology 2015, 54, 1423–1425

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Milia en plaque on the nose

Figure 2 Subepidermal multiple keratin-filled cysts surrounded by mild inflammatory infiltrate (hematoxylin and eosin, 9 10)

taining central keratinous material, enveloped by stratified squamous epithelium and a granular cell layer.1 Milia are classified as primary and secondary types. Milia can arise spontaneously without known cause (primary milia), or they can be secondary to genodermatoses (epidermolysis bullosa or hereditary porphyria), medication (benoxaprofen, topical steroid, 5-fluorouracil, cyclosporine, penicillamine), bullous disease (bullous pemphigoid, herpes zoster, bullous lupus erythematosus, etc.), and trauma (traumatic abrasion, second degree burn, dermabrasion, chemical peels, ablative laser therapy).1 Milia en plaque is a rare clinical variant of primary milia, characterized as numerous milia arising on an erythematous plaque. The etiology, pathogenesis, and significance of this rare phenomenon have not been clarified.1,2 Lesions usually develop on the head and neck. They can be unilateral or bilateral and have a special predilection for the ears and eyes.2 In adults, they most commonly occur on the periauricular area (48%), and the periorbital area is another common site (19%). They may also be found on the forehead,4 cheek,5 submandibular,2 and chin.6 MEP occasionally involves the supraclavicular2 and limb region.7 One case of MEP on the nasal bridge has been described; however, it seems to be the case of MEP of the inner canthus.8 Milia en plaque has been reported in different age groups. Typically, adults between the ages of 32 and 84 years are affected. It has also been reported in children.3 In this age group, MEP favors involvement first on the periocular region (six cases) then the periauricular region (two) and cheek (one). Our case is the first report of MEP on the nasal ala. Moreover, it is the youngest patient yet reported with this dermatosis. It seems MEP is more common in women (male/female ratio in adults: 14 : 23; in children: 3 : 6), although it is International Journal of Dermatology 2015, 54, 1423–1425

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not entirely clear that this represents gender predilection rather than case selection bias due to cosmetic factors. As MEP is a benign process and lacks symptoms, only cosmetic concerns lead to a medical visit. Coexistence of MEP with pseudoxanthoma elasticum2 and previously identified plaque of discoid lupus erythematosus9 as well as in a renal transplant recipient taking cyclosporine have been reported.7 It has also been reported as a de novo manifestation of chronic cutaneous lupus erythematosus.6,10 Therefore, it should be considered as a differential diagnosis of cutaneous lupus erythematosus, particularly when biopsy of the lesion reveals typical alteration of this disease, such as pronounced lichenoid infiltration with basal vacuolar changes at the dermoepidermal junction of the cyst walls and mucin deposition in the reticular dermis along with positive direct immunofluorescence.6,10 Milia en plaque generally remains unchanged if untreated; however, occasional spontaneous regression has been reported.2 Several treatments have been suggested, but no optimal treatment has been established because of few described cases that vary in size, degree of inflammation, and depth of lesion in skin.2 In general, simple extraction or topical retinoids are the most tried options; incision followed by extraction of the keratin core may result in recurrence or scarring. Good response to topical tretinoin 0.05% or Adapalen 0.1% over three months has been reported,2 particularly in cases with superficially located milia on histologic examination; however, treatment with topical retinoids may not be successful, and recurrence has been reported after discontinuation of therapy.2 Minocycline 100 mg/day for 2–3 months has been used successfully in four cases of MEP with dense dermal infiltration in histological examinations,2 while a 2-week application of doxycycline 100 mg/day was accompanied by no benefit.8 According to the anti-inflammatory and antikeratinization effects of systemic retinoids, etretinate (50 mg/day) has been applied in one case. After three months of therapy, a marked reduction of milia was observed.11 Photodynamic therapy with topical aminolevulinic acid 20% resulted in a partial improvement; however, its high cost makes it unacceptable.2 Surgical excision can be considered for small lesions.4 Although destructive procedures are possible predisposing factors for milia formation, they have been used for treatment of deeply located milia. Removing epidermis and superficial dermis via dermabrasion under local anesthesia has resulted in an acceptable outcome in a patient with MEP on the mandible.12 Open spray cryosurgery has been successfully applied in one case with bilateral retroauricular plaques. After three weeks, complete re-epithelialization occurred, and almost complete resolution of the lesions and no pigmentary changes were ª 2013 The International Society of Dermatology

Barzegar and Mozafari

observed.13 Other inexpensive and office surgical procedures such as electrodesiccation14 and radiosurgery15 have been applied for the treatment of periorbital milia with partial resolution, but they are associated with considerable risk of thermal injury and scarring. For periocular MEP, which cosmetic and functional results are highly advocated, more accurate destructive methods such as ablative lasers are preferred.16,17 In overall, the erbium:YAG laser, with more careful control of depth of ablation and less thermal injury than CO2 laser, represents a promising treatment option for this rare condition.17 In summary, we have presented a case of MEP on the nasal ala of a 4-year-old girl. This is an extremely rare presentation. To our knowledge, this is the first reported case at this site and the youngest patient with this dermatosis. In view of limited reported cases of MEP, there is no consensus on the best therapeutic options; therefore, treatment should be individualized. References 1 Berk DR, Bayliss SJ. Milia: a review and classification. J Am Acad Dermatol 2008; 59: 1050–1063. 2 Stefanidou MP, Panayotides JG, Tosca AD. Milia en plaque: a case report and review of the literature. Dermatol Surg 2002; 28: 291–295. 3 Zhang RZ, Zhu WY. Bilateral milia en plaque in a 6year-old Chinese boy. Pediatr Dermatol 2012; 29: 504–506. 4 Fujita H, Iguchi M, Kenmochi Y, et al. Milia en plaque on the forehead. J Dermatol 2008; 35: 39–41. 5 Cota C, Sinagra J, Donati P, et al. Milia en plaque: three new pediatric cases. Pediatr Dermatol 2009; 26: 717–720.

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Case report

6 Kouba DJ, Owens NM, Mimouni D, et al. Milia en plaque: a novel manifestation of chronic cutaneous lupus erythematosus. Br J Dermatol 2003; 149: 424–426. 7 Dogra S, Kaur I, Handa S. Milia en plaque in a renal transplant patient: a rare presentation. Int J Dermatol 2002; 41: 897–898. 8 Alsaleh QA, Nanda A, Sharaf A, et al. Milia en plaque: a new site. Int J Dermatol 2000; 39: 614–615. 9 Belhadjali H, Youssef M, Yahia S, et al. Milia en plaque and discoid lupus erythematosus. Clin Exp Dermatol 2009; 34: e356–e357. 10 Rose RF, Merchant W, Goulden V. Retroauricular milia en plaque: a rare presentation of lupus erythematosus. Clin Exp Dermatol 2008; 33: 715–717. 11 Ishiura N, Komine M, Kadono T, et al. A case of milia en plaque successfully treated with oral etretinate. Br J Dermatol 2007; 157: 1287–1289. 12 van Lynden-van Nes AM, der Kinderen DJ. Milia en plaque successfully treated by dermabrasion. Dermatol Surg 2005; 31: 1359–1362. 13 Noto G, Dawber R. Milia en plaque: treatment with open spray cryosurgery. Acta Derm Venereol 2001; 81: 370–371. 14 Al-Mutairi N, Joshi A. Bilateral extensive periorbital milia en plaque treated with electrodesiccation. J Cutan Med Surg 2006; 10: 193–196. 15 Wollina U. Bilateral milia en plaque of the eyelids: long eyelashes and unibrow – case report and review of literature. Dermatol Surg 2010; 36: 406–408. 16 Pozo J, Castineiras I, Fernandez-Jorge B. Variants of milia successfully treated with CO(2) laser vaporization. J Cosmet Laser Ther 2010; 12: 191–194. 17 Voth H, Reinhard G. Periocular milia en plaque successfully treated by erbium:Yag laser ablation. J Cosmet Laser Ther 2011; 13: 35–37.

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A new site of milia en plaque: report of a case and review of the literature.

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