Pediatric Dermatology Vol. 26 No. 6 717–720, 2009
Milia En Plaque: Three New Pediatric Cases Carlo Cota, M.D., Jolinda Sinagra, M.D., Pietro Donati, M.D., and Ada Amantea, M.D. San Gallicano Dermatologic Institute, IRCCS, Rome, Italy
Abstract: Milia en plaque (MEP) is an unusual and extremely rare clinical variant of milia, characterized by multiple milia-like lesions overlying an erythematous edematous plaque with histologic findings consistent with milia. MEP tends to affect the middle-aged patients and shows a predilection for women. Among children, this entity is rarely described and, to our knowledge, only four cases have been reported to date in the dermatologic literature. We add three new cases of children, one of whom had an unusual site of presentation.
Milia en plaque (MEP) is a rare localized plaque variant of primary milia, characterized by numerous tiny milia within an erythematous base that arise spontaneously on healthy skin in middle-aged predisposed individuals and without any apparent causative factors. The lesions are characteristically located in the periauricular area and other localizations have been sporadically reported (1). The presentation is very rare in children. In this study, we report three new cases of unilateral MEP occurring in pediatric patients including one with a novel site of presentation.
canthus (Fig. 1A). Isolated milia were observed on the contralateral upper eyelid. Biopsy specimen revealed numerous dermal cysts lined by stratified squamous epithelium and filled with keratin in laminated layers, which were consistent with milia; only few lymphocytic cells were seen around them (Fig. 1B). After a treatment with topical 0.05% tretinoin twice daily, the larger milia were evacuated with a comedo extractor with an acceptable cosmetic result. Case 2
CASE REPORTS Case 1 A 12-year-old girl presented to us with a 6-month history of periorbital multiple milia-like lesions. Neither use of cosmetics and medical creams, nor wearing of eyeglasses was implicated. No history of photosensivity, dermabrasion, or burns was present. On clinical examination, she presented multiple white to yellow cystic lesions varying in size between 0, 5, and 3 mm in diameter and scattered within a slightly erythematous plaque localized on the left upper eyelid, with extension on the inner
A 9-year-old female child presented to us with a 3-month history of an asymptomatic erythematous plaque on the right upper eyelid. She did not wear glasses and denied any history of local trauma, topical medications, or application of cosmetics to the affected area. Cutaneous examination revealed an erythematous, well-defined 2 · 1 cm plaque with numerous whitish-yellow colored cystic lesions over her right upper eyelid along the inner canthus (Fig. 2A). No other milia-like lesion was present on the face and the general examination revealed no further anomalies.
Address correspondence to Carlo Cota, M.D., San gallicano Dermatologic Institute, IRCCS, Via Elio Chianesi, 53, 00144 Rome, Italy, or e-mail:
[email protected]. DOI: 10.1111/j.1525-1470.2009.01020.x
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Case 3 A 9-year-old girl was referred to our department because of an 11-month history of an unusual unilateral plaque on the cheek. The patient’s mother denied the use of any topical preparations prior to the onset of the rash and there was no history of trauma, chemical peeling, dermabrasion, or burns on the affected area. The patient had tried various antibiotic and antimycotic creams without benefit. Dermatologic examination showed a well-defined erythematous 4 · 2, 5 cm plaque studded with multiple, tiny, yellowish cystic lesions localized on the left cheek, just over the nasolabial fold (Fig. 3A). Skin biopsy was characterized by multiple keratin-filled epidermal cysts, surrounded by an intense infiltration of lymphocytes with monocytes and rare eosinophils (Fig. 3B, C). The child was prescribed topical 0.05% tretinoin twice daily, and at 1 month of follow-up, the lesion had partially cleared with a significant decrease in erythema and milia count (Fig. 4).
A
DISCUSSION B
Figure 1. (A) White to yellow milia scattered within a slightly erythematous plaque localized on the left upper eyelid. Isolated milia are present on the contralateral upper eyelid. (B) Multiple well-formed cysts within the entire dermis.
A skin biopsy showed several keratin-filled cysts within the dermis, each surrounded by a mild lymphocytic infiltrate (Fig. 2B). Treatment with topical 0.05% tretinoin twice daily for two months produced only partial improvement. The mother of our patient refused further treatment.
A
Milia en plaque is considered a rare localized plaque variant of primary milia, histopathologically characterized by keratin-filled epidermoid cysts surrounded by mild to dense mononuclear infiltrate. MEP is now a well-recognized entity, with an increasing number of cases being described in the literature. To date 29 cases have been reported and in most of the previous reports, the patients were middle-aged women (median age 43 years; male ⁄ female ratio 9:19) (1,2). MEP is usually described to affect the periauricular area (1,2). Involvement of other sites including eyelids, inner canthus, side of nasal bridge, supraclavicular
B
Figure 2. (A) Numerous yellow colored cystic lesions over the right upper eyelid along the inner canthus. (B) Several keratin-filled cysts within the dermis, each surrounded by a mild lymphocytic infiltrate.
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B
A
C
Figure 3. (A) Well-defined erythematous plaque studded with multiple yellowish cystic lesions of the left cheek. (B, C) multiple keratin-filled epidermal cysts, surrounded by an intense inflammatory infiltrate.
Figure 4. After treatment, the lesion has improved with a significant decrease in erythema and milia count.
area, and forehead has been far more rarely described (3–7). Milia en plaque has been rarely documented in children and adolescents, and in these patients, the lesions were usually localized around the periocular region, particularly over the eyelids and the inner canthus (3,4,8). The only exception was a 14-year-old boy who had a retroauricular MEP with an unusual histologic picture of hybrid cyst with epidermal and trichilemmal keratinization (9). Two of our patients had the same localization with unilateral periorbital plaque of the upper eyelid. It seems that the ocular area is the more common localization in pediatric patients. However, to date, no known triggering factors that can cause secondary milia have
been found in our cases or any of the reported cases, and the physiopathogenic mechanisms which lead to primary MEP formation on the periocular area are unknown. Our last patient presented unilateral MEP on the cheek. This localization has never been reported in pediatric patients and, except for one report of milia arising within a previously identified plaque of discoid lupus erythematosus (10), only one case of a spontaneously appearing MEP covering the entire cheek in a 44year-old man has been described recently (2). Our case has to be considered as primary milia, as the patient did not reveal any known stimulus or external actions that may cause secondary milia and there was no evidence of a pre-existing skin disease. Milia en plaque is a benign and asymptomatic process, but, in most of the cases, entails cosmetic problems, especially in pediatric patients. Unfortunately, treatments for MEP are limited and, usually, the lesions persist unchanged without treatment. Occasionally, spontaneous regression of MEP has been reported (1). Topical retinoid or simple evacuation with a comedo extractor is the mostly frequently used therapy in pediatric patients. In a 10-year-old girl, after an initial treatment with azelaic acid cream and topical hydrocortisone, oral minocycline, 100 mg a day for a month, was used successfully (3). It is reported that responders to extraction or topical retinoid showed a superficial location of the milia on histologic examination, and minocycline administration was advisable in case of presence of dense inflammatory infiltrate of the dermis (1).
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In conclusion, we add three new cases of pediatric female patients with MEP and also report a novel site of presentation not previously described during childhood. From the analysis of the pediatric cases of MEP, it seems that the most common localization in children is the periocular area, but, to date, the physiopathogenic mechanisms that lead to primary MEP formation in the ocular area are unknown. REFERENCES 1. Stefanidou MP, Panayotides JG, Tosca AD. Milia en plaque: a case report and review of the literature. Dermatol Surg 2002;28:291–295. 2. 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.
3. Bridges AG, Lucky AW, Haney G et al. Milia en plaque of the eyelids in childhood: case report and review of the literature. Pediatr Dermatol 1998;15:282–284. 4. Buassida S, Meziou TJ, Mlik H et al. Milium en plaque, infantile, du canthus interne. Ann Dermatol Venereol 1998;125:906–908. 5. Alsaleh QA, Nanda A, Sharaf A et al. Milia en plaque: a new site. Int J Dermatol 2000;39:614–615. 6. Combenale P, Faisant M, Dupin M. Milia en plaque in the supraclavicular area. Dermatology 1995;191:262–263. 7. Fujita H, Iguchi M, Kenmochi Y et al. Milia en plaque on the forehead. J Dermatol 2008;35:39–41. 8. Dogra S, Kanwar AJ. Milia en plaque. J Eur Acad Dermatol Venereol 2005;19:263–264. 9. Lee DW, Choi SW, Cho BK. Milia en plaque. J Am Acad Dermatol 1994;31:107. 10. Boehm I, Schupp G, Bauer R. Milia en plaque arising in discoid lupus erythematousus. Br J Dermatol 1997;137:649–651.
