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Indian Journal of Dermatology
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E-IJD-CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 105
Congenital milia En plaque on scalp


Departments of Skin and VD, and PGIMS, Rohtak, Haryana, India

Date of Web Publication26-Dec-2014

Correspondence Address:
Sangita Ghosh
42\136, New Ballygunge Road, Kolkata - 39
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.147871

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   Abstract 

Milia en plaque is a rare disease entity characterized by confluence of multiple keratin-filled cysts resulting from the obstruction of hair follicle without any preceding primary dermatosis. Fewer than 40 cases have been reported so far in dermatological literature, and most cases are described to occur in adults and in the peri-auricular area. We describe a case of congenital MEP on scalp of a five-year-old boy with a blaschkoid extension into posterior nuchal area. This case report claims its uniqueness because of the unusual site and congenital presentation.


Keywords: Circumscribed cicatricial alopecia, milia en plaque, peri-auricular


How to cite this article:
Ghosh S, Sangal S. Congenital milia En plaque on scalp. Indian J Dermatol 2015;60:105

How to cite this URL:
Ghosh S, Sangal S. Congenital milia En plaque on scalp. Indian J Dermatol [serial online] 2015 [cited 2018 Jan 21];60:105. Available from: http://www.e-ijd.org/text.asp?2015/60/1/105/147871

What was known?
Primary milia en plaque is a rare clinical variant characterized by numerous tiny milia within an erythematous base that arise spontaneously on a healthy skin in a predisposed individual without any apparent causative factor. Pathogenesis behind this entity is unclear. Most cases are seen in adults and occur in peri-auricular areas.



   Introduction Top


Milia en plaque (MEP) is a rare form of primary milia characterized by the presence of milia on an erythematous, edematous infiltrated plaque with or without comedones. The condition was first described by Balzer and Fouquet in 1903 and denominated by Hubler et al. in 1978. [1] Since then, less than 40 cases have been reported in literature till date. [2] MEP has been known to effect mostly peri-auricular and peri-orbital region in adults with a mean age of onset at 44 years with a slight female predilection. [1] We describe this unusual case of progressive milia en plaque on scalp in a five-year-old boy since birth, presenting with circumscribed cicatricial alopecia. This particular case claims its uniqueness because of its unusual site and congenital presentation. Presentation of MEP at birth gives new dimension to the existing hypothesized pathogenesis behind MEP.


   Case Report Top


A five-year-old healthy boy presented to our outpatient department with a progressive, asymptomatic, circumscribed area of lost hair on the parietal scalp, studded with multiple tiny white, globoid, elevated lesions. The father gave history of this lesion being there since birth as a small area of alopecia with relative hardening of circumscribed scalp skin. Father denied any history suggestive of any long-standing pus-discharging infection, scaly dermatosis, or any preceding trauma at the site of the lesion. Over the last five years, the area of involvement increased in size and more such white elevated lesions appeared on the lesion and, despite treatment from multiple centers, there was no improvement. The boy was delivered by a normal vaginal delivery, and his parents had a non-consanguineous marriage. Nobody else in the family has similar lesions or any other significant history.

On examination, there was 5 × 6 cm 2 irregular, circumscribed erythematous and edematous plaque on the left parietal scalp, 6 cm above the mastoid tubercle. The surface of the plaque was devoid of hair and studded with multiple white milia of variable sizes [Figure 1]a. On needling one of the intact milia, white thick material was extruded from it, which was determined to be sterile after performing Gram stain. On palpation, there was no tenderness, induration, or increase in temperature. There were no comedones or any pustules or pus exudation on applying pressure over and around the lesion. There were areas of scarring and superficial crusting in few areas of the plaque. From the lower margin of the plaque, few discrete minute milia spread beyond the inferior margin of the original lesion, to be distributed in a linear fashion, extending up to the posterior nuchal area following a Blaschkoid pattern [Figure 1]b. However, bilateral retroauricular areas were spared of any milia. Potassium hydroxide (KOH) mount of hair uprooted from the margin of plaque did not reveal any fungal element.
Figure 1: (a) Irregular, circumscribed erythematous, infiltrated plaque with complete alopecia and an uneven surface studded with multiple whitish globoid structures, on the left parietal scalp of a 5-year-old boy, (b) Same plaque showing a marginal extension of milia in a linear fashion along the posterior nuchal area following blaschkoid patten

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Histopathology showed multiple keratin-filled inclusion cysts of variable sizes, lined by epithelia, interspersed between cross sections of hair adnexa in dermis with sparse lymphocytic infiltrates [[Figure 2]a, b, and c].
Figure 2: (a) H and E, ×4 scanner view showing multiple cysts lined by epithelia and filled with strands of keratin, in the dermis, interspersed between cross sections of hair follicles with sparse inflammatory infiltrates, (b) H and E, ×10 view showing a cross sectional view of a keratin-filled milium adjacent to a hair follicle, which is in the process of giving rise to a milium, (c) H and E, ×40 view showing a closer view of one of the milia in the dermis, lined by epithelia and the keratin strands within

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The child was started on minocycline 50 mg once daily, which he continued for three weeks without any considerable response. Oral isotretinoin 0.5 mg-1 mg/Kg od, for few weeks also failed to bring about any positive result. Parents did not consent for any surgical procedure.


   Discussion Top


Milia are small keratin-filled cysts that result from the obstruction of a hair follicle or eccrine sweat ducts. Milia are classified as primary when their onset is spontaneous and secondary when they appear following trauma (such as burn, dermabrasion, ablative laser etc.), inflammatory skin diseases (particularly those involving the basement membrane zone, such as sub-epidermal bullous diseases), or the use of topical medication (topical corticosteroids, 5-fluorouracil) or systemic drugs (cyclosporine and benoxaprofen). [1],[3] Primary milia en plaque is a rare clinical variant characterized by numerous tiny milia within an erythematous base that arise spontaneously on a healthy skin in a predisposed individual without any apparent causative factor. [1] Evidences show that primary milia originate in the hair follicle infundibulum, whereas the secondary forms may develop in various adnexal structures like sweat ducts, sebaceous glands, and hair follicle. [1],[4] Despite being an asymptomatic benign lesion, MEP can be a major cosmetic concern to the patient. Earlier reports described this entity as a retroauricular dermatosis; however, later more case reports emerged with different sites of involvement like peri-orbital, [5] pre-auricular, [6] earlobes, [7] sub-mandibular area, [8] and supraclavicular area. [9] Though most cases of MEP have been described in adults, there were three case reports describing MEP in children aged 6 years, 10 years, and 14 years. [5],[10],[11]

The pathogenesis of MEP is unclear. Several theories have been hypothesized including trauma, cold environment, and underlying diseases such as discoid lupus erythematosus, [12] pseudoxanthoma elasticum, [13] follicular mucinosis, and mycosis fungoides. [14] All of these reports suggest some amount of antecedent tissue injury by the underlying diseases, which resulted in development of milia en plaque. Alteration in the infundibular portion of the follicle, resulting in dysfunctional keratinization and outlet obstruction or degeneration of elastic fibers are considered as various etiopathogenesis of MEP by some authors. [1] Presence of MEP at birth probably suggests intrauterine tissue alteration in that area, and extension of few milia along line of Blaschko's was probably due to migration of the altered tissue during cell line alignment in utero. A lack of positive family history, congenital presence, argues for a primary type of milia in our case.

There is no standard treatment for MEP, but there are reports of MEP being successfully treated by various medical as well as surgical treatments. Medical treatments of MEP include topical tretinoin, [1] oral isotretinoin, [14] etretinate, [2] minocycline, [7] photodynamic therapies, [1] producing variable response. Surgical modalities like simple extraction, [10],[12] electrodessication, [11] dermabrasion, [15] and CO 2 laser evaporation [16] had been tried with variable success. Spontaneous regression has also been reported. [17]


   Conclusion Top


MEP is a rare dermatological entity without any optimal treatments. Though most cases reported in the literature were of adult onset or childhood onset, presence of MEP at birth with a Blaschkoid extension in this case reports points towards an in-utero tissue insult. MEP in scalp presenting as a circumscribed area of cicatricial alopecia is also a unique feature of this case. Successful medical and surgical treatment still remains a challenge in this case with failed therapy with minocycline and isotretinoin.

 
   References Top

1.
Stefanidou MP, Panayotides JG, Tosca AD. Milia en plaque: A case report and review of the literature. Dermatol Surg 2002;28:291-5.  Back to cited text no. 1
    
2.
Ishiura N, Komine M, Kadono T, Kikuchi K, Tamaki K. A case of milia en plaque successfully treated with oral etretinate. Br J Dermatol 2007;157:1287-9.  Back to cited text no. 2
    
3.
Rose RF, Merchant W, Goulden V. Retroauricular milia en plaque: A rare presentation of lupus erythematosus. Clin Exp Dermatol 2008;33:715-7.  Back to cited text no. 3
    
4.
Wong SS, Goh CL. Milia en plaque. Clin Exp Dermatol 1999;24:183-5.  Back to cited text no. 4
    
5.
Mendiratta V, Sarkar R, Sharma RC, Koranne RV. Milia en plaque- An unusual presentation. Indian J Dermatol 1999;44:64-5.  Back to cited text no. 5
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6.
Losada-Campa A, De La Torre-Fraga C, Cruces-Prado M. Milia en plaque. Br J Dermatol 1996;134:970-2.  Back to cited text no. 6
    
7.
Keohane SG, Beveridge GW, Benton EC, Cox NH. Milia en plaque-A new site and novel treatment. Clin Exp Dermatol 1996;21:58-60.  Back to cited text no. 7
    
8.
García Sánchez MS, Gómez Centeno P, Rosen E, Sánchez-Aguilar D, Fernández-Redondo V, Toribio J, et al. Milia en plaque in a bilateral submandibular distribution. Clin Exp Dermatol 1998;23:227-9.  Back to cited text no. 8
    
9.
Combemale P, Faisant M, Dupin M. Milia en plaque in the supraclavicular area. Dermatology 1995;191:262-3.  Back to cited text no. 9
    
10.
Bridges AG, Lucky AW, Haney G, Mutasim DF. Milia en plaque of the eyelids in childhood: Case report and review of the literature. Pediatr Dermatol 1998;15:282-4.  Back to cited text no. 10
    
11.
Lee DW, Choi SW, Cho BK. Milia en plaque. J Am Acad Dermatol 1994;31:107.  Back to cited text no. 11
    
12.
Boehm I, Schupp G, Bauer R. Milia en plaque arising in discoid lupus erythematosus. Br J Dermatol 1997;137:649-51.  Back to cited text no. 12
    
13.
Cho SH, Cho BK, Kim CW. Milia en plaque associated with pseudoxanthoma elasticum. J Cutan Pathol 1997;24:61-3.  Back to cited text no. 13
    
14.
Leverkus M, Rose C, Bröcker EB, Goebeler M. Follicular cutaneous T-cell lymphoma: Beneficial effect of isotretinoin for persisting cysts and comedones. Br J Dermatol 2005;152:193-4.  Back to cited text no. 14
    
15.
Van Lynden-van Nes AM, der Kinderen DJ. Milia en plaque successfully treated by dermabrasion. Dermatol Surg 2005;31:1359-62.  Back to cited text no. 15
    
16.
Sandhu K, Gupta S, Handa S. CO2 laser therapy for Milia en plaque. J Dermatol Treat 2003;14:253-5.  Back to cited text no. 16
    
17.
Tsoïtis G, Papadimitriou C, Asvesti C, Lefaki J, Lambroudi M, Hatzibougias J, et al. [Retroauricular dermatitis of the "milia en plaque" type]. Ann Dermatol Venereol 1993;120:58-64.  Back to cited text no. 17
    

What is new?
Scalp is a unique site of presentation for this entity. Though few cases of MEP with childhood onset have been described, congenital presentation of MEP on scalp with a Blaschkoid extension is unusual.


    Figures

  [Figure 1], [Figure 2]



 

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    Abstract
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   Case Report
   Discussion
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    References
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