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 Table of Contents  
CASE REPORT
Year : 2011  |  Volume : 3  |  Issue : 1  |  Page : 31-33  

Malherbe's calcifying epithelioma (pilomatrixoma): An uncommon periocular tumor


Ocular Oncology Service, L. V. Prasad Eye Institute, Banjara Hills, Hyderabad, Andhra Pradesh, India

Date of Web Publication16-Jun-2011

Correspondence Address:
Santosh G Honavar
Ocular Oncology Service, L. V. Prasad Eye Institute, Road No. 2, Banjara Hills, Hyderabad 500 034, Andhra Pardesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-7753.82134

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   Abstract 

Benign calcifying epithelioma of Malherbe or pilomatrixoma or pilomatricoma is an uncommon lesion of the periocular tissues, arising from the matrix cells at the base of the hair. In the periocular area, it usually arises from the lids and eyebrows. Pilomatrixoma has certain characteristic clinical and histopathologic features, but since it is not commonly suspected preoperatively, certain distinctive clinical features of tumor should suggest clinical diagnosis followed by histopathologic confirmation.

Keywords: Malherbe′s epithelioma, pilomatricoma, pilomatrixoma


How to cite this article:
Ali MJ, Honavar SG, Naik MN, Vemuganti GK. Malherbe's calcifying epithelioma (pilomatrixoma): An uncommon periocular tumor. Int J Trichol 2011;3:31-3

How to cite this URL:
Ali MJ, Honavar SG, Naik MN, Vemuganti GK. Malherbe's calcifying epithelioma (pilomatrixoma): An uncommon periocular tumor. Int J Trichol [serial online] 2011 [cited 2020 Sep 24];3:31-3. Available from: http://www.ijtrichology.com/text.asp?2011/3/1/31/82134


   Introduction Top


Benign calcifying epithelioma of Malherbe or pilomatrixoma or pilomatricoma is an uncommon lesion of the periocular tissues, arising from the matrix cells at the base of the hair. [1] In the periocular area, it usually arises from the lids and eyebrows. Since it is not commonly suspected preoperatively, certain distinctive clinical features of tumor should suggest clinical diagnosis followed by histopathologic confirmation.


   Case Report Top


A 25-year-old male presented with a small nodule in the right brow since the past 2 years. His best-corrected visual acuity was 20/20, N6 in both eyes and ocular examination was within normal limits.

The nodular lesion measuring 8×6 mm was present within the brow, subcutaneous in location, well-circumscribed, non-tender, freely movable and firm with a gritty surface on palpation. Skin over the swelling could not be well-visualized because of the dense overlying brow hair [Figure 1]. Since the patient was keen on surgical removal, an excision biopsy was performed through a small incision taken just over the lesion so that the scar could well be hidden within the brow hair.
Figure 1: External clinical photograph. The slight elevation and boundaries of the subcutaneous nodular lesion are marked by a circle

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Histopathology

Gross examination showed a well-circumscribed single grayish-white mass measuring 7×5×5 mm. Cut section showed grayish areas, firm and gritty. Microscopic examination showed numerous lobules with basophilic cells in the periphery and of ghost-like squamous cells toward the center with a few anucleated cells [Figure 2]. Various stages of maturation of the basaloid cells into shadow cells could be seen [Figure 3]. These islands are surrounded by foreign body giant cells with a few lymphoplasmacytic infiltrates [Figure 4]. There were numerous foci of calcification more so in the necrotic areas and in the periphery of cellular islands [Figure 2] and [Figure 3]. The histopathologic features were consistent with a diagnosis of pilomatrixoma (benign calcifying epithelioma of Malherbe).
Figure 2: Photomicrograph showing central area of calcification surrounded by shadow cells. Basaloid cells can be seen in the periphery (H and E, ×100)

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Figure 3: Various stages of maturation of basaloid cells into shadow cells seen (H and E, ×400)

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Figure 4: Photomicrograph showing numerous islands of basaloid cells and few foreign body giant cells (H and E, ×100)

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   Discussion Top


Pilomatrixoma is an uncommon lesion that arises from the matrix cells at the base of the hair. It was first described by Malherbe as benign calcifying epithelioma. [2] Subsequently numerous ultrastructural and electron microscopic studies [3],[4] provided strong evidence of its origin from the matrix cells and the term "pilomatrixoma" was then coined by Forbis and Helwig keeping the histogenesis into consideration. [1]

Pilomatrixoma is usually a solitary lesion affecting young individuals. Forty percent of them develop in the first decade of life and another 20% in the second decade. [5] They most commonly involve the head and neck region followed by upper extremities, trunk, and lower extremities. [6] Periocular tissues are involved in 10-17% of cases and 5% of cases are multifocal. [6],[7],[8]

Pilomatrixoma generally presents with subcutaneous red to blue mass that is fairly well circumscribed, freely movable and firm to gritty on palpation. [9] Clinical features as documented by Duran et al.[10] and later also by Perez and Nicholson.[11] should arise clinical suspicion and they include onset in childhood or early adulthood, average size of 10 mm or less, consistency ranging from firm to cystic, moderate pattern of growth, pink to purple hue with subepithelial yellowish tinge, and intact overlying skin with telangiectatic vessels.

Clinical differential diagnosis includes epidermoid cysts, dermoid cyst, sebaceous adenoma or carcinoma, juvenile xanthogranuloma, capillary hemangioma, chalazion, and rhabdomyosarcoma. [6],[9],[11] Although they grow slowly, they occasionally demonstrate rapid growth and may resemble keratoacanthoma. [12] They can rarely undergo malignant transformation into pilomatrix carcinoma. [13]

Histopathologic examination reveals the tumor to be grossly well circumscribed and firm to gritty in consistency. Microscopic examination shows numerous islands of epithelial cells with characteristic arrangement of basophilic cells in the periphery and shadow cells in the center. As the tumor matures the number of basophilic cells looses their nuclei and becomes shadow cells. Calcification is seen in 75% of the cases. Sheets of intensely eosinophilic keratinous material is seen within necrotic areas, and this may induce a foreign body giant cell reaction. [7],[9],[14] Histopathologic differential diagnosis include basal or squamous cell epitheliomas as well as a variety of skin and subcutaneous cysts. [15]

Management includes a complete excision biopsy if there is any clinical suspicion of pilomatrixoma based on the clinical features described since this tumor is often confined to the soft tissues. Various aesthetic approaches can be used for excision. A sub-brow approach using a small incision close the hairline is ideal in such lesions. A superior lid crease approach as for blepharoplasty can also be used but preferable if the brow lesion is larger.

Although pilomatrixoma is an uncommon benign tumor and frequently misdiagnosed as epidermoid or dermoid cyst, it has some distinctive clinical features that suggest the correct diagnosis.

 
   References Top

1.Forbis R Jr, Helwig EB. Pilomatrixoma (Calcifying epithelioma). Arch Dermatol 1961;83:606-18.  Back to cited text no. 1
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2.Malherbe A, Chenantais JE. Note sur l' epitheliome calcifie des glandes sebacees. Progres Med Par 1880;8:826-8.  Back to cited text no. 2
    
3.Lever WF, Griesemer RD. Calcifying epithelioma of Malherbe: report of fifteen cases with comments on its differentiation from calicified epithelial cyst and on its histogenesis. Arch Derm Syphilol 1949;59:506-18.  Back to cited text no. 3
[PUBMED]    
4.Mc Gavran MH. Ultra structure of pilomatrixoma (calcifying epithelioma). Cancer 1965;18:1445-56.  Back to cited text no. 4
    
5.Mencia-Gutierrez E, Gutierrez-Diaz E, Garcia-Suarez E, Ricoy JR. Eyelid pilomatricomas in young adults: A report of 8 cases. Cutis 2002;69: 23-6.  Back to cited text no. 5
    
6.Yap EY, Hohberger GG, Bartley GB. Pilomatrixoma of the eyelids and eyebrows in children and adolescents. Ophthal Plast Reconstr Surg 1999;15:185-9.  Back to cited text no. 6
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7.Boniuk M, Zimmerman LE. Pilomatrixoma (benign calcifying epithelioma) of the eyelid and eyebrow. Arch Ophthalmol 1963;70:399-406.  Back to cited text no. 7
[PUBMED]  [FULLTEXT]  
8.Orlando RG, Rogers GL, Bremer DL. Pilomatricoma in a pediatric hospital. Arch Ophthalmol 1983;101:1209-10.  Back to cited text no. 8
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9.Shields JA, Shields CL, Eagle RC Jr, Mulvey L. Pilomatrixoma of the eyelid. J Pediatr Ophthalmol Strabismus 1995;32:260-1.  Back to cited text no. 9
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10.Duran S, de Buen S. Epitelioma calcificante de Malherbe. Revision de la literatura y presentacion de un caso. An Soc Mex Oftalmol 1968;41:109-16.  Back to cited text no. 10
    
11.Perez RC, Nicholson DH. Malherbe's calcifying epithelioma (pilomatrixoma) of the eyelid. Arch Ophthalmol 1979;97:314-5.   Back to cited text no. 11
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12.Kang HY, Kang WH. Guess what! Perforating pilomatricoma resembling keratoacanthoma. Eur J Dermatol 2000;10:63-4.  Back to cited text no. 12
    
13.Martelli G, Giardini R. Pilomatrix carcinoma: A case report and review of the literature. Eur J Surg Oncol 1994;20:703-4.  Back to cited text no. 13
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14.Font RL. Eyelids and lacrimal drainage system. In: Spencer WH, editor. Ophthalmic Pathology. An Atlas and Textbook. 4th ed. Philadelphia: WB Saunders; 1996. p. 2321-2.  Back to cited text no. 14
    
15.O'Grady RB, Spoerl G. Pilomatrixoma (Benign calcifying epithelioma of Malherbe). Ophthalmology 1981;88:1196-7.  Back to cited text no. 15
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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