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| CASE REPORT |
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| Year : 2020 | Volume
: 12
| Issue : 2 | Page : 51-53 |
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Pilomatrixoma of the left upper eyelid
Pragati Garg, Akansha Sharma
Department of Ophthalmology, ERA's Lucknow Medical College, ERA University, Lucknow, Uttar Pradesh, India
| Date of Submission | 09-Jun-2019 |
| Date of Decision | 17-Jul-2020 |
| Date of Acceptance | 08-Sep-2020 |
| Date of Web Publication | 09-Mar-2021 |
Correspondence Address:
Dr. Akansha Sharma
Department of Ophthalmology, ERA's Lucknow Medical College, ERA University, Lucknow, Uttar Pradesh
India
 DOI: 10.4103/sjopthal.sjopthal_15_19 
To report an uncommon presentation of pilomatrixoma. We report a 72-year-old male attending the ophthalmology outpatient department of a tertiary care center of North India with a painless growth just above his left upper lid at the lateral end of the left eyebrow. He underwent radiological imaging, histopathological examination, and thorough ocular examination which were suggestive of a pilomatrixoma. Pilomatricoma, histologically, is characterized by a mass made up of basaloid cells in the periphery, ghost cells in the central part and calcification, and sometimes ossification. The ghost cells represent necrotic areas of previously vital basaloid cells. The calcification and ossification areas appear progressively in necrotic areas. Ghost cells are pathognomonic of pilomatricoma. Keywords: Calcification, lid, neoplasia
How to cite this article:
Garg P, Sharma A. Pilomatrixoma of the left upper eyelid. Sudanese J Ophthalmol 2020;12:51-3 |
| Introduction | |  |
Pilomatricoma or pilomatrixoma or calcifying epithelioma of Malherbe is a benign skin neoplasia originating from the hair follicle matrix cells.[1] It usually develops slowly and presents as a single or sometimes multiple benign solid lesions lying just under or in the skin.[2],[3] Pilomatricomas represent 0.12% of all skin tumors.[4]
Clinically, the lesion appears as a slowly enlarging, irregularly shaped, rock hard, nodular, and nontender mass freely movable under the subcutaneous tissue. The skin usually has reddish to blue discoloration due to dilated blood vessels and chalky white nodules may be seen through the skin. There is typically no history of inflammation or trauma.[5],[6],[7]
| Case Report | | |
A 72-year-old male reported in the ophthalmology outpatient department of a tertiary care center of North India with a painless growth just above his left upper lid at the lateral end of the left eyebrow [Figure 1]. The lesion first appeared 1 year ago as a small painless swelling which slowly grew to its present size. The patient gave a history of similar lesions present in the left forearm and both the thighs. The patient underwent a fine-needle aspiration cytology (FNAC) before our consultation. It reported blood mixed field and was advised biopsy. As stated by the patient, the lesion present at the apex of the mass was due to FNAC performed elsewhere. The patient had no ocular complaints or difficulty with lid functions but complained of diminution of vision. There was no history of trauma or similar complaints in the other family members. He was a known diabetic for the past 5 years but was well controlled by medication.
On examination, the visual acuity was 5/60 unaided bilaterally. He was diagnosed with a brown cataract in the left eye and was pseudophakic in the right eye. Examination of the adnexa revealed a single sessile, reddish-brown growth above the left upper eyelid measuring 2 cm × 1.5 cm in size. It was firm in consistency, noncompressible, and nonpulsatile. There was no mechanical ptosis or bruit. The computed tomography (CT)-scan orbit and the head-and-neck was advised before our consultation to assess the extent of the nodular mass and involvement of the deeper tissues of the eyelid. The patient has misplaced the CT scan and only had the report which revealed a heterogeneous hypoechoic tissue mass in the subcutaneous area with an attachment to the underlying muscle. There was no evidence of intraorbital extension or deeper tissue involvement.
The patient was subjected to surgical excision by modified Blair's technique using the radiofrequency machine after obtaining proper consent from the patient. During surgery, the growth was free from overlying skin but was attached to the underlying muscle. The mass was gray-white in color, smooth surface with a hair follicle present at the apex [Figure 2]. The wound was repaired primarily with interrupted sutures using 6–0 Vicryl [Figure 3]. Histopathological examination reported a single globular gray–white to gray–brown soft tissue measuring 2 cm × 1.5 cm. Cut surface showed chalky white solid areas. Microscopic examination of the excised tissue section showed the trichilemmal type of keratinization embedded in cellular stroma along with many ghost cells and nest of basaloid cells. These basaloid cells have round or elongated deeply basophilic nuclei and scanty cytoplasm. Few areas show calcification. There was no granulomatous or neoplastic pathology identified [Figure 4].
The patient was assessed weekly after the procedure and the sutures were removed 2 weeks postoperatively. The postoperative result was satisfactory and the patient was content with the aesthetic outcome.
Written and informed consent was obtained from the patient for the use of photographs for publication of this article. Ethical approval for this study was provided by the ethical committee of Era's Lucknow Medical College, Lucknow, Uttar Pradesh.
| Discussion | | |
Pilomatricoma is relatively rare skin neoplasia. It may affect individuals at any age, incidence peaks on the first and sixth decades of life. It is more common in women (1.5–2.5:1).[1],[2],[3],[4],[5],[6],[7],-8] The largest case series in the literature includes 346 pilomatricomas of which 15.3% were observed in the upper extremities [Figure 5].[9]
Pilomatricoma is associated with high levels of beta-catenin caused by either a mutation in the APC gene or a stabilizing mutation in the beta-catenin gene, CTNNB1. B-catenin is a component of the cadherin protein, and it plays a role in the hair follicle differentiation. High expression of B-catenin leads to activation of the Wnt signaling pathway, and this is seen in the matrix cells that are proliferating. B-catenin controls the adhesion between epithelial cells. Overexpression of BCL2 proto-oncogene also has been seen in immunohistochemical studies high levels of beta-catenin increases cell proliferation, inhibit cell death, and ultimately leads to neoplastic growth.[10]
These lesions are typically found in the head-and-neck region, but they have also been described in various upper extremity locations. It is a benign skin neoplasm that arises from hair follicle matrix cells.[7],[11],[12],[13],[14],[15],[16] Clinically, it manifests as asymptomatic, benign solitary, soft, and friable to hard nodule measuring 0.5 cm–5.0 cm. It is a subcutaneous slow-growing tumor which may or may not be attached to the skin and mobile over underlying structures. It may also present as multiple and nodular patterns in 2%–10% of the cases.[5]
Histologically, pilomatricoma is characterized by a mass made up of basaloid cells in the periphery, ghost cells in the central part and calcification, and sometimes ossification. The ghost cells represent necrotic areas of previously vital basaloid cells. The calcification and ossification areas appear progressively in necrotic areas. Ghost cells are pathognomic of pilomatricoma.[17]
Making a clinical diagnosis of pilomatrixoma can be difficult. The differential diagnosis includes dermoid, inclusion cysts, hemangiomas, and malignant soft-tissue tumors.
| Conclusion | | |
Pilomatricoma is a benign skin neoplasia originating from hair follicle matrix cells. In our case, the presenting age was 72 years, had an eyelid affliction, was of male sex, and there was the presence of a similar lesion in the forearm and thighs with the absence of any predisposing factors such as trauma and typical histopathological findings, thus making it an uncommon presentation of pilomatricoma and hence reported.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
| 1. | Fernandes R, Holmes J, Mullenix C. Giant pilomatricoma (epithelioma of Melherbe): Report of a case and review of literature. J Oral Maxillofac Surg 2003;61:634-6.  |
| 2. | Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma). A statistical study. Arch Dermatol 1973;108:532-4. |
| 3. | Urvoy M, Legall F, Toulemont PJ, Chevrant-Breton J. Multiple pilomatricoma. Apropos of a case. J Fr Ophthalmol 1996;19:464-6. |
| 4. | Greene RM, McGuff HS, Miller FR. Pilomatrixoma of the face: A benign skin appendage mimicking squamous cell carcinoma. Otolaryngol Head Neck Surg 2004;130:483-5. |
| 5. | Abalo-Lojo JM, Cameselle-Teijeiro J, Gonzalez F. Pilomatrixoma: Late onset in two periocular cases. Ophthalmic Plast Reconstr Surg 2008;24:60-2. |
| 6. | Kumar S. Rapidly growing pilomatrixoma on eyebrow. Indian J Ophthalmol 2008;56:83-4. [ PUBMED] [Full text] |
| 7. | Kumaran N, Azmy A, Carachi R, Raine PA, Macfarlane JH, Howatson AG. Pilomatrixoma-Accuracy of clinical diagnosis. J Pediatr Surg 2006;41:1755-8. |
| 8. | Pirouzmanesh A, Reinisch JF, Gonzalez-Gomez I, Smith EM, Meara JG. Pilomatrixoma: A review of 346 cases. Plast Reconstr Surg 2003;112:1784-9. |
| 9. | Birman MV, McHugh JB, Hayden RJ, Jebson PJ. Pilomatrixoma of the forearm: A case report. Iowa Orthop J 2009;29:121-3. |
| 10. | Hassanein AM, Glanz SM, Kessler HP, Eskin TA, Liu C. Beta-Catenin is expressed aberrantly in tumors expressing shadow cells. Pilomatricoma, craniopharyngioma, and calcifying odontogenic cyst. Am J Clin Pathol 2003;120:732-6. |
| 11. | Marrogi AJ, Wick MR, Dehner LP. Pilomatrical neoplasms in children and young adults. Am J Dermatopathol 1992;14:87-94. |
| 12. | Migirov L, Fridman E, Talmi YP. Pilomatrixoma of the retroauricular area and arm. J Pediatr Surg 2002;37:E20. |
| 13. | Schweitzer WJ, Goldin HM, Bronson DM, Brody PE. Solitary hard nodule on the forearm. Pilomatricoma. Arch Dermatol 1989;125:828-9, 832. |
| 14. | Meyer RS, Sanchez AA, Bannykh G, Donthineni-Rao R, Botte MJ. Forearm mass in a 31-year-old man. Clin Orthop Relat Res 2004;424:280-6. |
| 15. | Wagner AM. A 12-year-old girl with an enlarging nontender nodule on the arm. Pediatr Ann 2006;35:445-7. |
| 16. | Yencha MW. Head and neck pilomatricoma in the pediatric age group: A retrospective study and literature review. Int J Pediatr Otorhinolaryngol 2001;57:123-8. |
| 17. | Passi N, Chawala U, Jyoti A, Khurana AK. Pilomatrixoma of eyelid in a middle aged. Nepal J Ophthalmol 2011;3:88-90. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]
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