|Year : 2022 | Volume
| Issue : 3 | Page : 268-270
A case report of eyelid pilomatrixoma: A rare presentation
Prateek Jain1, Sapna Bhandari2, Anshuman Pattnaik1, Pooja Shivaji Shinde1
1 Department of Community Ophthalmology, Global Hospital Institute of Ophthalmology, Sirohi, Rajasthan, India
2 Global Hospital Trauma Centre, Sirohi, Rajasthan, India
|Date of Submission||30-Nov-2020|
|Date of Decision||15-Dec-2020|
|Date of Acceptance||22-Dec-2020|
|Date of Web Publication||22-Dec-2022|
Dr. Prateek Jain
Global Hospital Institute of Ophthalmology, Abu Road, Sirohi, Rajasthan
Source of Support: None, Conflict of Interest: None
Pilomatrixoma is a rare periocular benign tumor involving hair follicle matrix cells. It presents as a hard, painless, and gradually enlarging mass. It is more common in Caucasians, has bimodal age distribution (5–15 years and 50–65 years) and with female preponderance. We present a rare case report of a 35-year-old Indian male presenting with progressively enlarging; noninflammatory mass in the left upper eyelid for which excision biopsy was performed and a diagnosis of pilomatrixoma was arrived at. The main differential diagnosis includes chalazion and it is vital to distinguish them prior to any surgical intervention as both have different lines of management. This article also deals with the clinical as well as histopathological features and syndromic association of pilomatrixoma along with a brief mention of its malignant variation. This case report also includes intraoperative photographs of the lesion which the authors could not find during literature search.
Keywords: Chalazion, eyelid mass, pilomatrixoma
|How to cite this article:|
Jain P, Bhandari S, Pattnaik A, Shinde PS. A case report of eyelid pilomatrixoma: A rare presentation. Kerala J Ophthalmol 2022;34:268-70
| Introduction|| |
A pilomatrixoma, also known as calcifying epithelioma of Malherbe, is a benign appendageal tumor with differentiation toward hair follicle matrix cells.
| Case Report|| |
A 35-year-old male, computer operator by occupation, presented with a complaint of gradually increasing painless swelling in the left upper eyelid for 2 months. The history revealed the absence of redness around the lesion or in the eye during the entire period of progression. The patient had no history of trauma or systemic illness. There was no history of similar complaint in the family. The swelling was diagnosed as chalazion at first presentation and warm compresses, lid massage, and topical medication were advised. In spite of patient being compliant, swelling did not regress and he presented again after a month with an enlarged size.
On inspection, a single large mass with well-defined margins involving the left upper eyelid causing mechanical ptosis was observed. The eyelid margin was uninvolved and overlying skin showed reddish-blue discoloration [Figure 1]. On palpation, the mass was found to be around 9 mm × 5 mm size, nontender, hard in consistency, and located on pretarsal area, which could be moved over underlying structures. The overlying skin was adherent to the mass. On eyelid eversion, palpebral conjunctiva appeared normal. The uncorrected visual acuity was 20/20 and the rest of other ocular examination was unremarkable. Examination of the right eye was within normal limits. Systemic examination did not reveal any lymphadenopathy or nodular lesion elsewhere.
|Figure 1: Clockwise from top to left: Torch-light photograph of the left upper eyelid swelling with mechanical ptosis; Slit-lamp photograph depicting reddish blue discoloration of overlying skin due to dilated blood vessels; Photograph after lid eversion showing un-involved upper tarsal conjunctiva; postoperative 1 month photograph showing reversal of ptosis after excisional biopsy|
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In view of the mass increasing in spite of medical treatment, excisional biopsy was planned. Routine blood investigations were normal and the patient was taken to operating room. Local infiltration with 2% lignocaine followed by painting and draping was performed. Mass was approached from the skin side. Under microscope, a horizontal circumlinear skin incision was given along the superior border of the mass followed by meticulous dissection of surrounding adhesions. Single, isolated, and encapsulated nodule was excised without rupturing its wall. No hemorrhagic or ulcerative patterns were noted in the adjacent tissues. The root of follicles could be visualized in close proximity of the nodule [Figure 2]. Wound was closed by applying interrupted (8-0) Vicryl sutures. Excised sample was sent en bloc for histopathological (h/p) analysis in a vial containing 10% formalin. There were no intraoperative or postoperative complications. Routine postoperative medications were administered.
|Figure 2: Intraoperative photographs: clockwise from top to left: exposed subcutaneous mass after horizontal skin incision; encapsulated mass being delineated after surrounding adhesiolysis; completely excised nodule; hair follicles underneath the excised nodule, adding to the diagnosis|
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On H/P: Grossly, a single, globular pinkishbrown encapsulated mass with smooth outer surface measuring 6 mm × 3 mm × 2 mm was seen. Whole sample was processed and embedded in one block. Multiple sections of 5 μ each were cut and stained by Hematoxylin and Eosin stain, following which they were examined under microscope at ×10 and ×40 magnification.
Under microscope, the tumor showed sheets of bland, uniform, and basophilic hair matrix cells. Large masses of ghost/shadow cells, basaloid cells, and inflammatory cells were observed. Confluent foci of dystrophic calcification of the matrix with keratin were seen. Ghost cells are suggestive but not pathognomonic of pilomatrixoma and represent necrotic areas of previously vital basaloid cells. Careful serial sections did not show malignant change in any field of the lesion. Based on these features, a histopathological diagnosis of a pilomatrixoma was arrived at.
| Discussion|| |
Pilomatrixoma (pilomatricoma), a benign tumor of the hair follicle, was first described by Malherb in 1880 as “calcifying epithelioma” and was thought to be derived from the sebaceous gland. Later on, in 1949 Lever and Griesemer suggested the hair matrix cells to be the origin of the tumor and finally in 1961 Forbis and Helwig proposed the currently accepted name “pilomatrixoma.”
Pilomatrixoma while commonly encountered in other parts of the body is a rare periocular tumor. The most common periocular location is the eyebrow but can involve the eyelid. There are limited reports in the literature on eyelid pilomatrixomas.,
The age of presentation shows a bimodal pattern, with the first peak being 5–15 years and the second being 50–65 years with a female preponderance. A racial study revealed that most cases are presented in Caucasians and relatively few in Asians.
Isolated pilomatrixomas are non-hereditary type. Mutations in the exon 3 of the CTNNB1 gene, located on chromosome 3p22-p21.3, which encodes the beta-catenin protein implicate beta-catenin as the major cause of hair-matrix cell tumorigenesis. Multiple pilomatrixomas are familial type, associated with Steinert's myotonic dystrophy, Gardner Syndrome, Turner Syndrome, Trisomy 9, Rubinstein-Taybi syndrome, Xeroderma-pigmentosum, etc.,
Our patient was a 35-year-old Asian male, with no familial history or syndromic association, and the mass was located in the eyelid. Hence, portraying is a rare presentation of pilomatrixoma.
Clinically, the lesion appears as a slowly enlarging, irregularly shaped, hard, nodular, and nontender mass freely movable over the subcutaneous tissue, but not under the skin. The skin usually has reddishblue discoloration due to dilated blood vessels and chalky white nodules may be seen through the skin. Usually, there is no history of inflammation or trauma. The distribution of pilomatrixoma depends on the density of pilosebaceous glands in a given area.
Pilomatrix carcinomas can occur but are uncommon, they are locally invasive and can cause visceral metastases and death. It occurs more often in middle age or older individuals, more commonly in men than in women. Microscopically, it is characterized by exuberant proliferation of basaloid cell masses with varying degrees of cytological atypia, frequent mitoses, and areas of necrosis. The keratinization with shadow cell formation is less extensive than in benign pilomatrixoma. The infiltrative growth pattern which extends into deeper soft tissues may display vascular and perineural invasion. No such features suggestive of carcinomatous changes were noted clinically or histopathologically in our patient [Figure 3].
|Figure 3: Photomicrographs at ×40 (clockwise from top left): Abruptly keratinized ghost cells with mild calcification; overlying epithelium with increased subepithelial vascularity; Solid nests of basaloid cells undergoing abrupt trichilemmal type keratinization; calcification seen in ghost cells|
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Differential diagnoses of pilomatrixoma include epidermal inclusion cyst, sebaceous adenoma, dermoid cyst, epidermoid cyst, and vascular tumors such as capillary hemangioma. These tumors have similar clinical appearance, making histopathological analysis mandatory.
| Conclusion|| |
Pilomatrixoma is a benign condition often masquerading as chalazion. A 35-year-old man presenting with gradually enlarging, hard, painless swelling on the left upper eyelid underwent excision biopsy which later-on confirmed as pilomatrixoma. The mainstay of treatment involves complete surgical excision.
The authors would like to acknowledge the guidance of Dr. V C Bhatnagar, Head of Department and Medical Superintendent, Global Hospital Institute of Ophthalmology.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]