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Year : 2011  |  Volume : 2  |  Issue : 2  |  Page : 75-77  

Pilomatricoma: Forget me not

1 Department of Otolaryngorhinology, MM Institute of Medical Sciences and Research, Mullana, Ambala, India
2 Department of Medicine, MM Institute of Medical Sciences and Research, Mullana, Ambala, India
3 Department of Dermatology, MM Institute of Medical Sciences and Research, Mullana, Ambala, India
4 Department of Pathology, MM Institute of Medical Sciences and Research, Mullana, Ambala, India

Date of Web Publication14-Oct-2011

Correspondence Address:
Sanjeev Gupta
H.No. B-2, MM Medical College Residential Campus, Mullana, Ambala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.85995

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Pilomatricoma is a benign skin neoplasia, which is not commonly encountered in general practice. The diagnosis is often made only after histopathology. The present case report is of a 30-year-old woman who presented with swelling in neck, which was diagnosed as pilomatricoma only after excision. The idea of reporting this case is that pilomatricoma is not rare in occurrence but rarely diagnosed because of lack of confirmation of excised swelling and it is imperative that it should be kept in the differential diagnoses of all superficial skin tumors by dermatologists and surgeons.

Keywords: Benign tumor, neck, pilomarixoma, pilomatricoma

How to cite this article:
Garg LN, Arora S, Gupta S, Gupta S, Singh P. Pilomatricoma: Forget me not. Indian Dermatol Online J 2011;2:75-7

How to cite this URL:
Garg LN, Arora S, Gupta S, Gupta S, Singh P. Pilomatricoma: Forget me not. Indian Dermatol Online J [serial online] 2011 [cited 2020 Jul 6];2:75-7. Available from: http://www.idoj.in/text.asp?2011/2/2/75/85995

   Introduction Top

Pilomatricoma, or pilomatrixoma, or calcifying epithelioma of Malherbe is a benign skin neoplasia originating from hair follicle matrix cells. [1] It usually develops slowly and is known 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] We report here a case of pilomatricoma diagnosed after surgical excision with the help of histopathology.

   Case Report Top

A 30-year-old female presented to skin department with complaint of swelling over the middle of neck on right lateral side since 1 year. The onset was gradual and initially the size was very small (pea sized) but slowly over last 1 year it had grown to the size of an almond. It was painless to begin with but there was an occasional dull ache in the swelling. There was no associated fever or malaise. The patient denied any history of trauma prior to onset. Family history and past history of patient was unremarkable.

On examination there was swelling in the right side of neck of 2.5 × 2.0 cm size [Figure 1]. It was smooth on palpation, moderately firm to hard in consistency and minimally tender to touch. It could be moved laterally with a little restriction in caudocephalic movement. It appeared to be fixed with the skin with no attachment to the underlying tissues. There was no rise of local temperature, scar, sinus, or any other remarkable feature. General physical and systemic examination was found normal.
Figure 1: A subcutaneous swelling on right middle of neck

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A provisional clinical diagnosis of sebaceous cyst was made. Surgical excision was planned after blood investigations. The swelling was excised under local anesthesia and sent for histopathological examination. On gross examination the excised mass appeared to be calcified during excision. The histopathology features were suggestive of pilomatricoma [Figure 2] and [Figure 3].
Figure 2: Pilomatrixoma. Photomicrograph showing basaloid cell at the periphery and "ghost cells" on the right side of the field (H and E, ×100)

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Figure 3: Pilomatrixoma. Higher magnification showing abrupt keratinization with "ghost cells" (H and E, ×400)

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   Discussion Top

Pilomatricoma is a 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 to 2.5:1) among young people, 40% happen before 10 years of age and 60% before 20 years. [1],[5] New hair follicles are not formed after birth, only some are activated during puberty. If they are located in very deep layers, differentiation induction agents will not act properly on them. These partially differentiated follicles would form the pilomatricomas. [1] These tumors can be familial related to Gardner's syndrome, Steinerd's disease, and Sarcoidosis. [6]

Pilomatricomas are often mistaken for "small round blue cell" tumors in children, or for Merkel cell carcinoma, basalioma, and metastatic small cell carcinoma in adults, with possible over aggressive therapeutic approach. [7] A recent study suggests that trisomy-18 is a consistent feature in pilomatricoma, suggesting that genes carried on this chromosome such as that for the antiapoptotic oncoprotein BCL2 may have a role in the growth and differentiation of this benign self-limited tumor. [8]

The clinical history of pilomatricama is typical of a slowly enlarging mass, irregularly contoured, which is fixed to the skin but slides freely over the underlying tissues, often with a discoloration, which varies from red to bluish-purple. [9] Pilomatrixomas are often misdiagnosed on preoperative evaluation. In a series of 51 histologically proven pilomatrixomas, Wells et al found that the diagnosis during reference was incorrect in 94% of cases, and the preoperative diagnosis was incorrect in 57%. [10] In a recent series of 346 pilomatrixomas, the preoperative diagnosis was accurate and consistent with the pathological diagnosis of pilomatrixoma in only 28.9% of cases. [5] Finally, Kumaran et al reported a correct preoperative clinical diagnosis in 46% following retrospective review of 78 excised pilomatrixomas. [11] Incorrect preoperative diagnoses most commonly included unidentified masses, as well as epidermoid cysts, sebaceous cysts, dermoid cysts, nonspecified cysts, and foreign bodies. [5],[11]

Histological characteristics include ghost cells in the center with basophilic nucleated cells in the periphery. Calcification is present in 70%-95%. [1],[12] , Clinically skin and adjacent tissue infiltration, ulceration, and histologically - presence of nuclear pleomorphism, atypical mitosis, central necrosis is suggestive of malignancy [13] Cytomorphologic characteristics of PM are reliable enough for correct preoperative diagnosis in adequate specimens. However, the best results are achieved when fine-needle aspiration cytology is performed by an experienced cytologist after obtaining all clinical data. [7]

A rare malignant counterpart, pilomatrix carcinoma, has been described and nearly 90 cases have been reported in the literature. It is locally aggressive and can recur. In several cases, metastases have been observed. Many key features are similar between these benign and malignant counterparts; the primary differentiating characteristics include a high mitotic rate with atypical mitoses, central necrosis, infiltration of the skin and soft tissue, and invasion of blood and lymphatic vessels. [14],[15]

Surgical excision with clear margins is the treatment of choice, otherwise there may be recurrence due to incomplete resection. [9]

   Conclusion Top

Pilomatricoma is a benign tumor of the skin, which is not rare in occurrence but rarely diagnosed preoperatively and it is imperative that it be kept in the differential diagnoses of all benign skin tumors by dermatologists.

   References Top

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.  Back to cited text no. 1
2.Moehlenbeck FW. Pilomatrixoma (calcifying epithelioma). A statistical study. Arch Dermatol 1973;108:132-4.  Back to cited text no. 2
3.Urvoy M, Legall F, Toulemont PJ, Chevrant-Breton J. [Multiple pilomatricoma. Apropos of a case]. J Fr Ophthalmol 1996;19:464-6.  Back to cited text no. 3
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.  Back to cited text no. 4
5.Pirouzmanesh A, Reinisch JF, Gonzalez-Gomez I, Smith EM, Meara JG. Pilomatrixoma: A review of 346 cases. Plast Reconstr Surg 2003;112:1784-9.  Back to cited text no. 5
6.Hubbard VG, Whittaker SJ. Multiple Familial Pilomatricomas. An Unusual Case. J Cutan Pathol 2004;31:281-3.  Back to cited text no. 6
7.Seili-Bekafigo I, Jonjiæ N, Stemberger C, Rajkoviæ-Molek K. Additional cytomorphological criteria in diagnosis of pilomatricoma-benign tumor with bad reputation. Coll Antropol 2010;34:117-22.   Back to cited text no. 7
8.Agoston AT, Liang CW, Richkind KE, Fletcher JA, Vargas SO. Trisomy 18 is a consistent cytogenetic feature in pilomatricoma. Mod Pathol 2010;23:1147-50.  Back to cited text no. 8
9.Roche NA, Monstrey SJ, Matton GE. Pilomatricoma in children: Common but often misdiagnosed. Acta Chir Belg 2010;110:250-4.  Back to cited text no. 9
10.Wells NJ, Blair GK, Magee JF, Whiteman DM. Pilomatrixoma: A common, benign childhood skin tumor. Can J Surg 1994;37:483-6.  Back to cited text no. 10
11.Kumaran N, Azmy A, Carachi R, Raine PA, Macfarlane JH, Howatson AG. Pilomatrixoma - accuracy of clinical diagnosis. J Pediatr Surg 2006;41:1755-8.  Back to cited text no. 11
12.Lan MY, Lan MC, Ho CY, Li WY, Lin CZ. Pilomatricoma of the head and neck: A retrospective review of 179 cases. Arch Otolaryngol Head Neck Surg 2003;129:1327-30.  Back to cited text no. 12
13.Phyu KK, Bradley PJ. Pilomatrixoma in the parotid region. J Laryngol Otol 2001;115:1026-8.  Back to cited text no. 13
14.Dutta R, Boadle R, Ng T. Pilomatrix carcinoma: Case report and review of the literature. Pathology 2001;33:248-51.  Back to cited text no. 14
15.Mikhaeel NG, Spittle MF. Malignant pilomatrixoma with multiple local recurrences and distant metastases: A case report and review of the literature. Clin Oncol (R Coll Radiol) 2001;13:386-9.  Back to cited text no. 15


  [Figure 1], [Figure 2], [Figure 3]


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