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CORRESPONDENCE
Year : 2023 | Volume
: 41
| Issue : 2 | Page : 129-130
Giant perforating pilomatricoma: A case report
Jun Young Kim1, Woo Young Nho2, Se Kook Kee3, Dong Hun Kim4, Kyung Tae Yoon5
1 Department of Dermatology, School of Medicine, Kyungpook National University, Daegu, South Korea
2 Department of Emergency Medicine, School of Medicine, Kyungpook National University, Daegu, South Korea
3 Department of General Surgery, CHA Gumi Medical Center, School of Medicine, CHA University, Gumi, South Korea
4 Department of Pathology, CHA Gumi Medical Center, School of Medicine, CHA University, Gumi, South Korea
5 Department of Plastic and Reconstructive Surgery, CHA Gumi Medical Center, School of Medicine, CHA University, Gumi, South Korea
Correspondence Address:
Prof. Woo Young Nho
Department of Emergency Medicine, School of Medicine, Kyungpook National University, 130 Dongdeok-Ro, Jung-Gu, Daegu 41944
South Korea
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/ds.DS-D-23-00001
Dear Editor,
Pilomatricoma is a benign skin neoplasm usually <3 cm of the size. Furthermore, perforated pilomatricoma (PP) with the huge mass was barely reported. We present the case of pilomatricoma occurs exceptionally with excessive clinical features resembling malignancy.
A 43-year-old Asian woman with a large mass on her back presented to the outpatient surgical clinic. In her early 20s, she noticed a small lesion that had grown gradually. However, she did not seek any medical care for the mass for more than 20 years due to her economic circumstances. A few months ago, the patient visited a dermatology clinic and was advised to be evaluated for potential skin malignancy. She did not have any diagnostic tests at the time. She recently decided on a palliative excision due to the smelly purulent discharge from the mass regardless of whether it was malignant or not. The patient has no relevant medical history and no relatives with skin neoplasms. Physical examination revealed a 15 cm by 15 cm by 7 cm sized fungating polypoid lesion with a purulent discharge from an ulcerated surface [Figure 1]a. The results of laboratory tests were within the reference range. A computed tomography (CT) scan revealed a heterogeneously enhancing mass with calcification in the cutaneous level of the back, raising the possibility of skin cancer, such as soft tissue sarcoma [Figure 1]b. Multiple enlarged lymph nodes in the left axilla and left back area were considered to be metastatic. A bicameral gallbladder with a gallstone was discovered by chance, and radiologists suspect focal chronic cholecystitis. The surgical team successfully removed the mass, which weighed 365 g, with a 30 mm tissue margin. Histopathological examination confirmed the diagnosis of pilomatricoma, and the possibility of PP was suggested with the features of transepidermal elimination [Figure 1]c and [Figure 1]d. The skin defect was covered by a split-thickness skin graft from the posterior thigh. Due to intermittent upper abdominal pain, the patient underwent laparoscopic cholecystectomy. The diagnosis of gallbladder adenomyomatosis was confirmed after the operation. At the 3-month follow-up, there was no evidence of recurrence or wound dehiscence.
Figure 1: (a) 15 cm by 15 cm by 7 cm sized fungating polypoid lesion with a purulent discharge from an ulcerated surface on the upper back of the patient (b) Computed tomography revealed a heterogeneously enhancing mass with calcification in the cutaneous level of the back (c) Histopathological examination showed the features of transepidermal elimination (H and E, ×200) (d) The proliferation of the basaloid cells phase into the ghost cells that are characterized by abundant eosinophilic cytoplasm and loss of nuclei after keratinization (H and E, ×200).Pilomatricoma is a benign skin neoplasm from hair follicle matrix cells. The calcifying epithelioma was first described in the 19th century by Malherbe and Chenantais, and the terms pilomatrixoma and pilomatricoma were coined in 1961 and 1977, respectively.[1] The disease occurred within the first 2 decades to 3 decades of life in more than half of patients, and female preponderance has been reported.[1] The tumor is most common in the head, neck, and upper extremities, and the size of the mass is <3 cm mostly. Clinically, the term giant pilomatricoma is defined, in case of the diameter exceeds 5 cm.[2] Most pilomatricoma presents as a single nodular lesion, but multiple lesions have been linked to Gardner syndrome, Turner syndrome, myotonic dystrophy, and Rubinstein–Taybi syndrome.[2]
PP is a rare variant of pilomatricoma, with a significant histological feature of transepithelial elimination.[1] The exact etiology of PP is unknown. There is no clear dominant age or sex, and multiple lesions are unusual in PP.[1] In addition, PP with tremendous scale, as in this case, is extremely rare. The diagnosis of pilomatricoma is challenging and approximately 20% have been diagnosed correctly.[3] The “tent sign” or “teeter-totter sign” are characteristic physical examinations in classic pilomatricoma with their mobile, nontender calcifying nature in usual size.[4] Differential diagnosis includes other pilosebaceous origin cysts and tumors such as epidermoid cyst, pigmented follicular cyst, and steatocystoma.[4] Furthermore, malignant neoplasms such as basal cell carcinoma, pilomatrix carcinoma should be considered in a patient with a larger size, with excessive clinical features, or in the elderly.[2],[5] Notably, simple benign skin tumor of pilomatricoma exceptionally present with excessive clinical characteristics resembling malignancy, such as in this case. Further radiologic evaluation, such as CT or positron emission tomography, is recommended in cases of potential malignancy.[6] Simple surgical excision offers a curative option in benign pilomatricoma.[3] Extended preoperative evaluations may be required in cases of suspected malignancy.
The presence of extra-intestinal adenomyomatosis with pilomatricoma in this case clinically suggests Gardner syndrome. A remarkable aspect of this case is the significant possibility of Gardner syndrome in solitary PP. We advised the patient to have colonoscopic examination with suspicion for particular genetic disease. However, the patient flatly refused due to fear of further serious disease and cost. Further, we tried to identify the family histories related to Gardner syndrome from the patient. Unfortunately, the patient did not recognize the exact medical conditions of her relatives, either.
Ethical approval
This study was approved by CHA Gumi Medical Center (GM21-19, 2021.10.30). The patient consent was waived by the IRB.
Data availability statement
The datasets generated during and/or analyzed during the current study are available from the corresponding author on reasonable request.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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