Pilomatrixomas are rare, slow-growing tumors that derive from hair follicle cells, which are ectodermal in origin. They develop in the lower dermis, present as irregularly shaped, firm growths that are tethered to the overlying skin but are not, however, attached to the underlying tissue. They often cause a blue or red discoloration on the skin but are otherwise non-tender [1]. Moreover, pilomatrixomas have the ability to calcify or ossify.

They most frequently occur within the first decade of life and affect females more than males [2]. Furthermore, they are usually found in the head and neck region, but have also been reported in the upper extremities. Typically, pilomatrixomas are solitary, yet multiple growths can occur simultaneously. The occurrence of the latter may be linked to various conditions, exemplified by sarcoidosis, Gardner syndrome, and myotonic dystrophy [3].

An even rarer type of tumor is the malignant form of pilomatrixomas, that is, pilomatrix carcinoma. These may present in a similar manner to pilomatrixomas, but are distinguished by their rapid growth, and involvement of the hematologic and lymphatic systems [4]. They have a tendency to recur and sometimes metastasize.

The diagnosis of pilomatrixoma is often missed, with the correct identification of the mass being less than 30% pre-operatively [5]. The reason for the high diagnostic inaccuracy may be a combination of the rarity of the tumor, its similarity to other superficial masses such as dermoid cysts, lipomas, foreign bodies and branchial cleft cysts, and atypical features such as an uncommon location [6].

• Asymptomatic

  Sir, Pilomatrixomas are benign neoplasms of the ectodermal hair follicles which typically appear as solitary, firm, asymptomatic nodules1. When multiple, they may be associated with other diseases. [imj.ie]

  CASE REPORT A 9-year-old boy first visited the dermatology clinic due to an asymptomatic nodular mass on the left cheek, which had been presented for 2 months (Fig. 1). [opendermatologyjournal.com]

  The patient presented at 8 months of age with an 1.2-cm, red, asymptomatic lesion of the right ear. The mass was noted at 4 months of age and subsequently enlarged. [ncbi.nlm.nih.gov]

• Swelling

  Furthermore, FNA smears from a swelling located in head and neck region (comparable to our case with right posterior triangle neck swelling). [cytojournal.com]

  There was no associated pain or history of trauma prior to the appearance of the swelling. Local examination showed a firm subcutaneous swelling, 0.8 × 0.6 cm in size on the lateral aspect of the left arm. [hindawi.com]

  An excisional biopsy of the swelling was performed, and the histologic diagnosis of pilomatrixoma was made. [ncbi.nlm.nih.gov]

• Inflammation

  Overlying skin is usually normal without blood vessels, inflammation, or ulceration. [eyewiki.aao.org]

  The lesion was associated with pain and inflammation in 7 cases (18%) and abscess or ulceration in 4 cases (11%). Twenty-nine patients presented with single nodules and 4 presented with multiple occurrences. [ghorayeb.com]

  The overlying skin is usually normal, with no dilated blood vessels, inflammation, or ulceration. [sciencerepository.org]

  Pathologic diagnosis of pilomatrixoma is based on the finding of large masses of shadow cells, combined with basophilic cells, inflammation, foreign body giant cells, calcification, and ossification. [ncbi.nlm.nih.gov]

• Surgical Procedure

  One year after surgical procedure the patient observed the appearance of new nodules on both arms. In the therapy surgical excision was performed with histopathological examination of the tissues. [ncbi.nlm.nih.gov]

  A surgical procedure was performed under general anesthesia. The mass was exposed and removed along with surrounding tissue by extracapsular enucleation. [ijp.iranpath.org]

  Diagnostic Procedures Pathological evaluation of the surgical specimen is necessary to make the definitive diagnosis, although clinical findings are usually typical of pilomatrixoma. [sciencerepository.org]

  Pilomatrixoma removal is a fairly simple surgical procedure. It involves taking out all of the tissue in the lump and some of the surrounding tissues. The exact amount of tissue removed will vary from person to person. [webmd.com]

  The records of these patients were reviewed to determine sex, age at initial presentation, age at the time of surgical management, location and size of the tumor, clinical presentation, highlights of the surgical procedure, histological findings, and [jamanetwork.com]

• Nausea

  Symptoms include weak muscles, numbness, trouble walking, nausea, weight loss, irritability, fatigue, and increased heart rate. Treatment may include vitamin B12 supplements. It is also important to eat a well-balanced diet. [hopkinsmedicine.org]

  They can include: Diarrhea or constipation Nausea Vomiting Fatigue, lack of energy, or lightheadedness when standing up or with exertion Loss of appetite Pale skin (mild jaundice) Shortness of breath, mostly during exercise Heartburn Swollen, red tongue [medlineplus.gov]

• Vomiting

  They can include: Diarrhea or constipation Nausea Vomiting Fatigue, lack of energy, or lightheadedness when standing up or with exertion Loss of appetite Pale skin (mild jaundice) Shortness of breath, mostly during exercise Heartburn Swollen, red tongue [medlineplus.gov]

  Symptoms may include vomiting, diarrhea, fatigue, headache, inability to sleep (insomnia), lack of appetite, failure to thrive, a yellow coloration of the skin (jaundice), irritability, and/or a pale complexion. [rarediseases.org]

• Ulcer

  The present case is reported to sensitize the ophthalmologists to think beyond chalazion and meibomian gland carcinoma for a firm, nodular, non ulcerated, painless lid swelling. [ncbi.nlm.nih.gov]

  The lesion was ulcerated. We did a wide local excision for the lesion and the patient is following up with us regularly. [ispub.com]

  The lesion was associated with pain and inflammation in 7 cases (18%) and abscess or ulceration in 4 cases (11%). Twenty-nine patients presented with single nodules and 4 presented with multiple occurrences. [ghorayeb.com]

  Our patient presented with an ulcerated nodule in the left lower leg. Clinical diagnosis of squamous cell carcinoma was given. [ijtrichology.com]

• Subcutaneous Nodule

  Pilomatrixoma (pimatricoma) is a firm subcutaneous nodule often found on the head and neck of children. A pilomatrixoma is small, firm subcutaneous mass found under the skin. [eyewiki.aao.org]

  A five-year-old girl underwent fine needle aspiration (FNA) of a firm subcutaneous nodule on the lateral aspect of left arm. FNA smears showed scattered and few fragments of round to oval cells along with multinucleated giant cells. [hindawi.com]

  Search for more papers by this author First published: December 1989 Cited by: 4 Abstract A 26‐year‐old man with Gardner's syndrome had numerous subcutaneous nodules over almost his entire body. [doi.org]

  We report an 18-year-old girl presented with tender, subcutaneous nodule with overlying skin showing atrophy and hypopigmentation. Clinically, it was diagnosed as neurofibroma and sent for FNAC. [e-ijd.org]

• Skin Disease

  Diseases and STIs Institute of Dermatology Chinese Academy of Medical Science and Peking Union Medical College China Fang Fang Jiangsu Key Laboratory of Molecular Biology for Skin Diseases and STIs Institute of Dermatology Chinese Academy of Medical [alliedacademies.org]

  Non-malignant skin disease. References ↑ Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 387. [librepathology.org]

  Books about skin diseases See the DermNet NZ bookstore. [dermnetnz.org]

• Alopecia

  + Alopecia areata + Diffuse alopecia + Defluvium + Pseudopelade of Brocq + Alopecia in lupus erythematodes + Follicular mucinosis (alopecia mucinosa) + Primary follicular mucinosis + Secondary follicular mucinosis in mycosis fungoides + Alopecia androgenica [atlases.muni.cz]

  There are two reported cases of pilomatrixoma associated with alopecia, with one presenting with an area of hair loss surrounding a scalp lesion. The pilomatrixoma was on the left forehead in a 5-year-old Japanese girl in the second case. [statpearls.com]

  MalaCards based summary: Pilomatrixoma, also known as pilomatricoma, is related to epidermoid cysts and alopecia. [malacards.org]

  For example, pernicious anemia may occur together with autoimmune thyroid disease, type 1A diabetes mellitus, alopecia, vitiligo, and chronic atrophic gastritis in type III polyglandular autoimmune (PGA) syndrome—one of a rare group of disorders also [emedicine.medscape.com]

• Urticaria

  Conclusion: In descending order, the most frequent drug eruptions were exanthematous eruptions, acneiform eruptions, and urticaria, and the most common causative drugs were antibiotics, anticancer agents, and contrast media. [kiss.kstudy.com]

  + Progressive osseous heteroplasia + Gout, arthritis uratica + Hemosiderin + Argyrosis + Titanium pigmentation + Deposits of corticosteroids in the dermis + Ochronosis + Colloid milium + Subcutaneous emphysema + Tumorous infiltration of the corium + Urticaria [atlases.muni.cz]

• Irritability

  Symptoms include weak muscles, numbness, trouble walking, nausea, weight loss, irritability, fatigue, and increased heart rate. Treatment may include vitamin B12 supplements. It is also important to eat a well-balanced diet. [hopkinsmedicine.org]

  Another hypothesis proposes that the blister-like formation is caused by mechanical irritation. It is widely accepted that blister is caused by the lymphatic vessel occlusion. [alliedacademies.org]

  Sensory Impairment Loss of smell, taste, touch Irritability/Frustration/Impatience A general feeling of unease. Some patients will try to deal with this by seeking isolation for peace and quiet away from noises, crowds and bright lights. [pernicious-anaemia-society.org]

  Symptoms can include: Confusion Short-term memory loss Depression Loss of balance Numbness and tingling in the hands and feet Problems concentrating Irritability Hallucinations Delusions Optic nerve atrophy The goal of treatment is to increase your vitamin [medlineplus.gov]

  Symptoms may include vomiting, diarrhea, fatigue, headache, inability to sleep (insomnia), lack of appetite, failure to thrive, a yellow coloration of the skin (jaundice), irritability, and/or a pale complexion. [rarediseases.org]

• Onset in Adolescence

  ORPHA:91414 Classification level: Disorder Synonym(s): Epithelioma calcificans of Malherbe Pilomatricoma Prevalence: Unknown Inheritance: Not applicable Age of onset: Childhood, Adolescent, Adult ICD-10: D23.3 D23.4 D23.6 OMIM: 132600 UMLS: C0206711 MeSH [orpha.net]

The diagnosis of pilomatrixomas can be made based on clinical findings alone. The tent sign can be elicited and allows visualization of the irregularity of the mass. There is no development of lymphadenopathy. The modal location of tumors is the periorbital area, and a minority of cases are preceded by a history of trauma.

Histologically, pilomatrixomas are well circumscribed and surrounded by connective tissue. They consist of groups of metrical cells with cystic changes and central degeneration caused by ghost cells which lack a nucleus and are not exclusively found in pilomatrixomas [7]. Inflammation may be present in the surrounding tissue. Calcification occurs in the majority of tumors, whilst other changes that can occur include a local build-up of keratin, and foreign body granulomas [6].

Imaging studies are not routinely required. If X-rays are carried out, there may be visible calcifications. Pilomatrixomas are depicted on computerized tomography (CT) scans as soft tissue masses that may also contain calcifications. Magnetic resonance imaging (MRI) reveals ring-enhancing lesions, while ultrasonography may reveal peripherally hyperechoic masses with acoustic shadowing visible in the subcutaneous tissue [8].

Fine needle aspiration cytology (FNAC) remains the most accurate diagnostic test, although it also has a high margin of error [7]. On FNAC, basaloid and ghost cells are indicative of pilomatrixomas [9].

Surgical resection with wide margins was the optimal treatment for pilomatrixoma because local recurrence may occur. ETHICS APPROVAL AND CONSENT TO PARTICIPATE Not applicable. [opendermatologyjournal.com]

The treatment of choice is surgical excision, and the recurrence rate is low. [ncbi.nlm.nih.gov]

Medical therapy No medical treatment Surgery Surgery is the treatment for complete removal of the lesion. This is usually curative and also allows for pathologic diagnosis of the mass. [eyewiki.aao.org]

Prognosis Prognosis after surgical removal is excellent, but reports have shown a very low recurrence rate in some cases. Patients and their family should be aware that any new lesions should be evaluated appropriately. [eyewiki.aao.org]

diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE. [dovemed.com]

[…] inhomogeneous with multiple areas of intermediate signal or homogeneous with either intermediate signal intensity or high signal radiating from the center T1 C+ (Gd): variable post contrast T1 appearance, where some only enhance peripherally Treatment and prognosis [radiopaedia.org]

The prognosis is typically good and the treatment of choice is surgical removal. Recurrence is rare. An 11-year-old was referred to the Orthopaedic clinic following a fall. [imj.ie]

Pilomatrixoma is a rare skin neoplasm which is usually localized on face, neck and lower extremites and its etiology is still unknown. We report a case of a 32-year-old male patient with a rare localisation of pilomatrixoma on the right shoulder. [ncbi.nlm.nih.gov]

Pilomatrical carcinoma Matrical carcinoma Malignant pilomatrixoma Pilomatrix carcinoma Definitions Pilomatrixoma Benign dermal &/or subcutaneous tumor Derived from matrical portion of hair follicle Pilomatrical carcinoma Malignant counterpart of pilomatrixoma ETIOLOGY [basicmedicalkey.com]

Etiology Also known as benign calcifying epithelioma of Malherbe, this is a rare benign tumor that originates from the hair root matrix. It was first described by Malherbe and Chenantais in 1880. [eyewiki.aao.org]

The findings of the current case series, which is one of the largest published thus far in the ophthalmic literature, are in concordance with the other series of pilomatrixoma, confirming the epidemiologic, clinical, and histopathological features of [ncbi.nlm.nih.gov]

[…] pilomatrical carcinomas CTNNB1 gene encodes β-catenin Genetics Rare cases are associated with myotonic dystrophy and Rubinstein-Taybi syndrome Usually multiple pilomatrixomas present Cysts of Gardner syndrome can show pilomatricoma-like changes CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Painted sherbet 1880 You might also be interested in Dermatology Occurrence/Epidemiology This section has been translated automatically. [altmeyers.org]

In this study, we discuss the epidemiology of the tumor, clinical and histopathological findings, differential diagnosis and treatment outcome of pilomatrixoma in the light of our experiences with 20 cases. [go.gale.com]

Epidemiology Pilomatrikoma meets more often at children's and youthful age. Family cases are occasionally observed. [infomeds.net]

We demonstrate associations that are consistent with the proposed pathophysiology of pilomatrixoma. Surgical excision is curative. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved. [ncbi.nlm.nih.gov]

Pathophysiology The tumor arises from hair matrix cells. The cause has been suggested as the result of a mutation in the beta-catenin gene. [accessanesthesiology.mhmedical.com]

Pathophysiology Histologically, the cells in pilomatricomas resemble hair follicle matrix cells. [dermatologyadvisor.com]

Pilomatrixoma Pathophysiology One study which included individuals suffering from pilomatrixoma confirmed that the tumor is strongly related to very positive BCL2. [ic.steadyhealth.com]

Primary prevention There is no primary prevention. Diagnosis is often difficult to make clinically, and therefore can only be definitively established after histopathological examination of the lesion after surgical removal. [eyewiki.aao.org]

Prevention & Risk Assessment Prevention & Risk Assessment What causes pilomatrixoma? Pilomatrixomas develop from an abnormal formation of cells that are similar to hair cells, which become hardened or calcified. [childrensnational.org]

Oral prednisolone was administered and slowly tapered to manage the possible remnant lesion and to prevent disease recurrence. Two years after initiating therapy, there was no evidence of relapse. [ncbi.nlm.nih.gov]

1. Roche NA, Monstrey SJ, Matton GE. Pilomatricoma in children: Common but often misdiagnosed. Acta Chir Belg. 2010;110(2):250-254.
2. Pirouzmanesh A, Reinisch JF, Gonzalez-Gomez I, Smith EM, Meara JG. Pilomatrixoma: A review of 346 cases. Plast Reconstr Surg. 2003;112(7):1784-1789.
3. Urvoy M, Legall F, Toulemont PJ, Chevrant-Breton J. Multiple pilomatricoma. Apropos of a case. J Fr Ophtalmol. 1996;19(6-7):464-466.
4. Dutta R, Boadle R, Ng T. Pilomatrix carcinoma: case report and review of the literature. Pathology. 2001;33(2):248-251.
5. Julian CG, Bowers PW. A clinical review of 209 pilomatricomas. J Am Acad Dermatol. 1998;39(2 Pt 1):191-195.
6. Yencha MW. Head and neck pilomatricoma in the pediatric age group: a retrospective study and literature review. Int J Pediatr Otorhinolaryngol. 2001;57(2):123-128.
7. Wang J, Cobb CJ, Martin SE, Venegas R, Wu N, Greaves TS. Pilomatrixoma: clinicopathologic study of 51 cases with emphasis on cytologic features. Diagn Cytopathol. 2002;27(3):167-172.
8. Hwang JY, Lee SW, Lee SM. The common ultrasonographic features of pilomatricoma. J Ultrasound Med. 2005;24(10):1397–1402.
9. Agarwal RP, Handler SD, Matthews MR, Carpentieri D. Pilomatrixoma of the head and neck in children. Otolaryngol Head Neck Surg. 2001;125(5):510-515.