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Pilomatrixoma Clinical Presentation

  • Author: Jaggi Rao, MD, FRCPC; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Mar 20, 2015
 

History

Patients usually present with a solitary nodule that has been slowly growing over several months or years. Patients are usually asymptomatic, but some report pain during episodes of inflammation or ulceration. Rapid growth is rare, but reports indicate one lesion reaching 35 mm in 8 months and another reaching 1 cm in 2 weeks. Occurrence in more than one member of the same family is rare and is usually observed in association with myotonic dystrophy.

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Physical

Approximately 50% of the lesions occur on the head and neck, especially the cheek, preauricular area, eyelids, forehead, scalp, and lateral and posterior neck.[45, 46, 47] Lesions can also occur on the upper and lower extremities and trunk.[48] One lesion was observed in the middle ear and another in the ovary.[49, 50]

Most lesions measure 0.5-3 cm, but, rarely, giant lesions up to 15 cm are reported. Patients usually have a single, firm, stony, hard nodule. Lesions are usually the color of the normal skin, but reddish-purple lesions have been observed (probably resulting from hemorrhage). Stretching of the overlying skin can give the lesion a multifaceted, angulated appearance known as the "tent sign," likely due to calcification in the lesion.

One lesion showed the "dimple sign," which is often associated with dermatofibromas. Unusual morphological variants include perforating, cystic, bullous, lymphangiectatic, hornlike, keratoacanthomalike, pigmented, and lesions that show anetodermalike changes on the surface.[51, 52, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67]

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Causes

Investigators in one study showed that at least 75% of the lesions studied had mutations in the gene CTNNB1; these data directly implicate beta-catenin/LEF misregulation as the major cause of hair matrix cell tumorigenesis in humans.

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Contributor Information and Disclosures
Author

Jaggi Rao, MD, FRCPC Clinical Professor of Medicine, Division of Dermatology and Cutaneous Sciences, Director of Dermatology Residency Program, University of Alberta Faculty of Medicine and Dentistry

Jaggi Rao, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for Laser Medicine and Surgery, Canadian Medical Association, Pacific Dermatologic Association, Royal College of Physicians and Surgeons of Canada, Canadian Medical Protective Association, Canadian Dermatology Association

Disclosure: Nothing to disclose.

Coauthor(s)

Andrew Lin, MD, FRCPC Associate Professor, Department of Internal Medicine, Division of Dermatology, University of Alberta

Andrew Lin, MD, FRCPC is a member of the following medical societies: American Academy of Dermatology, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

Smeena Khan, MD Private Practice, Adult and Pediatric Dermatology Associates

Smeena Khan, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Andrei I Metelitsa, MD Chief Resident, Division of Dermatology and Cutaneous Sciences, University of Alberta, Canada

Disclosure: Nothing to disclose.

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Pilomatricoma with prominent basaloid cells.
Ghost cells (shadow cells) and basaloid cells, associated with a granulomatous reaction. Shadow cells are also seen in this high-power micrograph.
 
 
 
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