0
We're unable to sign you in at this time. Please try again in a few minutes.
Retry
We were able to sign you in, but your subscription(s) could not be found. Please try again in a few minutes.
Retry
There may be a problem with your account. Please contact the AMA Service Center to resolve this issue.
Contact the AMA Service Center:
Telephone: 1 (800) 262-2350 or 1 (312) 670-7827  *   Email: subscriptions@jamanetwork.com
Error Message ......
Special Feature |

Picture of the Month—Diagnosis FREE

[+] Author Affiliations

Section Editor: Samir S. Shah, MD, MSCE

More Author Information
Arch Pediatr Adolesc Med. 2009;163(10):956. doi:10.1001/archpediatrics.2009.163-b.
Text Size: A A A
Published online

DENOUEMENT AND DISCUSSION: EYELID PILOMATRICOMA

Both lesions underwent surgical excision (Figure 2), and histopathologic examination revealed well-demarcated tumors with distinct regions of darkly stained basophilic cells and pale-staining anuclear cells in both cases. There were also focal calcium deposits within the tumors.

Place holder to copy figure label and caption
Figure 2.

Patient 1 (A) and patient 2 (B) following oculoplastic excision and repair.

Graphic Jump Location

The images depict pilomatricomas involving the eyelid. Pilomatricomas, also referred to as pilomatrixomas and calcifying epitheliomas of Malherbe, represent benign tumors of hair follicle origin. They were first described in 1880 by Malherbe and Chenantais.1Although they can occur in any age group, they are most common in children and adolescents.2They typically appear as solitary, firm, painless dermal papules or nodules on the head and neck. Pilomatricomas may have a bluish coloration or may have central ulceration revealing underlying calcification (Figure 3). When palpated, they often exhibit the so-called teeter-totter sign, as pressure on one edge of the lesion causes elevation of the opposite side. When excised, these lesions are often larger than they appear on the surface.

Place holder to copy figure label and caption
Figure 3.

Calcified pilomatricoma.

Graphic Jump Location

Pilomatricomas uncommonly manifest on the eyelid areas, and when they do, they are often mistaken clinically for chalazions, granuloma annulare, epithelial cysts or dermoid cysts when they are bluish in color,3or keratoacanthomas or other malignant tumors if they have central ulceration.4

The appearance of multiple pilomatricomas has been occasionally associated with myotonic dystrophy,5Gardner syndrome,6Steiner disease,7Turner syndrome,8and sarcoidosis.9At least 75% of human pilomatricomas possess activating mutations in β-catenin, suggesting that β-catenin plays a role in the tumorigenesis of pilomatricomas.10β-Catenin is a member of the Wnt signaling pathway and plays an important role in the morphogenesis of hair follicles.11Although typically benign, malignant pilomatricomas have rarely been reported.12Treatment consists of surgical excision, and recurrence is rare after complete resection.

Return to Quiz Case.

Correspondence:Albert C. Yan, MD, Section of Dermatology, Children's Hospital of Philadelphia, 3550 Market St, 2nd Floor, Philadelphia, PA 19104 (yana@email.chop.edu).

Accepted for Publication:May 6, 2009.

Author Contributions:Study concept and design: Castelo-Soccio, J. A. Katowitz, and Yan. Acquisition of data: Castelo-Soccio, W. R. Katowitz, Shah, and Yan. Analysis and interpretation of data: Castelo-Soccio, W. R. Katowitz, Shah, Treat, and Yan. Drafting of the manuscript: Castelo-Soccio and Yan. Critical revision of the manuscript for important intellectual content: W. R. Katowitz, J. A. Katowitz, Shah, Treat, and Yan. Administrative, technical, and material support: Castelo-Soccio, W. R. Katowitz, J. A. Katowitz, Shah, Treat, and Yan. Study supervision: Yan.

Financial Disclosure:None reported.

Malherbe  AChenantais  J Note sur l’epithéliome calcifié des glandes sébacées. Prog Med (Paris) 1880;8826- 837
Yencha  MW Head and neck pilomatricoma in the pediatric age group. Int J Pediatr Otorhinolaryngol 2001;57 (2) 123- 128
PubMed Link to Article
Levy  JIlsar  MDeckel  YMaly  AAnteby  IPe’er  J Eyelid pilomatrixoma. Surv Ophthalmol 2008;53 (5) 526- 535
PubMed Link to Article
Lan  MYLan  MCHo  CYLi  WYLin  CZ Pilomatricoma of the head and neck. Arch Otolaryngol Head Neck Surg 2003;129 (12) 1327- 1330
PubMed Link to Article
Hino  SKondo  SSekiya  H  et al.  Molecular mechanisms responsible for aberrant splicing of SERCA1 in myotonic dystrophy type 1. Hum Mol Genet 2007;16 (23) 2834- 2843
PubMed Link to Article
Pujol  RMCasanova  JMEgido  RPujol  Jde Moragas  JM Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome? Pediatr Dermatol 1995;12 (4) 331- 335
PubMed Link to Article
Barberio  ENino  MDente  VDelfino  M Guess what! multiple pilomatricomas and Steiner disease. Eur J Dermatol 2002;12 (3) 293- 294
PubMed
Wood  SNguyen  DHutton  KDickson  W Pilomatricomas in Turner syndrome. Pediatr Dermatol 2008;25 (4) 449- 451
PubMed Link to Article
Julian  CGBowers  PW A clinical review of 209 pilomatricomas. J Am Acad Dermatol 1998;39 (2, pt 1) 191- 195
PubMed Link to Article
Park  SWSuh  KSWang  HYKim  STSung  HS Beta-catenin expression in the transitional zone of pilomatricoma. Br J Dermatol 2001;145 (4) 624- 629
PubMed Link to Article
Moreno-Bueno  GGamallo  CPérez-Gallego  LContrero  FPalacios  J Beta-catenin expression in pilomatrixomas. Br J Dermatol 2001;145 (4) 576- 581
PubMed Link to Article
Bassarova  ANesland  JMSedloev  TDanielsen  HChristova  S Pilomatrix carcinoma with lymph node metastases. J Cutan Pathol 2004;31 (4) 330- 335
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure 2.

Patient 1 (A) and patient 2 (B) following oculoplastic excision and repair.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 3.

Calcified pilomatricoma.

Graphic Jump Location

Tables

References

Malherbe  AChenantais  J Note sur l’epithéliome calcifié des glandes sébacées. Prog Med (Paris) 1880;8826- 837
Yencha  MW Head and neck pilomatricoma in the pediatric age group. Int J Pediatr Otorhinolaryngol 2001;57 (2) 123- 128
PubMed Link to Article
Levy  JIlsar  MDeckel  YMaly  AAnteby  IPe’er  J Eyelid pilomatrixoma. Surv Ophthalmol 2008;53 (5) 526- 535
PubMed Link to Article
Lan  MYLan  MCHo  CYLi  WYLin  CZ Pilomatricoma of the head and neck. Arch Otolaryngol Head Neck Surg 2003;129 (12) 1327- 1330
PubMed Link to Article
Hino  SKondo  SSekiya  H  et al.  Molecular mechanisms responsible for aberrant splicing of SERCA1 in myotonic dystrophy type 1. Hum Mol Genet 2007;16 (23) 2834- 2843
PubMed Link to Article
Pujol  RMCasanova  JMEgido  RPujol  Jde Moragas  JM Multiple familial pilomatricomas: a cutaneous marker for Gardner syndrome? Pediatr Dermatol 1995;12 (4) 331- 335
PubMed Link to Article
Barberio  ENino  MDente  VDelfino  M Guess what! multiple pilomatricomas and Steiner disease. Eur J Dermatol 2002;12 (3) 293- 294
PubMed
Wood  SNguyen  DHutton  KDickson  W Pilomatricomas in Turner syndrome. Pediatr Dermatol 2008;25 (4) 449- 451
PubMed Link to Article
Julian  CGBowers  PW A clinical review of 209 pilomatricomas. J Am Acad Dermatol 1998;39 (2, pt 1) 191- 195
PubMed Link to Article
Park  SWSuh  KSWang  HYKim  STSung  HS Beta-catenin expression in the transitional zone of pilomatricoma. Br J Dermatol 2001;145 (4) 624- 629
PubMed Link to Article
Moreno-Bueno  GGamallo  CPérez-Gallego  LContrero  FPalacios  J Beta-catenin expression in pilomatrixomas. Br J Dermatol 2001;145 (4) 576- 581
PubMed Link to Article
Bassarova  ANesland  JMSedloev  TDanielsen  HChristova  S Pilomatrix carcinoma with lymph node metastases. J Cutan Pathol 2004;31 (4) 330- 335
PubMed Link to Article

Correspondence

CME
Meets CME requirements for:
Browse CME for all U.S. States
Accreditation Information
The American Medical Association is accredited by the Accreditation Council for Continuing Medical Education to provide continuing medical education for physicians. The AMA designates this journal-based CME activity for a maximum of 1 AMA PRA Category 1 CreditTM per course. Physicians should claim only the credit commensurate with the extent of their participation in the activity. Physicians who complete the CME course and score at least 80% correct on the quiz are eligible for AMA PRA Category 1 CreditTM.
Note: You must get at least of the answers correct to pass this quiz.
You have not filled in all the answers to complete this quiz
The following questions were not answered:
Sorry, you have unsuccessfully completed this CME quiz with a score of
The following questions were not answered correctly:
Commitment to Change (optional):
Indicate what change(s) you will implement in your practice, if any, based on this CME course.
Your quiz results:
The filled radio buttons indicate your responses. The preferred responses are highlighted
For CME Course: A Proposed Model for Initial Assessment and Management of Acute Heart Failure Syndromes
Indicate what changes(s) you will implement in your practice, if any, based on this CME course.
Submit a Comment

Multimedia

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

See Also...
Articles Related By Topic
Related Collections
PubMed Articles