History

The clinical history may be nonspecific because this is usually a painless growth. Patients may report a change in nail color or nail thickening. Prior history of onychomycosis may be important; however, this is not yet well established.

Other important information to inquire about in the history is previous trauma to the region and a family or personal history of cancer.

Physical Examination

Occurrence is predominantly (63%) on the fingers.^[10]The percentage of lesions on the first, second, third, and fifth fingers is 37%, 15%, 37%, and 11%, respectively.^[9]Multiple-digit occurrences in a single patient has been reported.

The following clinical tetrad has been reported: (1) xanthonychia, (2) longitudinal ridging, (3) splinter hemorrhage, and (4) notable overcurvature. Erythema and swelling of the proximal nail fold, tenderness with nail compression, and longitudinal melanonychia have also been seen.^[10]Clinical presentation can be nonspecific and include pterygium.^[6]See the image below.

Courtesy of Dr. Militello.

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The ventral nail may have woodwormlike holes. See the image below.

Note the woodworm-like features. Courtesy of Dr. Militello.

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Nail avulsion exposes the filiform tumor arising from the matrix. Upon surgical removal or nail avulsion, the tumor has been described to have the macroscopic appearance of an "anemone."^[12]This is due to the nail matrix having developed multiple filamentlike digitations that extend up and ultimately insert and protrude into the bottom of the proximal nail. As a result, the bottom of the proximal nail has multiple small holes that are visible and correspond to the matrix projections.

Differential Diagnoses

Baran R, Kint A. Onychomatrixoma. Filamentous tufted tumour in the matrix of a funnel-shaped nail: a new entity (report of three cases). Br J Dermatol. 1992 May. 126(5):510-5. [QxMD MEDLINE Link].
Haneke E, Franken J. Onychomatricoma. Dermatol Surg. 1995 Nov. 21(11):984-7. [QxMD MEDLINE Link].
Kint A, Baran R, Geerts ML. The onychomatricoma: an electron microscopic study. J Cutan Pathol. 1997 Mar. 24(3):183-8. [QxMD MEDLINE Link].
Perrin C, Langbein L, Schweizer J, Cannata GE, Balaguer T, Chignon-Sicart B, et al. Onychomatricoma in the light of the microanatomy of the normal nail unit. Am J Dermatopathol. 2011 Apr. 33(2):131-9. [QxMD MEDLINE Link].
Goettmann S, Zaraa I, Moulonguet I. Onychomatricoma with pterygium aspect: unusual clinical presentation. Acta Derm Venereol. 2006. 86(4):369-70. [QxMD MEDLINE Link].
Perrin C, Baran R. Onychomatricoma with dorsal pterygium: pathogenic mechanisms in 3 cases. J Am Acad Dermatol. 2008 Dec. 59(6):990-4. [QxMD MEDLINE Link].
Burchette JL, Pham TT, Higgins SP, Cook JL, Soler AP. Expression of cadherin/catenin cell--cell adhesion molecules in a onychomatricoma. Int J Surg Pathol. 2008 Jul. 16(3):349-53. [QxMD MEDLINE Link].
Cañueto J, Santos-Briz Á, García JL, Robledo C, Unamuno P. Onychomatricoma: genome-wide analyses of a rare nail matrix tumor. J Am Acad Dermatol. 2011 Mar. 64(3):573-8, 578.e1. [QxMD MEDLINE Link].
Di Chiacchio N, Tavares GT, Tosti A, Di Chiacchio NG, Di Santis E, Alvarenga L, et al. Onychomatricoma: epidemiological and clinical findings in a large series of 30 cases. Br J Dermatol. 2015 May 12. [QxMD MEDLINE Link].
Tosti A, Piraccini BM, Calderoni O, Fanti PA, Cameli N, Varotti E. Onychomatricoma: report of three cases, including the first recognized in a colored man. Eur J Dermatol. 2000 Dec. 10(8):604-6. [QxMD MEDLINE Link].
Rashid RM, Swan J. Onychomatricoma: benign sporadic nail lesion or much more?. Dermatol Online J. 2006. 12(6):4. [QxMD MEDLINE Link].
Piraccini BM, Antonucci A, Rech G, Starace M, Misciali C, Tosti A. Onychomatricoma: first description in a child. Pediatr Dermatol. 2007 Jan-Feb. 24(1):46-8. [QxMD MEDLINE Link].
Fraga GR, Patterson JW, McHargue CA. Onychomatricoma: report of a case and its comparison with fibrokeratoma of the nailbed. Am J Dermatopathol. 2001 Feb. 23(1):36-40. [QxMD MEDLINE Link].
DiMaio DJ, Cohen PR. Trichilemmal horn: case presentation and literature review. J Am Acad Dermatol. 1998 Aug. 39(2 Pt 2):368-71. [QxMD MEDLINE Link].
Yasuki Y. Acquired periungual fibrokeratoma--a proposal for classification of periungual fibrous lesions. J Dermatol. 1985 Aug. 12(4):349-56. [QxMD MEDLINE Link].
Baran R, Perrin C. Bowen's disease clinically simulating an onychomatricoma. J Am Acad Dermatol. 2002 Dec. 47(6):947-9. [QxMD MEDLINE Link].
Patel MR, Desai S. Subungual keratoacanthoma in the hand. J Hand Surg [Am]. 1989 Jan. 14(1):139-42. [QxMD MEDLINE Link].
Bokszczanin A, Levinson AI. Coexistent yellow nail syndrome and selective antibody deficiency. Ann Allergy Asthma Immunol. 2003 Nov. 91(5):496-500. [QxMD MEDLINE Link].
Jaeger TNG, Canella C, Leverone AP, Nakamura RC. Onychomatricoma with Onychomycosis: A Case Report and Review of the Literature. Skin Appendage Disord. 2021 Aug. 7 (5):422-426. [QxMD MEDLINE Link].
Wynes J, Wanat KA, Huen A, Mlodzienski AJ, Rubin AI. Pigmented Onychomatricoma: A Rare Pigmented Nail Unit Tumor Presenting as Longitudinal Melanonychia That Has Potential for Misdiagnosis as Melanoma. J Foot Ankle Surg. 2015 Jul-Aug. 54 (4):723-5. [QxMD MEDLINE Link].
Spaccarelli N, Wanat KA, Miller CJ, Rubin AI. Hypopigmented onychocytic matricoma as a clinical mimic of onychomatricoma: clinical, intraoperative and histopathologic correlations. J Cutan Pathol. 2013 Jun. 40(6):591-4. [QxMD MEDLINE Link].
Kallis P, Tosti A. Onychomycosis and Onychomatricoma. Skin Appendage Disord. 2016 May. 1 (4):209-12. [QxMD MEDLINE Link].
Lesort C, Debarbieux S, Duru G, Dalle S, Poulhalon N, Thomas L. Dermoscopic Features of Onychomatricoma: A Study of 34 Cases. Dermatology. 2015. 231 (2):177-83. [QxMD MEDLINE Link].
Soto R, Wortsman X, Corredoira Y. Onychomatricoma: clinical and sonographic findings. Arch Dermatol. 2009 Dec. 145(12):1461-2. [QxMD MEDLINE Link].
Peruilh-Bagolini L, Dossi MT, Wortsman X, Montero T. Pigmented onychomatricoma: A clinical simulator that could not mislead ultrasound. Acta Biomed. 2021 Apr 30. 92 (S1):e2021158. [QxMD MEDLINE Link].
Sanchez M, Hu S, Miteva M, Tosti A. Onychomatricoma has channel-like structures on in vivo reflectance confocal microscopy. J Eur Acad Dermatol Venereol. 2014 Nov. 28 (11):1560-2. [QxMD MEDLINE Link].
Graves MS, Anderson JK, LeBlanc KG Jr, Sheehan DJ. Utilization of Mohs micrographic surgery in a patient with onychomatricoma. Dermatol Surg. 2015 Jun. 41 (6):753-5. [QxMD MEDLINE Link].
Perrin C, Baran R, Pisani A, et al. The onychomatricoma: additional histologic criteria and immunohistochemical study. Am J Dermatopathol. 2002 Jun. 24(3):199-203. [QxMD MEDLINE Link].
Miteva M, Cadore de Farias D, Zaiac M, Romanelli P, Tosti A. Nail clipping diagnosis of onychomatricoma. Arch Dermatol. 2011 Sep. 147(9):1117-8. [QxMD MEDLINE Link].

Media Gallery

Courtesy of Dr. Militello.
Note the woodworm-like features. Courtesy of Dr. Militello.

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Author

Annie Wester, MD, MS Resident Physician, Department of Dermatology, Vanderbilt University Medical Center

Disclosure: Nothing to disclose.

Coauthor(s)

James W Swan, MD Associate Professor of Medicine, Division of Dermatology, Loyola University Stritch School of Medicine; Attending Physician, Loyola University Medical Center; Attending Physician, Section of Dermatology, Hines Veterans Affairs Medical Center

James W Swan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Contact Dermatitis Society, Chicago Dermatological Society

Disclosure: Nothing to disclose.

Rashid M Rashid, MD, PhD Director, Mosaic Clinic Hair Transplant Center of Houston

Rashid M Rashid, MD, PhD is a member of the following medical societies: American Academy of Dermatology, Council for Nail Disorders, Houston Dermatological Society, International Society of Hair Restoration Surgery, Texas Dermatological Society, Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Military Dermatologists, Phi Beta Kappa, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Received income in an amount equal to or greater than $250 from: Elsevier; WebMD<br/>Served as a speaker for various universities, dermatology societies, and dermatology departments.

Additional Contributors

Richard K Scher, MD Adjunct Professor of Dermatology, University of North Carolina at Chapel Hill School of Medicine; Professor Emeritus of Dermatology, Columbia University College of Physicians and Surgeons

Richard K Scher, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American Medical Association, Association of Military Surgeons of the US, International Society for Dermatologic Surgery, Noah Worcester Dermatological Society, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Onychomatricoma Clinical Presentation

History

Physical Examination

References

Tables

Contributor Information and Disclosures

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