Onychomatricoma
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An onychomatricoma is a subungual tumor of the fingers and toes, described in 1992. [1]
The terminology describing onychomatricomas has slowly been adjusted over time. In 1992, the original description termed this tumor an onychomatrixoma, based on the description of a filamentous, tufted tumor in the matrix. Subsequently, the term onychomatricoma was introduced into the English literature in 1995. [2] The term onychomatricoma is most descriptive and breaks down as onycho (nail) matric (matrix) oma (tumor).
Currently, histologic criteria have been used to establish subclassifications of onychomatricomas as 1 of 3 types. The clinical relevance of these subclassifications has not yet been well established, but they are described in Histologic Findings.
The etiology of onychomatricomas is not yet known. Patients do not usually report any history of trauma.
One literature review has noted that onychomycosis may be found more commonly in association with toe onychomatricomas; almost half the reported cases have had a fungal association. However, the significance of onychomycosis in these patients is not yet well established and may or may not play a role in the pathophysiology of onychomatricomas.
Another theory espoused is that onychomatricomas are fibroproliferative in origin and are epithelial and connective-tissue hamartomas simulating the nail matrix. This theory is further supported by an electron microscopy study that associated onychomatricoma to a nail matrix origin. [3] On immunohistochemistry, onychomatricomas express the same pattern of cytokeratins and integrins as seen in normal nail matrix. Keratin expression patterns have also suggested differentiation toward the nail bed and the nail isthmus. [4]
One report has noted an onychomatricoma arising from the ventral part of the proximal nail fold, thus taking on a pterygium aspect. [5] A dorsal pterygium bridging the proximal nail fold may also be noted. In such cases, tumor is also produced by the ventral surface of the proximal nail fold (as opposed to most cases, in which only the matrix produces tumor). This odd variant produces 2 layers of plate sandwiched together, which makes diagnosis more difficult. [6]
Onychomatricomas were originally believed to be benign nail lesions. However, the literature suggests that malignant potential cannot be completely excluded.
In ultrastructure studies, E-cadherin and beta-catenin have been noted at the cell membrane in the epithelial invaginations, while P-cadherin is restricted to basal cells. In contrast to other matrical tumors, nuclear beta-catenin was not present, suggesting that onychomatricoma may lack the transcriptional activating role of beta-catenin noted in follicular and odontogenic matrical tumors. [7]
Genome studies have suggested chromosome 11 plays a role in this tumor. [8]
The frequency of onychomatricomas in the United States has not been determined. One epidemiological study reported no more than 80 known cases in the literature. [9] The frequency of onychomatricomas internationally has not been determined.
In the English literature, one case occurred in a black patient, [10] while all other reports have been in white persons. [11]
The most current literature suggests the male-to-female ratio for onychomatricomas is approximately 1:1.
The mean age of presentation is 47.9 years, as of the last tabulation. [9] One report now exists of a presentation in a child. [12]
Few long-term studies are available on onychomatricomas. In one case, surgical removal yielded no recurrence at 2-year follow-up. Similar results were also noted in a 2-year follow-up. Nail regrowth occurs without incident.
A continuum with potentially malignant onychomatricoma may exist. Awareness of the possibility is prudent. [11]
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. [Medline].
Haneke E, Franken J. Onychomatricoma. Dermatol Surg. 1995 Nov. 21(11):984-7. [Medline].
Kint A, Baran R, Geerts ML. The onychomatricoma: an electron microscopic study. J Cutan Pathol. 1997 Mar. 24(3):183-8. [Medline].
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. [Medline].
Goettmann S, Zaraa I, Moulonguet I. Onychomatricoma with pterygium aspect: unusual clinical presentation. Acta Derm Venereol. 2006. 86(4):369-70. [Medline].
Perrin C, Baran R. Onychomatricoma with dorsal pterygium: pathogenic mechanisms in 3 cases. J Am Acad Dermatol. 2008 Dec. 59(6):990-4. [Medline].
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. [Medline].
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. [Medline].
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. [Medline].
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. [Medline].
Rashid RM, Swan J. Onychomatricoma: benign sporadic nail lesion or much more?. Dermatol Online J. 2006. 12(6):4. [Medline].
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. [Medline].
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. [Medline].
DiMaio DJ, Cohen PR. Trichilemmal horn: case presentation and literature review. J Am Acad Dermatol. 1998 Aug. 39(2 Pt 2):368-71. [Medline].
Yasuki Y. Acquired periungual fibrokeratoma–a proposal for classification of periungual fibrous lesions. J Dermatol. 1985 Aug. 12(4):349-56. [Medline].
Baran R, Perrin C. Bowen’s disease clinically simulating an onychomatricoma. J Am Acad Dermatol. 2002 Dec. 47(6):947-9. [Medline].
Patel MR, Desai S. Subungual keratoacanthoma in the hand. J Hand Surg [Am]. 1989 Jan. 14(1):139-42. [Medline].
Bokszczanin A, Levinson AI. Coexistent yellow nail syndrome and selective antibody deficiency. Ann Allergy Asthma Immunol. 2003 Nov. 91(5):496-500. [Medline].
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. [Medline].
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. [Medline].
Kallis P, Tosti A. Onychomycosis and Onychomatricoma. Skin Appendage Disord. 2016 May. 1 (4):209-12. [Medline].
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. [Medline].
Soto R, Wortsman X, Corredoira Y. Onychomatricoma: clinical and sonographic findings. Arch Dermatol. 2009 Dec. 145(12):1461-2. [Medline].
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. [Medline].
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. [Medline].
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. [Medline].
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. [Medline].
Annie Wester, MD, MS Resident Physician, Department of Dermatology, Vanderbilt University Medical Center
Disclosure: Nothing to disclose.
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, Texas Medical Association, Texas Dermatological Society, International Society of Hair Restoration Surgery, Council for Nail Disorders, Houston Dermatological Society
Disclosure: Nothing to disclose.
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, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa
Disclosure: Nothing to disclose.
Jeffrey Meffert, MD † Former Associate Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio
Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, Texas Dermatological Society
Disclosure: Nothing to disclose.
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.
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
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