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Peer Reviewed

Photo Essay

An Atlas of Nail Disorders, Part 8

Volume 60 - Issue 6 - June 2020

AUTHORS:
Alexander K. C. Leung, MD1,2 • Benjamin Barankin, MD3 • Kin Fon Leong, MD4 • Joseph M. Lam, MD5

AFFILIATIONS:
1Department of Pediatrics, University of Calgary, Calgary, Alberta, Canada
2Alberta Children’s Hospital, Calgary, Alberta, Canada
3Toronto Dermatology Centre, Toronto, Ontario, Canada
4Pediatric Institute, Kuala Lumpur General Hospital, Kuala Lumpur, Malaysia
5Department of Pediatrics and Department of Dermatology and Skin Sciences, University of British Columbia, Vancouver, British Columbia, Canada

CITATION:
Leung AKC, Barankin B, Leong KF, Lam JM. An atlas of nail disorders, part 8. Consultant. 2020;60(6):e9. doi:10.25270/con.2020.06.00001

DISCLOSURES:
The authors report no relevant financial relationships.

CORRESPONDENCE:
Alexander K. C. Leung, MD, #200, 233 16th Ave NW, Calgary, AB T2M 0H5, Canada (aleung@ucalgary.ca)

EDITOR’S NOTE:
This article is part 8 of a 15-part series of Photo Essays describing and differentiating conditions affecting the nails. Parts 9 through 15 will be published in upcoming issues of Consultant. To access previously published articles in the series, visit the Consultant archive at www.Consultant360.com and click the “Journals” tab.

Glomus Tumor

A glomus tumor is a rare benign tumor that originates from a neuromyoarterial glomus body.1 Glomus bodies are located throughout the body but are concentrated in the fingertips, especially in the subungual region.1 These glomus bodies are a thermoregulatory apparatus of cutaneous microvasculature.2,3 The peak occurrence of glomus tumor is between 30 and 50 years of age.4-6 There is a female predominance.5-7

The classic triad of symptoms is intense paroxysmal pulsatile or shooting pain, pinpoint tenderness, and hypersensitivity to cold.4,5,8-10 Because of its small size, the tumor may not be visible clinically.8 Rather, it often appears as a small, round, erythematous to purple area visible through the nail plate in the fingertip.3,5,11

Glomus tumor photo

Less commonly, the tumor may elevate, discolor, and deform the overlying nail and may form a palpable nodule (Figure).5,7 Rarely, a glomus tumor may present with longitudinal erythronychia.5,12 Ridging and distal onycholysis may be present.3,5 The first and second fingernails are more commonly affected.8 Occasionally, pulp lesions have also been reported.11 Rarely, glomus tumor in the subungual area of the toe has been reported.13 The tumor is usually solitary.1,5 Multiple tumors are rare2,10 and are usually associated with neurofibromatosis type 1.1,7 Multiple glomus tumors are often painless and tend to occur in children.1,5,7

The diagnosis can sometimes be made clinically, based on the history and clinical findings. Several clinical tests can help to diagnose a glomus tumor. In the Love pin test, the area containing the glomus tumor is exquisitely painful when pressure is applied to the area with a pinhead, the end of a paperclip, or the end of a ballpoint pen.7,9 In the Hildreth test, the pain is relieved by applying a tourniquet to the ipsilateral arm to induce ischemia.5,7 When the Love pin test is repeated, the area containing the glomus tumor is no longer painful when pressure is applied.5,7 There is sudden return of pain in the area of the tumor when the tourniquet is released.5,7,9 In the cold sensitivity test, pain in the affected area is increased when an ice cube or cold water is applied to the affected area.7 In one study, the sensitivity and specificity of the Love pin test, Hildreth test, and cold sensitivity test were 100% and 78%, 77.4% and 100%, and 100% and 100%, respectively.14

In addition, dermoscopy can also be helpful in the diagnosis. Dermoscopy typically shows a deep red-purple discolored area with blurred borders or a band of longitudinal erythronychia that does not reach the distal margin.8 The tumor appears as a pinkish glow under the ultraviolet light (the pink glow sign), suggesting the vascular nature of the tumor.6,8 If necessary, ultrasonography and uncommonly magnetic resonance imaging can be used to locate the tumor and aid the diagnosis.11

Complete surgical excision of the tumor is the treatment of choice and should be considered for pain relief.4,11,15

REFERENCES:

  1. Lipner SR, Scher RK. Evaluation of nail lines: color and shape hold clues. Cleve Clin J Med. 2016;83(5):385‐391. doi:10.3949/ccjm.83a.14187
  2. Joseph VM, Maryada VR, Venkata GR, Mulpur P, Sagi M. Bilateral solitary glomus tumour of thumb: a case report. J Clin Diagn Res. 2017;11(5):RD04‐RD06. doi:10.7860/JCDR/2017/22374.9949
  3. Perera E, Sinclair R. Diagnosis using the nail bed and hyponychium. Dermatol Clin. 2015;33(2):257‐263. doi:10.1016/j.det.2014.12.006
  4. Flint WW, Cain JD. Nail and skin disorders of the foot. Med Clin North Am. 2014;98(2):213‐225. doi:10.1016/j.mcna.2013.11.002
  5. Lenz BL, Beachkofsky TM, Kobayashi TT. Painful nail with longitudinal erythronychia. J Fam Pract. 2013;62(8):429‐432. Accessed May 15, 2020. https://www.mdedge.com/familymedicine/article/76653/dermatology/painful-nail-longitudinal-erythronychia
  6. Thatte SS, Chikhalkar SB, Khopkar US. “Pink glow”: A new sign for the diagnosis of glomus tumor on ultraviolet light dermoscopy. Indian Dermatol Online J. 2015;6(suppl 1):S21‐S23. doi:10.4103/2229-5178.171041
  7. Morey VM, Garg B, Kotwal PP. Glomus tumours of the hand: review of literature. J Clin Orthop Trauma. 2016;7(4):286‐291. doi:10.1016/j.jcot.2016.04.006
  8. Alessandrini A, Starace M, Piraccini BM. Dermoscopy in the evaluation of nail disorders. Skin Appendage Disord. 2017;3(2):70‐82. doi:10.1159/000458728
  9. Lee W, Kwon SB, Cho SH, Eo SR, Kwon C. Glomus tumor of the hand. Arch Plast Surg. 2015;42(3):295‐301. doi:10.5999/aps.2015.42.3.295
  10. Lee J-K, Kim T-S, Kim D-W, Han S-H. Multiple glomus tumours in multidigit nail bed. Handchir Mikrochir Plast Chir. 2017;49(5):321‐325. doi:10.1055/s-0043-115115
  11. Santoshi JA, Kori VK, Khurana U. Glomus tumor of the fingertips: a frequently missed diagnosis. J Family Med Prim Care. 2019;8(3):904‐908. doi:10.4103/jfmpc.jfmpc_88_19
  12. Da Silva DR, Gaddis KJ, Hess S, Rubin AI. Nail unit glomus tumor with myxoid and symplastic change presenting with longitudinal erythronychia. Dermatopathology (Basel). 2018;5(2):74‐78. doi:10.1159/000488980
  13. Sprinkle RLB III, Sangueza OP, Schwartz GA. Glomus tumor of the toe. J Am Podiatr Med Assoc. 2017;107(3):257‐260. doi:10.7547/15-161
  14. Bhaskaranand K, Navadgi BC. Glomus tumour of the hand. J Hand Surg Br. 2002;27(3):229‐231. doi:10.1054/jhsb.2001.0746
  15. Altinel D, Serin M, Leblebici C, Toplu G. Transungual resection of subungual glomus tumour. BMJ Case Rep. 2017;2017:bcr2017221211. doi:10.1136/bcr-2017-221211

NEXT: Digital Clubbing

Digital Clubbing

Digital clubbing refers to a focal bulbous uniform swelling of the soft tissue of the terminal phalanx of a digit with increased transverse and longitudinal curvature of the nail plate (Figure).1 Digital clubbing is said to be present if the angle between the nail plate and the proximal nail fold (the Lovibond angle) when viewed from the side is greater than 180°.1 There is also a loss of the normal diamond-shaped window created by placing the back surfaces of terminal phalanges of similar fingers together (the Schamroth sign).2 Clubbed digits are asymptomatic in most cases.3

Digital clubbing photo

Digital clubbing can be primary (hereditary or idiopathic) or secondary (acquired). Primary nail clubbing typically is congenital. The involvement is almost always bilateral and symmetric, although different fingers and digits may be involved to varying extent.3 Some digits may be spared, but the thumbs are usually involved.3 Causes of primary digital clubbing include primary hypertrophic osteoarthropathy (also known as pachydermoperiostosis), familial clubbing, and idiopathic causes.3,4 Primary hypertrophic osteoarthropathy is characterized by digital clubbing, periostosis, thickening of the skin, coarse facial features, seborrhea, and hyperhidrosis.1,4 The condition is due to mutations in the 15-hydroxyprostaglandin-dehydrogenase gene (HPGD) and is inherited in an autosomal dominant or autosomal recessive fashion.1,3,4 Isolated congenital digital clubbing is considered as a forme fruste, or an incomplete form of primary hypertrophic osteoarthropathy, and accounts for cases of familial clubbing.1

Secondary or acquired digital clubbing can be bilateral or unilateral.1 Acquired bilateral digital clubbing is by far the most common. Causes of acquired bilateral digital clubbing include pulmonary diseases (eg, cystic fibrosis, bronchiectasis, pulmonary tuberculosis, lung abscess, empyema, interstitial pulmonary fibrosis, sarcoidosis, asbestosis, pulmonary arteriovenous fistula, plural mesothelioma, pulmonary cancer), cardiovascular diseases (eg, cyanotic congenital heart disease, subacute bacterial endocarditis, brachial arteriovenous fistula, left atrial myxoma, thoracic aortic aneurysm), gastrointestinal diseases (eg, celiac disease, Crohn disease, ulcerative colitis, primary biliary cirrhosis, cirrhosis of the liver, chronic active hepatitis, juvenile polyposis coli), endocrine diseases (eg, Graves disease, thyroid acropachy, acromegaly, severe secondary hyperparathyroidism), hematologic diseases (sickle cell disease, thalassemia, polycythemia rubra vera), and infections (eg, HIV, schistosomiasis).1,3,5-11

Acquired unilateral digital clubbing may be secondary to ipsilateral subclavian artery thrombosis, aneurysm, arteriovenous fistula, causalgia, hemiplegia, and Pancoast tumor.1,9,11-15 Acquired single digital clubbing may be secondary to digital mucoid cyst, enchondroma, osteoid osteoma, and myxochondroma.3

Treatment should be directed to the underlying cause if possible. The prognosis depends on the underlying cause.

REFERENCES:

  1. Rich P. Overview of nail disorders. UpToDate. Updated December 5, 2019. Accessed May 15, 2020. https://www.uptodate.com/contents/overview-of-nail-disorders
  2. Nguyen K, Aronowitz P. Drumstick digits: a case of clubbing of the fingers and toes. J Hosp Med. 2010;5(3):196. doi:10.1002/jhm.630
  3. Sarkar M, Mahesh DM, Madabhavi I. Digital clubbing. Lung India. 2012;29(4):354‐362. doi:10.4103/0970-2113.102824
  4. Krugh M. Osteoarthropathy hypertrophic. StatPearls. Updated April 16, 2019. Accessed May 15, 2020. https://www.ncbi.nlm.nih.gov/books/NBK540968/
  5. Helvaci MR, Acipayam C, Davran R. Autosplenectomy in severity of sickle cell diseases. Int J Clin Exp Med. 2014;7(5):1404‐1409. Accessed May 15, 2020. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4073764/
  6. Jamieson A. The causes of finger clubbing: a list worth learning. Am J Med. 2011;124(7):e1‐e3. doi:10.1016/j.amjmed.2011.01.020
  7. Marrie TJ, Brown N. Clubbing of the digits. Am J Med. 2007;120(11):940‐941. doi:10.1016/j.amjmed.2007.06.029
  8. Rutherford JD. Digital clubbing. Circulation. 2013;127(19):1997‐1999. doi:10.1161/CIRCULATIONAHA.112.000163
  9. Shiji PV, Narayanan S, Niyaz, George KC. Polycythemia rubra vera presenting as unilateral clubbing due to left subclavian artery thrombosis. J Assoc Physicians India. 2018;66(5):90‐91. Accessed May 15, 2020. https://www.researchgate.net/publication/324840524
  10. Sinniah D, White JC, Omar A, Murugasu R, Iyngkaran N. Digital clubbing—a clinical sign in thalassemia. J Pediatr. 1978;92(4):597‐599. doi:10.1016/s0022-3476(78)80300-x
  11. Spicknall KE, Zirwas MJ, English JC III. Clubbing: an update on diagnosis, differential diagnosis, pathophysiology, and clinical relevance. J Am Acad Dermatol. 2005;52(6):1020‐1028. doi:10.1016/j.jaad.2005.01.006
  12. Kahtan S, Kahtan N. Unilateral finger clubbing. Lancet. 1991;338(8766):576. doi:10.1016/0140-6736(91)91145-k
  13. Roozbeh J, Sagheb MM, Yavari V, Bastani B, Raeesjalali GA. Unilateral digital clubbing in a hemodialysis patient. Hemodial Int. 2010;14(1):84‐86. doi:10.1111/j.1542-4758.2009.00401.x
  14. Saunders PR, Hanna M. Unilateral clubbing of fingers associated with causalgia. BMJ. 1988;297(6664):1635. doi:10.1136/bmj.297.6664.1635-b
  15. Velur P, Kalamangalam GP. Teaching neuroimages: unilateral clubbing in hemiplegia. Neurology. 2012;78(19):e122. doi:10.1212/WNL.0b013e3182553cab