Chondrodermatitis nodularis chronica helicis

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Chondrodermatitis helicis nodularis in a 67-year-old man.


1_ Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1];Associate Editor(s)-in-Chief: Karnik Raju Paila Bangaru, M.B.B.S.[2] Kiran Singh, M.D. [3]

Synonyms and keywords:Chondrodermatitis nodularis helicis, winkler disease, ear pressure sore, chondrodermatitis nodularis auricularis

Overview

Chondrodermatitis nodularis chronica helicis (also known as "Chondrodermatitis nodularis helicis"[1]:782) (CNCH) is a small, nodular, tender, chronic inflammatory lesion occurring on the helix of the ear, occurring most often in men.[2]:610


Historical Perspective[edit | edit source]

  • Chondrodermatitis nodularis chronica helicis was first discovered by Max Winkler, a dermatologist, in 1915, in a paper titled Knötchenförmige Erkrankung am Helix . [3][4]
  • in 1925, Foerster further developed the clinical, microscopic and treatment options for CNCH.

Pathophysiology

  • The pathophysiology of Chondrodermatitis nodularis chronica helicis remains unclear. It may be due to an inflammatory condition from chronic pressure on helix of the ear, but can occur on anti-helix too.
  • CNCH is seen mostly in elderly men. There are few cases seen in women and young, especially when CNCH is associated with autoimmune or connective tissue disorders. [5][6][7][8]
  • The anatomy of ear pinna can contribute to the pathophysiology of CNCH. The pinna has limited blood supply and little subcutaneous tissue, which easily lead to formation of sore with pressure or trauma or cold temperature. [9]
  • In 2009, Perichondrium vasculitis theory explained above anatomical features. Because of the vasculature and subcutaneous tissue padding limitations, there is easy development of ischemia and necrosis of underlying cartilage, causing a severe inflammatory condition, leading to CNCH. [10][11]
  • There could be a possibility of genetic relation to disease, as observed in a case of monozygotic twins, but not studied enough to make a point. [12]
  • On gross pathology, CNCH looks like any other sore. Microscopically, it shows hyperkeratosis, parakeratosis with epithelial hyperplasia, dermal tissue damage with underlying cartilage destruction and dermal vessel proliferation. [13][14]

Causes[edit | edit source]

  • Chondrodermatitis nodularis chronica helicis may be caused due to chronic pressure or trauma or anatomic features or temperature changes or actinic damage to the ear. Prolonged pressure seemed the most commonly reported cause. Sleeping on the one side can cause this pressure.
  • Other causes can be continuous use of headphones/ ear phones/ cell phones, hearing aids, or can be idiopathic. There are some theories on association with autoimmune disorders and connective tissue diseases like scleroderma, autoimmune thyroiditis, lupus erythematosus, or dermatomyositis. [15][16][17][18]


Differentiating [disease name] from other Diseases[edit | edit source]

  • Chondrodermatitis nodularis chronica helicis can present as painful nodular lesion with central crust, so it must be differentiated from Basal cell carcinoma which is generally painless and with more vascularity.
  • It must be differentiated from other diseases that cause a bump on the ear, such as:
  • Actinic Keratosis
  • Keratoacanthoma
  • Squamous cell carcinoma
  • Gouty tophi

Epidemiology and Demographics[edit | edit source]

  • The disease is much common than it is reported.

Age[edit | edit source]

  • CNCH is more commonly observed among patients aged 50-80 years old, but has been seen in young adults and children too. [19] Unilateral occurrence is more common.

Gender[edit | edit source]

  • CNCH is seen mostly in elderly men, but may occur with women and children too.

Race[edit | edit source]

  • There is no racial predilection for CNCH, but is seen more commonly in fair skinned with sun exposure.

Risk Factors[edit | edit source]

  • Common risk factors in the development of CNCH are pressure, trauma, sun damage, and systemic conditions.

Natural History, Complications and Prognosis[edit | edit source]

  • Early clinical features include just a painful sore. Spontaneous resolution is rare.
  • Prognosis is generally very good with treatment, although long term morbidity is common. Remissions can be seen when undertreated.


Diagnosis


History and Symptoms[edit | edit source]

  • CNCH mostly presents as a spontaneously appearing round to oval tender nodule with raised edges on helix or anti-helix with a central crust/ulcer, commonly on ear on usually sleeping side, with nocturnal pain.
  • Like said, this is most common presentation, but it can also present as bilateral lesion, or with diurnal pain.
  • It usually grows and becomes stable after reaching it's maximum size.

Physical Examination

Ear





Laboratory Findings[edit | edit source]

  • There are no specific laboratory findings associated with CNCH.
  • Skin biopsy may be necessary in conditions to differentiate it from carcinomatous lesions.

Other Diagnostic Studies[edit | edit source]

  • Investigating for the systemic illness( autoimmune diseases, connective tissue diseases or chronic non-communicable diseases) for underlying associations to the disease is indicated in some studies.[21]


Treatment[edit | edit source]

Medical therapy and Surgery are the treatment options. Surgical procedures have less chance of recurrence of disease.

Medical/Conservative Therapy[edit | edit source]

  • The mainstay of therapy is to avoid/eradicate the triggers of CNCH, like pressure, trauma or solar damage. Pressure-relieving prosthesis is most cost-effective method. They are available in various forms like foam sponge or foam bandages, doughnut pillows[22] or CNH pillows, or a home-made prosthesis. [23][24][25]
  • Intralesional and topical steroids are reported to be used in treating CNCH with a treatment success rate of 30-40%, especially with an initial benefit. [26][27][28]
  • Local collagen injections may provide cushioning and insulation. [29]
  • Topical antibiotics are used for secondary bacterial infections.
  • Carbon dioxide laser vaporizes the nodule and underlying cartilage. One study reports 92% cure rate with it. [30]
  • Nitroglycerin gel has been used by some to help reverse the cartilage necrosis by vasodilating arteriolar smooth muscles.[31][32]
  • Photodynamic therapy improves blood flow to the lesion and provides cytotoxic effect on the lesion.[33][34]

Surgery[edit | edit source]

  • Various surgical procedures have been implemented over years in the treatment of CNCH, which popularly include cartilage excision, curettage and wedge excision. Surgery is still the gold standard for treatment of CNCH, with a success rate of 89%-96% with wedge excision.[35][36][37]

Prevention[edit | edit source]

  • Effective measures for the primary prevention of CNCH include avoiding ear pressure/ pressure offloading techniques.


References

  1. ↑ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
  2. ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  3. ↑ Wagner G, Liefeith J, Sachse MM (2011). "Clinical appearance, differential diagnoses and therapeutical options of chondrodermatitis nodularis chronica helicis Winkler". J Dtsch Dermatol Ges. 9 (4): 287–91. doi:10.1111/j.1610-0387.2011.07601.x. PMID 21276202.
  4. ↑ Kechichian E, Jabbour S, Haber R, Abdelmassih Y, Tomb R (2016). "Management of Chondrodermatitis Nodularis Helicis: A Systematic Review and Treatment Algorithm". Dermatol Surg. 42 (10): 1125–34. doi:10.1097/DSS.0000000000000817. PMID 27399947.
  5. ↑ García-García B, Munguía-Calzada P, Aubán-Pariente J, Junceda-Antuña S, Zaballos P, Argenziano G; et al. (2018). "Dermoscopy of chondrodermatitis nodularis helicis". Arch Dermatol Res. 310 (7): 551–560. doi:10.1007/s00403-018-1844-6. PMID 29926164.
  6. ↑ Salah H, Urso B, Khachemoune A (2018). "Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis". Cureus. 10 (3): e2367. doi:10.7759/cureus.2367. PMC 5969795. PMID 29805936.
  7. ↑ Kumar P, Barkat R (2017). "Chondrodermatitis nodularis chronica helicis". Indian Dermatol Online J. 8 (1): 48–49. doi:10.4103/2229-5178.198767. PMC 5297272. PMID 28217474.
  8. ↑ Shah S, Fiala KH (2017). "Chondrodermatitis nodularis helicis: A review of current therapies". Dermatol Ther. 30 (1). doi:10.1111/dth.12434. PMID 27723195.
  9. ↑ Elsensohn A, Getty S, Shiu J, de Feraudy S (2018). "Intradermal Proliferative Fasciitis Occurring With Chondrodermatitis Nodularis Helicis". Am J Dermatopathol. 40 (2): 139–141. doi:10.1097/DAD.0000000000001027. PMC 6075668. PMID 29210713.
  10. ↑ Kumar P, Barkat R (2017). "Chondrodermatitis nodularis chronica helicis". Indian Dermatol Online J. 8 (1): 48–49. doi:10.4103/2229-5178.198767. PMC 5297272. PMID 28217474.
  11. ↑ Shah S, Fiala KH (2017). "Chondrodermatitis nodularis helicis: A review of current therapies". Dermatol Ther. 30 (1). doi:10.1111/dth.12434. PMID 27723195.
  12. ↑ Chan HP, Neuhaus IM, Maibach HI (2009). "Chondrodermatitis nodularis chronica helicis in monozygotic twins". Clin Exp Dermatol. 34 (3): 358–9. doi:10.1111/j.1365-2230.2008.02915.x. PMID 19175786.
  13. ↑ Shah S, Fiala KH (2017). "Chondrodermatitis nodularis helicis: A review of current therapies". Dermatol Ther. 30 (1). doi:10.1111/dth.12434. PMID 27723195.
  14. ↑ Juul Nielsen L, Holkmann Olsen C, Lock-Andersen J (2016). "Therapeutic Options of Chondrodermatitis Nodularis Helicis". Plast Surg Int. 2016: 4340168. doi:10.1155/2016/4340168. PMC 4748103. PMID 26925262.
  15. ↑ García-García B, Munguía-Calzada P, Aubán-Pariente J, Junceda-Antuña S, Zaballos P, Argenziano G; et al. (2018). "Dermoscopy of chondrodermatitis nodularis helicis". Arch Dermatol Res. 310 (7): 551–560. doi:10.1007/s00403-018-1844-6. PMID 29926164.
  16. ↑ Salah H, Urso B, Khachemoune A (2018). "Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis". Cureus. 10 (3): e2367. doi:10.7759/cureus.2367. PMC 5969795. PMID https://www.ncbi.nlm.nih.gov/pubmed/29805936 Check |pmid= value (help).
  17. ↑ Kumar P, Barkat R (2017). "Chondrodermatitis nodularis chronica helicis". Indian Dermatol Online J. 8 (1): 48–49. doi:10.4103/2229-5178.198767. PMC 5297272. PMID 28217474.
  18. ↑ Shah S, Fiala KH (2017). "Chondrodermatitis nodularis helicis: A review of current therapies". Dermatol Ther. 30 (1). doi:10.1111/dth.12434. PMID 27723195.
  19. ↑ Magro CM, Frambach GE, Crowson AN (2005). "Chondrodermatitis nodularis helicis as a marker of internal disease [corrected] associated with microvascular injury". J Cutan Pathol. 32 (5): 329–33. doi:10.1111/j.0303-6987.2005.00317.x. PMID 15811116.
  20. ↑ 20.0 20.1 20.2 20.3 20.4 20.5 20.6 20.7 20.8 20.9 "Dermatology Atlas".
  21. ↑ Magro CM, Frambach GE, Crowson AN (2005). "Chondrodermatitis nodularis helicis as a marker of internal disease [corrected] associated with microvascular injury". J Cutan Pathol. 32 (5): 329–33. doi:10.1111/j.0303-6987.2005.00317.x. PMID 15811116.
  22. ↑ Sanu A, Koppana R, Snow DG (2007). "Management of chondrodermatitis nodularis chronica helicis using a 'doughnut pillow'". J Laryngol Otol. 121 (11): 1096–8. doi:10.1017/S0022215107008535. PMID 17524165.
  23. ↑ Travelute CR (2013). "Self-adhering foam: a simple method for pressure relief during sleep in patients with chondrodermatitis nodularis helicis". Dermatol Surg. 39 (2): 317–9. doi:10.1111/dsu.12027. PMID 23205696.
  24. ↑ Sehgal VN, Singh N (2009). "Chondrodermatitis nodularis". Am J Otolaryngol. 30 (5): 331–6. doi:10.1016/j.amjoto.2008.04.001. PMID 19720252.
  25. ↑ Moncrieff M, Sassoon EM (2004). "Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach". Br J Dermatol. 150 (5): 892–4. doi:10.1111/j.1365-2133.2004.05961.x. PMID 15149500.
  26. ↑ Shah S, Fiala KH (2017). "Chondrodermatitis nodularis helicis: A review of current therapies". Dermatol Ther. 30 (1). doi:10.1111/dth.12434. PMID 27723195.
  27. ↑ Cox NH, Denham PF (2002). "Intralesional triamcinolone for chondrodermatitis nodularis: a follow-up study of 60 patients". Br J Dermatol. 146 (4): 712–3. doi:10.1046/j.1365-2133.2002.47047.x. PMID 11966717.
  28. ↑ Moncrieff M, Sassoon EM (2004). "Effective treatment of chondrodermatitis nodularis chronica helicis using a conservative approach". Br J Dermatol. 150 (5): 892–4. doi:10.1111/j.1365-2133.2004.05961.x. PMID 15149500.
  29. ↑ Greenbaum SS (1991). "The treatment of chondrodermatitis nodularis chronica helicis with injectable collagen". Int J Dermatol. 30 (4): 291–4. doi:10.1111/j.1365-4362.1991.tb04643.x. PMID 2050461.
  30. ↑ Taylor MB (1991). "Chondrodermatitis nodularis chronica helicis. Successful treatment with the carbon dioxide laser". J Dermatol Surg Oncol. 17 (11): 862–4. doi:10.1111/j.1524-4725.1991.tb03275.x. PMID 1757647.
  31. ↑ Yélamos O, Dalmau J, Puig L (2013). "Chondrodermatitis nodularis helicis: successful treatment with 2% nitroglycerin gel". Actas Dermosifiliogr. 104 (6): 531–2. doi:10.1016/j.ad.2012.07.016. PMID 23098568.
  32. ↑ Sanz-Motilva V, Martorell-Calatayud A, Gutiérrez García-Rodrigo C, Hueso-Gabriel L, García-Melgares ML, Pelufo-Enguix C; et al. (2015). "The Usefulness of 0.2% Topical Nitroglycerin for Chondrodermatitis Nodularis Helicis". Actas Dermosifiliogr. 106 (7): 555–61. doi:10.1016/j.ad.2015.04.004. PMID 26001657.
  33. ↑ García-Malinis AJ, Turrión-Merino L, Pérez-García B, Saceda-Corralo D, Harto-Castaño A, Gilaberte Y (2017). "Observational study of chondrodermatitis nodularis helicis treated with methyl aminolevulinate photodynamic therapy". J Am Acad Dermatol. 76 (6): 1103–1108. doi:10.1016/j.jaad.2016.12.019. PMID 28215445.
  34. ↑ Kechichian E, Jabbour S, Haber R, Abdelmassih Y, Tomb R (2016). "Management of Chondrodermatitis Nodularis Helicis: A Systematic Review and Treatment Algorithm". Dermatol Surg. 42 (10): 1125–34. doi:10.1097/DSS.0000000000000817. PMID 27399947.
  35. ↑ Jacob K J, Satheesh S, Menon P, Saju KG (2005). "Winkler's disease". Indian J Otolaryngol Head Neck Surg. 57 (4): 323–4. doi:10.1007/BF02907700. PMC 3451457. PMID 23120207.
  36. ↑ de Ru JA, Lohuis PJ, Saleh HA, Vuyk HD (2002). "Treatment of chondrodermatitis nodularis with removal of the underlying cartilage alone: retrospective analysis of experience in 37 lesions". J Laryngol Otol. 116 (9): 677–81. doi:10.1258/002221502760237939. PMID 12437799.
  37. ↑ Salah H, Urso B, Khachemoune A (2018). "Review of the Etiopathogenesis and Management Options of Chondrodermatitis Nodularis Chronica Helicis". Cureus. 10 (3): e2367. doi:10.7759/cureus.2367. PMC 5969795. PMID https://www.ncbi.nlm.nih.gov/pubmed/29805936 Check |pmid= value (help).


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