Otology & Neurotology 35:e157Ye158 Ó 2014, Otology & Neurotology, Inc.
Temporal Bone Histopathology Case of the Month
Chondrodermatitis Nodularis Chronica and External Auditory Canal *Giuseppe Magliulo, *Giannicola Iannella, †Valentina Moretti, and ‡Massimo Re *Organi di Senso Department, and ÞPathology Department, University ‘‘la Sapienza’’, Rome; and þDepartment of Clinical Sciences, Polytechnic University of Marche, Ancona, Italy
Chondrodermatitis nodularis chronica helicis (CNCH) is a common benign condition characterized by degeneration of the perichondrium and the clinical appearance of single or multiple nodules on the helix or less frequently antihelix of the ear (1, 2). The authors describe the case of a woman in whom CNCH developed within the external auditory canal (EAC). To our knowledge, this is the first case of CNCH localized in this site to be reported in the English language literature.
(Fig. 3). These findings were consistent with a diagnosis of chondrodermatitis nodularis chronica. At 1-year follow-up examination, the patient is lesion-free.
DISCUSSION Chondrodermatitis nodularis chronica helicis was first described by Winkler in 1915 (1). CNCH is characterized by the presence of small, intensely painful nodules on the free border of the helix. Typically, the lesion is localized unilaterally, affects men more than women, and usually appears on the side that the patient sleeps on (1, 2). The etiology of CNCH remains unclear. Possible factors include tissue ischemia from pressure during sleep, frostbite, sun exposure, or trauma (2, 3). The histopathologic characteristics of CNCH are epithelial hyperplasia,
CASE REPORT A 56-year-old woman came to our department because of the appearance of a mass in the right EAC about 4 months. Otoscopy revealed a round, reddish swelling mass, measuring approximately 0.5 cm in diameter, localized in the inferior portion of the EAC, at the level of the cartilaginous portion. The mass was painful and presented central ulceration and perifocal erythema. The tympanic membrane could be only partially visualized. No cervical lymphadenopathy or other symptoms and sign were reported; moreover, there was no history of ear trauma or skin cancer. Pure-tone audiometry was within normal limits. We performed an excisional biopsy of the lesion under local anesthesia to determine its nature, followed by a retroauricular skin graft. Histology revealed an ulcerated epidermis with adjacent epithelium showing acanthosis, parakeratosis, and hyperplasia (Fig. 1). In the dermis, collagen hyalinosis and chronic inflammatory cells infiltrate were evident (Fig. 2). The inflammatory process extended to involve the perichondrium and cartilage
Address correspondence and reprint requests to Giuseppe Magliulo, M.D., Via Gregorio VII n.80, Rome 00165, Italy; E-mail: giuseppemagliuloorl@ yahoo.com The authors disclose no conflicts of interest.
FIG. 1. CNCH; ulcerated epidermis (arrowhead) with adjacent epitheliumshowingacanthosis,parakeratosis,andpseudoepitheliomatous hyperplasia. Collagen hyalinosis associated to a chronic inflammatory cell infiltrate (hematoxylin-eosin; original magnification, 5).
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G. MAGLIULO ET AL.
collagen degeneration, focal fibrinoid necrosis, and inflammatory process. Beneath the dermis, the perichondrium often shows inflammatory infiltrate, hemorrhage, and necrosis. Degenerative changes in the underlying cartilage may also be present (1Y4). CNCH can usually be diagnosed from patient history and physical examination, although biopsy is necessary to confirm the diagnosis (in atypical cases such as the one we describe) or to rule out several entities that may have a similar clinical appearance. The most important differential diagnoses include an actinic keratosis, squamous cell carcinoma or basal cell carcinoma or remotely a gouty tophus (1Y3). Various types of treatment, both medical and surgical, have been described for CNCH. Medical approaches in clude therapeutic decompression, cryotherapy, or intralesional injection of corticosteroids that, in some trials, proved to be effective in eradicating the lesion (3, 5). Complete surgical excision is the treatment of choice and consists of it includes wedge resection associated with cartilage resection or curettage. Cartilage excision alone
FIG. 3. CNCH; clear perichondrium infiltrate. Underlying cartilage now shows degenerative changes (hematoxylin-eosin; original magnification, 20).
produces cure rates up to 84% for helix lesions and 75% for antihelix lesions (1, 2, 4). We preferred the surgical removal to reduce the risk of EAC stenosis and to determine the nature of the lesion. To conclude, the differential diagnosis of EAC masses should also include CNCH because, as shown by the case described here, this lesion can also develop in other sites, not only in the helix or 99antihelix areas. REFERENCES
FIG. 2. CNCH; granulation tissue and chronic inflammatory process extended to involve dermis and perichondrium (hematoxylin-eosin; original magnification, 20).
1. Oelzner S, Elsner P. Bilateral chondrodermatitis nodularis chronica helicis on the free border of the helix in a woman. J Am Acad Dermatol 2003;49:720Y2. 2. Yaneza MM, Sheikh S. Chondrodermatitis nodularis chronica helicis excision and reconstruction. J Laryngol Otol 2013;127:63Y4. 3. Kuen-Spiegl M, Ratzinger G, Sepp N, Fritsch P. Chondrodermatitis nodularis chronica helicis - a conservative therapeutic approach by decompression. J Dtsch Dermatol Ges 2011;9:292Y6. 4. Hudson-Peacock MJ, Cox NH, Lawrence CM. The long-term results of cartilage removal alone for the treatment of chondrodermatitis nodularis. Br J Dermatol 1999;14:703Y5. 5. Wade TR. Chondrodermatitis nodularis chronica helicis. A review with emphasis on steroid therapy. Cutis 1979;24:406Y9.
Otology & Neurotology, Vol. 35, No. 4, 2014
Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
Temporal Bone Histopathology Case of the Month
Chondrodermatitis Nodularis Chronica and External Auditory Canal *Giuseppe Magliulo, *Giannicola Iannella, †Valentina Moretti, and ‡Massimo Re *Organi di Senso Department, and ÞPathology Department, University ‘‘la Sapienza’’, Rome; and þDepartment of Clinical Sciences, Polytechnic University of Marche, Ancona, Italy
Chondrodermatitis nodularis chronica helicis (CNCH) is a common benign condition characterized by degeneration of the perichondrium and the clinical appearance of single or multiple nodules on the helix or less frequently antihelix of the ear (1, 2). The authors describe the case of a woman in whom CNCH developed within the external auditory canal (EAC). To our knowledge, this is the first case of CNCH localized in this site to be reported in the English language literature.
(Fig. 3). These findings were consistent with a diagnosis of chondrodermatitis nodularis chronica. At 1-year follow-up examination, the patient is lesion-free.
DISCUSSION Chondrodermatitis nodularis chronica helicis was first described by Winkler in 1915 (1). CNCH is characterized by the presence of small, intensely painful nodules on the free border of the helix. Typically, the lesion is localized unilaterally, affects men more than women, and usually appears on the side that the patient sleeps on (1, 2). The etiology of CNCH remains unclear. Possible factors include tissue ischemia from pressure during sleep, frostbite, sun exposure, or trauma (2, 3). The histopathologic characteristics of CNCH are epithelial hyperplasia,
CASE REPORT A 56-year-old woman came to our department because of the appearance of a mass in the right EAC about 4 months. Otoscopy revealed a round, reddish swelling mass, measuring approximately 0.5 cm in diameter, localized in the inferior portion of the EAC, at the level of the cartilaginous portion. The mass was painful and presented central ulceration and perifocal erythema. The tympanic membrane could be only partially visualized. No cervical lymphadenopathy or other symptoms and sign were reported; moreover, there was no history of ear trauma or skin cancer. Pure-tone audiometry was within normal limits. We performed an excisional biopsy of the lesion under local anesthesia to determine its nature, followed by a retroauricular skin graft. Histology revealed an ulcerated epidermis with adjacent epithelium showing acanthosis, parakeratosis, and hyperplasia (Fig. 1). In the dermis, collagen hyalinosis and chronic inflammatory cells infiltrate were evident (Fig. 2). The inflammatory process extended to involve the perichondrium and cartilage
Address correspondence and reprint requests to Giuseppe Magliulo, M.D., Via Gregorio VII n.80, Rome 00165, Italy; E-mail: giuseppemagliuloorl@ yahoo.com The authors disclose no conflicts of interest.
FIG. 1. CNCH; ulcerated epidermis (arrowhead) with adjacent epitheliumshowingacanthosis,parakeratosis,andpseudoepitheliomatous hyperplasia. Collagen hyalinosis associated to a chronic inflammatory cell infiltrate (hematoxylin-eosin; original magnification, 5).
e157
Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
e158
G. MAGLIULO ET AL.
collagen degeneration, focal fibrinoid necrosis, and inflammatory process. Beneath the dermis, the perichondrium often shows inflammatory infiltrate, hemorrhage, and necrosis. Degenerative changes in the underlying cartilage may also be present (1Y4). CNCH can usually be diagnosed from patient history and physical examination, although biopsy is necessary to confirm the diagnosis (in atypical cases such as the one we describe) or to rule out several entities that may have a similar clinical appearance. The most important differential diagnoses include an actinic keratosis, squamous cell carcinoma or basal cell carcinoma or remotely a gouty tophus (1Y3). Various types of treatment, both medical and surgical, have been described for CNCH. Medical approaches in clude therapeutic decompression, cryotherapy, or intralesional injection of corticosteroids that, in some trials, proved to be effective in eradicating the lesion (3, 5). Complete surgical excision is the treatment of choice and consists of it includes wedge resection associated with cartilage resection or curettage. Cartilage excision alone
FIG. 3. CNCH; clear perichondrium infiltrate. Underlying cartilage now shows degenerative changes (hematoxylin-eosin; original magnification, 20).
produces cure rates up to 84% for helix lesions and 75% for antihelix lesions (1, 2, 4). We preferred the surgical removal to reduce the risk of EAC stenosis and to determine the nature of the lesion. To conclude, the differential diagnosis of EAC masses should also include CNCH because, as shown by the case described here, this lesion can also develop in other sites, not only in the helix or 99antihelix areas. REFERENCES
FIG. 2. CNCH; granulation tissue and chronic inflammatory process extended to involve dermis and perichondrium (hematoxylin-eosin; original magnification, 20).
1. Oelzner S, Elsner P. Bilateral chondrodermatitis nodularis chronica helicis on the free border of the helix in a woman. J Am Acad Dermatol 2003;49:720Y2. 2. Yaneza MM, Sheikh S. Chondrodermatitis nodularis chronica helicis excision and reconstruction. J Laryngol Otol 2013;127:63Y4. 3. Kuen-Spiegl M, Ratzinger G, Sepp N, Fritsch P. Chondrodermatitis nodularis chronica helicis - a conservative therapeutic approach by decompression. J Dtsch Dermatol Ges 2011;9:292Y6. 4. Hudson-Peacock MJ, Cox NH, Lawrence CM. The long-term results of cartilage removal alone for the treatment of chondrodermatitis nodularis. Br J Dermatol 1999;14:703Y5. 5. Wade TR. Chondrodermatitis nodularis chronica helicis. A review with emphasis on steroid therapy. Cutis 1979;24:406Y9.
Otology & Neurotology, Vol. 35, No. 4, 2014
Copyright © 2014 Otology & Neurotology, Inc. Unauthorized reproduction of this article is prohibited.
