CHONDRODERMATITIS HELICIS

Transcription

1 CHONDRODERMATITIS HELICIS CHONDRODERMATITIS NODULARIS CHRONICA HELICIS RICHARD SHUMAN, M.D., AND ELSON B. HELWIG, M.D. Armed Forces Institute of Pathology, Washington, D. C. A small painful nodule, situated primarily on the helix of the ear, has been recognized for many years under the designations "chondrodermatitis nodularis chronica helicis" and "painful nodular growth of the ear." It is a benign condition of relatively infrequent occurrence but of significance in differential diagnosis because the epidermal changes may be mistaken both clinically and pathologically for other benign and malignant keratoses involving the auricle. We prefer the term chondrodermatitis helicis to chondrodermatitis nodidaris chronica helicis inasmuch as it is less cumbersome but equally effective in elucidating the nature of this lesion. A number of authors have described the lesion, but most of their reports have appeared in the dermatologic literature and are largely clinical studies of small groups of cases or of individual cases. It is the purpose of this presentation to outline the present knowledge of the subject and to describe in detail the histopathologic features which have not been sufficiently emphasized in previous communications. HISTORICAL REVIEW The original description of chondrodermatitis nodularis chronica helicis was published in 1915 by Max Winkler 22 of Lucerne under this title. In a careful study based on 8 cases, including 3 biopsy specimens, Winkler called attention to an unusual growth located on the upper margin of the helix which he had observed only in males and which presented a constant clinical picture. In discussing the pathogenesis he considered the possibility that degeneration of the auricular cartilage, resulting from injury, was the primary factor in the initiation of the process, and that subsequently infection supervened and was maintained by the altered cartilage acting as a foreign bod}'. Two years later, before the American Dermatological Association, Otto Foerster 8 presented 4 identical cases which he had investigated independently without knowledge of Winkler's work. His name for the lesion was painful nodular growth of the ear. In 1925 Foerster 9 reported 8 additional cases and gave a classical account of the clinical, microscopic and therapeutic aspects of the disease. Like Winkler, he emphasized the remarkable uniformity in clinical symptomatology and histologic features and pointed out the striking frequency with which the lesion was situated on the helix in the adult male. In regard to pathogenesis, he alluded to the possible significance of the anatomic peculiarities of the part of the ear affected. Received for publication August 13, Dr. Shuman is Chief of the Section of Soft Tissue Pathology, and Dr. Helwig is Chief of the Section of Skin and Subcutaneous Tissue Pathology. 126

2 FEB CHONDRODERMATITIS HELICIS 127 Rost, 16 in 1926 described 7 cases he had observed over a 9-year period. Three lesions were in women, the first to be reported in this sex. Case 7 in which the nodule occurred on the anthelix of a 35-year-old nursing sister was discussed in complete detail with histologic findings. From his microscopic and clinical description this appears to be a bona fide example of chondrodermatitis nodularis chronica helicis, but no photomicrographic illustrations are included. The author believed that the unusual position of the nodule might be ascribed to trauma caused by the starched hood of the nun's habit. Ebenius 7 in 1941 reported a series of 33 cases, 26 of which were examined microscopically. He gave special attention to the differential diagnostic and therapeutic aspects of the disease and introduced the question of possible malignant transformation in the involved epidermis. Although he was unable to prove that carcinoma developed on the basis of chondrodermatitis nodularis chronica helicis, he mentioned several cases in which he had observed precancerous changes. Ebenius considered the possibility that circulatory disturbances of long duration might play a major role in the production of the lesion. Carol and Van Haren 3 in 1941 made histologic studies of 10 nodules from 8 patients. On the basis of a series of sections in 4 of these nodules, they concluded that the lesion should be regarded as a clavus and that the term "clavus helicis" would be preferable to chondrodermatitis nodularis chronica helicis since it better describes the pathogenesis. It was their belief that the cornified plug of hyperkeratosis and parakeratosis perforates the epidermis into the corium or that the perforation may come about from the opposite direction as a result of a hard mucoid or chondrin-like substance forming between the cartilage and skin. Numerous other reports have appeared in the literature; they are chiefly clinical studies with limited microscopic observations. MATERIALS AND METHODS The material forming the basis of this report was derived from a systematic survey of the Armed Forces Institute of Pathology (AFIP) files for all cases recorded as chondrodermatitis nodularis chronica helicis, painful nodular growth of the ear, or "perichondritis" a term used at the AFIP in former years as a synonym for chondrodermatitis helicis. Of this group of approximately 150 cases accumulated during a period of approximately 15 years, 100 had adequate fixed tissue and were otherwise suitable for the present study. Several cases previously diagnosed as chondrodermatitis nodularis chronica helicis were found to be benign or malignant keratotic lesions of the helix. They were excluded from the series but served as a control group and afforded an opportunity for comparative studies. The majority of specimens included the totally excised nodule but in a few cases tissue had been removed for biopsy prior to complete excision. Through the kindness of Doctor E. Uehlinger, Pathologisches Institut Kantonsspital, St. Galen, Switzerland, we were able to obtain Winkler's original sections. Comparison of these 3 cases with ours leaves no doubt that they are identical.

3 128 SHUMAN AND HELWIG VOL. 24 CLINICAL ANALYSIS The clinical information used in this study was obtained from the clinical records and from patients or their physicians on follow-up questionnaires. Age and race incidence. The age and race distribution are summarized in Table 1. The largest percentage (47 per cent) of lesions occurred between 40 and 60 years of age, 38 per cent between 20 and 40 years and 14 per cent between 60 and 79 years. The age recorded is that of the patient when he first came under medical observation. Since patients have only a vague recollection of when they were first conscious of the lesion, revision of their ages to correspond with the/ remembered date of onset would be unreliable. In a summary of 20 cases recorded up to 1924, Foerster found that the ages ranged from 27 to 68 years. The age range in our series was from 20 to 74 years. All patients in our series were men. Since the material used in this study was obtained primarily from a male population, it does not reflect the true sex incidence; however, most reports reveal a high preponderance of men, and some investigators even deny that the lesion occurs in women. Incidence and site of origin. The situation of the lesion is shown in Table 2. Of 100 lesions examined, 60 were situated on the right ear, 33 on the left ear, and TABLE 1 DISTRIBUTION BY ACE AND RACE NUMBER* White NUMBER Colored Total with age stated Age unknown * All of the patients were males. TABLE 2 LOCATION OF LESION IN RELATION TO NUMBER OF PATIENTS AND INCIDENCE OF LESIONS NUMBER Total Right ear Left ear Both ears Patients Incidence

4 FEB CHONDRODERMATITIS HELICIS were bilateral. This distribution approaches that recorded by Ebenius, Avho noted a predilection for the right ear. The site in 25 of his 33 cases was the right ear, in 7 the left ear and in 1 bilateral. In most reported series a fairly even distribution has been noted. Ninety per cent of the lesions were single and confined to one ear, and 10 per cent were single on both ears or multiple on one or both ears. In the accompanying diagram (Fig. 1) it will be noted that in 85 instances the nodules were limited to the outer margin of the helix. Seventy-three of these lesions were in the region of the crown angle, 8 on the descending helix, and the exact position on the helix of 4 was not specified. Six nodules on the anthelix and 1 on the antitragus were of interest since a number of authors have noted the lesion on this part of the ear in women, but rarely, except in Culver's Case 2, in men. In 3 instances the nodule was on the tragus, in 2 on the concha and in 3 the specific site was not noted. Clinical symptoms. The most characteristic symptom associated with the nodule was pain or tenderness. This was generally present at the beginning and prompted the patient to seek medical advice. Sometimes there was a latent period before the pain was felt, or the symptoms were very slight or even absent and the crusted nodule itself attracted the patient's attention. The pain was usually initiated on pressure, although in some cases it occurred spontaneously CHONDRODERMATITIS NODULARIS CHRONICA HEUCIS Helix 100 PATIENTS Antihelix 6 Concha 2 Antitrogus 3 Nodules not specified FIG. 1. Diagrammatic representation of location of lesion in 100 cases of chondrodermatitis helicis. AFIP Ace

5 130 SHTJMAN AND HELWIG VOL. 24 or was associated with climatic change. It varied in intensity but was usually very severe and was described by the patient as stabbing or piercing in quality. This discomfort was paroxysmal or continuous and persisted from a few minutes to several hours. Patients frequently complained that the pain awakened them from sleep when the head rested on the affected side, but they soon learned to protect the ear by cupping the palm over it. Some of the patients gained temporary relief by removing the central crust. Clinical appearance. The fully developed lesion (Fig. 2) was a relatively small, firm, well-defined nodule varying from 3 to 18 mm. in greatest diameter, except in 2 cases where the lesions were between 2 and 3 cm. in greatest diameter. The average diameter was 7 mm. The nodule was generally raised several millimeters above the surrounding skin surface. As a rule it was convex, round to ovoid, with a dome-shaped or slightly flattened surface and sloping margins. The color sometimes differed only slightly from normal skin but usually was grayish white or translucent. After a time a bluish or reddish brown discoloration appeared as a result of concomitant capillary dilatation. A hyperemic zone of inflammatory redness often surrounded the periphery. An adherent scale or crust usually covered the summit and its removal disclosed a small central depression or ulcer. Course of the disease. The condition appeared spontaneously in the form of a circumscribed nodular thickening or as a minute body embedded within the epidermis. As a rule the nodule reached its maximal size within a relatively short time unless altered by trauma or secondary infection. The disease usually followed a very protracted course, sometimes lasting for years (3 weeks to 13 years in our series). In none of our cases was there evidence of spontaneous regression or complete disappearance of the nodule, although this has been reported. In most of these cases there was subsequent recurrence, but Foerster noted the spontaneous disappearance of a nodule in his Case 3, which had been present for about 18 months, with no recurrence 5 years later. Clinical diagnosis. No clinical diagnosis was submitted in 42 cases. Of the remainder only 26 per cent (15 out of 58 cases) were correctly diagnosed as chondrodermatitis nodularis chronica helicis. Table 3 lists the clinical diagnoses made in our cases. It should be noted that in approximately 30 per cent (17 cases) the lesion was interpreted either as basal- or squamous-cell carcinoma. In 12 per cent (7 cases) the clinical picture was mistaken for senile keratosis and precancerous dermatosis. This serves to point up the confusion that exists in the clinical evaluation of this entity. Pathologic diagnosis. The correct pathologic diagnosis was made by the contributing pathologist in approximately 41 per cent of the cases in our series. Fifty-eight lesions were incorrectly diagnosed. The wide range of pathologic diagnoses is shown in Table 4. A diagnosis of basal- or squamous-cell carcinoma was made in 9 per cent, and in 29 per cent the diagnosis submitted implied that the condition was either cancerous or precancerous. This indicates that confusion exists not only on clinical but on pathologic grounds. Treatment. Treatment of the patients in this series included excision in all instances. In 16 lesions various methods of treatment, including roentgen rays, radium, fulguration, carbon dioxide snow and caustics, were tried prior to ex-

6 FEB CHONDRODERMATITIS HELICIS 131 FIG. 2 (left). Typical nodule of chondrodermatitis helicis located on the outer rim of the helix in 34-year-old white man. Duration 7 months. AFIP Ace FIG. 3 (right). Section of the entire nodule showing the cystic degeneration of the auricular cartilage and changes in the surface epidermis. Elastica. AFIP Ace. 2S6414. X 21. TABLE 3 CLINICAL DIAGNOSES MADE BY CONTRIBUTORS Total 100 Not stated 42 Chondrodermatitis nodularis chronica helicis 15 Senile keratosis 6 Epithelioma, basal-cell or squamous-cell carcinoma 17 Hyperkeratosis 6 Cyst 5 Ulcer 2 Precancerous dermatosis 1 Fibrosis or dermatofibroma 1 Irradiation necrosis 1 Granuloma 1 Wart 2 Uric acid tophi 1 cision. Fourteen or 88 per cent had one or more recurrence after therapy other than excision, and 5 had a recurrence after excision (Table 5). Of 84 nodules in which excision only had been employed 15 recurred and these were successfully treated by secondary excision. Surgical removal of the nodule proved to be the

7 132 SHTJMAN AND HELWIG VOL. 24 PATHOLOGIC TABLE 4 DIAGNOSES MADE BY CONTRIBUTORS Total 100 Not stated 1 Chondrodermatitis nodularis chronica helicis 41 Senile keratosis 14 Epithelioma, basal-cell epidermoid carcinoma, etc. 9 Hyperkeratosis 8 Cyst 3 Dermatitis 3 Ulcer 3 Precancerous lesion 2 Dyskeratosis (malignant) 2 Acanthoma 2 Fibrosis or dermatofibroma 2 Papilloma Chondromyoma Irradiation necrosis Lupus erythematosus, discoid Degeneration of dermal collagen with chronic inflammation Syringocystadenoma Keloid Hemangioma TABLE 5 TYI\E OP TREATMENT IN RELATION TO NUMBER OP PATIENTS AND RECURRENCE AFTER SPECIFIC TREATMENT TYPE OF TREATMENT WITH RECURRENCES TOTAL WITH EXCISION EXCISION ONLY Number Per Cent EXCISION WITH OTHER TREATMENT Number Per Cent Total with excision No recurrence after excision Recurrence after excision S Total with excision and other treatment Recurrence after other treatment Total with excision, with or without other treatment Recurrence after excision (88%) most successful method of treatment. When the entire rim of degenerated cartilage was not completely removed, however, pain often persisted and the possibility of recurrence was greatly enhanced. MICROSCOPIC DESCRIPTION General criteria. The histopathologic aspect of the lesion was highly characteristic and several basic features which were generally reproduced in combina-

8 FEB CHONDRODERMATITIS HELICIS 133 ""^^sfe^ F r c 4 (upper). Nodular acanthosis of the epidermis surmounted b y a hyperkcratotic and parakeratotic scale. Note the proliferation of granulation tissue in the superficial dermis. Hematoxylin and eosin. A F I P Ace X 55. F r c 5 (lower). Centrally ulcerated epidermis bridged by a keratotic scale. T h e ulceration extends to t h e underlying cartilage forming a sinus t r a c t containing amorphous debris. Fibrinoid collagen necrosis surrounds the base of the ulcer and the entire area is outlined by an ingrowth of granulation tissue. Note the marginal downgrowth of the epidermis. Hematoxylin and eosin. A F I P Ace X 48.

9 134 SHUMAN AND HELWIG VOL. 24 tion suggested the nature of the condition. These included (1) nodular hyperplasia of the epidermis which was centrally depressed or ulcerated and topped by a hyperkeratotic and parakeratotic scale; (2) edema, homogenization and fibrinoid necrosis of the dermal collagen; (3) proliferation of the highly vascularized granulation tissue surrounding and partly replacing the altered dermis; (4) an inflammatory infiltrate of varying amount, usually small, made up predominantly of lymphocytes and plasma cells and (5) perichondritis with or without degeneration of the auricular cartilage (Fig. 3). Several additional details were observed and will be elucidated as the microscopic picture is described more completely. Epidermis. The epidermis was altered in a fairly characteristic manner in nearly all instances. Most remarkable was the circumscribed acanthosis which tended to form a plaque-like nodule situated directly over the intracutaneous portion of the lesion (Fig. 4). This feature was not always readily apparent in tangentially oriented sections. The surface of the acanthotic nodule invariably had a central depression or ragged ulcer, which was either filled with or covered by a laminated and heaped-up hyperkeratotic and parakeratotic scale (Fig. 5). Occasionally the scale covering the ulcerated surface was absent or replaced by a crust composed of fibrin and epithelial debris with occasional polymorphonuclear leukocytes throughout (Figs. 6 and 7). The rete pegs were thickened and elongated, particularly about the margins of the acanthotic epidermis, and dipped down into the dermis. Sometimes they were fused together, compressing or completely obliterating the dermal papillae. When an area of central ulceration was present, the extensive marginal downgrowth of the rete often suggested pseudoepitheliomatous hyperplasia. All layers of the epidermis participated in the epidermal hyperplasia. The stratum corneum was thickened by hyperkeratosis but gradually became thinned out as it approached the normal epidermis. Frequently, the central portion gave away to complete parakeratosis. The epidermis showed no loss of continuity, and there was a normal progression of maturation. Malignant dyskeratotic changes, such as keratinization of individual cells, clumping of nuclei, and frequent mitoses suggestive of senile keratosis or squamous-cell carcinoma, were noticeably absent, although penetration of the elongated epidermal processes deep into the dermis and occasional slight disorientation of the palisaded basal-cell layer were sometimes observed. In places spongiosis and intracellular edema of the epidermis led to vesiculation which appeared to be the beginning stage of ulceration. In Case 25 there was a small focus of benign dyskeratosis which presented the histologic features of Darier's disease, but this was thought to be purely fortuitous since it was not reproduced in any other instance. Dermis. The fundamental changes in the dermis were essentially similar in all lesions examined, although they varied greatly in degree and extent. These changes consisted of (1) degeneration of the collagenous connective tissue which was associated with a fairly intense edema, (2) formation of cystlike spaces and clefts which often communicated with the surface ulceration, (3) ingrowth of highly vascularized connective tissue and (4) chronic inflammatory infiltrate. The most striking feature was the degeneration of the dermal collagen, which

10 FEB CHONDRODERMATITIS HELICIS Fro. 6 (upper). Evacuated punched-out ulcer from which the surface scale has been removed. Cartilage shows cystic degeneration and bulbous expansion. Hematoxylin and eosin. AFIP Ace. 1637S6. X 4S. FIG. 7 (lower). Surface ulceration plugged by an inflammatory crust. Fibrinoid necrosis and granulation tissue are prominent. Hematoxylin and eosin. AFIP Ace X

11 136 SHUMAN AND HELWIG VOL. 24 occurred either in bands and isolated foci disrupting the normal collagen bundles in the affected area, or as diffuse involvement of the entire dermis (Fig. 8). The collagen fibers were swollen, homogenized and fused, producing compact bundles. There were diminution, shrinking and pyknosis of nuclei and loss of fibrils in the collagen so altered. The collagen stained intensely acidophilic in hematoxylin-and-eosin preparations. A fairly constant change was the fibrinoid necrosis of collagen progressing to dissolution and conversion into amorphous debris. It was present either in scattered foci or as a single large focus which stood out clearly, most conspicuous along the upper margin of the papillary dermis and directly above the altered perichondrium. Edema was sometimes fairly prominent in both the papillary and reticular layers, separating the less altered collagen fibers which assumed a granular appearance and sometimes lost their sharp definition. Often the fibers throughout the altered dermis were fragmented and ragged. In approximately 30 per cent of the lesions a cystlike space was located at various levels in the dermis, frequently extending to the cartilage, and generally communicating directly with the surface ulcer to form a small sinus tract. The lining wall of the cystlike space was formed by the surrounding connective tissue, which in many instances was completely transformed by fibrinoid necrosis. Since the floor of the cyst was often layered by fibrinoid collagen, which was also contained free within the space itself, it was tempting to assign it an origin in an evacuated focus of disintegrated connective tissue. In 3 instances the lumen contained a small amount of purulent exudate; a small venule filled by organized thrombus had eroded into the cyst in a fourth case and a detached portion of auricular cartilage protruded into the cyst in a fifth. At the periphery a rich vascular proliferation originating from the vessels situated outside the lesion invaded and partly replaced portions of the altered collagen. It is remarkable that similar invasion and replacement were never observed in foci of fibrinoid necrosis (Fig. 9). Surrounding these vascular channels, some of which were in the nature of dilated lymphatic spaces, were numerous plump fibroblastic and epithelioid cells, which occasionally simulated the pattern of a glomus tumor or hemangioma. A mild to moderate inflammatory infiltrate made up predominantly of lymphocytes and plasma cells was scattered here and there, concentrated chiefly about the newly formed vessels in the upper and mid dermis (Fig. 10). Elastic tissue stains showed extensive destruction of the elastic fibers where the dermal changes were most prominent and in the heavy inflammatory foci. In other areas the elastic fibers were swollen, fragmented and clumped, and often merged with the collagen in so-called collacin formation reminiscent of that observed in senile elastosis. Reticulin staining showed a dense framework of argyrophilic fibers in the granulation tissue. Stains for amyloid were negative. In the region of the nodule the adnexal structures were atrophic or absent and only an occasional nerve filament was observed. Where the cutaneous nerves remained intact, there was slight perineural fibrosis, but this was uncommon. No obi iterative or degenerative changes of significance were noted in the arterioles or venules surrounding the dermal portion of the lesion. Perichondrium and cartilage. The perichondrium invariably showed evidence

12 / * F I G. 8 (left upper). Area of degenerated collagen in the mid-dermis. T h e perichondrial margin and adjacent dermis also show a small focus of fibrinoid necrosis. Hematoxj'lin and eosin. A F I P Ace X 76. F r o. 9 (right upper). Necrotic collagen surrounded but not invaded by the vascular granulation tissue. Hematoxj'lin and eosin. A F I P Ace X 220. F I G. 10 (left lower). Richly vascularized granulation tissue infiltrated by chronic inflammatory cells. Hematoxylin and eosin. A F I P Ace X 75. FIG. 11 (right lower). Detail of the degenerated cartilage. Hematoxylin and eosin. A F I P Ace X

13 138 SHUMAN AND HELWIG VOL. 24 of perichondritis. In the affected area the dense perichondrial connective tissue was thickened and layered, producing a hyalin-like cap which sometimes resulted in a cartilaginous exostosis protruding into the dermis. In some instances the changes were less prominent but more severe and consisted of slight thickening, homogenization, and fibrinoid necrosis, resembling those observed in the dermis. Fibrinoid necrosis often occurred in the hyaline cap as well. The cartilage on occasion showed the most prominent changes, but in a few instances it appeared to be normally formed. The most frequent alteration of the elastic cartilage was swelling of the interstitial ground substance and chondrocytes, resulting in separation of the latter and loss of orientation. In more advanced lesions there was subsequent loss of homogeneity of the matrix with unmasking of the fibrillary component (Fig. 11). The chondrocytes in such areas were sparse and frequently disappeared, leaving a clear space or cyst. Cysts and cleftlike spaces thus formed usually contained an amorphous pink-staining material. When these clefts and cysts were produced at the periphery, the cartilage became thinned out and roughened, whereas the centrally located cysts usually produced a greatfy expanded and bulbous tip. Granulation tissue ingrowth, blood vessel penetration and fibrous partitioning of the cartilage were encountered but only rarely. Sections stained for elastic tissue fibers showed fragmentation or complete disintegration of these elements in the degenerated portions. Diffuse metachromasia was noted in toluidine blue preparations. Bone formation was observed in 1 case, but calcification of the cartilage was not encountered in a single instance. DISCUSSION Our analysis of 100 cases of chondrodermatitis helicis tends to indicate that the lesion is a separate and distinct clinicopathologic entity, as previously maintained by others. This concept gains support from the relative uniformity of the clinical picture and of the unique histologic features. While no single microscopic feature, of itself, was believed to be pathognomonic, the combination of the following changes was regarded as characteristic and diagnostic: (1) nodular epidermal hyperplasia, depressed or ulcerated centrally and capped by a hyperkeratotic and parakeratotic scale, (2) edema, homogenization, and fibrinoid degeneration of collagen, with or without cyst formation, (3) proliferation of a richly vascularized granulation tissue associated with a chronic inflammatory infiltrate and (4) perichondritis, with or without degeneration of the elastic cartilage. When these histologic changes are associated clinically with a painful, crusted or scale-topped nodule situated on the upper pole of the helix or anthelix (rarely on other portions of the ear), the diagnosis can be firmly established. The histologic picture of chondrodermatitis helicis may at times resemble that of the nonspecific keratoses, senile keratosis, carcinoma and various inflammatory processes, but the changes are usually so characteristic that they should not give rise to a mistaken pathologic diagnosis. From a clinical diagnostic standpoint the lesion must be differentiated from basal-cell carcinoma, squamous-cell carcinoma, cutaneous horn, senile keratosis, squamous papilloma,

14 FEB CHONDRODERMATITIS HEL1CIS 139 clavus, tophi, tuberculosis verrucosa cutis, discoid lupus erythematosus and numerous other keratotic and inflammatory lesions. As a rule, this distinction can be made without microscopic studies when the clinician is familiar with the entity. Ebiologic considerations. The etiology of chondrodermatitis helicis is obscure. Numerous authors including Winkler, Foerster, Halter, Ebenius and others have pointed out the embryologic and anatomic variations in the external form of the human ear, especially of that portion where the nodule develops most frequently. Foerster regarded it of interest and possible etiologic importance that the lesion occurred only in males and was situated on that part of the ear which, according to Schwalbe's and Streeter's investigations on the development of the human auricle, had been subjected to the greatest embryologic variation. In this regard he summarized the morphologic characteristics of the human auricle based on the studies mentioned and emphasized the belief of these 2 investigators that variations in the form and modeling of the ear were more frequent in the male than the female. Winkler cited Jadassohn's observations that changes in the cartilage could be seen clinically where whitish cartilage is visible through the thinned-out skin, and where in addition, there are elevations, retractions and nodule formation. On that basis Winkler suggested that cartilage so altered may be the point of origin of the lesion, since it is a point of minor resistance and can be readily damaged by traumatic, chemical or thermal irritants. We have reviewed a large series of classical studies on the development of the human auricle, including those of Streeter, His, Tartaroff, Gradenigo, Schwalbe, Munch, Hammar, Keith, Henneberg, Sera, and others, and are of the opinion that the mass of evidence favors the belief that there are marked anatomic variations of the human ear, particularly in the male, at the site where the painful nodule is most commonly encountered. It would appear reasonable, therefore, to consider the possibility that these developmental abnormalities resulting in cartilaginous aberrations may be points of diminished resistance. Halter has emphasized that circulatory disturbances are a factor of major importance and has pointed out that the vascular supply of the upper helix is so arranged as to favor the development of the disorder. Our own observations seem to bear this out. In our material it was apparent that there was a circulatory impoverishment in the region where the lesion was produced. This area appeared to be almost devoid of vessels of any major size, particularly at the free margin of the cartilaginous plate. Where small and large arterioles were present, the lesion seemed to end abruptly. Occasionally when these arterioles were encountered at the peripheral margins of the lesion, their walls were slightly thickened, but this was never of major significance. The skin covering the convex surface of the ear at its free margin is thin and closely adherent to the cartilage, since it is not provided with a distinct subcutaneous layer. It does not appear to support any major vessels and does not possess a liberal anastomosis. Assuming that there is a relationship between the peculiar anatomic development of the cartilage and the disturbances in the circulation which may produce

15 140 SHUMAN AND HELWIG VOL. 24 TABLE 6 POSSIBLE INITIATING FACTORS BASED ON PATIENTS' STATEMENT ETIOLOGY NUMBER Total 73* Sleeping on side where growth appeared 31 Wearing tight-fitting headgear 3 Injury to ear 3 Frostbite 5 Sunburn 2 Insect bite 1 Telephone receiver 1 Ear grasped during numerous ear examinations 1 Combinations Sleeping on side where growth appeared and tight-fitting headgear 3 Sleeping on side where growth appeared and injury 4 Sleeping on side where growth appeared and frostbite 8 Sleeping on side where growth appeared and sunburn 4 Sleeping on side where growth appeared and on coarse pillows 1 Frostbite and sunburn 1 Sleeping on side where growth appeared and earphones 1 Sleeping on side where growth appeared, frostbite and sunburn 2 Sleeping on side where growth appeared, injury and frostbite 1 Sleeping on side, tight-fitting headgear, frostbite and sunburn 1 * Not stated, 27. a point of minor resistance, it follows that some precipitating factor, as suggested by Winkler, must initiate the development of the lesion. Inquiry into the clinical history in our cases for possible inciting factors revealed a history of direct and indirect trauma in 73 instances. Fifty-six patients associated the presence of the nodule with pressure on the ear caused by sleeping on the affected side. This was the only reason given in 31 instances. In 26 others it was mentioned in association with other factors such as wearing tight-fitting headgear, direct injury, frostbite and sunburn. In 1 case the physician noted that a nodule developed at the site where the ear had been grasped during 16 examinations for a middle ear infection. Table 6 shows the incidence of possible inciting factors. Although it could not be proved that the factors enumerated in Table 6 played any significant part in the development of chondrodermatitis helicis, they have been given emphasis by numerous authors. Ebenius thought that the distinct difference in frequency between right and left-sided lesions was related to the position in sleep whereby the right ear is subjected to more prolonged pressure. The rarity of the disease in women is difficult to explain on the basis of these observations. It has been pointed out, however, that the female ear is subject to less anatomic variation in the folding of the helix, has less prominent Darwinian points, and a greater padding of soft tissue over the free border of the helix. Culver also believed that the female ear may be protected by the cushioning ef-

16 FEB CHONDRODERMATITIS HELICIS 141 feet of the mass of hair about the ears. The only adequate microscopic description of the lesion in women that has come to our notice is in 1 case reported by Rost; we were unable, therefore, to compare the lesion in women with the nodules Ave have studied. The nodular lesions of the anthelix reported by Ivlauder and observed only in women were said by that author to be clinically unlike the lesions which characterize chondrodermatitis nodularis chronica helicis. Other examples with different pathologic features, described as chondrodermatitis nodularis chronica helicis, probably belong to this unrelated category, being similar only insofar as they are painful nodules. These problems of etiology and pathogenesis are further complicated by the controversy which exists concerning the region in which the earliest histopathologic changes occur. The cartilage and its perichondrium, the dermis and the epidermis have all been implicated as the site of origin. In our attempts to explain the histogenesis of the condition we studied our cases after they were separated into various groups according to duration estimated from the time of clinical onset to excision. It was hoped that by this method the earliest group would provide a clue as to the initial changes and point of localization. It was then anticipated that the subsequent groups would enable us to trace the full development and natural course of the disease. In the early lesions the fibrinoid necrosis of collagen was the single most striking histologic.finding. This change was most conspicuous in the upper dermis and in the adjacent perichondrium. The foci of fibrinoid necrosis in some instances merged with one another, or the entire zone between the epidermis and perichondrium, including the latter, was involved as a single major focus. When, in addition, the auricular cartilage showed degenerative changes (and this was usually the case) one gained the impression that the chondritis was primary and that the disturbance in the soft tissues and epidermis developed secondarily, or the secondary infection permitted the soft tissue changes to be maintained by the degenerated cartilage. Winkler, Davies and others have held this view, although Ebenius has correctly pointed out that while there were cases with pronounced chondritis, other typical cases of chondrodermatitis nodularis chronica helicis were seemingly devoid of cartilaginous changes. Edema was also prominent in early lesions, and in most of them a moderate to heavy infiltrate of chronic inflammatory cells of the lymphocytic and plasma cell varieties. Occasionally a few polymorphonuclear leukocytes were present, but abscess formation was never encountered. Giant cells were noticeably absent except in 2 of our cases. The capillary lymphatic and blood vessels were greatly dilated and a proliferation of fibroblastic cells was taking place. The elastic fibers had disappeared in the area of fibrinoid change and in the region where the inflammatory reaction was most pronounced. The epidermis overlying the fibrinoid collagen and inflammatory zone had become thickened and showed prominent intercellular and intracellular edema and in some cases these changes had progressed to colliquation and ulceration. In cases of 3 to 6 months the edema and chronic inflammatory reaction appeared to have subsided. Homogenization and sclerosis of the dermal collagen were more conspicuous than in the earlier lesions, but the fibrin-

17 142 SHUMAN AND HELWIG VOL. 24 oid foci were never completely removed unless evacuated when an ulcer formed at the surface. Generally the perichondrium at this stage showed layering, frequently forming the so-called hyaline cap in which there was cartilaginous metaplasia. It was usually at this stage that a sinus tract extending to the perichondrium was first noted. The lesions in all subsequent groups appeared to be maintained essentially unchanged. In none of our cases did the nodule disappear spontaneously, although the characteristic pain subsided in a few cases. The recurrent nodules in several of our cases which had been previously treated by various methods were almost identical, microscopically and clinically, with the primary lesion. In several instances 2 or more lesions appeared at or in immediate proximity to the site of removal. In cases treated by x-ray for some time prior to excision, the cutis showed extensive fibrosis of the collagen bundles, and the epidermis had undergone dyskeratotic changes bordering on senile keratosis. It is possible that Ebenius, who raised the question of possible precancerous changes in individual cases, had observed such postirradiation changes. In none of our cases did the epidermal changes progress to squamous-cell carcinoma. Because the outstanding changes were always manifested by degeneration of the collagenous connective tissue of the dermis and perichondrium, the possibility was suggested that a relationship might exist between chondrodermatitis nodularis chronica helicis and a wide variety of conditions showing the same basic pathologic features, particularly the localized collagen vascular diseases. Klemperer, however, has stressed the fact that fibrinoid changes can be provoked by a variety of factors and that their occurrence in different pathologic entities must not be interpreted as indicating that the diseases are identical or even related. It has also been noted by numerous investigators that allergic factors, toxins, specific bacteria, etc., and other factors may be responsible for the production of fibrinoid degeneration and that this change may also be encountered in inflammatory foci. Tsai Tung Wu has demonstrated that squeezing of the rat's tail results in fibrinoid alteration in the absence of specific sensitization. It would appear that the collagen changes in the painful nodule are unrelated to specific sensitization but result simply from mechanical tissue damage or climatic sensitivity. On the basis of our observations it was impossible to draw specific conclusions regarding the point of origin or pathogenetic background of chondrodermatitis helicis. The characteristic dermal and epidermal changes were invariably present, whereas the cartilaginous changes varied greatly in degree and were absent or minor in approximately 20 per cent of our cases. This suggested that the initial fixed reaction may have taken place in the dermis, the injury having been transmitted through the epidermis. It is logical to assume, however, that when the initial injury is severe, the process may involve skin and cartilage simultaneously or it may extend secondarily upon the cartilage. Regardless of how the changes develop, it appears fairly certain that once the dermis is altered the condition is maintained and even intensified and the possibility of complete healing is remote. We are inclined to accept the view held by other writers (Foerster,

18 FEB CHONDRODERMATITIS HELICIS 143 Halter, Ebenius) that circulatory factors and topographic variations create a point of minor resistance on which the superimposition of a slight injury is sufficient to initiate the development of the lesion. SUMMARY One hundred cases of chondrodermatitis helicis have been analyzed and the pertinent literature reviewed. The histopathologic features which provide fairly reliable criteria for diagnosis are: (1) nodular hyperplasia of the epidermis, (2) fibrinoid alteration of the dermal collagen, (3) proliferation of a richly vascularized granulation tissue associated with an inflammatory infiltrate and (4) perichondritis with or without degenerative changes of the auricular cartilage. Clinically the lesion was characterized by a small painful nodule situated on the helix in 85 instances and on other specified portions of the ear in 12. It was centrally depressed or ulcerated and surmounted by an adherent scale or crust. In this study the nodule was limited entirely to men. The lesion can be readily differentiated from other benign and malignant keratotic and inflammatory conditions, although the clinical.and pathologic pictures were mistaken for senile keratosis or carcinoma in a relatively high percentage of cases. Several possible etiologic and pathogenetic factors were suggested although no definite conclusions were reached. On the basis of our studies it is believed that chondrodermatitis helicis is an acceptable clinicopathologic entity. REFERENCES 1. BELGODERE, G.:.Nodule douloureux de l'oreille. Ann. de dermat. et syph., 10: S73-S76, BROERS, J. H.: Painful nodules on margin of auricle. Nedei'l. tijdschr. v. geneesk., 2: , CAROL, W. L. L., AND VAN HAREN, H. B.: liber Clavus helicis, bzw. Chondrodermatitis nodularis chronica helicis. Dermatologica, 83: , CULVER, G. D.: Painful ear nodule of Winkler and Foerster; report of case. California & West. Med., 31: , DA VIES, J. H. T.: Chondrodermatitis nodularis chronica helicis. Rev. franc, de dermat. et de v6n6rol., 4: 3SS-390, 192S. 6. DUBREUILH, W., AND PIGEARD DE GURBERT, M.: Le nodule douloureux de l'orielle. Ann. do dermat. et syph., 9: , 192S. 7. EBENIUS, B.: Chondrodermatitis nodularis chronica auricula. Acta radiol., 22: , FOERSTER, 0. H.: A painful nodular growth of the ear. J. Cutan. Dis., 36: , FOERSTER, 0. H.: Painful nodular growth of the ear. Arch. Dermat. & Syph., 11: , Fox, H.: Chondrodermatitis nodularis chronica helicis. Arch. Dermat. & Syph., 19: , HALTER, K.: Zur Pathogenese der Chondrodermatitis nodularis chron. helicis. Dermat. Ztschr., 73: , KLAUDER, J. V.: Nodule of the ear simulating chondrodermatitis nodularis chronica helicis. Arch. Dermat. & Syph., 22: S33-S3S, MEIROWSKY, E.: Zur Kenntnis der sogennanten Chondrodermatitis nodularis chronica helicis. Dermat. Wchnschr., 88: 2S9-292, MITCHELL, J. H.: Chondrodermatitis nodularis helicis. Arch. Dermat. & Syph., 7: , POTII, D. O.: Sex incidence of chondrodermatitis nodularis chronica helicis; case reports. Texas State J. Med., 33: 19-21, ROST, G. A.: Uber die sogenannte Chondrodermatitis nodularis chronica helicis. Wein. med. Wchnschr., 76: , 1926.

19 144 SHUMAN AND HELWIG VOL ROXBURGH, A. C: Chondro-dermatitis nodularis chronica helicis. Brit. J. Derniat., 39: , IS. SCHWA LB E, G.: Das iiussere Ohr. Handbuoh des Menschen. Jena: K. von Bardeleben, 1897, vol. 5, pt. 2, pp SEQUEIRA, J. H.: Diseases of the Skin. Ed. 3, London: Churchill, 1919, p STREETER, G. L.: Development of the Auricle in the Human Embryo. Contributions to Embryology, Carnegie Institute of Washington 14: Nos , WISE, F.: Painful nodular lesion on right ear. Arch. Derniat. & Syph., 11: 6S0-6S2, WINKLER, M.: Knotchenformige Erkrankung am Helix. Chondrodermatitis nodularis chronica helicis. Arch. f. Derniat. u. Syph., 121: 27S-2S5, WORINGER, F., AND ZOON, J. J.: Note prijliminaire sur I'abondance anormale de filets nerveux dans le nodule douloureux de l'oreille. Bull. Soc. franc, de derniat. et syph., (Reunion derniat., Strasbourg), 45: 66S-671, 193S.