J. clin. Path. (1959), 12, 179.

ON THE HISTOPATHOLOGY OF CHONDRODERMATITISNODULARIS HELICIS CHRONICA

BY

JAMES CALNAN AND BRUNO ROSSATTIFrom the Department of Plastic Surgery, Churchill Hospital, Oxford

(RECEIVED FOR PUBLICATION JULY 4, 1958)

The histological features of chondrodermatitishave been well described in recent papers byCarol and van Haren (1941), Newcomer, Steffen,Sternberg, and Lichstenstein (1953), Shuman andHelwig (1954), and McConnell (1957). Theepidermis shows acanthosis, with irregularbroadening and deepening of the dermal papillaeand hyperkeratosis with keratotic plugging of thefollicles. The epithelium is often ulcerated, withfibrinous material in the base; deep to this round-celled infiltration extends down into the coriumwhere there is a proliferation of fibrovasculartissue. The latter reaction may be so markedas to resemble a vascular hamartoma. Theperichondrium may be thickened or be involvedby the down growth of the vascular granulationtissue. The underlying cartilage, often normal,

may show degenerative changes or even necrosisdue to direct involvement in the ulcer.Although the histopathology of this condition

has been repeatedly studied, several features andsymptoms draw attention to its similarity to theglomus tumour. Pain in the helix appeared to bepromoted not only by pressure but also byalteration in the temperature of the part. Also apainful cutaneous nodule, in an area where thereare arteriovenous anastomoses, is almostdiagnostic of a glomus tumour. These pointssuggested a re-examination of the histologicalspecimens of patients already treated, and a studyof new cases by more suitable histologicaltechniques.

In the past four years, 21 cases referred bydermatologists have been seen. Of these, 18 have

i 'R. iFIG. 1.-Papillary layer of the dermis in a case of chondrodermatitis helicis. Extensive infiltration of

epithelioid cells (E). Mallory-Azan; x 220.

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FIG. 2.-Artery (a) with epithelioid differentiation of the media probably intermediate portion of an arteriovenous anastomose).(b) Normal arteriole for comparison. Note the proliferation of epithelioid cells (E) in the surrounding tissue and the distinctiveclear cytoplasm of these. Mallory-Azan; x 290.

FIG. 3.-A clump of epithelioid cells: note the fine stroma. Mallory-Azan; x 83).

FIG. 4.-Infiltration of epithelioid cells (E) in the deeper layers of the stratum MAlpig'ii. Mallory-Azan; x 293.

FiG. 5.-The normal network of elastic fibres is absent in the areas of epithelioid cell proliferation (arrows). Weigert; x 53.

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HISTOPATHOLOGY OF CHONDRODERMATITIS

been treated, in one the lesion resolvedspontaneously, one refused further treatmenthaving had three lesions excised previously, andone has been retained for observation on thenatural history of the complaint.

Material and MethodsThe present study includes 21 cases, diagnosed as

chondrodermatitis, of which four have beenexamined by serial section. Specimens were fixed informol-saline, Susa, or alcohol. Paraffin sections 6 Mthick were stained with Mallory-Azan, Masson,haematoxylin and eosin, Unna-Taenzer and Weigertfor elastic tissue. The method of Cajal-De Castrofor nerve fibres was also used.

Personal ObservationsThe accepted histological features were

confirmed, but in three-quarters of the cases clearevidence was found of an unusual proliferation ofepithelioid cells of the walls of arteriovenousanastomoses, a finding characteristic of glomustumours of the type described by Masson (1924)in the finger tips.The affected area is very rich in vessels with

small lumina (Fig. 1), mainly arterioles, the wallsof which are formed by stratified layers ofepithelioid cells. It is often possible to ascertain,that such proliferation initiated from the wall ofthese vessels and then flared out into thesurrounding tissue (Fig. 2). There are also areasin which epithelioid cells have assumed the usualinfiltrative appearance of a tumour; they may bearranged in clumps or in palisade formation witha fine network of stromal tissue (Fig. 3). Thisproliferation mainly occupies the superficial layerof the dermis or the dermal papillae with littletendency to infiltrate the cartilage. Migration ofepithelioid cells from the papillae into thesuperficial layers of the epidermis has also beenobserved (Fig. 4). In several cases accompanyinginflammatory and degenerative changes werenoted: round cells and localized areas of mucoiddegeneration may be found scattered among theglomus tumour cells, mainly in the superficiallayers. Such inflammatory infiltration maycompletely mask the presence of epithelioid cellsand so lead to an incorrect conclusion.

Epithelioid cells are readily recognized insections stained by the Mallory-Azan method.The normal network of elastic fibres in the dermisis absent in the zones occupied by solid clumpsof epithelioid cells (Fig. 5). Nerve fibre stainsdid not show any unusual pattern in theinnervation of the skin. The rate of proliferationof these cells does not appear to be very high, for

R

mitoses are extremely rare. Ulceration of skinseems to be related to the lack of blood supply dueto tumour infiltration in the underlying papillae,and hence degenerative changes in the epidermisand in the cartilage could be regarded assecondary.

DiscussionThere is no doubt that the cellular proliferation

observed in many cases of chondrodermatitis,reported here, has to be regarded as a typicalproliferation of the specific cells present in thewall of arteriovenous anastomoses, which havebeen demonstrated in the human ear by Prichardand Daniel (1956). Their peculiar characteristicsare readily recognized when suitable stainingmethods are employed. It follows that whenepithelioid cells proliferate in this fashion, thelesion should be classified under the heading of" glomus tumour." It should not be inferred thatall cases of chondrodermatitis are to beconsidered glomus tumours of the helix, but somecases can be associated with the presence of asmall glomus tumour and the symptoms are likelyto be due to the latter.Cases of glomus tumour of the auricle have

been recorded by Opitz (1954), Ratzenhofer(1941), Sannicandro (1936), and Ertl (1943).None of these had been previously classified aschondrodermatitis, most likely because of theirsize (which was larger than the usual nodule) orbecause of their different situation (neither on thehelix margin nor the antihelix).The only report in which the association of

chondrodermatitis and glomus tumour is clearlydemonstrated is that of Fernaindez and Monserrat(1931), who described one case in a man aged 38.These authors concluded that this case was oneof glomus tumour and felt that all cases ofchondrodermatitis should be so classified. Stout(1935) did not accept the diagnosis, whileFreudenthal, Anderson, and Weber (1937)commented, "This case is apparently achondrodermatitis nodularis helicis and not aglomus tumour," without any further explanation.The opinion of Fernandez and Monserrat,however, is supported by photomicrographs whichshow a proliferation of epithelioid cells. Theirinterpretation was correct for that case, but weare unable to share their enthusiasm in advocatingthe classification of chondrodermatitis as glomustumour on every occasion. Only certain of thecases examined, although a majority, exhibitedevidence of proliferation of epithelioid cells forwhich the diagnosis of glomus tumour isjustified.

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JAMES CALNAN and BRUNO ROSSATTI

SummaryTwenty-one cases of chondrodermatitis

nodularis helicis chronica are reviewed. In morethan three-quarters of these cases there isevidence of extensive proliferation of epithelioidcells from the wall of arteriovenous anastomoses,and therefore such cases have to be considered assmall glomus tumours of the helix.

REFERENCESCarol, W. L. L., and Haren, H. B. van (1941). Dermatologica (Basel)

83, 353.Ertl, E. (1943). Mschr. Ohrenheilk, 77, 1c5.

Fernandez, A. A., and Monserrat, J. L. E. (1931). Sem. mid. (B.Aires), 38 (2), 1693.

Freudenthal, W., Anderson, R. G., and Weber, F. Parkes (1937).Brit. J. Derm., 49, 151.

Masson, P. (1924). Lyon chir., 21, 257.McConnell, E. M. (1957). J. clin. Path., 10, 46.Newcomer, V. D., Steffen, C. G., Sternberg, T. H., and Lichtenstein,

L. (1953). A.M.A. Arch. Derm. Syph., 68, 241.Opitz, K. (1954). Zbl. Allg. Path. path. Anat., 92, 172.Prichard, M. M. L., and Daniel, P. M. (1956). J. Anat. (Lond.),

90, 309.Ratzenhofer, M. (1941). Zbl. Allg. Path. path. Anat., 77, 173.Sannicandro, G. (1936). Dermosifilografo, 11, 424.Shuman, R., and Hel,vig, E. B. (1934). A ner. J. cliii. Path., 24,

126.Stout, A. P. (1935). Amer. J. Canicer. 21, 255.

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