DOI: 10.5152/eurjrheumatol.2014.

140048

Case Report

Chondrodermatitis nodularis chronica helicis in a

patient with systemic sclerosis associated with primary
biliary cirrhosis (Reynolds syndrome): A case report
Walter Alberto Sifuentes Giraldo1, Carmen Gonzlez-Garca2, Elena de las Heras Alonso3,
Carlos de la Puente Bujidos1

Abstract
Chondrodermatitis nodularis chronica helicis is a rare non-neoplastic inflammatory and degenerative process of the external ear, characterized by necrobiotic changes in the dermis that extend down to the perichondrium. This condition has been occasionally reported in
patients with limited cutaneous systemic sclerosis but not in those with concomitant primary biliary cirrhosis; this association is known
as Reynolds syndrome. We report a 70-year-old woman diagnosed with primary biliary cirrhosis at age 47 and with limited cutaneous
systemic sclerosis at age 54 who developed a painful ulcerated nodule on the helical rim of the left ear shortly after the last diagnosis.
The lesion was excised because of the suspicion of malignancy, but the histopathology was consistent with chondrodermatitis nodularis
chronica helicis. Although this condition is infrequent, it is necessary to know, because it may occur in patients with systemic sclerosis
and be mistaken for neoplasms, such as basal cell and squamous cell carcinoma, and these patients have an increased risk for the development of skin malignancies.
Key words: Chondrodermatitis nodularis chronica helicis, systemic sclerosis, primary biliary cirrhosis, Reynolds syndrome, skin malignancies

Introduction
Systemic sclerosis (SSc) is a multisystem connective tissue disease, the hallmarks of which are autoimmunity, inflammation, functional and structural alterations in small blood vessels, and interstitial and vascular
fibrosis in the skin and internal organs (1). Primary biliary cirrhosis (PBC), a chronic cholestatic liver disease
characterized by immune-mediated chronic non-suppurative cholangitis and associated with anti-mitochondrial antibodies (AMAs), is considered the most common liver disorder in patients with SSc, with an estimated prevalence of 8% and occurring mostly as the limited cutaneous variant (lcSSc) (2). The association
of SSc and PBC is known as Reynolds syndrome (RS) because of its original description in the early 1970s (3).
Chondrodermatitis nodularis chronica helicis (CNCH) is a rare non-neoplastic inflammatory and degenerative process of the external ear, characterized by necrobiotic changes in the dermis that extend down to
the perichondrium (4). This condition has been occasionally reported in association with SSc (5, 6) but not
in patients with concomitant PBC. We report a patient with RS who developed CNCH.
1 Department of Rheumatology,
University Hospital Ramn y Cajal,
Madrid, Spain
2 Department of Pathology, University
Hospital Ramn y Cajal, Madrid, Spain
3 Department of Dermatology,
University Hospital Ramn y Cajal,
Madrid, Spain
Address for Correspondence:
Walter Alberto Sifuentes Giraldo,
Department of Rheumatology,
University Hospital Ramn y Cajal,
Madrid, Spain
E-mail: albertosifuentesg@gmail.com
Submitted: 10.06.2014
Accepted: 08.07.2014
Copyright 2014 Medical Research and
Education Association

Case Presentation
A 70-year-old white female was diagnosed with PBC at age 47, based on elevated levels of alkaline phosphatase (672 U/L), positive AMA test, and liver biopsy with a periportal inflammatory infiltrate and mild
portal fibrosis (stage I of Scheuers classification). Since then, she was treated with ursodeoxycholic acid
(Ursochol; Zambon, Madrid, Spain). Alkaline phosphatase levels were stable, and pruritus and signs of portal hypertension were absent. Concomitant with the diagnosis of PBC, the patient began to have episodes of Raynauds phenomenon and progressively developed sclerodactyly, xerostomia, xerophthalmia,
and esophageal dysmotility, being diagnosed with lcSSc at age 54. The physical examination at that time
showed telangiectasia on the fingers, a beaked nose, and mild perioral furrowing; capillaroscopy demonstrated giant capillary loops with minimal loss of capillaries; and serologic investigations found increased
titers of antinuclear antibodies (1/320) and positive anti-centromere antibodies.
Shortly after the diagnosis of lcSSc, the patient noticed a painful nodule that involved the helical rim of the
left ear (Figure 1). The nodule grew slowly over the course of several months, and she stated that the pain

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Sifuentes Giraldo et al. Chondrodermatitis nodularis helicis in Reynolds syndrome

was exacerbated by rubbing the telephone
and supporting the head on the affected side
during sleep. On physical examination, the
lesion measured 10 mm, erythema was present, and a central 3-mm-diameter ulcer was
covered by a crust. The right ear was normal.
Because a skin malignancy was suspected,
surgical resection of the lesion was performed.
Histopathologic study revealed that the ulcer
was composed of necrotic and granulation tissue in the dermis, with a marked mixed acute
and chronic inflammatory cell infiltrate and
isolated multinucleated giant cells. The epidermis surrounding the ulcer showed reactive
acanthosis, and there were elastic fiber degeneration and telangiectatic vessels in the underlying dermis. The cartilage showed chondroid
matrix degeneration and chronic inflammatory infiltrate that was immediately adjacent
(Figure 2). These findings were consistent with
CNCH. No dysplasia or malignancy was identified.
The patient experienced complete resolution
of the lesion after the resection. However, she
developed a recurrence in the same location 2
years after and underwent a new surgical reFigure 1. Nodule of chondrodermatitis
nodularis chronica helicis on the helix
showing erythema, superficial scaling, and a
central crust

section. There have been no recurrences since

then.

Discussion
Clinically, CNCH manifests as a single (rarely
multiple or bilateral), exquisitely tender, dull
erythematous nodule on the helix (less frequently on the antihelix, scapha, or concha)
and varies from 3 to 10 mm. The surface of the
lesion is often covered with a scale or crust
over an underlying central depression. CNCH
shows a marked male dominance (up to 80%
of cases) and occurs typically in the elderly
(average age 58-72 years) (6, 7). Some pediatric cases have rarely been reported (8). Histopathology of the lesions demonstrates dermal
inflammation associated with either a central
hyperkeratotic plug or ulceration and crust.
Ulcer margins often show acanthosis, hyperkeratosis, and numerous dermal telangiectasias.
Cartilage beneath the granulomatous and fibrotic dermis is disrupted, hemorrhagic, and
even necrotic, although occasionally it can appear undamaged (4, 6, 7).
The pathogenesis of CNCH has not yet been
fully elucidated. The Swiss dermatologist Max
Winkler was the first to describe this condition
in 1915 and suspected the influence of thermal, traumatic, or chemical factors on the anatomically prominent cartilage portions of the
ear (7). However, lesions often appear to arise
spontaneously, and patients may not identify
a triggering factor (6). In the following decades, further causes of CNCH, such as actinic
damage, disorders of cornification, collagen
defects, and perichondrial vasculitis, were
proposed (7). Only the last hypothesis has recently received support from histopathological
studies, which have demonstrated arteriolar
narrowing in the perichondrium as the possible cause of underlying cartilage necrosis re-

Figure 2. a, b. Photomicrograph of the nodule on the helix of the left ear (a) showing a central
crateriform ulcer covered by a crust (hematoxylin and eosin; original magnification 4x). Bottom of
the ulcer (b) showing granulation tissue and degenerative changes in the cartilage (hematoxylin
and eosin; original magnification 20x)

Eur J Rheumatol 2014; 4: 161-3

sulting in CNCH (9). The association between
CNCH and autoimmune diseases has also been
observed (5, 6, 8). Newcomer et al. reported a
series of 99 cases of CNCH and found autoimmune diseases in 3 of them (2 rheumatoid
arthritis and 1 SSc) (8). Three additional cases
of CNCH in SSc have been reported by Bottomley and Goodfield (5). The ages of the patients
were 41, 54, and 47 years, respectively, and all
of them were diagnosed with lcSSc, but none
had concomitant PBC. No other predisposing
factors were identified, except the presence
of SSc (5). Our patient also developed CNCH
spontaneously, but the symptoms were exacerbated by mechanical factors. These authors
suggested that CNCH may be underreported
in SSc, because those 3 cases were identified in
a total of 23 patients (5). This condition has also
been reported in juvenile dermatomyositis (8).
Factors that may increase the risk of developing
CNCH in SSc include vascular pathology and
autoimmunity. The most characteristic vascular
finding in patients with established SSs is bland
intimal proliferation in the small- and mediumsized arteries that produce progressive luminal
occlusion (1), similar to that observed in the
perichondrial arterioles in CNCH (9). Raynauds
phenomena may also exacerbate the vascular occlusion and lead to ischemia in exposed
areas, such as the ears (5). Antibodies to denatured type II collagen have been detected in
CNCH and may contribute to its pathogenesis
(10). These antibodies also occur in SSc, but their
exact role in this disease is unknown (1). Skin disorders associated with PBC include xanthomas,
vitiligo, melanosis, xeroderma, lichen planus,
and cutaneous vasculitis (2), but CNCH has not
been previously reported.
The conditions most frequently encountered
with a similar clinical appearance to CNCH are
basal cell and squamous cell carcinoma (7). It is
important to take this into account, as patients
with SSc have an increased risk of developing
skin malignancies (1). Therefore, a biopsy of
the lesion must be performed to rule out malignancy (4). CNCH can be treated surgically
with a wedge-shaped excision or medically
with more conservative therapies, including
laser, pressure-relieving padding, collagen injections, and topical or intralesional glucocorticoids (7). Recurrence after surgical excision occurs in up to 34% of patients, as in our case (6).
In conclusion, CNCH is an inflammatory and
degenerative skin disorder of the external ear
that may occur in patients with SSc. Although
this condition is rare, it is necessary to know,
because it may be confused with skin malignancies due to its clinical appearance.

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Ethics Committee Approval: N/A.
Informed Consent: Informed consent was obtained
from patient who participated in this study.
Peer-review: Externally peer-reviewed.
Author contributions: Concept - W.A.S.G., C.P.B.; Design
- W.A.S.G., C.G.G., C.P.B.; Supervision - C,G.G., E.H.A.;
Resource - W.A.S.G., C.G.G., C.P.B.; Materials - W.A.S.G.,
C.G.G., E.H.A., C.P.B.; Data Collection&/or Processing
- W.A.S.G., C.G.G., C.P.B.; Analysis&/or Interpretation
- W.A.S.G., E.H.A., C.G.G.; Literature Search - W.A.S.G.,
C.P.B.; Writing - W.A.S.G., C.P.B.; Critical Reviews C.G.G., E.H.A.
Conflict of Interest: No conflict of interest was declared by the authors.
Financial Disclosure: The authors declared that this
study has received no financial support.

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