# Metastatic Crohn's Disease of the Ear

Authors: J. H. Chuah; D. S. Kim; C. Allen; L. Hollis
Journal: International Journal of Otolaryngology
 (2009)

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## Abstract

Objective. We reported a very rare case of metastatic Crohn's disease involving the retro-auricular region. Method. A case report and a review of literature concerning metastatic Crohn's disease.
Results. Metastatic Crohn's disease is an uncommon extraintestinal cutaneous manifestation of Crohn's disease and a very rare case involving the retro-auricular region is reported here. Given the limited existing literature little is known about this condition. The skin lesions appear to have a predilection for the lower trunk and genitalia regions. There is no clear association with the severity of Crohn's disease and in some cases, the cutaneous lesions predate the onset of gastrointestinal Crohn's disease. Treatment with immune-modulating medications together with the antitumour necrosis factor monoclonal antibody therapy appears to offer the best chance of remission. Conclusion. By reporting this interesting and rare condition we also hope to highlight the importance of considering underlying chronic systemic disorders, such as Crohn's disease, when presented with skin lesions resistant to simple local treatments.


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## Body

## 1. Introduction

Metastatic cutaneous Crohn’s disease is a rare manifestation of
Crohn’s disease. Its hallmark features include the presence of cutaneous
granulomatous lesions noncontiguous with the gastrointestinal tract or fistulae
[1]. The clinical appearance can vary and biopsy is required to
confirm the diagnosis.We report a very rare case of metastatic Crohn’s disease
affecting the ear in a man with longstanding Crohn’s disease. We aim to
highlight the need for clinical suspicion of systemic diseases that may present
as a localised ear lesion, in particular, the rare entity of metastatic Crohn’s
disease.


## 2. Case Report

A 43-year-old man with an 18 years history of Crohn’s disease
developed a severe erythematous and exudative skin lesion in the right postauricular
cleft region. Prominent purulent discharge and microabscesses suggested an
infective lesion. The patient had recently completed a course of the
therapeutic drug infliximab, a monoclonal antibody for tumour necrosis factor
(TNF), which is a key inflammatory agent in systemic inflammatory conditions.
Gastroenterologists were concerned that the skin lesion was an infective
complication of the new therapeutic regimen due to a possible immunosuppressive
action. Several antibacterial agents were used with no clinical benefit, and
the patient was then referred to the ENT department for a second opinion.
Concurrently, the patient had a recurrence of severe perineal Crohn’s disease
and warranted further course of infliximab. However, given the issue of
possible association with the skin lesion, the treatment decision was deferred
pending resolution of the skin condition.Initial ENT examination excluded an otitis externa causing local
inflammation from overspill onto the adjacent skin area. Appearance of the skin
lesion was in keeping with either an infection or severe eczematous
inflammation. A skin swab for culture was taken and triadcortyl ointment
applied. During the initial consultation, the patient complained of a similar
lesion starting in the umbilicus region. The swab result reported heavy growths
of beta-hemolytic streptococcus andStaphylococcus aureus with moderate anaerobes.
Triple therapy with flucloxacillin, erythromycin, and metronidazole proved to
be ineffective. During this period, there was gradual deterioration of the
lesion, which prompted a biopsy of the periauricular skin lesion. The
pathologist reported the biopsy to represent a chronic noncaseating
granulomatous lesion consistent with a diagnosis ofmetastatic Crohn’s
disease (please refer pathology report).Subsequently, with dermatology advice, treatment with high-dose
betnovate cream, oral steroids, and infliximab resulted in rapid remission of
the skin condition (both the postaural and umbilical areas) and the perineal
Crohn’s disease.


## 3. Pathology Report

Histological haematoxylin
and eosin sections (Figure1) demonstrated chronic inflammation of the dermis with
admixed epithelioid and giant cell granulomas. Schaumann’s bodies were seen within the giant
cells. There was no caseation, and elastic fibres were absent from the
granulomas. Furthermore, there was an absence of fungi, staining for acid and
alcohol fast bacilli were
negative, and birefringent foreign bodies were not seen. These appearances were reported as
consistent with cutaneous Crohn’s disease.Figure 1
Skin
biopsy (x10 objective, haematoxylin,
and eosin stain) showing a dermal chronic inflammatory cell infiltrate with noncaseating
epithelioid and
giant cell granulomas. Although there are no pathognomonic features, this is
consistent with metastatic Crohn’s disease.


## 4. Discussion

Metastatic cutaneous Crohn’s disease refers to the presence of a
skin lesion containing epithelioid
and giant cell granulomas as seen in the affected bowel segments of patients with
Crohn’s disease, but is physically separated from the gastrointestinal tract [2].
This condition is distinct from other more well-characterized cutaneous
manifestations which include pyoderma gangrenosum, erythema nodosum,
polyarteritis nodosa, and epidermolysis acquisita. Although the estimated
incidence of cutaneous manifestation of Crohn’s disease varies widely between
2% and 44% [3],
true metastatic cutaneous Crohn’s disease is exceedingly rare.Metastatic Crohn’s disease does not appear to have any distinct
relationship with the severity of the Crohn’s bowel disease [3], and indeed may
even precede gastrointestinal involvement [3].
However, there is a suggestion in the limited literature available that
there may be an association with perianal Crohn’s disease [3]. Of the reported
cases of metastatic Crohn’s disease involvement primarily of the lower limbs
and trunk regions with a predilection for skin fold areas and genitalia has been described [4].
There has been only one reported case of facial involvement documented more
than 30 years ago.To date, given the rarity of the condition, there is no
gold-standard therapy for metastatic Crohn’s disease [2]. The use of systemic
and topical steroids, antibiotics, and immunosuppressive agents such as
azathioprine, sulfasalazine, and methotrexate has been described with variable success [4]. The
use of Infliximab, an antitumour necrosis factor monoclonal antibody, as one of
the latest treatment options has been described in a few case reports to be
more effective in maintaining disease remission, especially with repetitive
administration and when used concomitantly with other more mainstream therapies
[5–7]. Further
evidence is needed to substantiate this. However, given their recent introduction
to clinical use, in addition to a better evidence for clinical efficacy in
various disorders, new side effects may also become apparent with time and
should be carefully evaluated.In the case being presented, the possibility of an unusual
diagnosis for the skin lesion was raised by several features: the sudden onset
of postauricular rash in a gentleman with no previous ear or skin condition;
the aggressive nature of the purulent rash; the observed resistance to several
common treatments used including multiple antimicrobial agents and topical
steroid cream. The diagnosis of the rare cutaneous Crohn’s disease was first
suggested by the biopsy demonstrating chronic granulomatous inflammation in a
patient known to be suffering from active Crohn’s disease. Biopsies
demonstrating chronic granulomatous inflammation of the skin require the
pathologists to consider other and more common disorders including sarcoidosis,
tuberculosis and fungal or parasitic infections, foreign body granulomas, and
annular elastocystic granuloma. Histological analysis failed to demonstrate any
of the pathognomonic features of the other granulomatous conditions. The
diagnosis of metastatic Crohn’s was further supported by the rapid remission
both of the skin rash and perineal Crohn’s disease in response to treatment
with high-dose
local steroid cream, oral steroids, and concomitant infliximab infusion.Despite its rarity, a meticulous evaluation including a thorough
history and examination, and biopsy in cases unresponsive to simple measures,
together with a high index of clinical suspicion in those with an established
diagnosis or presenting with concomitant clinical features of Crohn’s disease,
may provide early diagnosis of this skin condition which can be disfiguring and
emotionally distressing to the patient. The diagnosis of metastatic Crohn’s
disease may be relatively easier in patients with established Crohn’s disease
compared to those cases where the skin lesions precede bowel involvement. In
the latter cases, diagnosis by biopsy should then lead to appropriate
counseling about the diagnosis of Crohn’s disease and the future onset of the
more significant bowel disease.We hope by reporting this rare and interesting case that we may highlight the
need to consider systemic diseases, in particular, chronic inflammatory
conditions such as Crohn’s disease, as causes of skin lesions of the ear and
face that are unresponsive to simple treatments.


## 5. Summary

(i)
Metastatic Crohn’s disease is an uncommon cutaneous manifestation of Crohn’s disease.(ii)
Metastatic Crohn’s lesions have a predilection for the
skin fold, limbs, and trunk.(iii)
Metastatic
Crohn’s involving ear is extremely rare.(iv)
To date, there is no gold standard treatment for
metastatic Crohn’s disease.(v)
Treatment with
repetitive administration of Infliximab together with other mainstream
therapies is promising in maintaining disease remission.



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*Source: 871567-2009-03-16.xml*