Download Unilateral, nontender, vulvar swelling as the presenting sign of

doi: 10.1111/j.1346-8138.2010.00991.x
Journal of Dermatology 2011; 38: 303–307
LETTER TO THE EDITOR
Unilateral, non-tender, vulvar swelling as the
presenting sign of Crohn’s disease: A case report and
our suggestion for early diagnosis
Dear Editor,
We report a patient with metastatic Crohn’s disease
(MCD) who presented with vulvar swelling preceding
the diagnosis of gastrointestinal disease. Vulvar
involvement of Crohn’s disease (CD) is rare and difficult to diagnose, especially when it precedes the
onset of overt gastrointestinal symptoms. In our case,
the diagnosis was enigmatic because no radiological
and laboratory examinations gave a clue for diagnosis. Here, we suggest two diagnostic clues which
may help early diagnosis of vulvar MCD after reviewing reported cases of MCD in children whose vulvar
swelling or edema preceded gastrointestinal symptoms in the English-language published work.
A 10-year-old girl presented to our dermatological
clinic with a 1-month history of unilateral, non-tender,
vulvar swelling. Before visiting our clinic, she had
been treated with empirical antibiotics for 2 weeks at
local pediatric and gynecological clinics under the
impression of infectious disease but her vulvar swelling had not improved. She and her parent denied any
trauma, sexual abuse or a history of infectious disease. She had no notable medical history and did not
have any systemic symptom. Physical examination
revealed non-tender, erythematous swelling on the
left vulva (Fig. 1a,b) and small skin tags on the anal
orifice (Fig. 1c). Height and weight percentiles were
normal for her age. Laboratory test results were within
normal range except for a slightly increased erythrocyte sedimentation rate of 18 mm ⁄ h (reference range
0–15 mm ⁄ h). Magnetic resonance imaging of the pelvis showed focally enhancing subcutaneous tissue
on the anterior portion of the left labia majora which
primarily suggested focal cellulitis (Fig. 1d). We prescribed cefprozil 250 mg ⁄ day for 10 days under the
impression of infection of the vulva.
Ten days later, the lesion did not improve, so we
decided to perform skin biopsy. The histopathological finding revealed non-caseating granulomatous
inflammation throughout the dermis. The granulomas
were composed of epithelioid histiocytes, a few giant
cells and lymphocytes (Fig. 1e). Neither special stains
and cultures for bacteria, fungus and mycobacterium
nor polarizing light microscopy for foreign bodies
were positive. Chest X-ray and tuberculin skin test
were also normal. Even though the patient did not
have overt abdominal symptom, diagnostic colonoscopy was performed to rule out CD. It disclosed multiple aphthous ulcers ranging from rectosigmoid
colon to ascending colon with ileal involvement and
biopsy specimens of the colon revealed granulomatous inflammation, consistent with CD. Treatment
was commenced with oral corticosteroid and metronidazole with marked improvement in 2 months’
treatment. During one and half year follow up, several
diarrheal episodes occurred but her symptom was
generally tolerable.
Crohn’s disease is a chronic relapsing, multisystemic, inflammatory disorder of uncertain etiology,
characterized by non-caseating granulomatous
inflammation that can affect any part of the gastrointestinal tract from mouth to anus. Twenty-two to
forty-four percent of CD patients present four types
of cutaneous manifestations: (i) direct extension
from involved bowel leading to perioral or perianal
diseases; (ii) MCD; (iii) nutritionally-associated skin
lesions; and (iv) reactive lesions like pyoderma
gangrenosum and erythema nodosum.1
Metastatic Crohn’s disease is defined as noncaseating, granulomatous skin lesions that are not
contiguous to the gastrointestinal tract. It usually
presents as solitary or multiple papules, nodules or
Correspondence: Hyun-Chang Ko, M.D., Department of Dermatology, School of Medicine, Medical Research Institute, Pusan National
University, 305 Guduk-ro, Seo-gu, Busan 602-739, Korea. Email: [email protected]
2010 Japanese Dermatological Association
303
J.-H. Mun et al.
(a)
(d)
(b)
(c)
(e)
Figure 1. Erythematous swelling on left vulva (a,b) and skin tags on the perianal region (c). Magnetic resonance imaging of
pelvis showing focal subcutaneous thickening with low signal intensity in T1-weighted image (d). Histopathological examination
of the vulva shows multiple non-caseating granulomas extending throughout the dermis and the granuloma consist of
epithelioid histiocytes, lymphocytes and multinucleated giant cells (e) (hematoxylin–eosin, original magnification ·40).
plaques that are red to purple or violaceous and commonly involves the limbs, especially legs, vulva, penis
and face.2 MCD on the vulva in children is rare and
difficult to diagnose. Furthermore, if a dermatological
manifestation precedes gastrointestinal symptoms,
the diagnosis becomes enigmatic. Only several cases
of which vulvar swelling or edema antedates the clinical appearance of intestinal CD in children have been
reported in the English-language published work.3–9
The diseases which can cause vulvar swelling
include pregnancy, hidradenitis suppurativa, sexual
abuse, tumor, vascular malformation, lymphedema,
genital cheilitis granulomatosa, factitial dermatitis,
304
contact dermatitis, CD, mastocytosis and various
infectious diseases. For differential diagnosis of vulvar
swelling, prudent history taking and physical examinations are important. Laboratory and radiological
examinations could also give some diagnostic clues.
When clinical diagnosis cannot be made easily, skin
biopsy is necessary. MCD is distinguished histologically by granulomatous inflammation. However,
because various conditions can also cause granulomatous inflammation, diverse investigations are
necessary.7 After common infectious or inflammatory
diseases are excluded, performing diagnostic colonoscopy is warranted to check for CD. In our case, as
2010 Japanese Dermatological Association
Letter to the Editor
Table 1. Reported cases of vulvar swelling or edema precede gastrointestinal symptoms of Crohn’s disease in children in
English-language published work
Reference
Age
(years)
Mountain3
14
Lally et al.4
Werlin et al.5
Guerrieri et al.6
Initial
diagnosis
Vulvar
symptom
Concomitant
perianal lesion
Edema and
inflammation
of labia
Tuberculosis†
N⁄D
Anal fistula
8
Vulvar erythema
and edema
Non-tender
Anal fistula
8
Erythematous,
firm, unilateral,
labial hypertrophy
Swelling of right
labium majus
Contact
dermatitis,
candidiasis and
sexual abuse
Foreign body
reaction
Non-tender
Skin tags
Vulvitis
granulomatosa
Non-tender
Anal tags
Erythema and
edema of the
labial majora
Cellulitis
N⁄D
Swelling of lower
lip, vulvar swelling
and perivaginal
abscess
Swelling and
induration of
the labia majora
Erythematous, firm
tumescence
of left labial majora
Left vulvar swelling
N⁄D
N⁄D
Skin tags
and fissures
with anal
hemorrhoids
None
Sexual abuse
Genital itching
Infectious
disease
Asymptomatic
Cellulitis
Non-tender
11
Ploysangam
et al.7
6
Ploysangam
et al.7
10
Porzionato
et al.8
7
Pinna et al.9
10
Present case
10
Presentation
Perianal
fissures and
skin tags
Rose-grayish
vegetations
Skin tags
Vulvar pathology
Chronic
inflammatory
changes without
tubercules or
caseation
Noncaseating
granulomatous
inflammation
Non-caseating
granulomatous
inflammation
Non-necrotizing
granulomatous
inflammation
Granulomatous
inflammation
Granulomatous
inflammation
Non-caseating
granulomatous
inflammation
Non-caseating
granulomatous
inflammation
Non-caseating
granulomatous
inflammation
†
The author did not describe initial diagnosis but the lesion was treated with a 2-month course of streptomycin and isoniazid without benefit.
N ⁄ D, Not described.
the dermatological manifestation antedated the onset
of typical intestinal symptoms, the diagnosis was
difficult because no laboratory and radiological examinations gave a hint for MCD.
After reviewing reported MCD cases in children
including the present case whose vulvar swelling or
edema preceded gastrointestinal symptoms of Crohn’s disease (Table 1), we suggest two diagnostic
characteristics which may help early diagnosis of
MCD on the vulva. First, if the vulvar swelling is nontender or painless, MCD rather than infectious diseases should be included in the differential diagnosis.
MCD represents another ‘‘great imitator’’ and it
resembles many other dermatoses.1 Most of the
reported cases have been clinically misdiagnosed as
2010 Japanese Dermatological Association
cellulitis, erysipelas, lichenoid eruptions and various
sexually transmitted diseases1 and vulvar swelling as
a manifestation of CD commonly misdiagnosed as
cellulitis or sexual abuse.10 So, non-tenderness or
painlessness in MCD could be an important clinical
feature in the differential diagnosis with infectious diseases. Many reported vulvar CD were painless, especially when occurring in children.4–6,8–11 In Table 1,
pain was not observed in six out of nine cases, and in
the remaining three cases, vulvar symptom was not
described. We speculate that those three cases
would have been painless because the authors would
have probably stated the symptom if there was pain
or tenderness. In addition, many reported MCD cases
in male patients presenting penile and scrotal swelling
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J.-H. Mun et al.
or edema did not accompany pain.12–19 In perianal
CD, Alexander-Williams et al.20 previously suggested
that the relative lack of pain, multiplicity of lesions and
edema of skin tags can be important clinical indicators if perianal CD occurred as the first manifestation.
We do not think that every vulvar CD shows lack of
pain but we assume that most genital swelling in
MCD at its first manifestation without secondary
lesions such as fissure, erosion, ulcer or abscess
would be painless or non-tender. We suggest that following MCD cases need to address the presence of
tenderness or pain because such a clinical feature
can be a useful information for clinicians. Second, if a
patient has perianal lesions such as skin tags, anal
fissures, ulcers, fistulas, perianal abscesses and anorectal strictures with vulvar swelling, MCD should be
considered. In our case, anal skin tags were small
and asymptomatic, and were not of clinical interest in
the first place. Presence of perianal disease was
noted on presentation in 81% of pediatric patients
with MCD.10 Therefore, if a patient presents vulvar
swelling, careful examination of the anus as well as
the vulva is needed.
Because vulvar CD is very rare, there is no consistent treatment for genital CD. The most effective
treatment for cutaneous CD seems to be oral metronidazole, and other treatments such as systemic
corticosteroids, topical corticosteroids, azathioprine,
cyclosporine, sulfasalazine and tetracycline have
been used sucessfully.1
Metastatic Crohn’s disease usually postdates or
coincides with gastrointestinal symptoms, but in
25% of cases, dermatological lesions precede the
onset of overt intestinal symptoms.1 Without intestinal manifestations, the diagnosis is extremely
difficult. So we suggest that if vulvar swelling or
edema is non-tender or painless and accompanies
perianal diseases, CD should be taken into consideration. For definite diagnosis, skin biopsy and
colonoscopy need to be performed. Vulvar CD has
been a diagnostic challenge not only for dermatologists but also for gastroenterologists, gynecologists, pediatricians and urologists. Delayed
diagnosis of CD can result in many complications
such as malnutrition, delayed pubertal development,
intestinal obstruction, hemorrhage and emotional
issues. In this context, our suggestion for early diagnosis has clinical significance.
306
Je-Ho MUN, Su-Han KIM, Do-Sang JUNG,
Hyun-Chang KO, Moon-Bum KIM,
Kyung-Sool KWON
Department of Dermatology, School of Medicine,
Pusan National University, Busan, Korea
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2010 Japanese Dermatological Association
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