Crohn Disease of the Vulva without Gastrointestinal Manifestations

CASE REPORT
Crohn Disease of the Vulva without Gastrointestinal
Manifestations in a 16-Year-Old Girl
Ai-jun Zhang, Shu-hui Zhan, Hong Chang, Yu-qiang Gao, and Yan-qing Li
Background: Crohn disease of the vulva is a rare disease that is difficult to diagnose. There are limited reports describing
treatment of this condition.
Objective: To describe the diagnosis and treatment of a 16-year-old girl with Crohn disease of the vulva, without onset of
intestinal symptoms.
Methods: Crohn disease was diagnosed by histopathology. The patient was treated with corticosteroids and followed for 1 year.
Results: After the final diagnosis, cutaneous lesions responded rapidly to corticosteroid treatment, which was gradually stopped
after 6 months. The disease was well controlled at the 1-year follow-up.
Conclusion: Crohn disease of the vulva can develop alone without the onset of intestinal symptoms. Diagnosis relies on special
pathologic findings. Corticosteroid treatment is effective for this condition.
Contexte: La maladie de Crohn vulvaire est une affection rare, difficile à diagnostiquer, et seul un petit nombre de rapports font
état du traitement de la maladie.
Objectif: L’article vise à décrire la marche suivie dans l’exploration diagnostique et le choix des traitements dans un cas de
maladie de Crohn vulvaire, non précédée de symptômes intestinaux, chez une fille de 16 ans.
Méthode: Le diagnostic de la maladie de Crohn a été posé d’après les résultats de l’examen histopathologique, et le traitement a
consisté en l’administration de corticostéroı̈des. La patiente a été suivie pendant 1 an.
Résultats: Après la pose du diagnostic formel, les lésions cutanées ont réagi rapidement à la corticothérapie, qui a pris fin au bout
de 6 mois après une réduction graduelle de la posologie. La maladie était bien maı̂trisée au bout de 1 an de suivi.
Conclusions: La maladie de Crohn peut affecter la vulve seule, sans être précédée de symptômes intestinaux. Le diagnostic
repose sur des constatations pathologiques particulières, et le traitement par les corticostéroı̈des se montre efficace.
ROHN DISEASE is a chronic, relapsing and remitting, inflammatory bowel disease of unknown
etiology.1 The terminal ileum and colon are the most
commonly affected organs, but other regions of the
alimentary tract, from the mouth to the anus, may also
be affected. Vulvar involvement in Crohn disease is rare
and difficult to diagnose.2 This report describes a case of
Crohn disease affecting only the vulva.
C
From the Department of Gastroenterology, Qilu Hospital of Shandong
University, Jinan, and Departments of Gastroenterology and Pathology,
Qingdao Municipal Hospital, Qingdao, P.R. China.
Address reprint requests to: Yan-Qing Li, MD, PhD, Department of
Gastroenterology, Qilu Hospital of Shandong University, 107# Wenhua Xi
Road, Jinan 250012, P.R. China; e-mail: [email protected].
DOI 10.2310/7750.2014.14005
# 2014 Canadian Dermatology Association
Figure 1. Crohn disease of the vulva before treatment. Note the
ulcerations, erythema, and swelling of the labia majora. There is some
destruction of the vulval architecture.
Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 19, No 1 (January/ February), 2015: pp 81–83 81
Zhang et al
Figure 2. Histologic examination
revealing ulceration of the dermis.
A, Low-power (hematoxylin-eosin stain;
1003) microscopic image showing
diffuse inflammatory cell infiltration.
B, High-power (hematoxylin-eosin stain;
2003) image showing multiple, noncaseating epithelioid cell granulomas.
Case Report
A 16-year-old girl with a history of intermittent fever for
1 month and a high temperature of 39.3uC was
hospitalized. She had no respiratory, digestive, or urinary
tract symptoms. She had no oral ulcers, arthralgia, or skin
rash. Clinical examination revealed multiple oval ulcers
distributed on and around the vulva (Figure 1). The labia
majora was biopsied. The patient’s blood count, procalcitonin level, liver function, and blood culture were normal.
Her erythrocyte sedimentation rate was 74 mm/h. Serum
antituberculosis antibody (TB-Ab), rapid plasma reagin
(RPR), antihuman immunodeficiency virus antibody
(HIV-Ab), anti–herpes simplex virus antibody (HSV-Ab,
IgM), perinuclear antineutrophil cytoplasmic antibody (pANCA), and purified protein derivative (PPD) tests were
negative, whereas the anti-Sjögren syndrome A antibody
(SSA-Ab) and the anti-Ro-52 antibody tests were positive
(+++). The chest computed tomographic (CT) scan was
normal, but the abdominal CT scan showed thickening of
the sigmoid wall. A colonoscopy (< 20 cm into the
terminal ileum) was normal. Gastroscopy showed only
chronic superficial gastritis.
Histologic analysis of the vulval biopsy showed vascular
endothelium proliferation, diffuse infiltration of chronic
inflammatory cells, and multiple, noncaseating epithelioid cell granulomas in the deeper dermis (Figure 2).
Ziehl-Neelsen, Gram, and periodic acid–Schiff stains were
negative for bacterial infection. No fungi were found.
Topical povidone-iodine, systemic oseltamivir, ceftriaxone
sodium, and thalidomide treatments failed, but the patient
responded rapidly to systemic methylprednisolone administered at 40 mg/d for 1 week. The methylprednisolone
dosage was gradually reduced, and the ulcers healed after
4 weeks of treatment (Figure 3).
A final diagnosis of Crohn disease of the vulva was
made. Methylprednisolone treatment was gradually
stopped within 6 months. At the 1-year follow-up visit,
the patient’s skin condition was well controlled, and she
reported no bowel problems during the year.
Discussion
Vulvar localization of Crohn disease is unusual and can
precede gastrointestinal disease by many years3,4 or remain
isolated.5 Typical clinical symptoms include erythema,
edema, and ulcerations. Before a diagnosis of Crohn disease
can be made, other causes of ulcerative vulvar lesions, such
as HSV infection (especially in immunosuppressed individuals), syphilis, chancroid, granuloma inguinale, lymphogranuloma venereum, secondary bacterial infections, fungal
infections,6 HIV, tuberculosis, Epstein-Barr virus, leishmaniasis, amebiasis,7 and mycoplasma,8 must be ruled out.
Figure 3. Vulval ulcerations after
glucocorticoid treatment. A, Two
weeks after treatment. B, Four weeks
after treatment.
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Crohn Disease of the Vulva in a Girl
Skin disorders, including contact dermatitis,9 drug reaction,
trauma,6 squamous cell carcinoma, sarcoid of the vulva, and
metastatic carcinoma, 10 must also be considered.
Uncommon reasons for vulvar ulcers include Behçet
disease11 and self-induced (factitial) ulcer.12
In the present case study, a careful physical examination, pathogenic microorganism cultures, and histopathologic examination were all helpful in making the diagnosis
of Crohn disease. The definitive diagnosis was facilitated
by the histologic finding of multiple, noncaseating
epithelioid cell granulomas and the effectiveness of
glucocorticoid therapy. There is no consensus for the
treatment of Crohn disease of the vulva. Treatment
choices are based on limited anecdotal case reports. In
agreement with the current literature,13 glucocorticoid
therapy led to significant, long-term patient improvement.
If glucocorticoid treatment is not effective, metronidazole,
sulfasalazine, mesalazine, azathioprine, infliximab, thalidomide, or even vulvectomy may be considered.5 Because
this disease tends to relapse, follow-ups must be
continued for years. Vulvar involvement may be the only
manifestation of Crohn disease without gastrointestinal
localization.
Acknowledgment
Financial disclosure of authors and reviewers: None reported.
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