Crohn Disease of the Vulva without Gastrointestinal Manifestations
Transcription
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. 82 Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 19, No 1 (January/ February), 2015: pp 81–83 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. References 1. Makhija S, Trotter M, Wagner E, et al. Refractory Crohn’s disease of the vulva treated with infliximab: a case report. Can J Gastroenterol 2007;21:835–7. 2. Urbanek M, Neill SM, McKee PH. Vulval Crohn’s disease: difficulties in diagnosis. Clin Exp Dermatol 1996;21:211–4, doi:10. 1111/j.1365-2230.1996.tb00065.x. 3. Lally MR, Orenstein SR, Cohen BA. Crohn’s disease of the vulva in an 8-year-old girl. 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Behcet’s disease seen in China: analysis of 334 cases. Rheumatol Int 2013;33:645–8, doi:10.1007/s00296-012-2384-6. 12. Gattu S, Rashid RM, Khachemoune A. Self-induced skin lesions: a review of dermatitis artefacta. Cutis 2009;84:247–51. 13. Andreani SM, Ratnasingham K, Dang HH, et al. Crohn’s disease of the vulva. Int J Surg 2010;8:2–5, doi:10.1016/j.ijsu.2009.09.012. Canadian Dermatology Association | Journal of Cutaneous Medicine and Surgery, Vol 19, No 1 (January/ February), 2015: pp 81–83 83