Indian Journal of Dermatology
  Publication of IADVL, WB
  Official organ of AADV
Indexed with Science Citation Index (E) , Web of Science and PubMed
 
Users online: 1888  
Home About  Editorial Board  Current Issue Archives Online Early Coming Soon Guidelines Subscriptions  e-Alerts    Login  
    Small font sizeDefault font sizeIncrease font size Print this page Email this page


 
Table of Contents 
E-IJD-CASE REPORT
Year : 2015  |  Volume : 60  |  Issue : 1  |  Page : 105
Genital ulcerative pyoderma gangrenosum in Behçet's disease: A case report and review of the literature


1 Afyon Kocatepe University Department of Dermatology, Afyon, Turkey
2 Department of Pathology, Afyon Kocatepe University Dermatology, Afyon, Turkey
3 Department of Nephrology, Afyon Kocatepe University Dermatology, Afyon, Turkey

Date of Web Publication26-Dec-2014

Correspondence Address:
Pinar Ozuguz
Afyon Kocatepe University Dermatology, Afyon
Turkey
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.147866

Rights and Permissions

   Abstract 

Behçet's disease (BD), first described by Hulusi Behcet, is a multisystemic disease characterized by recurrent oral and genital ulcerations, ocular and cutaneous lesions, arthritis and vascular disease. Pyoderma gangrenosum (PG) is a rare, chronic, sterile pustular and progressive ulcerative process of unknown cause; sometimes can participate in the differential diagnosis of Behcet's ulceration. A 33-year-old woman complained a severe genital ulcer. She had a purulent oozing and stinky ulceration on the right side of labium minor measuring 5-8 cm. A punch biopsy at ulcer margin showed that the lymphocytic panniculitis was extending to the subcutaneous fat tissue without fibrin deposition or necrotic changes in the vessel wall. Based on the clinical and histological findings, she was diagnosed as genital ulcerative PG, which occurred during the exacerbation of BD.


Keywords: Behçet′s disease, genital ulcer, ulcerative pyoderma gangrenosum


How to cite this article:
Ozuguz P, Kacar SD, Manav V, Karaca S, Aktepe F, Ulu S. Genital ulcerative pyoderma gangrenosum in Behçet's disease: A case report and review of the literature. Indian J Dermatol 2015;60:105

How to cite this URL:
Ozuguz P, Kacar SD, Manav V, Karaca S, Aktepe F, Ulu S. Genital ulcerative pyoderma gangrenosum in Behçet's disease: A case report and review of the literature. Indian J Dermatol [serial online] 2015 [cited 2020 Feb 20];60:105. Available from: http://www.e-ijd.org/text.asp?2015/60/1/105/147866

What was known?
50% of pyoderma gangrenosum cases are associated with an underlying systemic illness, in particular infl ammatory bowel disease such as ulcerative colitis or Crohn disease, arthritis, monoclonal gammopathy, malignancy, myeloproliferative disorder.



   Introduction Top


Behcet's disease (BD) was described by Hulusi Behçet as a complex of multisystem disease characterized. Although oral ulceration, genital ulceration and eye disease are the classic triad of manifestations, the cardiovascular, gastrointestinal, musculoskeletal and central nervous systems can also be affected. The presence of skin reactivity to needle trauma and erythema nodosum are particularly helpful findings. [1] On the other hand, pyoderma gangrenosum (PG) is a rare, chronic, sterile, pustular and progressive ulcerative process with unknown cause. [2] Its clinical appearance is a characteristic burrowing ulcer. Approximately, 50% of the cases are associated with an underlying systemic illness, in particular inflammatory bowel disease such as ulcerative colitis or Crohn disease, arthritis, monoclonal gammopathy, malignancy, myeloproliferative disorder [3] and rarely Behçet's disease. [4] Four clinical and histological variants have been recognized: Ulcerative, pustular, bullous and vegetative. There are confusing cases involving the diagnosis of BD and PG in the literature. We report here a case of BD who had genital ulcerative PG afterwards.


   Case Report Top


A 33-year-old woman complained of severe genital ulcer in January 2012. On admission, the patient had painful ulcer on the genital area for the past 10 days with purulent discharge [Figure 1]. She was diagnosed as BD based on criteria of International study group [1] by the presence of pathergy together with oral and genital ulcers in 2004 and treated with colchicine 0.5 mg orally 3 times/day. In 2009, she had diplopia as a result of neurological manifestations of BD and was successfully treated with systemic steroids. There was no evidence for a systemic disease or malignancy. Her family history was positive for BD.
Figure 1: The purulent oozing and stinky ulceration on the right side of labium minor measuring 5-8 cm

Click here to view


On dermatological examination; she had a purulent oozing and stinky ulceration on the right side of labium minor measuring 5-8 cm. There was no lymphadenopathy. Repeated smears and cultures for bacteria and fungi were negative. Physical examination revealed no systemic involvement. On laboratory examination increased erythrocyte sedimentation rate, white blood cell count and C-reactive protein (CRP); with anemia were detected. The X-ray imaging of the chest was normal. The punch biopsy of ulcer margin showed the lymphocytic panniculitis, which was extending to the subcutaneous fat tissue without fibrin deposition or necrotic changes in the vessel wall [Figure 2] and [Figure 3]. The patient of erythrocyte sedimentation rate and CRP levels were higher and she had three oral ulcers. Although the histological findings are not very typical for PG, based on the clinical and histological findings, our case was diagnosed as genital ulcerative PG, which occurred during the exacerbation of BD. Ulcer markedly improved and healed with scarring after oral prednisolone therapy with 20 mg/day for 8 weeks together with topical sucralfate and mometasone furoate therapy [Figure 4].
Figure 2: The histopathological image stained with hematoxylin eosin X10 magnification

Click here to view
Figure 3: The histopathological image stained with hematoxylin eosin X400 magnification

Click here to view
Figure 4: The patients lesion healed with scar

Click here to view



   Discussion Top


Since, the first report by Munro and Cox in 1988 [5] 13 cases of PG associated with BD have been reported in the literature [6],[7],[8],[9],[10],[11],[12],[13],[14],[15],[16],[17] [Table 1]. Both BD and PG belong to the category of the neutrophilic dermatoses, in which primary pustular lesions occur and neither of them has a characteristic histological appearance. Oral and genital ulcerative lesions can be observed in both diseases. [4] On the other hand, the frequency of the mucosal ulcers is absolutely different between two diseases. Orogenital ulcers are commonly seen in BD, whereas rarely seen in PG. [5] In BD, oral ulcers tend to be small and round, with well-circumscribed margins, erythematous halo and yellow-grey floor. The lesions resemble those seen with a recurrent aphthous stomatitis. Genital ulcers are similar in appearance to oral ulcers and can sometimes cause scar formation. [6] Pustular lesions can be the initial skin manifestation and skin hyperreactivity or pathergy is often found in both diseases. In PG, the ulcers of the genital mucosa appear similar to those skin, which are comprised of a shallow burrowing ulcers with irregular margins and a ragged purple overhanging edges. [2] Rapid local destruction may occur in PG. [7] Thus, the clinical features of mucosal ulcers are different to some degree between the two diseases. However, there is a histopathological distinction between PG and BD. [18] In PG, vascular involvement ranges from none to fibroid necrosis, and in most cases a neutrophilic infiltrate is present with limited vascular damage. Conversely, in BD, mononuclear cell vasculitis with variable fibrin deposition or leukocytoclastic vasculitis is usually found.
Table 1: The reported cases of Behçet's disease with pyoderma gangrenosum

Click here to view


According to the clinical appearance, the rate of progression, the associated systemic disease and the histopathological changes, PG has been classified into four clinical variants: As ulcerative, pustular, bullous and vegetative. [3] Our case had vegetative types of PG. Vegetative form of PG is a limited, non-aggressive, chronic and superficial variant, which is also termed superficial granulomatous pyoderma. Unlike classic PG, many patients with vegetative PG do not have an associated systemic disease. However, Langan and Powell reported that 18% of patients had other medical conditions, including diabetes mellitus, rheumatoid arthritis, chronic lymphocytic leukemia, polycythemia rubra vera, paraproteinemia and pulmonary sarcoidosis. [19] There is a clinical and histological overlap between PG and BD. [4],[5] Skin lesions of PG and BD may show similar range of histological appearances, including a neutrophilic vascular reaction. In our case, there were not any necrotic changes with fibrinoid material in the vessel wall in favor of PG.

Glucocorticoids, sulfa-drugs, dapsone and immunosuppressive agents, such as cyclosporine, azathioprine or cyclophosphamide are therapeutic agents used to treat both PG and BD.

In the present case, genital ulcerative PG developed concurrently with an exacerbation of BD. Therefore, this clinical course accepted as the overlap of PG and BD. In this report, our patient had been diagnosed with BD before and she had ulceration of the labia minora and major afterwards. The genital ulcers appeared as large burrowing ulcers, which were characteristic of PG. This finding suggested that our case might be PG. We reported the second case of PG in the literature, showing mucosal ulcers, which were difficult to distinguish from BD at the first medical examination.

 
   References Top

1.
International Study Group for Behcet's disease. Criteria for diagnosis of Behçet's disease. International study group for Behçet's disease. Lancet 1990;335:1078-80.  Back to cited text no. 1
    
2.
Wolff K, Stingl G. Pyoderma gangrenosum. In: Freedberg IM, Eisen AZ, Wolff K, Austen KF, Goldsmith LA, Katz SI, editors. Fizpatrick's Dermatology in General Medicine. 6 th ed., Vol. 1. New York: McGraw-Hill; 2003. p. 969-76.  Back to cited text no. 2
    
3.
Powell FC, Su WP, Perry HO. Pyoderma gangrenosum: Classification and management. J Am Acad Dermatol 1996;34:395-409.  Back to cited text no. 3
    
4.
Kaklamani VG, Vaiopoulos G, Kaklamanis PG. Behçet's disease. Semin Arthritis Rheum 1998;27:197-21.  Back to cited text no. 4
    
5.
Munro CS, Cox NH. Pyoderma gangrenosum associated with Behçet's syndrome - Response to thalidomide. Clin Exp Dermatol 1988;13:408-10.  Back to cited text no. 5
    
6.
Brunsting LA, Goeckerman WH, O'Leary PA. Pyoderma (ecthyma) gangrenosum: Clinical and experimental ob-servations in five cases occurring in adults. Arch Dermatol Syph 1930;22:655-80.  Back to cited text no. 6
    
7.
Lilford RJ, Tindall VR, Batchelor AG. Post-surgical pyoderma gangrenosum of the vaginal vault associated with ulcerative colitis and Behçet's disease; a case report. Eur J Obstet Gynecol Reprod Biol 1989;31:93-4.  Back to cited text no. 7
    
8.
Rustin MH, Gilkes JJ, Robinson TW. Pyoderma gangrenosum associated with Behçet's disease: Treatment with thalidomide. J Am Acad Dermatol 1990;23:941-4.  Back to cited text no. 8
    
9.
Armas JB, Davies J, Davis M, Lovell C, McHugh N. Atypical Behçet's disease with peripheral erosive arthropathy and pyoderma gangrenosum. Clin Exp Rheumatol 1992;10:177-80.  Back to cited text no. 9
    
10.
Chams-Davatchi C, Shizarpour M, Davatchi F, Shahram F, Chams H, Nadji A, et al. Extensive pyoderma gangrenosum-like lesion in two cases of Behçet's disease, responding only to cyclosporin. Adv Exp Med Biol 2003;528:337-8.  Back to cited text no. 10
    
11.
Joshi A, Mamta. Behçet's syndrome with pyoderma-gangrenosum-like lesions treated successfully with dapsone monotherapy. J Dermatol 2004;31:806-10.  Back to cited text no. 11
    
12.
Singh G, Sethi A, Okade R, Harish MR. Bullous pyoderma gangrenosum: A presentation of childhood Behcet's disease. Int J Dermatol 2005;44:257-8.  Back to cited text no. 12
    
13.
Nakamura T, Yagi H, Kurachi K, Suzuki S, Konno H. Intestinal Behcet's disease with pyoderma gangrenosum: A case report. World J Gastroenterol 2006;12:979-81.  Back to cited text no. 13
    
14.
Akay N, Boyvat A, Heper AO, Soykan I, Arica IE, Bektas M, et al. Behçet's disease-like presentation of bullous pyoderma gangrenosum associated with Crohn's disease. Clin Exp Dermatol 2006;31:384-6.  Back to cited text no. 14
    
15.
Kim JW, Park JH, Lee D, Hwang SW, Park SW. Vegetative pyoderma gangrenosum in Behçet's disease. Acta Derm Venereol 2007;87:365-7.  Back to cited text no. 15
    
16.
Kim DW, Lee BI, Park SH. Accelerated healing of pyoderma gangrenosum in Behçet patient treated with cyclosporine and split thickness skin graft. Ann Plast Surg 2008;61:552-4.  Back to cited text no. 16
    
17.
Tsuboi H. Case of pyoderma gangrenosum showing oral and genital ulcers, misdiagnosed as Behcet's disease at first medical examination. J Dermatol 2008;35:289-92.  Back to cited text no. 17
    
18.
Elder D, Elenitsas R, Johnson B Jr, Murphy G. Cutaneous manifestations of nutritional deficiency states and gas-trointestinal disease. In: Elder DE, Elenitsas R, Johnson BL, Murphy GF, editors. Lever's Histopathology of the Skin. 9 th ed. Philadelphia: Lippincott-Williams and Wilkins; 2005. p. 422-6.  Back to cited text no. 18
    
19.
Langan SM, Powell FC. Vegetative pyoderma gangrenosum: A report of two new cases and a review of the literature. Int J Dermatol 2005;44:623-9.  Back to cited text no. 19
    

What is new?
Pyoderma gangrenosum(PG) is rarely associated with Behηet′s disease (BD). PG and BD may overlap.


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1]



 

Top
Print this article  Email this article
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (1,257 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures
    Article Tables

 Article Access Statistics
    Viewed2022    
    Printed37    
    Emailed0    
    PDF Downloaded56    
    Comments [Add]    

Recommend this journal