Saudi Journal of Gastroenterology
Home About us Instructions Submission Subscribe Advertise Contact Login    Print this page  Email this page Small font sizeDefault font sizeIncrease font size 
Users Online: 885 


 
REVIEW ARTICLE Table of Contents   
Year : 2007  |  Volume : 13  |  Issue : 4  |  Page : 159-162
Cutaneous manifestations of inflammatory bowel disease


Dermatology Department, College of Medicine, King Saud University, Riyadh, Saudi Arabia

Click here for correspondence address and email

Date of Submission28-May-2007
Date of Acceptance29-Jun-2007
 

   Abstract 

Inflammatory bowel disease (IBD) has many extraintestinal manifestations, and skin lesions are one of the most frequently described extraintestinal findings. Reports indicate an incidence of cutaneous manifestations ranging from 2 to 34%, Cutaneous manifestations are usually related to the activity of the bowel disease but may have an independent course. In this review we aim to address the various cutaneous manifestations associated with IBD, their impact on the disease course, and the treatment options available.

Keywords: Inflammatory bowel disease, skin, ulcerative colitis, Crohn′s disease, pyoderma gangrenosum, erythema nodosum

How to cite this article:
Al Roujayee A. Cutaneous manifestations of inflammatory bowel disease. Saudi J Gastroenterol 2007;13:159-62

How to cite this URL:
Al Roujayee A. Cutaneous manifestations of inflammatory bowel disease. Saudi J Gastroenterol [serial online] 2007 [cited 2019 May 20];13:159-62. Available from: http://www.saudijgastro.com/text.asp?2007/13/4/159/36744


Cutaneous manifestations of inflammatory bowel disease (IBD) are relatively common wherein a variety of skin lesions may develop. These are generally secondary to reactive skin eruptions, granulomatous cutaneous disease and other associated conditions. [1]


   Pyoderma Gangrenosum Top


Pyoderma gangrenosum (PG) is a severe ulcerating noninfectious neutrophilic dermatosis. It has been reported in 1-10% of patients with ulcerative colitis (UC) and in 0.5-20% of patients with Crohn's disease (CD). [2],[3] It occurs equally in men and women with a peak age incidence between 25 and 54 years of age; [4] 50-78% of patients with PG have an underlying systemic disease, [5] such as IBD, myeloproliferative disease or rheumatological disease. [6]

Four variants of PG have been described: ulcerative, pustular, bullous and vegetative. Ulcerative and pustular PG are associated with IBD; PG can occur before, during or after the onset of IBD, and both diseases can occur independent of one another. [4] Typically, PG begins with pain, followed by pustule formation and rapid ulceration. A sterile, purulent necrotic center surrounded by a bluish border most commonly occurs on the lower extremities, although it can occur on any part of the body [Figure - 1]. [7],[8] PG is a diagnosis of exclusion where every possible effort must be made to identify the underlying systemic disease and subsequently treat it. [9] Tissue should be examined histologically to rule out other diseases. It consists of a neutrophilic infiltrate centrally in the ulcer and a lymphocytic infiltrate in the periphery. [4] Routine testing for bacteria, mycobacteria and deep fungal infection is recommended. Additional studies may include syphilis serology, antiphospholipid antibody levels, serum protein electrophoresis and immunoelectrophoresis. Management should be directed at both the lesions of PG and at the underlying disorder. [10]

Lesion-specific management can include topical and intralesional therapies, local injections of triamcinolone acetonide (10-40 mg/ml), with or without systemic corticosteroids. [11],[12] Other therapies include topical tacrolimus (FK 506). More extensive disease may require large daily doses of prednisone or other immunosuppressive agents such as cyclosporin or FK 506. In several reports, patients treated with infliximab have shown the rapid healing of their lesions and a successful response to infliximab in severe peristomal PG has also been observed. [13] Because the course of PG can be independent of the course of IBD and has even been reported years after proctocolectomy, bowel resection is not a primary therapy. [14],[15]


   Erythema Nodosum Top


Erythema nodosum (EN), another common cutaneous manifestation of IBD, consists of tender red nodules that occur primarily on the anterior portion of the lower extremities [Figure - 2]. The incidence of EN in patients with IBD is around 2-4% and up to 20% of patients are in an active phase of the disease. [16],[17] Although the underlying pathogenesis is unknown, EN is thought to be a hypersensitivity reaction (Type 4) to a number of inciting agents. As opposed to PG, whose lesions ulcerate, EN does not ulcerate and heals without scarring or atrophy. Histopathologically, the nodules represent a septal panniculitis. The treatment consists of controlling the underlying flare of IBD, oral corticosteroids, potassium iodide, dapsone and colchicine.


   Aphthous Stomatitis Top


Aphthae are shallow round ulcers with a central fibrinous membrane and erythematous halo [Figure - 3]. It is observed in around 10% of patients with IBD. Aphthae associated with CD and UC cannot be differentiated clinically from common aphthae. [18] Aphthae are also associated with celiac sprue, human immunodeficiency virus/acquired immunodeficiency syndrome, Behcet's disease and Reiter's syndrome. The treatment of the underlying condition may be curative. [19] For symptomatic pain relief, 2% viscous lidocaine is frequently used; topical corticosteroids such as triamcinolone 0.1% paste once to three times per day is effective for healing. Systemic corticosteroids should only be used in refractory cases or in persistent or severe aphthae. [20]


   Mucosal Nodularity (Cobblestoning) Top


Mucosal nodularity, known as cobblestoning, appears as mucosal coloured papules forming firm plaques on the buccal mucosa and palate. Lesions, although infrequent, are specific for CD. [21] Cobblestoning may be painful and interferes with speaking and eating. [22] The treatment of the underlying disease is necessary. In addition, topical and systemic corticosteroids are present in severe cases. [23]


   Pyostomatitis Vegetans Top


Pyostomatitis vegetans is a rare oral ulcerative disorder observed in patients with UC and less frequently in patients CD [Figure - 4]. Pustules, erosions and vegetative plaques appear on the buccal and gingival mucosa and form a ''snail track'' appearance. Pyostomatitis vegetans is a specific marker for IBD. [24],[25] Generally, IBD precedes the onset of pyostomatitis vegetans and mimics the activity of the bowel disease, [26] although it has been reported with asymptomatic UC. [27] Treatment with topical corticosteroids has been useful in some cases. However, the treatment of choice is systemic corticosteroids; dapsone or azathioprine may be added. [24]


   Perianal and Peristomal Ulcers and Fistulae Top


Perianal disease is often the first cutaneous manifestation of CD. In a study of patients with mainly colonic (41%) and ileocolonic (35%) CD, perianal disease was observed in 7% of patients at diagnosis and in 45% of patients during the course of this disease. [28] The clinical spectrum of perianal lesions is variable with the most common early lesions being perianal erythema, aphthous ulcers in the anal canal and perianal fissures. Aggressive forms include profound ulcers that destroy the anal sphincter, abscesses complicating the ano/perianal region, or rectovaginal fistulae and scarring leading to deformations.

Perirectal fistulae and abscesses can be well-evaluated by magnetic resonance imaging (MRI). [13],[29] Recently, endoscopic ultrasonography (EUS), has been proven to be a valuable diagnostic tool. A prospective study was performed to determine the accuracy of EUS in the diagnosis of perianal fistulas. Thirty-four patients with CD underwent EUS, MRI and an examination under anesthesia. The accuracy in detecting a fistula was 91% for EUS, 87% for MRI and 91% for examination under anesthesia. The authors concluded that although EUS, MRI and examination under anesthesia are all reasonably accurate tests for determining fistula anatomy, an optimal strategy would combine any two of the three methods that produced 100% accuracy rates. [30]

Potential therapies for complex fistulae include antibiotics, azathioprine/mercaptopurine, infliximab and surgery. Infliximab has been proven to be effective in placebo-controlled trials in reducing the number of draining fistulae and maintaining that reduction. [31]


   Conclusion Top


Cutaneous manifestations of IBD are relatively common and disabling. Patients presenting with IBD should be examined for cutaneous manifestations. Treatment should be directed at both the bowel and cutaneous lesions, Early aggressive therapy can minimize severe complications.

 
   References Top

1.Tavarela Veloso F. Skin complications associated with inflammatory bowel disease. Aliment Pharmacol Ther 2004;20:50-3.  Back to cited text no. 1    
2.Greenstein AJ, Janowiz HD, Sachar PB. The extra-intestinal complications of Crohn's disease and ulcerative colitis. A study of 700 patients. Medicine (Baltimore) 1976;55:401-12.  Back to cited text no. 2    
3.Lebwohl M, Lebwohl O. Cutaneaous manifestations of inflammatory bowel disease. Inflamm Bowel Dis 1998;4:142-8.  Back to cited text no. 3  [PUBMED]  
4.Powell FC, Su WR, Perry HO. Pyoderma gangrenosum: Classification and management. J Am Acad Dermatol 1996;34:395-409.  Back to cited text no. 4    
5.Bennett ML, Jackson JM, Jorizzo JL, Fleischer AB Jr, White WL, Callen JP. Pyoderma gangrenosum: A comparison of typical and atypical forms with an emphasis on time to remission. Case review of 86 patients from 2 institutions. Medicine (Baltimore) 2000;79:37-46.  Back to cited text no. 5    
6.Wollina U. Clinical management of pyoderma gangrenosum. Am J Clin Dermatol 2002;3:149-58.  Back to cited text no. 6  [PUBMED]  
7.Schwaegerle SM, Bergfeld WF, Senitzer D, Tidrick RT. Pyoderma gangrenosum: A review. J Am Acad Dermatol 1988;18:559-68.  Back to cited text no. 7  [PUBMED]  
8.Schorr-Lesnick B, Brandt LJ. Selected rheumatologic and dermatologic manifestations of inflammatory bowel disease. Am J Gastroenterol 1988;83:216-23.  Back to cited text no. 8  [PUBMED]  
9.Chow RK, Ho VC. Treatment of pyoderma gangrenosum. J Am Acad Dermatol 1996;34:1047-60.  Back to cited text no. 9  [PUBMED]  [FULLTEXT]
10.Berkowitz EZ, Lebwohl M. Cutaneaous manifestations of inflammatory bowel disease. J Eur Acad Dermatol Venereol 2000;15:349-50.  Back to cited text no. 10    
11.Boh EE, al-Smadi RM. Cutaneaous manifestations of gastrointestinal diseases. Dermatol Clin 2002;20:533-46.  Back to cited text no. 11  [PUBMED]  
12.Schwaegerle SM, Bergfeld WF, Senitzer D, Tidrick RT. Pyoderma gangrenosum: A review. J Am Acad Dermatol 1988;18:559-68.  Back to cited text no. 12  [PUBMED]  
13.Sandborn WJ, Fazio VW, Feagan BG, Hanauer SB; American Gastroenterological Association Clinical Practice Committee. AGA technical review on perianal Crohn's disease. Gastroenterology 2003;125:1508 30.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]
14.Levitt M, Ritchie J, Lennard-Jones J, Phillips RK. Pyoderma gangrenosum in inflammatory bowel disease. Br J Surg 1991;78:676-8.  Back to cited text no. 14    
15.Mir-Madjlessi SH, Taylor JS, Farmer RG. Clinical course and evolution of erythema nodosum and pyoderma gangrenosum in chronic ulcerative colitis: A study of 42 patients. Am J Gastroenterol 1985;80:615-20.  Back to cited text no. 15  [PUBMED]  
16.Das KM. Relationship of extraintestinal involvements in inflammatory bowel disease new insights into autoimmune pathogenesis. Digest Dis Sci 1999;44:1-13.  Back to cited text no. 16  [PUBMED]  [FULLTEXT]
17.Lebwohl M, Fleischmajer R, Janowitz H, Present D, Prioleau PG. Metastatic Crohn's disease. J Am Acad Dermatol 1984;10:33-8.  Back to cited text no. 17  [PUBMED]  
18.Gregory B, Ho VC. Cutaneaous manifestations of gastrointestinal disorders. Part II. J Am Acad Dermatol 1992;26:371-83.  Back to cited text no. 18    
19.MacPhail L. Topical and systemic therapy for recurrent aphthous stomatitis. Semin Cutan Med Surg 1997;16:301-7.  Back to cited text no. 19  [PUBMED]  
20.McBride DR. Management of aphthous ulcers. Am Fam Physician 2000;62:149-54.  Back to cited text no. 20  [PUBMED]  [FULLTEXT]
21.Trost LB, McDonnell JK. Important cutaneaous manifestations of inflammatory bowel disease. Postgrad Med J 2005;81:580-5.  Back to cited text no. 21  [PUBMED]  [FULLTEXT]
22.Lisciandrano D, Ranzi T, Carrassi A, Sardella A, Campanini MC, Velio P, Bianchi PA. Prevalence of oral lesions in inflammatory bowel disease. Am J Gastroenterol 1996;91:7-10.  Back to cited text no. 22  [PUBMED]  
23.Williams AJ, Wray D, Ferguson A. The clinical entity of orofacial Crohn's disease. Q J Med 1991;79:451-8.  Back to cited text no. 23  [PUBMED]  [FULLTEXT]
24.Soriano ML, Martνnez N, Grilli R, Fariρa MC, Martνn L, Requena L. Pyodermatitis-pyostomatitis vegetans: Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:322-6.  Back to cited text no. 24    
25.Brinkmeier T, Frosch PJ. Pyodermatitis-pyostomatitis vegetans: A clinical course of two decades with response to cyclosporine and low-dose prednisone. Acta Derm Venereol 2001;81:134-6.  Back to cited text no. 25  [PUBMED]  
26.Calobrisi SD, Mutasim DF, McDonald JS. Pyostomatitis vegetans associated with ulcerative colitis: Temporary clearance with fluocinonide gel and complete remission after colectomy. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1995;79:452-4.  Back to cited text no. 26  [PUBMED]  
27.Chaudhry SI, Philpot NS, Odell EW, Challacombe SJ, Shirlaw PJ. Pyostomatitis vegetans associated with asymptomatic ulcerative colitis. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87:327-30.  Back to cited text no. 27  [PUBMED]  [FULLTEXT]
28.Veloso FT, Ferreira JT, Barros L, Almeida S. Clinical outcome of Crohn's disease: Analysis according to the Vienna classification and clinical activity. Inflamm Bowel Dis 2001;7:306-13.  Back to cited text no. 28  [PUBMED]  
29.Borley NR, Mortensen NJ, Jewell DP. MRI scanning in perianal Crohn's disease: An important diagnostic adjunct. Inflamm Bowel Dis 1999;5:231-4.  Back to cited text no. 29  [PUBMED]  
30.Schwartz DA, Wiersema MJ, Dudiak KM, Fletcher JG, Clain JE, Tremaine WJ, et al. A comparison of endoscopic ultrasound, magnetic resonance imaging and exam under anesthesia for evaluation of Crohn's perianal fistulas. Gastroenterology 2001;121:1064-72.  Back to cited text no. 30  [PUBMED]  [FULLTEXT]
31.Sandborn WJ, Fazio VW, Feagan BG, Hanauer SB; American Gastroenterological Association Clinical Practice Committee. AGA technical review on perianal Crohn's disease. Gastroenterology 2003;125:1508-30.  Back to cited text no. 31  [PUBMED]  [FULLTEXT]

Top
Correspondence Address:
Abdulaziz Al Roujayee
P.O. Box 55760 College of Medicine, King Saud University, Riyadh 11544
Saudi Arabia
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1319-3767.36744

Rights and Permissions


    Figures

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

This article has been cited by
1 Skin signs of systemic diseases
Dimitris Rigopoulos, George Larios, Andreas Katsambas
Clinics in Dermatology. 2011; 29(5): 531
[VIEW] | [DOI]



 

Top
 
  Search
 
  
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Pyoderma Gangrenosum
    Erythema Nodosum
    Aphthous Stomatitis
    Mucosal Nodulari...
    Pyostomatitis Ve...
    Perianal and Per...
    Conclusion
    References
    Article Figures

 Article Access Statistics
    Viewed5981    
    Printed116    
    Emailed2    
    PDF Downloaded822    
    Comments [Add]    
    Cited by others 1    

Recommend this journal