Cutaneous manifestations in inflammatory bowel disease[]*

 

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Summary

Numerous extraintestinal manifestations in various organ systems have been reported to be associated with inflammatory bowel disease (IBD). Aim of the present paper was to evaluate the frequency of cutaneous manifestations in Crohn’s disease (CD) and ulcerative colitis (UC) with respect to their location, the activity and location of the underlying disease, the treatment options and the time to remission.

Methods: The medical records of 1043 inpatients with CD and UC were screened retrospectively for extraintestinal symptoms with special regard to cutaneous manifestations.

Results: The prevalence of cutaneous manifestations in IBD was 22/1043 (2.1 %; 18 women, 4 men; age: 31.41 ± 9.9 [21 51] yrs.). In 15/22 patients (68.2 %) the cutaneous manifestations were associated with CD, in 7/22 patients (31.8 %) UC was confirmed. In 6/22 patients (27.3 %) pyoderma gangrenosum (PG) was diagnosed, in 16/22 patients (72.7 %) erythema nodosum (EN). EN and PG predominately occurred at the lower legs: In 68.1 % the tibia was the main affection site. Other locations like breast or anus were rare. In 16/22 patients (72.7 %) an acute phase of the underlying disease was evident, in 6/22 patients (27.3 %) CD or UC were in remission. In patients with CD a colonic involvement was found in 86.7 %. Arthritis was the most frequent coexisting extraintestinal manifestation in CD (53.3 %) and UC (28.8 %). Drug treatment was performed with high doses of glucocorticoids and salicylates. The time to remission in patients with EN was significantly shorter as compared to PG (5.3 ± 1.8 vs. 19.6 ± 14.2 weeks; p < 0.001). In 5/22 patients (22.7 %) cutaneous manifestations reoccurred after a symptom-free interval. All efflorescenses reoccurred during an active phase of the underlying disease at the same manifestation site as the initial presentation.

Conclusion: In this series the prevalence of cutaneous manifestations in IBD was 22/1043 (2.1 %). EN and PG were more frequent in women with IBD, in CD, and during the acute phases of the underlying disease. EN and PG predominately affect the lower legs. Cutaneous manifestations respond well to an acute phase therapy of the underlying disease. The time to remission was significantly shorter in EN as compared to PG. However, relapses have to be considered in a relevant subgroup of patients.

Hautveränderungen bei chronisch-entzündlichen Darmerkrankungen

Zahlreiche extraintestinale Manifestationen in unterschiedlichen Organsystemen sind bei chronisch-entzündlichen Darmerkrankungen (CED) beschrieben worden. Ziel der vorliegenden Arbeit war es, die Häufigkeit kutaner Manifestationen bei Morbus Crohn (MC) und Colitis ulcerosa (CU) unter besonderer Berücksichtigung der Lokalisation, der Aktivität und Lokalisation der Grunderkrankung, der Behandlungsregime und der Zeit bis zur Remission zu untersuchen.

Methoden: Die Krankenakten von 1043 stationären Patienten mit MC und CU wurden hinsichtlich extraintestinaler Manifestationen im Bereich der Haut retrospektiv ausgewertet.

Ergebnisse: Die Häufigkeit kutaner Manifestationen bei CED betrug 22/1043 (2,1 %; 18 Frauen, 4 Männer; Alter: 31,41 ± 9,0 [21 51] Jahre). Bei 15/22 Patienten (68,2 %) lag ein MC vor, bei 7/22 Patienten (31,8 %) eine CU. Bei 6/22 Patienten (27,3 %) wurde ein Pyoderma gangrenosum (PG) diagnostiziert, bei 16/22 Patienten (72,7 %) ein Erythema nodosum (EN). EN und PG traten bevorzugt an den unteren Extremitäten auf: Bei 68,1 % war die Tibia die Prädilektionsstelle. Andere Manifestationsorte wie Brust oder Anus waren selten. Bei 16/22 (72,7 %) der Patienten (27,3 %) waren MC oder CU in Remission. Bei Patienten mit MC wurde in 86,7 % eine Kolonbeteiligung festgestellt. Arthritiden waren mit 53,3 % (MC) bzw. 28,8 % (CU) das häufigste gleichzeitig auftretende extraintestinale Symptom. Die medikamentöse Therapie wurde mit hohen Steroiddosen und Aminosalizyten durchgeführt. Die Zeit bis zum Abheilen der Effloreszenzen war bei EN signifikant kürzer als bei PG (5,3 ± 1,8 vs. 19,6 ± 14,2 Wochen; p < 0,001). Bei 5/22 Patienten (22,7 %) kam es zu einem Rezidiv der Hautsymptome nach einem symptomfreien Intervall. Alle Rezidive traten während akuter Krankheitsphasen und an den Stellen der Primäreffloreszenz auf.

Folgerung: PG und EN sind seltene extraintestinale Symptome bei CED, die mehrheitlich während akuter Krankheitsphasen auftreten. Meist sind Frauen und Patienten mit MC betroffen. EN und PG treten bevorzugt an den unteren Extremitäten auf und sprechen recht gut auf die Akute-Phase-Therapie der Grunderkrankung an. Die Abheilungszeiten sind bei Patienten mit EN signifikant kürzer als bei PG. Rezidive müssen jedoch berücksichtigt werden.

1 Dedicated to Prof. Dr. B. May (Bochum) on occasion of his 65^th birthday

References

• 1 Danzi J T. Extraintestinal manifestations of idiopathic inflammatory bowel disease.  Arch Intern Med. 1988;  148 297-302
  Google Scholar
• 2 Greenstein A J, Janowitz H D, Sachar D. The extraintestinal complications of Crohn’s disease and ulcerative colitis: A study of 700 patients.  Medicine. 1976;  55 401-412
  Google Scholar
• 3 Monsen U, Sorstad J, Hellers G, Johansson C. Extracolonic diagnoses in ulcerative colitis: An epidemiological study.  Am J Gastroenterol. 1985;  85 711-716
  Google Scholar
• 4 Rankin G B, Watts H D, Melnyk C S, Kelley M L. National Cooperative Crohn’s Disease Study: Extraintestinal manifestations and perianal complications.  Gastroenterology. 1979;  77 914-920
  Google Scholar
• 5 Veloso F T, Carvalho J, Magro F. Immune-related systemic manifestations of inflammatory bowel disease. A prospective study of 792 patients.  J Clin Gastroenterol. 1996;  23 29-34
  Google Scholar
• 6 Apgar J T. Never aspects of inflammatory bowel disease and its cutaneous manifestations: A selective review.  Sem Dermatol. 1991;  10 138-147
  Google Scholar
• 7 Farmer R G, Hawk W A, Turnbull R B. Clinical patterns in Crohn’s disease: A statistical study of 615 cases.  Gastroenterology. 1975;  68 627-635
  Google Scholar
• 8 Goebell H, Förster S, Dirks E. et al . Morbus Crohn: Klinische Erkrankungsmuster in Beziehung zur Lokalisation.  Med Klin. 1987;  82 1-8
  Google Scholar
• 9 Linaker B D. Erythema nodosum presenting before inflammatory bowel disease.  Lancet. 1977;  29 931
  Google Scholar
• 10 Adler G. Morbus Crohn - Colitis ulcerosa. Heidelberg; Springer-Verlag 1996
  Google Scholar
• 11 May B. Extraintestinale Manifestationen der chronisch entzündlichen Darmerkrankungen.  Leber Magen Darm. 1997;  29 17-20 (3)
  Google Scholar
• 12 Areias E, Garcia e  Silva L. Cutaneous manifestations of ulcerative colitis (Portogese).  Med Cutan Ibero Lat Am. 1987;  15 185-187
  Google Scholar
• 13 Kirsch B, Gerhardt H, Gladisch R. et al . Dermatosen bei chronisch entzündlichen Darmerkrankungen. Ergebnisse einer Untersuchung von 119 M.-Crohn-/C.-ulcerosa-Patienten.  Akt Dermatol. 1992;  18 17-22
  Google Scholar
• 14 Yates V M, Watkinson G, Kelman A. Further evidence for an association between psoriasis, Crohn’s disease and ulcerative colitis.  Br J Dermatol. 1982;  106 323-330
  Google Scholar
• 15 Sigl I, Bauerdorf R. Granulomatous rosacea associated with ulcerative colitis: 2 case reports (German).  Z Hautkr. 1989;  64 499-502
  Google Scholar
• 16 Shum D T, Guenther L. Metastatic Crohn’s disease.  Arch Dermatol. 1990;  126 645-648
  Google Scholar
• 17 Lamers C BHW. Treatment of extraintestinal complications of ulcerative colitis.  Eur J Gastroenterol Hepatol. 1997;  9 850-853
  Google Scholar
• 18 Gregory B, Ho V C. Continuing medical education: Cutaneous manifestations of gastrointestinal disorders.  Part II. J Am Acad Dermatol. 1992;  26 371-383
  Google Scholar
• 19 Marshall J K, Irvine E J. Successful therapy of refractory erythema nodosum associated with Crohn’s disease using potassium sodide.  Can J Gastroenterol. 1997;  11 501-502
  Google Scholar
• 20 Levine J B, Lukawski-Trubish D. Extraintestinal considerations in inflammatory bowel disease.  Gastroenterol Clin North Am. 1995;  24 633-646
  Google Scholar
• 21 Phillips W G, Marsden J R. Skin disease in gastroenterology. Gastroenterology: Clinical science and practice 2 Philadelphia; WB Saunders Company 1993: 1328-1340
  Google Scholar
• 22 Basler R SW. Ulcerative colitis and the skin.  Gut. 1980;  64 941-944
  Google Scholar
• 23 Ochsendorf F R. Cutaneous manifestations of IBD and IBD-associated inflammatory lesions. Lembcke B, Kruis W, Sartor RB Kluver Academic Publishers Systemic manifestations of IBD - The pending challenge for subile diagnosis and treatment Frankfurt, Köln, Chapel Hill; 1998: 67-93
  Google Scholar
• 24 McCord M L, Hall R P. Cutaneous manifestations of inflammatory bowel disease. Targan Sr, Shanahan F Inflammatory Bowel Disease: From bench to bedside Baltimore, Philadelphia, Hong Kong, London, Munich, Sydney, Tokyo; Williams & Wilkins 1994: 682-694
  Google Scholar
• 25 Bargen J A. Complications and sequelae of chronic ulcerative colitis.  Ann Intern Med. 1929;  3 335-352
  Google Scholar
• 26 Brunsting L A, Goeckermann W H, O’Leary PA. Pyoderma (ecthyma) gangrenosum. Clinical and experimental observations in 5 cases occurring in adults.  Arch Dermatol. 1930;  22 655-680
  Google Scholar
• 27 Schoetz D J, Coller J A, Veidenheimer M C. Pyoderma gangrenosum and Crohn’s disease. Eight cases and a review of the literature.  Dis Colon Rectum. 1983;  26 155-158
  Google Scholar
• 28 Levitt M D, Ritchie J K, Lennard-Jones J E, Phillips R K. Pyoderma gangrenosum in inflammatory bowel disease.  Br J Surg. 1991;  78 676-678
  Google Scholar
• 29 Hüppe D, Schenck B, Tromm A. Extraintestinale Manifestation und Komplikationen bei chronisch aktivem Verlauf der Colitis ulcerosa im Kindes- und Jugendalter.  Dtsch Med Wochenschr. 1999;  124 824-828
  Google Scholar
• 30 Lysy J, Zimmerman J, Ackerman Z, Reifen E. Atypical auricular pyoderma gangrenosum simulating fungal infection.  J Clin Gastroenterol. 1989;  11 561-564
  Google Scholar
• 31 Cerdan F J, Cantero R, Martinez S. et al . Die gangränöse Pyodermie am Kolostoma eines Patienten mit Morbus Crohn.  Coloproctology. 1994;  16 407-410
  Google Scholar
• 32 Cairns B A, Herbst C A, Sartor B R, Brigamann R A, Koruda M J. Peristomal pyoderma gangrenosum and inflammatory bowel disease.  Arch Surg. 1994;  129 769-772
  Google Scholar
• 33 Giroux J M, Quellet A, Heppell J, Lacroix M. Peristomal pyoderma gangrenosum in a patient with ulcero-hemorrhagic rectocolitis.  Ann Dermatol Venerol. 1987;  114 935-939
  Google Scholar
• 34 Holmlund D E, Wahlby L. Pyoderma gangrenosum after colectomy for inflammatory bowel disease. Case report.  Acta Chir Scand. 1987;  153 73-74
  Google Scholar
• 35 Keltz M, Lebwohl M, Bishop S. Peristomal pyoderma gangrenosum.  J Am Acad Dermatol. 1992;  27 360-364
  Google Scholar
• 36 Ng C S, Wolfsen H C, Kozarek R A, Brubacher L L, Kayne A L. Chronic parastomal ulcers: Spectrum of dermatoses.  J Et Nurs. 1992;  19 85-90
  Google Scholar
• 37 Tjandra J J, Hughes L E. Parastomal pyoderma gangrenosum in inflammatory bowel disease.  Dis Colon Rectum. 1994;  37 938-942
  Google Scholar
• 38 Graham J A, Hansen K K, Rabinowitz L G, Esterly N B. Pyoderma gangrenosum in infants and children.  Pediatr Dermatol. 1994;  11 10-17
  Google Scholar
• 39 Snyder R A. Pyoderma gangrenosum involving the head and neck.  Arch Dermatol. 1986;  12 295-302
  Google Scholar
• 40 Lilford R J, Tindall V R, Batchelor A G. Post-surgical pyoderma gangrenosum of the vaginal fault associated with ulcerative colitis and behcet’s disease: A case report.  Eur J Obstet Gynecol Reprod Biol. 1989;  31 93-94
  Google Scholar
• 41 Sanusi I D, Gonzales E, Venable D D. Pyoderma granulomatosa of penile and scrotal skin.  J Urol. 1982;  117 547-549
  Google Scholar
• 42 Klein J D, Biller J A, Leape L I, Grand R J. Pyoderma gangrenosum occurring at multiple surgical incision sites.  Gastroenterology. 1987;  92 810-813
  Google Scholar
• 43 Powell F C, Schroeter A L, Su W P, Perry H O. Pyoderma gangrenosum: A review of 86 patients.  Q J Med. 1995;  55 173-186
  Google Scholar
• 44 Cox N H, Peebes-Brown D A, MacKie R M. Pyoderma gangrenosum occurring 10 years after proctocolectomy for ulcerative colitis.  Br J Hosp Med. 1986;  36 363
  Google Scholar
• 45 Talansky A L, Meyers S, Greenstein A J, Janowitz H D. Does intestinal resection heal the pyoderma gangrenosum of inflammatory bowel disease?.  J Clin Gastroenterol. 1980;  5 207-210
  Google Scholar
• 46 Novell L M, Malkinson F D. Commentary: Pyoderma gangrenosum.  Arch Dermatol. 1982;  118 769-773
  Google Scholar
• 47 Warneke B, Breitbart E W, Nasemann T. Pyoderma gangrenosum with special reference to immunologic and hematologic parameters.  Z Hautkr. 1988;  63 398-408
  Google Scholar
• 48 Das K M, Squillante L, Chitayet D, Kalloussek D K. Simultaneous appearance of a unique common epitope in fetal colon, skin, and biliary epithelial cells. A possible link for extra-colonic manifestations in ulcerative colitis.  J Clin Gastroenterol. 1992;  15 311-316
  Google Scholar
• 49 Hüner A, Hornstein O P, von den Driesch P. Pyoderma gangraenosum: Klinik, assoziierte Erkrankungen und Therapiemöglichkeiten.  Dtsch Ärztebl. 1995;  92 B1467-1471
  Google Scholar
• 50 Hoogkamp-Korstanje J A, de Koning J. Clinical aspects, diagnosis and therapy of Yersinia enterocolitica infections.  Immun Infekt. 1990;  18 192-197
  Google Scholar
• 51 Stolk-Engelaer V M, Hoogkamp-Kortanje J A. Clinical presentation and diagnosis of gastrointestinal infections by Yersinia enterocolitica in 261 Dutch patients.  Scand J Infect Dis. 1996;  28 571-575
  Google Scholar
• 52 Klein S, Mayer L, Present D H. et al . Extraintestinal manifestations in patients with diverticulitis.  Ann Intern Med. 1988;  108 700-702
  Google Scholar
• 53 Chow R KP, Ho V C. Treatment of pyoderma gangrenosum.  J Am Acad Dermatol. 1996;  34 1047-1060
  Google Scholar
• 54 Schwaegerle S M, Bergfeld W F, Wenitzer D, Tidrick R T. Pyoderma gangrenosum: A review.  J Am Acad Dermatol. 1988;  18 559-568
  Google Scholar
• 55 Stone O J. Sulfapyridine and sulfones decrease glycosaminoglycans viscosity in dermatitis herpetiformis, ulcerative colitis, and pyoderma gangrenosum.  Med Hypotheses. 1990;  31 99-103
  Google Scholar
• 56 Prochazka V, Rozkovcova J. Successful treatment with the Imuran and prednisone combination.  Cesk Dermatol. 1985;  56 173-177
  Google Scholar
• 57 Elgart G, Storek P, Larson K. et al . Treatment of pyoderma gangrenosum with cyclosporine: Results in seven patients.  J Am Acad Dermatol. 1991;  24 83-86
  Google Scholar
• 58 Matis W L, Ellis C N, Griffiths C E, Lazarus G S. Treatment of pyoderma gangrenosum with cyclosporine.  Arch Dermatol. 1992;  128 1060-1064
  Google Scholar
• 59 Shelley E D, Shelley W B. Cyclosporine therapy of pyoderma gangrenosum associated with sclerosing cholangitis and ulcerative colitis.  J Am Acad Dermatol. 1988;  18 1084-1088
  Google Scholar
• 60 Kaplan B, Trau H, Soefer E, Feinstein A, Schwebach-Millet M. Treatment of Pyoderma gangrenosum with clofacimine.  Int J Dermatol. 1992;  31 591-593
  Google Scholar
• 61 Callen J P, Case J D, Sager D. Chlorambucil is effective in pyoderma gangrenosum.  J Am Acad Dermatol. 1989;  21 515-519
  Google Scholar
• 62 Galun E, Flugelman M Y, Rachmilewitz D. Pyoderma gangrenosum complicating ulcerative colitis: Successful treatment with methylprednisolone pulse therapy and Dapsone.  Am J Gastroenterol. 1986;  81 988-989
  Google Scholar
• 63 Schorr-Lesnick B, Brandt L J. Selected rheumatological and dermatological manifestations of inflammatory bowel disease.  Am J Gastroenterol. 1988;  83 216-223
  Google Scholar
• 64 Cave D R, Burakoff R. Pyoderma gangrenosum associated with ulcerative colitis: Treatment with disodium cromoglycate.  Am J Gastroenterol. 1987;  82 802-804
  Google Scholar
• 65 Saffouri B, Horn B M, Metersdorf J M, Gardiner J E. Treatment of pyoderma gangrenosum with disodium cromoglycate.  Dig Dis Sci. 1984;  29 183-185
  Google Scholar
• 66 Richardson J B, Callen J P. Pyoderma gangrenosum treated successfully with potassium iodide.  J Am Acad Dermatol. 1993;  28 1005-1007
  Google Scholar
• 67 Goldstein F, Krain R, Thorton J J. Intralesional steroid therapy of pyoderma gangrenosum.  J Clin Gastroenterol. 1991;  7 499-501
  Google Scholar
• 68 Mir-Madjlessi S H, Taylor J S, Farmer R G. Clinical course and evolution of erythema nodosum and pyoderma gangrenosum in chronic ulcerative colitis: A study of 42 patients.  Am J Gastroenterol. 1985;  80 615-620
  Google Scholar
• 69 Bennent M L, Jackson J M, Jorizzo J L. et al . 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. 2000;  79 37-46
  Google Scholar

1 Dedicated to Prof. Dr. B. May (Bochum) on occasion of his 65^th birthday

Address for correspondence

Priv.-Doz. Dr. med. Andreas Tromm

Klinik für Innere Medizin Evangelisches Krankenhaus Hattingen Akademisches Lehrkrankenhaus der RUB

Bredenscheider Straße 54

45525 Hattingen

Email: Andreas.Tromm@ruhr-uni-bochum.de