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Inflammatory Bowel Disease with Acute Leukocytoclastic Vasculitis of Skin Aamir Dam, MD, Marina Kim MD, and Xinjun Zhu, MD
Division of Gastroenterology and Hepatology, Albany Medical Center, Albany, NY 12208
BACKGROUND
Leukocytoclastic vasculitis (LCV) is an immune complex syndrome involving small vessels characterized by neutrophilic invasion, endothelial damage and fibrinoid necrosis. The most common etiologies include infections, drugs, connective tissue diseases, and malignancy. Lesion classically appearing as palpable purpura but also may appear as necrotic ulcers. Rarely, cutaneous LCV results from inflammatory bowel disease (IBD), and the pathogenesis is unclear. It has been postulated that the disrupted mucosal barrier in the inflamed gut may lead to the formation of immune complexes by the exposure of fecal antigens to intestinal immune cells. These immune complexes may then precipitate within the wall of the small vessels leading to cutaneous lesions.
DISCUSSION HPI: 28 year old man with no significant past medical history who presented initially to an outside hospital after 1 month of intermittent rectal bleeding, diffuse abdominal pain, and weight loss. One week prior to admission, he reported increased bloody diarrhea up to 16 bowel movements per day. Simultaneously, he noted a non-pruritic, maculopapular rash on the medial aspect of his right lower extremity that began at the ankle and subsequently migrated into the groin. In addition, he developed oligoarticular arthralgias in the upper and lower extremities, splinter hemorrhages under the fingernails and severe pain in his toes with associated bluish-purple discoloration.
LCV is an uncommon extraintestinal manifestation of IBD. Approximately, fourteen previous cases of LCV in association with Crohn’s disease and Ulcerative colitis have been described. In the majority of cases, LCV either preceded diagnosis of IBD or occurred synchronous with intestinal symptoms. Two cases did report onset of LCV years after diagnosis. Generally, the treatment can be directed at the underlying etiology. Case reports involving IBD and LCV have described a clinical response to pulse dose steroids, mesalamine, and biologic therapies. One case required total colectomy for refractory Ulcerative colitis. Our case report is the first describe the use of combination of biologic and immunomodulator therapy in the setting of IBD and small vessel vasculitis. The increasing literature supports that cutaneous lesions of unknown cause, may warrant further investigation of the gastrointestinal tract for IBD. In addition, given the fulminant presentation our case with associated hypercoagulability implies LCV may portend a more severe clinical course of IBD that may be refractory to first line therapies. Further studies are needed to confirm these observations.
CASE PRESENTATION (Cont.)
PMHx: Seasonal allergies, no hx tobacco use
Medications: Loratadine
Physical Exam: Abdomen nontender, nondistended. normactive bowel sounds. No
hepatosplenomegaly. Extremeties: Dusky purplish discoloration of the lower extremity
digits
Significant Labs/Studies:
WBC 32, Hgb 8.5, Hct 25.1, PLT 286 Na 129, K 4.2, CL 101, CO2 19, BUN 7, Cr 1.1 Albumin 1.0, LFTs WNL, CRP 214 P-ANCA 1:80, C3 low, C4, cryoglobulins, RF, ANA, C-ANCA ,
Hepatitis B and C serologies were normal. CT A/P: Pancolitis with mild ascites and nonspecific mesenteric
adenopathy
CASE PRESENTATION
REFERENCES
1.Karatoprak, C., et al., Gastroenterology cases of cutaneous leukocytoclastic vasculitis. Case Rep Med, 2013. 2.Limdi, J.K., H.M. Doran, and J.R. Crampton, Cutaneous vasculitis in Crohn's disease. J Crohns Colitis, 2010. 4(3): p351-2. 3.Tsiamoulos, Z., et al., Leukocytoclastic vasculitis as an onset symptom of Crohn's disease. Case Rep Gastroenterol, 2008. 2(3): p.410-4. 4.Plaza Santos, R., et al., [Leukocytoclastic vasculitis associated with Crohn's disease]. Gastroenterol Hepatol, 2010. 33(6): p. 433-5. 5.Zlatanic, J., et al., Crohn's disease and acute leukocytoclastic vasculitis of skin. Am J Gastroenterol,1996. 91(11): p.2410-3. 6.Martin, D., T. Handler, and J. McDermott, Leucocytoclastic vasculitis in severe ulcerative colitis. Mil Med, 2011. 176(5): p. 581-3. 7.Sipponen, T., S. Virolainen, and P. Arkkila, A patient with ulcerative colitis and an unusual rash. Gastroenterology, 2010. 139(6): p. e5-6. 8.Tripodi Cutri, F., et al., Ulcerative colitis associated with leukocytoclastic vasculitis of the skin. Dig Liver Dis, 2009. 41(7): p. e42-4. 9.Akbulut, S., et al., Ulcerative colitis presenting as leukocytoclastic vasculitis of skin. World J Gastroenterol, 2008. 14(15): p. 2448-50. 10.Iannone, F., et al., Leucocytoclastic vasculitis as onset symptom of ulcerative colitis. Ann Rheum Dis, 2003. 62(8): p785-6.
AIM
Intervention: During hospitalization, treated initially with IV methylprednisolone with improvement in lower extremity rash and digital necrosis. He continued to have significant bloody diarrhea and was initiated on Remicade 5mg/kg and after the second dose of induction therapy at week two, his bloody diarrhea improved with a decrease frequency in bowel movements. CRP upon discharge was 7.3.
Readmitted 2 months later with a significant flare further complicated by lower extremity deep vein thrombosis. Patient was started on 6-mercaptopurine 50mg and Remicade increased to 10mg/kg q8 weeks with improvement in clinical symptoms and inflammatory markers.
Case,
Author
Age of
Patient at
Diagnosis
of LCV
Crohn’s
(CD) or
Ulcerativ
e Colitis
(UC)
Timing of Rash with
Gastrointestinal (GI)
symptoms
Treatment and response of LCV and IBD
Zlatanic et
al.
39 CD Prolonged hx of CD, onset
of rash with flare
Rash and GI symptoms responded to IV
hydrocortisone.
Karatoprak
et al.
28 CD Simultaneous Methylprednisolone 60mg/day; ciprofloxacin
1000mg/day and metronidazole 1500mg/day. CRP
improved but rash did not resolve. Switched to pulse
dosed methylprednisolone (1g/day) and rash
resolved.
Tsiamoulos
, Z.
80
CD Rash preceded bloody
diarrhea by 2 months
Prednisolone 50mg IV; ciprofloxacin 500 IV bid,
mesalamine 500mg tid. Skin rash and diarrhea
resolved on day 5 of treatment.
Limdi, J.K 52 CD Prolonged hx of CD, then
rash developed without
concomitant flare
Steroid therapy for ten days
Akbulut et
al.
20 UC and
PSC
Rash preceded bloody
diarrhea by 8 months
Diarrhea and skin lesions resolved after 1 month of
mesalamine at 2g/day
Iannone et
al.
22 UC 3.5 year history of skin
lesions prior to diagnosis
of UC
Sulfasalazine 3g/day and rectal steroids. No skin
lesions at one year follow up.
Skin lesions did not previously respond to
hydroxychloroquine, prednisone, and cyclosporine.
Tripodi
Cutri et al.
33 UC 4 months of diarrhea then
skin lesions developed
Oral mesalamine 4g/day with rectal mesalamine
2g/day led to resolution of both GI symptoms and
rash in 2 weeks
Sipponen
et al.
28 UC UC diagnosed 4 months
before rash appeared
GI symptoms and rash improved with Infliximab
5mg/kg.
Martin et
al.
26 UC Hx of UC, onset of rash
with flare
Oral and rectal mesalamine, IV hydrocortisone and
infliximab was administered with improvement in GI
and skin symptoms. Ultimately, UC proved refractory
to medical therapy and patient underwent a total
abdominal colectomy.
Figure 1: Cutaneous rash/necrotic ulcers involving lower extremity digits.
Figure 2: Skin biopsy histological view
Figure 3: Colonoscopy and pathology.
A. HE x 100: 2 foci LCV B. HE x 400: Extravasated red blood cells and inflammatory cells within the
vascular wall. Fibrin thrombi also noted.
B. Crypt architectural distortion and cryptitis.
A. Colonoscopy: severe friable, hemorrhagic, inflamed, ulcerated mucosa in entire examined colon.
REVIEW OF LITERATURE ON LCV AND IBD
The authors had no financial disclosures
• Illustrate an unusual case of a patient with LCV in the setting of IBD. • Present the current literature regarding the association between these
two conditions.
P017
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