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Inflammatory Bowel Disease Presenting as a Rash

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Authors:
Jane Alookaran, MD, and John J. Sarandria, MD
Department of Pediatrics, University of Florida College of Medicine–Jacksonville

Jose Zayas, DO
Division of Hospital Pediatrics, Department of Pediatrics, University of Florida College of Medicine–Jacksonville

Citation:
Alookaran J, Sarandria JJ, Zayas J. Inflammatory bowel disease presenting as a rash [published online October 25, 2017]. Consultant for Pediatricians.

 

A 13-year-old girl with a history of constipation and anemia presented with abdominal pain and a new rash. She and her mother reported that the girl had had daily, intermittent episodes of epigastric and lower abdominal cramping that had often been followed by watery, nonbloody, nonmucoid diarrhea over the past 2 months. Her pain was not associated with any particular food or with eating. The pain was not severe and did not radiate. She took no medications to alleviate it.

Her rash was painless, nonpruritic, and limited to her upper and lower extremities. Additionally, her mother noted that the girl had experienced fatigue and a 3.6-kg weight loss during this same 2-month period. The patient denied a history of fever, joint pain, urinary symptoms, easy bruising, prolonged bleeding, oral ulcerations or other mucosal involvement, and abdominal trauma.

She had previously received a diagnosis of iron-deficiency anemia, presumed to be secondary to menstrual blood loss, and she had been taking oral iron supplementation. She took no other medications. She noted that her fatigue had continued despite the iron supplementation. Her father had a history of irritable bowel syndrome. She reported no surgical procedures or hospitalizations, and she had no known allergies.

Physical examination. Initial examination revealed an afebrile, sallow, but pleasant girl in no distress. Her vital signs were normal for her age. Her height was 157 cm (55th percentile) and her weight was 55 kg (87th percentile), corresponding to a body mass index of 22.3 kg/m2 (87th percentile).

The examination findings were notable for several tender, nonblanching, red, slightly raised and palpable discrete lesions on the lateral and extensor surface of her upper arms and on the anterolateral aspect of her lower legs bilaterally (Figures 1 and 2). These lesions turned purplish brown over the next several days. She also had an anal skin tag and bilateral brachymetatarsia.

 

IBD purpura figure 1

IBD purpura figure 2

She had no alopecia or oral lesions except for a geographic tongue. There was no conjunctival injection or drainage. There was no cervical mass or lymphadenopathy. Cardiac and lung examination findings were unremarkable. Her abdomen was soft and not tender or distended; no organomegaly or mass was appreciated. The remainder of physical examination findings were unremarkable.

Diagnostic testing. Her stool tested positive for occult bleeding, but infectious study results were nondiagnostic. Her hemoglobin level was low at 8.5 g/dL (reference range, 11.3-13.4 g/dL) with a mean corpuscular volume of 77.8 µm3 (reference range, 79.5-85.2), and her C-reactive protein level was elevated at 6.8 mg/dL (reference range, <1.0 mg/dL). Her stool calprotectin level was significantly elevated at more than 2000 µg/g (reference range, ≤50 µg/g). The remainder of the findings of a complete blood cell count, a basic metabolic panel, and liver function tests were within normal limits.

An abdominal computed tomography scan revealed thickening from the distal ascending to transverse colon and terminal ileum, suggestive of inflammatory bowel disease (IBD). A colonoscopy revealed patchy areas of chronic colitis with multiple noncaseating granulomas on a biopsy specimen, a finding consistent with Crohn colitis.

Discussion. Extraintestinal conditions associated with IBD (including nonspecific symptoms such as arthralgias) are identified in approximately 10% of patients at presentation and up to 30% of patients within the first few years after IBD diagnosis. The conditions do not consistently correlate with the degree of intestinal inflammation.1,2

The skin manifestations that have been described with IBD are erythema nodosum (most common), pyoderma gangrenosum, neutrophilic dermatoses, cutaneous vasculitis, and epidermolysis bullosa acquisita. However, the occurrence of skin manifestations prior to the development of IBD is rare.3,4

In patients with IBD, sometimes the vasculitic lesions coalesce, ulcerate, or become surrounded by hemorrhagic bullae.

Patient outcome. This patient presented with a mild, nonblanching, palpable, purpuric rash that was clinically consistent with a vasculitis. Although we did not biopsy any of the lesions, the skin findings likely represent a leukocytoclastic vasculitis that has been described in IBD.5 The patient was started on metronidazole, mesalamine, and infliximab. Her rash began to fade by the time of discharge.

REFERENCES:

  1. Jose FA, Garnett EA, Vittinghoff E, et al. Development of extraintestinal manifestations in pediatric patients with inflammatory bowel disease. Inflamm Bowel Dis. 2009;15(1):63-68.
  2. Dotson JL, Hyams JS, Markowitz J, et al. Extraintestinal manifestations of pediatric inflammatory bowel disease and their relation to disease type and severity. J Pediatr Gastroenterol Nutr. 2010;51(2):140-145.
  3. Bernstein CN, Blanchard JF, Rawsthorne P, Yu N. The prevalence of extraintestinal diseases in inflammatory bowel disease: a population-based study. Am J Gastroenterol. 2001;96(4):1116-1122.  
  4. Chiu G, Rajapakse CN. Cutaneous polyarteritis nodosa and ulcerative colitis. J Rheumatol. 1991;18(5):769-770.
  5. Akbulut S, Ozaslan E, Topal F, Albayrak L, Kayhan B, Efe C. Ulcerative colitis presenting as leukocytoclastic vasculitis of skin. World J Gastroenterol. 2008;14(15):2448-2450.