Jason Schairer, MD, on Skin Lesions in IBD

Properly assessing any extraintestinal manifestations of inflammatory bowel disease (IBD) on the skin of patients “can lead to faster and more accurate diagnosis, get patients on appropriate therapy sooner and overall just lead to a better quality of life for our patients with IBD,” Jason Schairer, MD, stated at the Advances in Inflammatory Bowel Diseases regional meeting September 30.

Dr Schairer is the director of the Henry Ford IBD Center in Detroit, Michigan.

“Humans exist on a continuous and variable scale” of skin tones, he said, which makes it very important for clinicians to understand how certain lesions may appear on darker skin as opposed to fair skin in order to avoid mistakes in diagnosis.

Among the more common skin lesions seen among patients with IBD is pyoderma gangrenosum, which causes painful lesions. Dr Schairer shared a case in which a patient in the first stage of 3-stage surgery for ileal pouch anal anastomosis suffered leakage of the ostomy and the breakdown of skin around the ostomy, resulting in a lesion with the deep edges and violaceous border characteristic of pyoderma gangrenosum. Dr Schairer noted that to confirm the diagnosis biopsy of the lesion may be needed.

“The hallmark is pathergy, in which small lesions can rapidly expand,” he explained. “You need to do something to ensure that stool is kept away from the skin” via different type of adhesive or use of a belt.

It is unknown if pyoderma gangrenosum is related to IBD disease activity, he said. Even in the absence of the colon, there may still be inflammation in Hartmann’s pouch, which can be determined via a quick scoping procedure. If there is inflammation, then the patient may need systemic therapy to control it. Anti-tumor necrosis factor (TNF) agents are the first-line treatment, “but in my practice in 2022, I don’t have a lot of bio-naïve patients coming in the door,” Dr Schairer stated. If the patient has been exposed to anti-TNFs and has not responded, then anti-integrins or interleukin inhibitors can be used to help control any remaining inflammation.

He shared another case in which a dark-skinned patient showed hyperpigmentation with an ulceration, which could be mistaken for pyoderma gangrenous. However, photos of a light-skinned patient with similar ulceration and hyperpigmentation revealed that the actual diagnosis is dermatitis herpetiformis, often associated with celiac disease.

“Patients with IBD are more likely to develop celiac disease,” Dr Schairer noted, especially those with Crohn disease. In small bowel Crohn disease patients often have villious atrophy and present with anemia, which are common in celiac disease. The diagnosis of celiac disease requires a duodenal biopsy to assess the Marsh lesion. If the clinician suspects a patient with IBD may also have celiac—or vice versa—Dr Schairer stressed the importance of discussing the clinical scenario with the pathologist to ensure accurate diagnosis.

“Why would we test for celiac disease in African-Americans?” Dr Schairer asked. “In my training we were told African-American patients a low rate of celiac disease so maybe we didn’t have to check. And that is obviously a problematic statement.” He noted that people come to the US from all over the world and there is significant mingling of various genetic populations here. “It’s exceedingly difficult to look at one person in the office and say, ‘I do or don’t think you have certain types of genes.’ So it’s appropriate to treat all of our patients equally and make sure we’re testing because anyone could have a genetic condition.” He also noted that patients may present with hyperpigmentation or with melanocyte dropout leading to hypopigmentation, “so there can be variations in the appearance of dermatitis herpetiformis.”

Dr Schairer illustrated the variations in presentation of another skin manifestation based on skin tone. On darker skin the appearance of hyperpigmentation and ulcerations could be seen as pyoderma gangrenosum. On lighter skin in a different location—the bottom of the feet—it might be seen as paradoxical psoriasis. “But both these lesions are the same— acrodermatitis enteropathica.” This condition is caused by zinc deficiency and generally thought to occur most often around the mouth and anus, Dr Schairer said. “But it can manifest anywhere in the body,” including the hands, as illustrated by another image.

There are 2 forms of acrodermatitis enteropathica: hereditary and acquired. The former tends to present very early in life. The acquired form is primarily due to the inability to absorb zinc in the GI tract or because the patient is on total parenteral nutrition that does not include zinc in the replacement fluid. Up to 15% of patients with IBD have zinc deficiency, which is associated with higher risk of hospitalization and surgery. If these patients receive zinc sulfate replacement therapy their risks return to the same level as those of IBD patient without zinc deficiency.

“The difficult part is that zinc does not exist in the body by itself. It exists in a balance with other minerals,” Dr Schairer explained. Vitamin D supplementation can drive zinc levels down; boosting zinc can drive iron and copper levels. “So we have to be thoughtful. We can’t just replace without monitoring.”

Reactivation of varicella zoster, the virus that causes chicken pox, results in the development of shingles, a painful outbreak of lesions that can occur on any part of the body but often presents on the trunk. Many patients with IBD who are 20 to 25 years old never had chicken pox but did get the vaccine, which is a live virus that can be reactivated as shingles.

Among the risk factors for developing shingles are several agents used to treat IBD, including steroids, thiopurines, and JAK inhibitors but “this can happen with most of our agents,” Dr Schairer said. Treatments include acyclovir and valcyclovir.

Using 3 different photos of darker, medium, and light skin tones, Dr Schairer illustrated how the presentation on the lightest skin of macules and papules on the chest and back make it clear that these patients all have acute febrile neutrophilic dermatosis, or Sweet syndrome.

He reiterated importance of assessing the skin during examination of patients with IBD. “I want us to be aware of differences between patients and their lesions, and how recognizing them can allow us to help more people,” Dr Schairer concluded.

—Rebecca Mashaw

Schairer, J. What lies beneath: skin lesions in IBD. Presented at: Advances in Inflammatory Bowel Diseases regional meeting; September 30, 2022. Virtual.