Abstract
A 6-year-old boy with severe very early-onset inflammatory bowel disease (VEO-IBD) was admitted for 1 week of high fevers, loose stools, joint pains, and myalgias. He subsequently developed a progressive, papular, and vesiculopustular eruption on his face with rapid spread to his trunk and extremities. Histopathology demonstrated dense dermal neutrophilic inflammation. Findings were consistent with bowel-associated dermatosis-arthritis syndrome (BADAS), which is rarely reported in children and requires further characterization.
Keywords: bowel-associated dermatoses-arthritis syndrome, inflammatory bowel disease, neutrophilic dermatosis, sweet syndrome
1 |. CASE REPORT
A 6-year-old boy with severe very early-onset inflammatory bowel disease (VEO-IBD), immune dysregulation, recurrent fevers, uveitis, oral ulcers, and markedly elevated inflammatory markers, refractory to medical management (infliximab, vedolizumab, canakinumab, antibiotics, mesalamine, and steroids) and requiring diverting ileostomy, was admitted for 1 week of high fevers, increased rectal output, vomiting, and pain with walking. On day 3 of admission, he developed tender, erythematous papules and vesiculopustules across his face, which spread over hours to his trunk and arms (Figure 1). Infectious workup was negative.
FIGURE 1.
Cutaneous lesions in a child with BADAS. Pink edematous papules coalescing into plaques across the face with pinpoint pustules scattered on the face, trunk, and upper extremities. Right arm with a focal vesicle overlaying a pink edematous plaque (day 3 of admission)
Histopathology from a punch biopsy showed a dense, dermal, neutrophilic infiltrate with prominent epidermal spongiotic vesicles (Figure 2). Special stains for infectious organisms were negative. Given his clinical picture, he was diagnosed with bowel-associated dermatosis-arthritis syndrome (BADAS) and improved with topical triamcinolone. Five weeks later, he developed erythematous patches with superimposed pustules involving his trunk near the stoma site, presumed to be BADAS recurrence. The eruption improved after subtotal colectomy, continuation of topical triamcinolone, and initiation of ruxolitinib for his refractory IBD.
FIGURE 2.
Medium power view shows prominent spongiotic vesicles, neutrophils in the epidermis, papillary dermal edema, and a dense dermal suppurative inflammatory infiltrate (H&E, 65×)
2 |. DISCUSSION
Bowel-associated dermatoses-arthritis syndrome (BADAS) is a rare neutrophilic dermatosis that presents episodically with flu-like symptoms followed by the development of progressive papules, vesiculopustules, and tender subcutaneous nodules on the trunk and extremities.1,2
Bowel-associated dermatosis-arthritis syndrome has been associated with several gastrointestinal conditions in adults, including bariatric surgery, peptic ulcers, diverticulitis, appendicitis, IBD, and cystic fibrosis.1,3 Very few cases have been reported in pediatric patients with IBD and short bowel syndrome.2,4,5
For most patients, cutaneous symptoms emerge after a flu-like prodrome, including fever, malaise, polyarthralgia, and tenosynovitis, as our patient experienced. Skin findings typically last for 1 week and then spontaneously regress, with the potential for ongoing flares every 4–6 weeks.1,2 Pathophysiology is thought to be secondary to bacterial overgrowth within inflamed areas of the gastrointestinal tract, leading to immune-complex formation and deposition in the skin. Histopathology shows a neutrophilic infiltrate, dermal edema, and absence of fibrinoid necrosis.2 While not always present, spongiosis may also be seen. Histopathology is similar to Sweet syndrome, but clinical history differentiates the two.
Treatment for BADAS includes steroids, antibiotics, therapies for the underlying gastrointestinal disease, and in some cases surgery. Our patient responded to topical steroids, subtotal colectomy, and was subsequently treated with ruxolitinib, a janus kinase (JAK) inhibitor, due to his persistent hyperinflammatory VEO-IBD. While there is no data for ruxolitinib use in IBD, this medication was chosen due to his pattern of immune dysregulation, similar to patients with known mutations in the JAK/Stat pathway.
Our case highlights that IBD may be a more likely clinical scenario for BADAS in children and that treatment of the underlying gastrointestinal disease with medication or surgery is a critical element of therapy. Increased awareness of BADAS in pediatrics is important, given the rising rates of IBD among children and the fact that BADAS may be the initial presentation of IBD, as described in previous case reports.5
REFERENCES
- 1.Thrash B, Patel M, Shah KR, Boland CR, Menter A. Cutaneous manifestations of gastrointestinal disease: Part II. J Am Acad Dermatol 2013;68(2):211.e1–211.e33. 10.1016/j.jaad.2012.10.036 [DOI] [PubMed] [Google Scholar]
- 2.Lee GL, Chen AYY. Neutrophilic dermatoses: Kids are not just little people. Clin Dermatol 2017;35(6):541–554. 10.1016/j.clindermatol.2017.08.005 [DOI] [PubMed] [Google Scholar]
- 3.Rosen JD, Stojadinovic O, McBride JD, Rico R, Cho-Vega JH, Nichols AJ. Bowel-associated dermatosis-arthritis syndrome (BADAS) in a patient with cystic fibrosis. JAAD Case Rep 2019;5(1):37–39. 10.1016/j.jdcr.2018.08.029 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 4.Pereira E, Estanqueiro P, Almeida S, Ferreira R, Tellechea O, Salgado M. Bowel-associated dermatosis-arthritis syndrome in an adolescent with short bowel syndrome. J Clin Rheumatol 2014;20(6):322–324. 10.1097/RHU.0000000000000137 [DOI] [PubMed] [Google Scholar]
- 5.Guerre-Schmidt AR, Pelletier F, Carbonnel F, Humbert P, Aubin F. Syndrome arthrocutané associé à une maladie de Crohn chez une adolescente. Rev Med Interne 2006;27(11):874–877. 10.1016/j.revmed.2006.07.017 [DOI] [PubMed] [Google Scholar]