Table 1.
Information obtained from reviewing published IBD-related BADAS cases
| References | Sex, age-associated diseases | Type of IBD; time from diagnosis of IBD to onset of symptoms | Skin lesions | Other symptoms of BADAS | GI symptoms | Laboratory tests findings; endoscopy finding | Treatment |
|---|---|---|---|---|---|---|---|
| Jorizzo et al. (1983) | F, 53; disseminated leiomyosarcoma | UC diagnosed simultaneously with BADAS | Erythematous papulovesicular, papulopustules with purpuric bases | Fever, arthralgia, oligoarthritis | Unknown | Leukocytosis, anemia; pancolitis | PRED |
| Fenske et al. (1983) | M, 32; none | UC diagnosed simultaneously with BADAS | Vesicles and pustules with necrosis | Fever | Weight loss, abdominal cramping, watery diarrhea | Leukocytosis; colitis | PRED, SSZ |
| Jorizzo et al. (1984) | F, 42; None | CD unknown | Erythematous pustules | Polyarthralgia | Unknown | Unknown | Colectomy, no additional therapy |
| Delaney et al. (1989) | M, 46; none | CD diagnosed 1 year before the first symptoms of BADAS | Erythematous macules, vesicopustules | Fever, arthritis | Weight loss, perianal ulceration | Leukocytosis, accelerated ESR; unknown | Underwent proctocolectomy, PRED, Cloxacillin, AZA |
| Matheson et al. (1996) | F, 16; none | CD diagnosed simultaneously with BADAS | Erythematous, maculopapular, plaques, lesions with pruritis | Fever | Weight loss, abdominal pain, hematemesis, hematochezia | Leukocytosis, accelerated ESR; deep ulcerations in anus, rectum | MP, oral H1 antagonists, topical corticosteroids |
| Vázquez et al. (2003) | M, 34; none | UC was diagnosed 12 years before the first symptoms of BADAS | Erythematous plaques, pustules | Fever | Severe diarrhea, abdominal pain | In normal ranges; diagnosis of IBD | PRED, SSZ |
| Mendoza et al. (2003) | F, 63; episcleritis | CD diagnosed simultaneously with BADAS | Erythematous plaques, nodules, vesicopustules | Fever, asymmetrical polyarthralgia | Weight loss, abdominal pain, mucosal stool movement | Anemia; multiple deep, wide ulcers alternating with normal mucosal from the sigmoid colon and ascending 70 cm | MP |
| Guerre-Schmidt et al. (2006) | F, 13, none | CD diagnosed simultaneously with BADAS | Erythematous pustules | Fever, oligoarthritis | Weight loss, rectal bleeding | Anemia, elevated CRP; ulcerated appearance of area of appendix | PRED |
| Ashok and Kiely (2007) | M, 23, none | CD was diagnosed 4 years before the first BADAS symptoms | Erythematous macules, papules, vesicopustular | Fever, arthritis, arthralgia, malaise | Stomal diarrhea, abdominal pain, vomiting | Leucocytosis, accelerated ESR, elevated CRP; unknown | PRED |
| Kinyó et al. (2011) | M, 32, none | CD diagnosed simultaneously with BADAS | Vesiculopustu les on erythematous base | Fever, arthritis | Bloody diarrhea | Leucocytosis, accelerated ESR, elevated CRP; Pancolitis. An ulcerated, granulomatous with abscess formation | MP, Metronidaz ole, cefuroxime, CsA, AZA |
| Lacey et al. (2011) | F, 24, none | UC diagnosed simultane ously with BADAS | Pustular lesions, non-tender | Fever, malaise | Abdominal pain, diarrhea, hematochezia | Leucocytosis; Severe UC | MP pulses, Metronidazole, Ciprofl oxacin, PRED, AZA |
| Truchuelo et al. (2013) | F, 35, none | UC diagnosed previous than BADAS | Erythematous plaques, nodules, vesicopustules with erythematous halo, painful | Fever, polyarthritis, malaise | Asthenia | Accelerated ESR; unknown | PRED, metronidazole |
| DeFilippis et al. (2014) | F, 64; depression, osteoporosis, spinal stenosis | UC was diagnosed 20 years before the first | Erythematous-violaceous macules | Arthralgia, arthritis | Abdominal pain, diarrhea | Accelerated ESR, elevated CRP; active microscopic inflammation on a right colon | PRED, MMF, SSZ |
| DeFilippis et al. (2014) | F, 64; depression, osteoporosis, spinal stenosis, gastroesophageal reflux | UC was diagnosed 20 years before the first symptoms of BADAS | Erythematous-violaceous macules | Arthralgia, arthritis | Abdominal pain, diarrhea | Accelerated ESR, elevated CRP; active microscopic inflammation on a right colon | Infliximab, MMF, SSZ |
| Aounallah et al. (2016) | F, 39, unknown | UC diagnosed previous than BADAS | Vesicles and pustules | Fever, arthralgia | Unknown | Unknown | PRED, AZA, CsA |
| Oldfield et al. (2016) | F, 4, sickle cell disease, autoimmune hepatitis | UC diagnosed previous than BADAS | Hemorrhagic vesicles | Fever, arthritis, malaise | Abdominal pain, congestion, hematochezia | Leucocytosis, anemia, elevated CRP; unknown | PRED, Metronidazole, Mesalazine, AZA, infliximab |
| Barland et al. (2016) | F, 42, unknown | CD was diagnosed 10 years before the first symptoms of BADAS | Erythematous nodules, vesiculopustules | Arthalgia | Unknown | Anemia, elevated CRP; unknown | Mesalazine, doxycycline |
| Heard et al. (2020) | F, 49, depression, anxiety, mitral valve prolapse, stroke | CD was diagnosed earlier than the onset of BADAS, but the date is unknown | Erythematous vesiculopustule, erosions | Fever, arthralgia | Diarrhea, rectal bleeding, dehydration | Anemia; severe ulceration in rectum and sigmoid | Piperacillin + tazobactam, ustekinumab |
| Havele et al. (2021) | M, 6, immune dysfunction, recurrent fever, uveitis | VEO-IBD diagnosed simultaneously with BADAS | Erythematous papules, vesiculopustules tender | Fever, arthralgia | Increase rectal output, vomiting, oral ulcers | Unknown | Infliximab, cedolizuma b, canakinum ab, mesalazine, ileostomy |
| Havele et al. (2021) | M, 6, Immune dysfunction, recurrent fever, uveitis | VEO-IBD diagnosed simultaneously with BADAS | Erythematous papules, vesiculopustules tender | Fever, arthralgia | Increase rectal output, vomiting, oral ulcers | Unknown | Triamcinol one topical |
| Havele et al. (2021) | M, 6, immune dysfunction, recurrent fever, uveitis | VEO-IBD diagnosed simultaneously with BADAS | Erythematous papules, vesiculopustules tender | Fever, arthalgia | Increase rectal output, vomiting, oral ulcers | Unknown | Subtotal colectomy, triamcinol one topical, ruxolitinib |
| Caro et al. (2021) | F, 78, erythema nodosum | UC diagnosed simultaneously with BADAS | Erythematous and purple plaques, nodules, | Arthralgia, arthritis | Abdominal pain, diarrhea, tenesmus, mucus in stool | accelerated ESR, elevated CRP; a recto sigmoid UC | Mesalazine, rifaximin |
| Russell Johns et al. (2022) | F, 14; none | UC diagnosed simultaneously with BADAS | Violaceous nodules, indurated plaques, painful, | Fever, migratory arthritis, malaise | Abdominal cramping, bloody diarrhea | Leucocytosis, anemia, accelerated ESR, elevated CRP; eosinophilic colitis from the rectum to the sigmoid colon | MP pulses, followed by PRED + SS Z followed by infliximab + SSZ |
| Alshahrani et al. (2022) | M, 39; none | UC was diagnosed 3 years before the symptoms of BADAS | Erythematous papular, maculopapular, urticaria-like, vesiculopustular lesions | Fever, Polyarteritis, Achilles tendinitis, malaise | Weight loss, bloody diarrhea, | Anemia, accelerated ESR, elevated CRP; hyperemia with mild edematous mucosal changes in the sigmoid colon | PRED, Mesalazine, Metronidaz ole, Fluoroquinolone, AZA |
| Case 1 (in our present publication) | F, 30; beta thalassemia | UC diagnosis in 2012, first symptoms of BADAS in 2017, BADAS diagnosis in 2021 | Edematous erythematous macules, maculopapular lesions | Fever, arthritis, arthralgia malaise | Bloody diarrhea | Anemia, elevated CRP, high D-dimer – 6478 ng/mL; unknown | PRED, Mesalazine, Cefuroxime, Fluoroquinolone, CsA, AZA |
| Case 2 (in our present publication) | M, 67; benign prostatic hyperplasia, osteoarthritis | CD diagnosed in 2020, BADAS diagnosed in 2023 | Edematous erythematous macules, maculopapular, vesicopustular lesions | Fever, arthritis, arthralgia | Abdominal pain, diarrhea, mucus in stool | Leucocytosis, anemia, elevated CRP, high D-dimer – 7200 ng/mL; the entire length of the sigmoid colon and the final section of the descending colon with a thickened, swollen wall | Metronidazole, PRED, ciprofloxacin, topical clobetasol propionate, infliximab |
[i] AZA, azathioprine; BADAS, bowel-associated dermatosis and arthritis syndrome; CD, Crohn’s disease; CRP, C-reactive protein; CsA, cyclosporine; ESR, erythrocyte sedimentation rate; F, woman; GI, gastrointestinal; IBD, inflammatory bowel disease; M, male; MMF, mycophenolate mofetil; MP, methylprednisolone; PRED, prednisone; SSZ, sulfasalazine; UC, ulcerative colitis.
Fig 1.
(A) Ankle swelling. (A, B) Erythematous macules and papules on the patient’s lower limbs.
Fig 2.
(A, B) Red spots and papules on the patient’s upper limbs. (C) After treatment, on 24-month follow-up.
Fig 3.
(A, B) Swelling and erythematous spots on the skin and asymmetric swelling of the ankle joints and wrist. (C, D) After treatment, the lesions on the upper and lower limbs healed.
Table 2.
Clinical symptoms of BADAS
| Enteric-Associated Dermatosis and Arthritis Syndrome (BADAS) | ||
|---|---|---|
| GI symptoms (%) | Skin symptoms | Rheumatological symptoms |
|
|
|
Fig 4.
(A–D) The dermis shows intense perivascular, multicellular inflammatory infiltrates with a dominant neutrophilic component. Damaged blood vessels with fibrinoid necrosis are infiltrated by inflammatory cells. Dermis with elastosis and edema. Image provided by the Dermatology Clinic, PIM MSWIA.
Fig 5.
(A, B) Epidermal skin biopsy with focal hyperkeratosis and parakeratosis. Epidermal spinous layer with local acanthosis and flattening of the retinal ridges. Image provided by the Dermatology Clinic, PIM MSWIA.
Table 3.
Differential diagnosis of BADAS with other neutrophilic dermatoses
| Disease | Clinical features | Skin lesions | Diagnosis |
|---|---|---|---|
| BADAS syndrome |
|
|
|
| Sweet syndrome |
|
|
|
| Pyoderma gangrenosum |
|
|
|
| Erythema nodosum | -Rarely general symptoms in cases associated with infections or sarcoidosis |
|
|
| PPV |
|
|
|
Table 4.
Treatment algorithm for BADAS with the focus on biologic therapies
| 4A. Practical treatment algorithm | |||
|---|---|---|---|
| Disease severity | Recommended therapy | Notes | |
| Mild–moderate | Antibiotics (e.g., metronidazole, ciprofloxacin), Optimize IBD treatment (e.g., 5-ASA, mesalazine) | Targeting bacterial overgrowth and intestinal inflammation may be sufficient in early stages and mild flares | |
| Moderate–severe/refractory | Systemic GCS – prednisone (0.5–1 mg/kg/day) or MP | Remains first-line therapy, choose i.v. MP in severe flares. Can be combined with other immunosuppressants | |
| Steroid-sparing/maintenance | Immunosuppressants (e.g., cyclosporine, azathioprine, MMF), Biologics (anti-TNF, anti-IL-12/23, anti-integrin, anti-IL-1) | Choice depends in comorbidities, IBD activity, recurrence rest. Biologics are a key to long-term management | |
| 4B. Focus on biologic agents | |||
| Agent/class | Mechanism of action | Evidence in BADAS or other neutrophilic dermatoses | Practical notes |
| Infliximab (anti-TNF-alfa) | Monoclonal antibody against TNF-α; suppresses systemic and mucosal inflammation | Most frequently reported biologic in BADAS (≥4 cases); strong evidence from IBD and neutrophilic dermatoses literature, including RCTs for pyoderma gangrenosum | Preferred option in BADAS with active IBD; rapid onset with great efficacy for the control of bowel, joint, and skin symptoms |
| Ustekinumab (anti-IL-12/23) | Blocks p40 subunit shared by IL-12 and IL-23, reducing Th1/Th17 activity | Reported in one BADAS case; broader evidence in IBD alone; retrospective, robust case series suggest benefit in neutrophilic dermatoses | Consider if anti-TNF is contraindicated or failed; slower onset than infliximab |
| Vedolizumab (anti-α4β7 integrin) | Intestine-selective blockade of lymphocyte trafficking | Reported in one BADAS case (pediatric); effective in IBD but less reliable for neutrophilic dermatoses (localized mode of action) | Best for cases where gut activity drives BADAS – severe IBD flare-up; may be insufficient for systemic/skin inflammation |
| Canakinumab (anti-IL-1β) | Monoclonal antibody against IL-1β; innate immunity inhibitor | Reported in one BADAS case (pediatric); low level of evidence | Reserve for highly refractory BADAS; use only after careful risk–benefit assessment |