sweet syndrome and ibd

Sweet's syndrome (SS) is a neutrophilic dermatosis characterised by the acute onset of erythematous papules or plaques and a constellation of symptoms including fever, leucocytosis, and histopathology demonstrating nodular, pustular, or diffuse infiltrate of neutrophils with marked papillary oedema. SS can be a manifestation of inflammatory bowel disease and often coincides with periods of disease flares. Only a few cases of SS associated with ulcerative colitis are reported in the literature, and no cases have involved pulmonary manifestations in the paediatric population...

This case report highlights the clinical challenge and need to distinguish Sweet syndrome and erythema nodosum (EN) in a 50-year-old woman with newly initiated azathioprine for inflammatory bowel disease. While she initially presented with clinical features concerning for drug-induced Sweet syndrome, a subsequent histopathological examination confirmed early-stage EN. Both Sweet syndrome and EN share common triggers and therapeutic responses, but have distinctive clinical characteristics. Subtle histologic differences also exist in lesion distribution and depth of infiltration...

Neutrophilic dermatosis of the dorsal hands (NDDH) is a variant of Sweet syndrome that presents with erythematous bullae, papules/plaques, or pustules on the dorsal hands. It is most commonly associated with hematologic and solid organ malignancies, though cases of NDDH associated with inflammatory bowel disease, rheumatologic disorders, and medication exposure have also been described in the literature. Felty syndrome is a rare complication of long-standing rheumatoid arthritis characterized by neuropathy, splenomegaly, and neutropenia...

Neutrophilic dermatosis of the hands (NDDH) is a localized variant of Sweet's syndrome which has been recently introduced. Strutton et al.in 1996 and then in Galaria et al. in 2000 reported cases with violaceous papulonodules on the dorsal surfaces of the hands with histopathological findings of a neutrophilic dermatosis in association with leukocytoclasia, but clinically and histologically without true vasculitis findings. Eventually, they proposed the term NDDH for these lesions (1,2). A 46-year-old man was referred to our outpatient dermatology clinic with a painful ulcerative lesion on the dorsal side of the left hand that had been present for one year...

Sweet's syndrome is an acute febrile neutrophilic dermatosis. Drug-induced Sweet's syndrome typically occurs soon after drug administration, with rapid resolution of symptoms with cessation of the offending agent. We report a man in his early 40s who presented with fever and widespread erythematous rash on a background of recently diagnosed mild stricturing ileal Crohn's disease. He was commenced on 6-mercaptopurine 12 days before presentation. Skin biopsy demonstrated diffuse infiltration of neutrophils in the upper dermis, dermal oedema, eosinophils and fibrin deposition...

Aseptic abscess syndrome (AAS) is a medical rarity. The combination of multiple abscess collections in different organs, negative microbiological studies, and the association with an inflammatory bowel disease is highly suggestive for an AAS. The AAS is an acute neutrophilic dermatosis, so "generalized pyoderma gangraenosum" or "generalized bullous sweet syndrome" might be used synonymously. It is important to note that the diagnosis of an AAS can be made only after careful exclusion of an infectious disease...

Sweet syndrome is a neutrophilic dermatosis associated with multiple disorders. This retrospective case-series study of patients with Sweet syndrome in a tertiary hospital in Spain from 2001 to 2021, explores clinicopathological characteristics of Sweet syndrome and variables associated with malignancy, presence of autoinflammatory disorders and differences between histological subtypes. A total of 93 patients were identified: 30% idiopathic, 34% malignancy-associated, 29% reactive to infections or drug-associated, and 6% with an autoimmune/inflammatory condition...

Mevalonate kinase deficiency is a group of rare metabolic autoinflammatory disorders that present with recurrent fevers, abdominal pain, arthralgias, adenopathy, and a variety of cutaneous manifestations. The skin findings may mimic cellulitis, erythema elevatum diutinum, IgA vasculitis, and Sweet syndrome, and there is often a morbilliform or urticarial rash and aphthous stomatitis. Mevalonate kinase deficiency is one of the identified monogenic variants that can cause very early onset inflammatory bowel disease (IBD)...

BACKGROUND: Sweet syndrome (SS), also known as acute febrile neutrophilic dermatosis, is an immunologic syndrome characterized by widespread neutrophilic infiltration. Histiocytoid Sweet syndrome (H-SS) is a histopathologic variant of SS. While SS most commonly occurs in adults, this case report discusses an infant patient who presented with H-SS. CASE PRESENTATION: Through a multidisciplinary approach, this patient was also found to have very early onset inflammatory bowel disease (VEO-IBD) and Mevalonate kinase-associated disease (MKAD)...

Bowel-associated arthritis-dermatosis syndrome (BADAS) is a rare neutrophilic dermatosis that was first described in 1971 in patients who underwent bypass surgery for obesity. Over the years, the number of reported cases associated with medical gastroenterological conditions, particularly inflammatory bowel disease (IBD), has progressively increased. To date, there are no systematic reviews in the literature on BADAS. The design of an a priori protocol was based on PRISMA guidelines, and a search of PubMed and Scopus databases was conducted for articles published between 1971 and 2023 related to the topic...

BACKGROUND: Cutaneous extra-intestinal manifestations (EIM) occur in up to 20% of patients with IBD. Information about Sweet syndrome (SS)'s clinical course as a rare cutaneous EIM in IBD is limited to case reports. We present the largest retrospective cohort on the occurrence and management of SS in IBD. STUDY: Electronic medical records and paper charts since 1980 were retrospectively reviewed at a large quaternary medical center to identify all adult IBD patients with histopathology-proven SS...

BACKGROUND: Opportunistic infections associated with immunosuppressive treatments for inflammatory bowel disease pose an important safety concern. Here we report the case of a patient with active ulcerative colitis and cryptococcal pneumonia who was treated with vedolizumab combined with fluconazole. CASE PRESENTATION: A 56-year-old Japanese man with ulcerative colitis and a history of Sweet's syndrome who was taking prednisolone and azathioprine presented with a moderate exacerbation of ulcerative colitis, abdominal pain, diarrhea, and bloody stools along with cytomegalovirus infection...

UNLABELLED: Sweet's syndrome or acute febrile neutrophilic dermatosis is characterized by an acute inflammatory skin eruption of oedematous and erythematous papules, plaques or nodules, accompanied by fever, and leucocytosis with possible extracutaneous involvement. Aetiologies include infections, inflammatory bowel disease, pregnancy or malignancy, or the syndrome may be drug-induced by many classes of medications or very rarely, radiocontrast exposure. Herein, the authors report a case of radiocontrast-induced bullous Sweet's syndrome and contrast-induced acute kidney injury in a woman in her 60s with a complex medical history...

Ustekinumab, an inhibitor of the interleukin-12/23 pathway, received Food and Drug Administration (FDA) approval in 2019 for induction and maintenance therapy in patients with moderate-to-severe ulcerative colitis (UC). Data regarding the efficacy of ustekinumab in the treatment of extraintestinal manifestations of UC are unclear. Sweet syndrome, an acute febrile neutrophilic dermatosis, is a cutaneous manifestation of inflammatory bowel disease that parallels disease activity. In this study, we present the first case of subcutaneous Sweet syndrome with sterile osteomyelitis in a patient with UC successfully treated with ustekinumab...

Inflammatory bowel diseases (IBD) are chronic inflammatory disorders of the digestive tract, and they involve systemic inflammatory diseases known as extra-intestinal manifestations (EIMs). Timely and correct diagnosis of mucocutaneous EIMs could assist with detecting and monitoring IBD. We present a case of 52-year-old male patient of ulcerative colitis with 2 rare EMIs together at the same time: pyostomatitis vegetans in the oral cavity and Sweet syndrome on the skin. They presented as multiple small white or yellow pustules on the surface of the hyperemic fragile oral mucosa and abrupt appearance of painful, swollen, and erythematous papules on the skin, respectively...

Neutrophilic dermatosis (ND) is a category of diseases characterized by trauma-induced, autoinflammatory cutaneous eruption. Comorbid systemic disease is common with a predilection for malignancy, inflammatory bowel disease, and rheumatologic disease. Rarely, it can manifest with aseptic shock, an entity referred to as necrotizing neutrophilic dermatosis (NND). NND may occur in the postoperative setting and is often misdiagnosed as a necrotizing soft tissue infection. Unfortunately, the treatment for a necrotizing soft tissue infection, namely, wide debridement, is often detrimental in the setting of NND...

Neutrophilic dermatoses (ND) are a group of inflammatory skin conditions characterized by a neutrophilic infiltrate on histopathology with no evidence of infection. ND are classified based upon the localization of neutrophils within the skin and clinical features. Recent findings suggest that ND are due to two main mechanisms: i) a polyclonal hereditary activation of the innate immune system (polygenic or monogenic); or ii) a clonal somatic activation of myeloid cells such as encountered in myelodysplastic syndrome or VEXAS syndrome...

Sweet syndrome, also known as Acute Febrile Neutrophilic Dermatosis, is a rare inflammatory condition. The exact pathogenesis of Sweet syndrome is unclear, however, autoimmune and inflammatory conditions including inflammatory bowel disease have been linked as underlying etiologies. Since its description, in 1964, there have been published less than fifty reports of Crohn's-associated Sweet syndrome. We report a 43-year-old male patient with a medical history of Crohn's disease who subsequently developed Sweet syndrome...

We present a rare case of Sweet's syndrome. A 39-year-old woman with subjective fevers, polyarthralgia, and malaise presented with worsening painful erythematous plaques on the trunk, arms, and legs. Further examination with biopsy revealed a diagnosis of acute febrile neutrophilic dermatosis, or Sweet's syndrome. Diagnosis by skin biopsy is crucial, and onset requires prompt evaluation for serious associated disorders such as leukemias, inflammatory bowel disease, thyroid disease, sarcoidosis, and infectious etiologies...

Cutaneous involvement is one of the most common extraintestinal manifestations of inflammatory bowel disease (IBD). More commonly, pyoderma gangrenosum and erythema nodosum are noted, but psoriasis, aphthous stomatitis, Sweet's syndrome, and vasculitis may also occur. Leukocytoclastic vasculitis (LCV) is a rare cutaneous manifestation, characterized by the appearance of palpable purpura, urticaria, and ulcer-necrotic lesions predominantly in the lower extremities that improve with immunosuppressive therapy...