A continuum of neutrophilic disease occurring in a patient with ulcerative colitis

A case of Sweet's syndrome (acute febrile neutrophilic dermatosis) occurring concurrently with bullous pyoderma gangrenosum is reported to emphasize the close relationship between these two disorders, Aiypical pyoderma gangrenosum and Sweet's syndrome have been described as occurring simultaneously in haematological) dyscrasias but not. to our knowledge, in ulcerative colitis. It has been proposed that pyoderma gangrenosum,; Sweet's syndrome, erythema elevatum diutinum and. subcorneal pustular dermatosis may represent manifestations along a continuum of neutrophilic dermatoses.     

Psoriasis associated with ulcerative colitis and Crohn's disease

Background  Numerous reports have demonstrated the epidemiological, pathogenic, and genetic association between psoriasis and Crohn's disease. Nevertheless, the association between psoriasis and ulcerative colitis was rarely described.
Objective  This study aims to investigate the association between psoriasis and inflammatory bowel disease.
Study design  Case-control study.
Setting  The study was performed utilizing the large medical dataset of Clalit Health Services.
Methods  Psoriasis patients were compared to controls regarding the prevalence of inflammatory bowel disease in a case–control study using logistic multivariate models.
Results  The study included 12 502 psoriasis patients aged 20 years and above and 24 287 age- and sex-matched controls. The prevalence of both Crohn's disease and ulcerative colitis was significantly higher in psoriasis patients compared with the control group. In multivariate analyses, psoriasis was associated with Crohn's disease [odds ratio (OR), 2.49; 95% confidence interval (95% CI), 1.71–3.62] as well as ulcerative colitis (OR, 1.64; 95% CI, 1.15–2.33). This association was independent of anti-tumour necrosis factor-α therapy.
Conclusion  Psoriasis is associated both with Crohn's disease and ulcerative colitis. Future studies on comorbidities in patients with psoriasis should focus on ulcerative colitis.

Conflicts of interest

None declared.     

Neutrophilic pustulosis and ulcerative colitis

Neutrophilic pustulosis is currently considered as a part of the spectrum of Sweet's syndrome, and has been associated with inflammatory bowel disease and several other diseases. We report the case of a 34-year-old male who had been suffering from ulcerative colitis (UC) for several years and who experienced the manifestation of a pustular eruption on both forearms and the abdominal wall during an exacerbation of his bowel disease. Both processes were controlled with steroids per os. The histological picture showed an inflammatory infiltrate composed mainly of neutrophils with scattered neutrophilic epidermal abscesses. Certain distinct clinical and histological characteristics have been described as diagnostic of the pustular eruption of UC. This picture should be included in the spectrum of the neutrophilic dermatosis, in our case associated with an ulcerative colitis. This is a well documented clinical and histologic case report of a recognized association of inflammatory pustulosis in the course of ulcerative colitis.     

Sweet''s syndrome (acute febrile neutrophilic dermatosis) associated with adenocarcinoma of prostate and transitional cell carcinoma of urinary bladder

Association of Sweet's syndrome (SS) with solid tumours is found in about 15% of all malignancy-associated cases, but an association with two malignancies occurring in the same patient has been rarely reported. In the present report, we describe an 82-year-old male with SS in association with adenocarcinoma of the prostate and transitional cell carcinoma of the urinary bladder.     

以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例

 报告以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例。患者女,50岁,反复全身红色斑块伴疼痛4个月,加重2 d。皮肤科检查：颈部及双上肢分布大小不等红色斑块,皮损边缘略隆起于皮面,呈环状,未见水疱及破溃,触痛阳性。上唇少许糜烂面。组织病理符合急性发热性嗜中性皮病改变。入院后查血液系统异常,诊断急性髓系白血病,以急性发热性嗜中性皮病为首发症状。予EA方案预化疗。三个月后患者因严重脑出血死亡     

Neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis

This report describes a case of chronic neutrophilic urticarial dermatosis as a presenting feature of systemic juvenile idiopathic arthritis. When encountered in children, neutrophilic urticarial dermatosis should raise suspicion of autoimmune or autoinflammatory disease.     

Lymphocytic infiltrates as a presenting feature of Sweet's syndrome with myelodysplasia and response to cyclophosphamide

Sweet's syndrome has a well-recognized association with malignancies, around half of which have been acute myelogenous leukaemia. There are also numerous reports of Sweet's syndrome in association with myelodysplasia. We report two patients with Sweet's syndrome in whom the classical histological appearances were preceded by dermal lymphocytic infiltrates. A literature search using PubMed indicates that this phenomenon has not been previously reported. The cases demonstrate the chronicity of Sweet's lesions in association with haematological disease and the need for repeat biopsies to make the diagnosis. We also describe successful treatment with cyclophosphamide, which adds to the list of second-line drugs that may be used in Sweet's syndrome.     

药物超敏综合征的遗传易感性研究进展

药物超敏综合征是一类严重的药物不良反应,患者有发热、皮肤损害、嗜酸粒细胞升高、淋巴结肿大及肝脏受累等,流行病学调查发现其死亡率高达10%~20%。近年来,研究发现药物超敏综合征的发生与个体的遗传易感性,尤其是某些人类白细胞抗原(HLA)等位基因有关。本文综述了卡马西平,别嘌呤醇,氨苯砜,阿巴卡韦及奈韦拉平所致药物超敏综合征的遗传易感性研究进展。     

Neutrophilic dermatosis of the dorsal hands in a farmer

Neutrophilic dermatosis of the dorsal hands is a recently described disorder, which is similar to Sweet's syndrome. It is characterized by erythematous plaques, pustules and haemorrhagic bullae located solely on the dorsal surface of the hands. We describe a 57-year-old man with neutrophilic dermatosis of the dorsal hands that occurred following exposure to a chemical fertilizer. There are few cases reported in the literature regarding neutrophilic dermatosis and the aetiology remains unclear. For the present case, we propose that neutrophilic dermatosis of the dorsal hands might have been induced by the chemical fertilizer.     

Pyodermatitis–pyostomatitis vegetans after subtotal colectomy for ulcerative colitis

A 51‐year‐old Japanese woman underwent subtotal colectomy for ulcerative colitis 5 years before her first visit to our hospital, and her symptoms stabilized. She presented with a 2‐month history of proliferative, pustular lesions on her face, scalp, and gingival and anal mucosae. Histological examination showed features similar to those of pemphigus vegetans. However, the results of immunofluorescence studies, immunoblot analysis and enzyme‐linked immunosorbent assay for pemphigus were negative, and colonoscopy revealed a relapse of ulcerative colitis in the remaining rectal mucosa, so a diagnosis of pyodermatitis–pyostomatitis vegetans was made. This is the first report of mucocutaneous lesions that developed with a relapse of ulcerative colitis in the remaining rectum after subtotal colectomy. Moreover, a close correlation was found between the condition of bowel inflammation and that of mucocutaneous lesions.     

Cerebral vasculitis and multi‐focal neurological deficits due to allopurinol‐induced hypersensitivity syndrome

Drug‐induced hypersensitivity syndrome (DIHS) is a multi‐system syndrome resulting from an idiosyncratic reaction to medication. While it commonly results in multi‐organ involvement, particularly the liver, there are few reports of DIHS causing cerebral vasculitis and neurological deficits. We report the case of a 63‐year old woman with DIHS secondary to allopurinol leading to multiple neurological deficits with magnetic resonance imaging findings consistent with a cerebral vasculitis.     

Clinicopathlogical features and prognosis of drug rash with eosinophilia and systemic symptoms: a study of 30 cases in Taiwan

Background  Drug rash with eosinophilia and systemic symptoms (DRESS), a group of non-blistering severe cutaneous adverse drug reactions (SCADRs), is characterized by skin rash and multiorgan involvement. Details of this reaction have not been reported in the literature so far.
Aim  We investigate clinical and pathological features and prognosis of DRESS and hope this study will provide data concerning this disorder in Taiwan.
Methods  From January 2001 to June 2006, a total of 30 patients, diagnosed with DRESS, were enrolled and evaluated for demographic characteristics, pathological findings, complications and outcome.
Results  Patient ages ranged from 13 to 78, with an equal sex ratio. The most common offending drug was allopurinol followed by carbamazepine. Pathologic changes observed were lichenoid dermatitis, erythema multiforme, pseudolymphoma and vasculitis. Impairment of liver and renal functions and blood dyscrasia were frequent complications. Active infection or reactivation of HHV-6 was observed in 7 of 11 patients studied serologically. Two patients developed type 1 diabetes mellitus. The mortality rate was 10% (3 of 30).
Conclusions  DRESS is a heterogeneous group of life-threatening conditions. The leading drug in DRESS in Taiwan is allopurinol. High eosinophil count and multiple underlying diseases are poor prognostic factors in patients with DRESS.     

A case of abatacept associated neutrophilic dermatosis and a review of the literature

Abatacept is a novel biological agent that dampens the immune response by blocking the co‐stimulation of T‐cells, thus downregulating T‐cell activation. It is currently approved for the treatment of rheumatoid arthritis (RA). The group of novel immunomodulatory agents, referred to as biologics, have now been used extensively, with established safety and side‐effect profiles. There are, however, increasing reports of adverse paradoxical reactions, most notably resulting from anti‐tumour necrosis factor (TNF) therapy. While cutaneous adverse reactions to abatacept are rare, there are a few reports of such paradoxical reactions. We report a case of an idiosyncratic paradoxical neutrophilic dermatosis associated with the use of abatacept.     

Case of lamotrigine‐induced drug adverse reaction under tocilizumab treatment with clinical and virological features of drug‐induced hypersensitivity syndrome

The pathological mechanisms and immunological kinetics of drug‐induced hypersensitivity syndrome (DIHS), including the relevance of interleukin (IL)‐6, remain unclear. We report a case of drug adverse reaction that does not fulfill the diagnostic criteria of DIHS but mimics its characteristic features. Because the patient was under anti‐IL‐6 therapy at the onset, some symptoms typically seen in DIHS were absent, such as fever and leukocyte count abnormalities. However, the characteristic features of DIHS were clearly observed in the subsequent course, including the repeated recurrence of skin rash, prolonged liver dysfunction and reactivation of herpes viruses. This case suggested that IL‐6 role at the onset is not a main factor to determine the subsequent pathomechanism of DIHS and attention should be paid to the preceding therapy for achieving accurate diagnosis.     

Drug‐reaction eosinophilia and systemic symptoms and drug‐induced hypersensitivity syndrome

Drug reaction with eosinophilia and systemic symptoms (DRESS), also known as drug‐induced hypersensitivity syndrome (DIHS), is a rare, severe cutaneous adverse reaction characterised by fever, rash, lymphadenopathy, eosinophilia and/or other leukocyte abnormalities, and internal organ involvement and often has a relapsing–remitting course despite withdrawal of the drug. The drugs that are most implicated include aromatic anticonvulsants, allopurinol, sulphonamides, antiretrovirals (abacavir and nevirapine), and minocycline. The pathogenesis of DRESS/DIHS is far from clear but probably involves a combination of impaired pharmacokinetics and the accumulation of drug metabolites, the sequential reactivation of the herpesvirus family and genetic susceptibility conferred by the association with certain human leukocyte antigen (HLA) class I alleles. The strong association between abacavir and HLA‐B*5701 has enabled pharmacogenetics screening to be employed successfully to minimise the occurrence of hypersensitivity. A prolonged course of oral corticosteroids is required to treat DRESS/DIHS, given the relapsing–remitting nature of the condition with i.v. immunoglobulin and valgangciclovir reserved for refractory or life‐threatening cases.     

Dabrafenib-induced neutrophilic panniculitis in a child undergoing dual BRAF-MEK inhibitor therapy for glioblastoma multiforme

BRAF inhibitor-induced neutrophilic panniculitis is a rare event that is well-characterized in adults undergoing therapy for metastatic melanoma. To date, there are very few reports of this event in children undergoing BRAF inhibitor therapy for low-grade gliomas, all of which were seen with vemurafenib. We report a case of dabrafenib-induced neutrophilic panniculitis in a 9-year-old girl that manifested within several weeks of initiating dual BRAF-MEK inhibitor therapy for glioblastoma multiforme. This case highlights neutrophilic panniculitis as a side effect of dabrafenib in children and serves as a reminder to consider cutaneous side effects of BRAF inhibitors as they are increasingly used to treat children with primary brain tumors.     

Colchicine treatment in a patient with neutrophilic dermatosis associated with rheumatoid arthritis

Neutrophilic dermatoses (ND) are characterized by epidermal and dermal infiltrates of polymorphonuclear leukocytes (PMN), without any infectious or other detectable agents as a cause. We describe a case of ND, which very rapidly improved upon colchicine treatment. The patient was a 67-year-old female with an 8-month history of dusky-red, tender, swollen plaques and nodules with superimposed vesicles and bullae on the buttocks, hands, and ankles associated with rheumatoid arthritis. The diagnosis of ND was made on the basis of the clinical and histopathological features of the skin lesions. She was treated with 0.6 mg of oral colchicine twice daily. Within one week, the erythematous plaques and arthralgia began to recede and disappeared within four weeks. In our case, colchicine seemed to improve the signs and symptoms of ND and appeared to be more effective than the other drugs we had used.