Fatal infectious mononucleosis with staphylococcal pyoderma in a girl with hereditary immunological dysregulations

We describe a 14-year-old girl with fatal infectious mononucleosis with high fever, pancytopenia, and multiple skin ulcers due toStaphylococcus aureus. Immunological studies revealed low serum IgM, low natural killer (NK) activity, and high CD4/CD8 ratio. Her father had also low NK activity and high CD4/CD8 ratio. It is suggested that she had a dominantly inherited immunodeficiency predisposing to severe Epstein-Barr virus infection.     

Acute Form of Pyoderma Gangrenosum Induced by Traumatic Injury

A 45-year-old man with the acute form of pyoderma gangrenosum is reported. He developed characteristic ulcers that rapidly followed violaceous erythema at sites of traumatic injury. The skin lesions repeatedly appeared six times within 7 years. Each time, the skin lesions were localized to sites of trauma, and no systemic symptoms nor abnormal laboratory findings were associated. Histological findings were acute superficial inflammation with neutrophilic infiltration into the dermis. Sweet's syndrome-like skin lesions were once associated, indicating that pyoderma gangrenosum and Sweet's syndrome have a close relationship. Nonsteroidal antiinflammatory drugs, potassium iodide, and minocycline were not effective. He was successfully treated with oral administration of corticosteroid. The pathergy of this patient must have been caused not only by the trauma itself but by additional factors such as epidermal components or contaminating micro-organisms, since traumatic injury did not always cause the skin lesions.     

中西医结合治疗骨髓增生异常综合征合并坏疽性脓皮病

骨髓增生异常综合征是一组起源于造血干细胞,以血细胞病态造血,高风险向急性髓系白血病转化为特征的异质性髓系肿瘤,其合并坏疽性脓皮病是一种罕见的情况,多意味着预后不良。现将天津中医药大学第一附属医院血液科收治的1例骨髓增生异常综合征合并坏疽性脓皮病诊疗进行整理,希冀能为临床的诊治提供新的治疗依据及理论思路。     

Bullous pyoderma gangrenosum of the bilateral dorsal hands

Pyoderma gangrenosum (PG) shows characteristic non‐infectious ulcers that are commonly associated with systemic diseases such as inflammatory bowel diseases, myeloproliferative disorders or aortitis syndrome. The typical clinical appearance is undermining ulcers with reddish and irregular borders on the legs. As PG has these notable signs, the diagnosis is relatively easy and its treatment depends on the severity of underlying complications. We report a case of a 60‐year‐old Japanese man, diagnosed with bullous PG, who also had been suffering from myeloperoxidase antineutrophil cytoplasmic antibody‐positive microscopic polyangiitis and pulmonary aspergillosis. This case displayed soft whitish ulcers that existed on the rough ulcer base, with irregular borders, on his bilateral dorsal hands. Initially, it seemed to be cutaneous secondary aspergillosis because the host was already infected with pulmonary aspergillosis in both lungs. The differential diagnosis of PG from aspergillosis was from the sterile bullae or neutrophilic bullae on his right forearm, which evolved into ulcers in a few days. This case was finally diagnosed as bullous PG and a topical glucocorticoid was very effective to epithelize the ulcers in 2–3 weeks.     

Bullous pyoderma gangrenosum: A case report and review of the published work

Pyoderma gangrenosum (PG) is an ulcerative skin disorder characterized by neutrophilic infiltrations. PG is generally classified into four types: (i) ulcerative; (ii) pustular; (iii) bullous; and (iv) vegetative. Among them, bullous PG is known as a rare type. Herein, we report a case of bullous PG together with a summary of the 12 PG cases treated in our department over the previous 15 years, and we review 38 well‐documented bullous PG cases (65.8% female; aged 18–80 years [mean ± standard deviation, 51.6 ± 16.8]) in the published work, including the present case, from 1972–2011. Although the disease most frequently associated with PG is inflammatory bowel disease, bullous PG is most commonly associated with hematological disorders (25/38, 65.8%), which indicates the characteristic pathophysiology specific to bullous PG.     

Treatment of recalcitrant ulcers in pyoderma gangrenosum with mycophenolate mofetil and autologous keratinocyte transplantation on a hyaluronic acid matrix

Pyoderma gangrenosum sometimes takes a recalcitrant course that is unresponsive to standard immuno-suppression with corticosteroids and/or cyclosporin A. In these cases improvement of painful ulcerations is a therapeutic challenge. We report a 17-year-old boy with severe pyoderma gangrenosum treated successfully with mycophenolate mofetil and autologous keratinocyte transplantation using an esterified hyluronic acid delivery system.     

Extraintestinal manifestations in inflammatory bowel disease – epidemiology,genetics, and pathogenesis

Introduction: Inflammatory bowel disease (IBD) is a chronic inflammatory disorder, primarily of, but not restricted to the gut. Extraintestinal manifestations (EIMs) are frequently observed and involve the joints, eyes, hepatobiliary tract, and skin.

Areas covered: In this review, we discuss classical EIM focusing on epidemiology, genetics, and pathogenesis, highlighting recent advances in the understanding of EIM. We further discuss treatment-induced immunological phenomena, which are increasingly recognized and might challenge IBD-treating physicians in the era of biological treatment.

Expert opinion: EIM considerably contributes to morbidity and mortality. Genetic studies have revealed a common genetic background between EIM and IBD and among specific EIM. Identified protein interactions have been shown to cluster in shared biological pathways. However – despite these recent advances – pathogenesis of EIM is at best partially understood. Several pathogenic mechanisms have been proposed such as upregulation of tumor necrosis factor, aberrant lymphocyte homing, and cross-reactive antigen presentation. It still remains unclear whether EIM is a direct result of the inflammatory process in the gut or rather a consequence of a shared genetic background leading to dysfunctional immune responses to environmental stimuli. Exploration and understanding of EIM genetics and pathophysiology will pave the road for better and more efficacious treatment options in the future.     

Blastomycosis-like Pyoderma with Good Response to Acitretin

Blastomycosis-like pyoderma is a rare, vegetating skin lesion that is an unusual exaggerated tissue reaction possibly to prolonged primary or secondary bacterial infection. We report a case of blastomycosis-like pyoderma in a man with Down syndrome, diabetes mellitus and hypothyroidism who responsed poorly to conventional therapies, including antibiotics and correction of predisposing factors for a long time, but experienced dramatic improvement after 3 months of acitretin treatment.     

Pyoderma gangrenosum at multiple sites in a post‐colostomy ulcerative colitis patient with chronic hepatitis B virus: A case report

Pyoderma gangrenosum is an uncommon ulcerative cutaneous lesion manifesting as rapidly progressing single or multiple skin ulcers. Permanent stoma in inflammatory bowel disease patients remains an independent risk of pyoderma gangrenosum. In the current report, we describe a case of pyoderma gangrenosum in a post‐colostomy ulcerative colitis patient with chronic hepatitis B. Pyoderma gangrenosum began seemingly as peristomal dermatitis that rapidly developed into painful ulcerations with subsequent appearance of sterile pustules and ulcerations in the left lower leg. The patient significantly improved after active management with prednisolone, antiviral therapy with entecavir, and wound dressings. Our case suggests that physicians and surgeons should have a high index of suspicion of pyoderma gangrenosum in post‐colostomy ulcerative colitis patients who develop peristomal dermatitis.