Adalimumab in Japanese patients with active ulcers of pyoderma gangrenosum: Twenty-six-week phase 3 open-label study

This phase 3 multicenter study, including 26-week treatment and extension periods, evaluated the efficacy and safety of adalimumab in Japanese patients with active ulcers due to pyoderma gangrenosum. Patients received adalimumab 160 mg at week 0, 80 mg at week 2, and then 40 mg every week starting at week 4. Of the 22 enrolled patients, 12 (54.5%, P < 0.001) achieved the primary efficacy end-point of pyoderma gangrenosum area reduction 100 (PGAR 100, defined as complete skin re-epithelialization) for the target ulcer at week 26 assessed by digital planimetry. PGAR 100 response was observed as early as week 6 (13.6%) and continued to increase over time. The mean percent change from baseline in target ulcer area was −31.8% at week 6 and −63.8% at week 26. A Physician’s Global Assessment score of 0 (PGA 0, all ulcers completely clear) was achieved by two patients (9.1%) at week 6 and eight (36.4%) at week 26, while PGA 0/1 (completely/almost clear) was achieved by five (22.7%) and 12 patients (54.5%) at week 6 and 26, respectively. Mean changes from baseline in pain numeric rating scale (−1.8 at week 6 and −2.5 at week 26) and the Dermatology Life Quality Index (−3.1 at week 6 and −3.6 at week 26) improved over time. Adverse events were reported by 18 patients, most commonly infections (n = 11), and serious adverse events by four. These results suggest that adalimumab is effective and generally well tolerated in Japanese patients with active ulcers of pyoderma gangrenosum.     

PG‐TIME: A practical approach to the clinical management of pyoderma gangrenosum

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis which may be rapidly progressive. Standard guidelines for local treatment are lacking. Through our experience, we suggested a local treatment algorithm based on the tissue, infection, moisture balance, and epithelization (TIME) concept. The clinical and histopathological features of 52 patients with PG, the duration, and the evolution of the lesions were retrospectively evaluated. Systemic therapies, local treatments, and standard wound treatments were reported. We observed ulcerative PG in the majority of the patients (82.6%), followed by the pustular form (9.6%), the peristomal type (3.8%), the vegetative form (1.9%), and the bullous type (1.9%). The lower leg was the most commonly area affected (90.4% of cases). Pathergy was reported in 15.3% of cases. The first‐line treatment was the use of oral glucocorticosteroids (GCSs). We observed healing after 3 weeks of GCS in 17.3% of cases. In 25% of all cases, we obtained complete healing with long‐term low doses (<0.5 mg/kg) of GCS in the range of 2 to 6 months. We used second‐line treatments in 57.6% of patients. Local, systemic treatment, and correct wound management can be associated with better clinical results. We suggest a new local therapeutic algorithm in both the inflammatory and noninflammatory healing phases.     

A rare case of granulomatous Wegener with multiple pyoderma gangrenosum‐like ulcers

Wegener's granulomatosis, is a rare form of systemic vasculitis characterized by necrotic granulomas around the blood vessels. We report a case of a healthy 43‐year‐old man presented with multiple ulcerated lesions in head and all over the body, that has appeared spontaneously. It was characterized by a giant deep ulceration covered by thick purulent, in size from 3 to 4 cm, deep; sub febrile temperature, and minimal pain associated. Skin biopsies results pyoderma gangrenosum (PG)‐like features: anti‐neutrophil cytoplasmic antibody (cANCA) positive. Six months later, the patient developed dispea episodes. CT scan (CT) result with multiple focal opacities. He was diagnosed with wegener granulomatosis (WG) and was treated with corticosteroids and ciclophosphamide. Skin involvement in WG is not common and lesions in the skin vary from purpura, paniculitis to ulceration. In our case, skin elements are the first manifestations. Whether these elements are Wegener manifestation or we have coexistence of PG with WG, it remains a still uncertain question to answer. There is no histopathologic pathognomic elements for both. Skin involvement in WG with PG‐like lesions have been rarely reported. In front of this type of widespread ulcerations, resistant to treatment we should be aware that one of the reasons may be a disease of autoimmune etiology such as WG.     

Heterogeneity of blastomycosis‐like pyoderma: A selection of cases from the last 35 years

Blastomycosis‐like pyoderma is a form of pyoderma with variable clinical findings and histopathological features. We present a case series of 39 patients collected over a 35‐year period to demonstrate its clinical features and histological findings. The most common clinical presentations found were solitary plaques, solitary nodules, sinuses, crypts, verrucous plaques and discharge, usually on sun‐exposed skin. The most common histopathological findings were chronic granulomatous inflammation, suppurative inflammation, sinus and abscess formation, pseudoepitheliomatous hyperplasia, transepidermal elimination and scarring. We discuss its treatment and the recent literature that has focused on its response to acitretin.     

Multifocal and refractory pyoderma gangrenosum: Possible role of cocaine abuse

Pyoderma gangrenosum is a rare and severe inflammatory skin condition. There are different variants, including generalised and atypical forms, but the most common presentation is an enlarging ulcer on the lower extremities. Treatment can represent a challenge for physicians and there are no guidelines based on randomised controlled trials. We report an exceptional case of widespread and refractory pyoderma gangrenosum in a middle‐aged woman where cocaine use may have played a role. Treatment with i.v. pulse corticosteroids and cyclosporine was ineffective, and disease control was obtained with oral corticosteroids together with mycophenolic acid, infliximab and abstinence from cocaine consumption. There was a temporal relationship between disease outbreaks and cocaine consumption and improvement after its discontinuation. In the present case such clinical severity without associated pathology and the temporal association with cocaine abuse raises the possibility of cocaine playing an aetiological role as well as accounting for therapy resistance.     

坏疽性脓皮病合并炎症性肠病

报告1例坏疽性脓皮病合并炎症性肠病。患者男,30岁。腹痛、腹泻及稀薄脓血便1个月余;躯干及四肢出现散在的红色丘疹、脓疱疹,溃疡伴疼痛12 d入院。皮损组织病理检查:表皮角化过度,棘层增厚,真皮全层及皮下脂肪间大量中性粒细胞浸润,可见红细胞外渗及血管壁纤维蛋白样变性。电子肠镜诊断为炎症性肠病。抗核周型中性粒细胞胞质抗体(anti perinuclear antineutrophil cytoplasmic antibody,p-ANCA)阳性。予糖皮质激素、美沙拉嗪及支持治疗后,患者病情明显好转。     

An aggressive course of pyoderma gangrenosum mimicking bacterial osteomyelitis after open reduction and internal fixation of a distal radius fracture with a titanium plate

Pyoderma gangrenosum (PG) is a rare condition characterized by the development of aseptic, non-healing skin ulcers. Any skin trauma, such as a surgical incision, can trigger an outbreak of lesions.Our case and literature review show that a physician should consider PG in every event of a non-healing, aseptic wound after surgery. The treatment of PG requires collaboration within a multidisciplinary team and immunosuppressive therapy is the first line of treatment, while surgical interventions should be avoided in the active stage of PG.     

A novel topical therapy for resistant and early peristomal pyoderma gangrenosum

Peristomal pyoderma gangrenosum (PPG) is an under‐recognised and difficult condition to treat. We describe a case series using a novel topical combination therapy that promotes wound healing and allows for adhesion of the stoma appliance. A crushed oral prednisolone tablet mixed with Stomahesive Protective Powder (ConvaTec) was applied topically to seven patients with PPG and resulted in pain relief and wound healing in six of seven patients. Only one patient experienced recurrence. The novel topical therapy we describe is cost‐effective, readily available, and easily applied in any inpatient or outpatient setting.