Niu Ruixian, Zheng Jiangtao, Ding Dongmei, Kuang Weian, Lu Fengyan, Yin Xunguo
Department of Dermatology, Qujing First People's Hospital affiliated to Kunming Medical University, Qujing, Yunnan, China.
Medicine (Baltimore). 2020 Feb;99(6):e18795. doi: 10.1097/MD.0000000000018795.
Pyoderma gangrenosum (PG) is a phenomenon of cutaneous ulceration with unknown etiology. About half the cases have associated extracutaneous manifestations or associated systemic diseases. The most commonly associated systemic disorders include inflammatory bowel disease (IBD), hematologic malignancies, autoimmune arthritis, and vasculitis. This is a case report about giant PG with ulcerative colitis (UC), which is extremely rare.
A 39-year-old female farmer with UC for the past 3 years presented with multiple painful ulcers, erosion, exudation, and crusting on the right leg for 1 month. A cutaneous examination showed diffusely distributed, multiple, well-defined, deep purulent ulcers on the right medial shank measuring 6 to 20 cm and sporadic worm-eaten ulceration on the right ectocnemial, with severe oozing and erosions. The ulcerations exhibited deep undermined borders, granulated tissue and a black eschar at the base. The right shank and feet were severely swollen, restricting movement. The arteria dorsalis pedis pulse was good, with normal sensation on the skin of the right shank and feet. Laboratory examinations showed a white cell count of 11.8 × 109/L, hemoglobin was 91 g/L, erythrocyte sedimentation rate was 82 mm/h, unelevated procalcitonin, serum C-reactive protein was 131.29 mg/L, and a negative tuberculin skin test. Enteroscopy demonstrated endoscopic evidence of UC. A skin lesion biopsy showed superficial erosion and scarring. Partial epidermal hyperplasia, partial epidermal atrophy and thinning, mild edema of the dermal papill. Most of the middle and lower part of the dermis, showed dense lymphocytes, histiocytes, multinucleated giant cells, and neutrophil infiltration. PG with UC was diagnosed based on clinical manifestations, laboratory examinations and enteroscopy results.
She was treated with topical applications of povidone iodine and kangfuxin solution twice daily, methylprednisolone sodium succinate 40 mg and compound glycyrrhizin 60 mg via intravenous drip once a day, along with thalidomide 50 mg twice daily. The UC was controlled with mesalazine.
She required multiple therapies to achieve PG healing 3 months later. No PG recurrence was observed during the 1-year follow-up.
Recognizing the clinical features of PG and its pathogenic nature, ensuring timely management fundamental for preventing severe destruction and deformity, and control of associated diseases are important aspects of treatment. Combination therapy is essential for PG patients with IBD.
坏疽性脓皮病(PG)是一种病因不明的皮肤溃疡现象。约半数病例伴有皮肤外表现或相关的全身性疾病。最常见的相关全身性疾病包括炎症性肠病(IBD)、血液系统恶性肿瘤、自身免疫性关节炎和血管炎。本文报告一例极其罕见的伴有溃疡性结肠炎(UC)的巨大型PG病例。
一名39岁女性农民,患UC已3年,右腿出现多处疼痛性溃疡、糜烂、渗出和结痂1个月。皮肤检查显示右小腿内侧弥漫性分布多个边界清晰的深脓性溃疡,大小为6至20厘米,右外髁有散在的虫蚀样溃疡,伴有严重渗液和糜烂。溃疡边界呈深部潜行性,底部有肉芽组织和黑色焦痂。右小腿和足部严重肿胀,活动受限。足背动脉搏动良好,右小腿和足部皮肤感觉正常。实验室检查显示白细胞计数为11.8×10⁹/L,血红蛋白为91g/L,红细胞沉降率为82mm/h,降钙素原未升高,血清C反应蛋白为131.29mg/L,结核菌素皮肤试验阴性。肠镜检查显示有UC的内镜证据。皮肤病变活检显示浅表糜烂和瘢痕形成。部分表皮增生,部分表皮萎缩变薄,真皮乳头轻度水肿。真皮中下部大部分区域可见密集的淋巴细胞、组织细胞、多核巨细胞和中性粒细胞浸润。根据临床表现、实验室检查和肠镜检查结果诊断为PG合并UC。
她接受了每日两次的聚维酮碘和康复新液局部应用治疗,每天一次静脉滴注40mg甲泼尼龙琥珀酸钠和60mg复方甘草酸苷,同时每日两次口服50mg沙利度胺。UC用美沙拉嗪控制。
3个月后她需要多种治疗方法才能使PG愈合。在1年的随访中未观察到PG复发。
认识PG的临床特征及其致病本质,确保及时治疗对于预防严重破坏和畸形至关重要,控制相关疾病是治疗的重要方面。联合治疗对合并IBD的PG患者至关重要。