Superficial granulomatous pyoderma: an idiopathic granulomatous cutaneous ulceration

A 70-year-old male with a superficial granulomatous ulcer is reported. Histopathological findings were the same as those described for superficial granulomatous pyoderma, a recognized variant of classic pyoderma gangrenosum. The differences between pyoderma gangrenosum and its variant superficial granulomatous pyoderma are highlighted.     

浅表性大疱型坏疽性脓皮病

报告1例浅表性大疱型坏疽性脓皮病。患者女,58岁。四肢红斑、水疱、溃疡伴疼痛9d入院。组织病理表现为表皮内有一大脓疱,脓疱两侧表皮内有较多中性粒细胞侵入,两侧表皮有明显细胞间及细胞内水肿,真皮浅层及中层有弥漫性中性粒细胞浸润,有明显核尘,核碎裂,真皮胶原纤维间水肿,皮下组织有部分区域出血。类似于典型坏疽性脓皮病,但是在真皮中的位置更表浅。给予小剂量糖皮质激素,米诺环素,雷公藤多苷治疗,2个月后痊愈。     

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.     

Superficial granulomatous pyoderma. A report of two cases

We report two cases of superficial granulomatous pyoderma (SGP). Unlike classical pyoderma gangrenosum this variant is characterized by a benign course, superficial ulceration and a granulomatous infiltrate. Although our cases share the typical features of SGP. they are noteworthy in that one had the disease for 18 years, and the other had concomitant sarcoidosis.     

多发性骨髓瘤伴坏疽性脓皮病一例

患者，女，71岁。双下肢反复溃疡伴疼痛20个月余。皮损组织病理检查符合坏疽性脓皮病。免疫球蛋白固定电泳示IgGκ链免疫电泳单克隆区带。骨髓穿刺结果示骨髓增生活跃，见异常浆细胞，幼稚浆细胞占29.5%。诊断：多发性骨髓瘤伴坏疽性脓皮病。     

间质肉芽肿性皮炎

报告1例间质肉芽肿性皮炎。患者女,17岁。因左胸部和左上肢出现呈带状分布、无自觉症状的丘疹和结节半年就诊。免疫学检查示类风湿因子阳性。组织病理检查显示真皮间质和血管周围弥漫而致密的以组织细胞为主的炎性细胞浸润,部分组织细胞核大,有异形：炎性浸润细胞中还有中性粒细胞和少量淋巴细胞、浆细胞、多核巨细胞,局部可见灶性胶原纤维变性、坏死,未见嗜酸性粒细胞浸润和血管炎改变。阿新蓝染色阴性。免疫组化染色示CD68强阳性,S-100蛋白、AE1／AE3、SMA、结蛋白、LCA、CD45RO、CD20、CD21、CD23、CD31、CD34、CD1a和Ki-67均阴性。符合间质肉芽肿性皮炎的诊断。     

芽生菌病样脓皮病

报告1例芽生菌病样脓皮病.患者女,62岁.而部皮肤出现结节、溃疡3个月余.皮肤科检查:双侧颊部、额部、鼻部有结节和疣状增殖性斑块,边缘隆起,表面旱菜花样,有脓性分泌物及结痂,双眼睑水肿.皮损组织病理检查示假上皮瘤样增生,真皮和增生的表皮内有多发性脓肿,主要成分为中性粒细胞,部分脓肿内可见多数嗜酸性粒细胞.脓性分泌物细菌培养见金黄色葡萄球菌生长.最终诊断:芽生菌病样脓皮病.     

浅表性血管黏液瘤

报告1例浅表性血管黏液瘤。患者男,53岁。头皮结节3年,时有疼痛,逐渐增大。皮损组织病理学检查示:肿瘤位于真皮内,周围有胶原纤维包绕。肿瘤由卵圆形较饱满的基质细胞组成,细胞质呈双染性,未见异形及核分裂像。肿瘤基质内有丰富的黏液样物质,散在分布中、小血管,未见大血管。诊断为浅表性血管黏液瘤。     

混合细菌感染引起的芽生菌病样脓皮病1例

报告一例４７岁男怀芽生菌病样脓皮病，该例存在２两种细菌混合感染。经抗菌治疗等综合处理，大部分皮损愈合，留小片上损未愈，行刮除术后痊愈。     

下疳样脓皮病伴非淋菌性尿道炎及尖锐湿疣1例

报告1例下疳样脓皮病伴尖锐湿疣及非淋菌性尿道炎。患者男，27岁。临床表现为尿频、尿急、尿痛，全身散在黄豆至蚕豆大梅毒硬下疳样溃疡和阴茎背侧赘生物。既往有非婚性接触史。尿道分泌物衣原体快速检测( )；反复暗视野查梅毒螺旋体及梅毒血清试验均阴性。诊断为下疳样脓皮病伴尖锐湿疣及非淋菌性尿道炎。给予口服米诺环素、左氧氟沙星及盐酸伐昔洛韦片，局部湿敷、尖锐湿疣皮损局部电凝等治疗，效果显著。     

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.     

嗜酸性肉芽肿性多血管炎一例

患者,女,29岁。头面、手足散在血疱样丘疹、结节3天。皮肤科检查：面部、手足散在绿豆至花生大丘疹、结节,质地稍硬,浸润明显,表面可见紫癜样改变,部分皮损破溃结痂。结合临床症状、实验室及组织病理检查,诊断为嗜酸性肉芽肿性多血管炎。     

Superficial granulomatous pyoderma: a localized vegetative form of pyoderma gangrenosum

Twenty-five patients had superficial ulcerative and vegetative pyoderma with granulomatous histologic findings. Healing occurred without systemic corticosteroid therapy in all but three patients. All patients had clinical pyoderma gangrenosum. In five patients the lesion occurred after surgery of the skin. Histopathologic study of 40 biopsy specimens showed focal neutrophilic abscesses of the papillary dermis, often with peripheral palisading histiocytes and foreign-body giant cells. Pseudoepitheliomatous, vegetative hyperplasia and sinus tract formation were observed frequently. All patient had massive areas of plasmacytosis, and 13 had eosinophils. Granulation tissue, hemorrhage, and fibrosis were additional features in some areas. Foreign material in the lesions was considered unimportant, except in one patient with a starch granuloma. Therapy with local corticosteroids, minocycline, tetracycline, or sulfa drugs resulted in healing in 15 patients. We believe that we have identified a localized, limited form of chronic superficial pyoderma gangrenosum with verrucous and ulcerative lesions and a granulomatous histologic appearance that represents a unique pattern of this disease in some patients.     

Squamous cell carcinoma arising in chronic perianal pyoderma a case report and review of Japanese literature

We report a rare case of squamous cell carcinoma developing from fistules of chronic perianal pyoderma in a 49-year-old Japanese man. He first noticed an abscess and nodule on his buttocks and perianal area 21 year previously (at the age of 28); the fistules formed later. These fistules were surgically removed, and an artificial anus was constructed 14 years ago (at the age of 35) in our hospital, when a histopathological examination revealed no malignant changes. However, he was recently admitted to our hospital with arterial bleeding from the ulcer of the buttock. On admission, the histological diagnosis of the ulcer was well differentiated squamous cell carcinoma. Wide local excision of the ulcer and scar tissue, including the sacrum, was performed. The defect was covered with a left latissimus dorsi flap and skin graft. He received radiation therapy after the operation. However, he died of cachexia and pneumonia. This case indicated that the CPP would better have been treated with wide excision before the development of SCC. Therefore, we recommend careful follow-up of patients affected by CPP and repeated biopsies of the lesion, particularly when the condition is severe, longstanding, and extensive. We discussed the term "CPP" and reviewed 22 cases of SCC arising in CPP reported in the Japanese literature.     

栅栏状中性粒细胞肉芽肿性皮炎合并强直性脊柱炎一例

患者,男,55岁。四肢暗红色斑块、脱屑5年。既往有强直性脊柱炎病史。皮损组织病理学检查示真皮内上皮细胞肉芽肿改变,部分区域上皮细胞呈栅栏状排列,局部伴有中性粒细胞、淋巴细胞浸润。实验室检查抗核抗体、SSA、SSB、抗双链DNA等为阴性,HLA-B27为阳性。诊断：栅栏状中性粒细胞肉芽肿性皮炎合并强直性脊柱炎。     

Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum

Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15‐year‐old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis.     

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.     

Superficial granulomatous pyoderma: a case in an 11-year-old girl and review of the literature

Superficial granulomatous pyoderma is a rare entity thought to be a variant of pyoderma gangrenosum and is often mistaken for classic pyoderma gangrenosum. Superficial granulomatous pyoderma has mainly been described in adults. We describe a case of Superficial granulomatous pyoderma in an 11-year-old girl and present a review of the literature. Superficial granulomatous pyoderma maintains clinical and histopathologic characteristics distinct from classic pyoderma gangrenosum and has a more favorable prognosis. It is often responsive to conservative treatment.     

Nodular secondary syphilis with associated granulomatous inflammation: case report and literature review

A 62‐year‐old male presented with a 10‐day history of a diffuse, erythematous papular rash sparing the palms and soles. Histopathologic examination of a skin lesion showed loose non‐caseating granulomas in a lymphoplasmacytic background. Scattered spirochetes were identified by Treponema pallidum immunohistochemistry, in keeping with a diagnosis of secondary syphilis. Granulomatous inflammation in secondary syphilis is uncommon. A review of the literature reveals that the majority of prior reported cases of granulomatous secondary syphilis share similar characteristics to this case; namely, a papular or nodular clinical presentation, sparing of the palms and soles, and collections of epithelioid histiocytes with associated lymphocytes and variable numbers of plasma cells.