以急性发热性嗜中性皮病皮疹为表现的白塞病1例

分析报告 1例以急性发热性嗜中性皮病皮疹为表现的白塞病。患者表现为急性发热性嗜中性皮病皮疹 ,伴口腔溃疡、关节疼痛 ,组织病理符合皮肤急性发热性嗜中性粒细胞增多性皮病 ,皮肤针刺反应阳性 ,诊断白塞病。     

急性发热性嗜中性皮病的皮下嗜中性白细胞浸润

本文报告一例白血病患者,伴发急性发热性嗜中性皮病(ND),其病理表现为限于皮下组织的嗜中性白细胞浸润。女性患者,52岁,因发热、皮下结节,疑有恶性血液肿瘤而住院。体检:T37.8℃,在臂及股内侧有多个2～3cm大小红斑结节,触痛。血细胞容积24％,白细胞计数16900,中性白细胞72％,髓母细胞4％。骨髓活检显示白血病前期改变,符合进行性急性髓样或髓性单核细胞白血病。皮肤结节活检可见致密的片状成熟的嗜中性白细胞浸润,局限在真皮下1～2mm处皮下脂肪组织小叶内,     

Sweet综合征与恶性肿瘤

Sweet于1964年首先以急性发热性嗜中性皮病报告的Sweet综合征(SS)有5个主要的特征:1.发热;2.嗜中性白细胞增多;3.多发性、高起的、不对称、红斑性、痛性皮肤斑块;4.由成熟的嗜中性细胞组成的浓密的皮肤浸润;5.对类固醇激素治疗迅速获效.本文复习了全世界有关伴有恶性肿瘤的急性发热性嗜中性皮病的文献,并进行了综述.SS与血液的恶性肿瘤最常发生于SS患者的肿瘤是急性髓细     

具有水疱及脓疱的急性发热性嗜中性皮肤病

报告1例具有水疱及脓疱的急性发热性嗜中性皮肤病。患者女，41岁。全身出现红色丘疹、斑块、水疱及脓疱伴疼痛1d。皮损组织病理检查示表皮下水疱形成，真皮全层有以中性粒细胞为主的弥漫性炎性细胞浸润。诊断：急性发热性嗜中性皮肤病。     

误诊为脓疱疮的急性发热性嗜中性皮病并发感染患者的护理

正急性发热性嗜中性皮病又称Sweet综合征,好发于头部、颈部和双上肢(包括手背),也可发生于身体任何部位。早期皮损为疼痛的红色丘疹或斑块,部分患者的斑块上形成真性水疱或脓疱,是一种少见疾病,平均发病年龄为30~60岁,婴儿、儿童及老年人亦可受累。20%以上患者伴有内脏恶性肿瘤。Sweet综合征皮损5~12周内可自行消退,但30%以上患者易复发[1]。现将我科收治的在院外误诊为脓疱疮的急性发热性嗜中     

大疱型急性发热性嗜中性皮病

报告1例大疱型急性发热性嗜中性皮病。患者男,51岁。面部及双上肢出现红色斑块及水疱伴发热10余天。实验室检查示外周血白细胞计数增高。皮损组织病理检查:表皮灶状水肿,真皮乳头高度水肿,局部真、表皮分离,真皮浅层致密团块状淋巴细胞及中性粒细胞浸润,局部红细胞溢出伴少许核尘,炎症累及中层血管周围。直接免疫荧光示Ig G、Ig M、Ig A及C3均(-)。诊断:大疱型急性发热性嗜中性皮病。     

Sweet综合征1例

Sweet综合征（Sweet’s Syndrome，SS）又称急性发热性嗜中性皮病（Acute febrile neutrophilic dermatosis），1964年由sweet首先报道，该病主要特点为发热、疼痛的红色丘疹、结节或斑块，中性粒细胞、白细胞升高和对真皮浅层的中性粒细胞浸润。本文报道1例无发热，白细胞及中性粒细胞均正常，误诊为单纯疱疹的患者。     

Sweet综合征的诊治要点

Sweet综合征又名急性发热性嗜中性皮病,以发热,四肢、面、颈部有疼痛性红色丘疹、斑块或结节,组织病理见真皮有密集的中性粒细胞浸润,末梢血中中性粒细胞增多为最突出的特点,女性较为多发。     

Sweet综合征的临床相关进展

Sweet综合征又称急性发热性嗜中性皮病,是一种少见的、病因尚未完全阐明的反应性疾病。临床上分为经典型/特发型、恶性肿瘤相关型和药物诱导型三个类型,以发热、疼痛性红色斑块、血中嗜中性粒细胞升高和真皮内中性粒细胞浸润为特征,可与多种炎症性疾病或肿瘤伴发。组织学上主要表现为真皮弥漫性成熟中性粒细胞浸润、白细胞核碎裂、真皮乳头水肿,通常缺乏表皮和原发性白细胞破碎性血管炎的改变。系统性糖皮质激素可快速缓解皮肤和系统症状。本文对Sweet综合征的相关进展作综述。     

Clinical progress in Sweet syndrome

Sweet综合征又称急性发热性嗜中性皮病,是一种少见的、病因尚未完全阐明的反应性疾病.临床上分为经典型/特发型、恶性肿瘤相关型和药物诱导型三个类型,以发热、疼痛性红色斑块、血中嗜中性粒细胞升高和真皮内中性粒细胞浸润为特征,可与多种炎症性疾病或肿瘤伴发.组织学上主要表现为真皮弥漫性成熟中性粒细胞浸润、白细胞核碎裂、真皮乳头水肿,通常缺乏表皮和原发性白细胞破碎性血管炎的改变.系统性糖皮质激素可快速缓解皮肤和系统症状.本文对Sweet综合征的相关进展作综述.     

Sweet's syndrome masquerading as facial cellulitis

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a cutaneous condition that typically occurs as tender red plaques or nodules. However, atypical presentations may occur and, in our case, Sweet's syndrome masqueraded as facial cellulitis and soft tissue infections of the extremities in a sporotrichoid pattern. Despite treatment with broad-spectrum antibiotics, the cutaneous lesions progressed. Results of skin biopsy specimens of the facial plaque and a nodule on the right upper extremity were diagnostic of Sweet's syndrome. Simultaneous to diagnosis, the patient also was found to have acute myelogenous leukemia (AML).     

Panniculitis in a 3‐year‐old child with Fanconi anemia‐associated bone marrow hypoplasia heralds transformation to acute myeloid leukemia

Fanconi anemia is a rare, autosomal recessive genomic instability disorder characterized by congenital limb anomalies, panmyelopathy and a high risk of malignancy, principally acute myeloid leukemia. Hematologic malignancy presenting with acute febrile neutrophilic dermatosis (Sweet syndrome), both deep and superficial forms, is well described in Fanconi anemia patients but is a rare phenomenon in otherwise healthy children. We present a case of panniculitis (presumptive subcutaneous Sweet syndrome) heralding transformation to acute myeloid leukemia in a 3‐year‐old boy with a severe Fanconi anemia phenotype.     

ACUTE FEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME) WITH MYELOID PROLIFERATION

A case of acute febrile neutrophilic dermatosis in a 21-year-old man is presented. Onset of the dermatosis followed sore throat, gastro-enteritis treated with sulphonamides and trimethoprim, and signs of meningism. Accompanying the pronounced neutrophilic leucocytosis and dermal polymorph infiltrate, there was marked myeloblastic proliferation in the bone marrow. While distinction from acute myeloid leukaemia was difficult, there was a rapid response to steroid therapy. The findings emphasize that myeloid proliferation may be a central feature in at least some cases of acute neutrophilic dermatosis.     

Facial Sweet's syndrome mimicking rosacea fulminans

A 36-year-old man presented with a non-pruritic, erythematous facial rash with peri-oral and peri-orbital sparing. The initial clinicopathological diagnosis was rosacea fulminans, which was treated with 25 mg oral prednisolone and cephalexin. The patient re-presented 1 week later with exacerbation of his rash in addition to constitutional symptoms of fever and malaise. A further skin biopsy was taken and the marked neutrophilic infiltrate in the absence of vasculitis made the diagnosis of Sweet's syndrome (acute febrile neutrophilic dermatosis). High-dose prednisolone (50 mg daily), topical hydrocortisone cream and ichthammol in zinc ointment were commenced with rapid clinical improvement. This case highlights the importance of considering Sweet's syndrome as a differential diagnosis when presented with a facial eruption.     

Acute febrile neutrophilic dermatosis and abnormal bone marrow chromosomes as a marker for preleukemia

Acute febrile neutrophilic dermatosis or Sweet's syndrome is a rare disease, which occasionally is seen in patients with myeloid leukemia. We present a case of Sweet's syndrome in a patient with an abnormal chromosome pattern in bone marrow aspirate. Initially the patient had flu-like symptoms with high fever. Two weeks later raised, erythematous and painful plaques appeared on the skin. Various antibiotics were ineffective, but the symptoms vanished after administration of prednisone. Six months later a fulminant acute myeloid leukemia developed, the course of which was complicated by a fatal subdural bleeding. It is concluded that Sweet's syndrome may be a cutaneous sign of a neoplastic myeloid proliferation and that a complete hematological examination including chromosome analysis is mandatory in these patients.     

Neutrophilic dermatosis of myeloproliferative disorders: Atypical forms of pyoderma gangrenosum and Sweet's syndrome associated with myeloproliferative disorders

Atypical forms of pyoderma gangrenosum (PG) and Sweet's syndrome (SS) (acute febrile neutrophilic dermatosis) have been separately reported in association with various forms of leukemia over the past decade. A case in which both atypical bullous PG and atypical SS occurred in a patient with myeloid metaplasia is presented, and the literature is reviewed concerning the association of these dermatoses with neoplasias of myeloid origin. The current case and review support the thesis that these dermatoses, when associated with myeloproliferative disorders, represent points on a continuum of noninfectious, nonmetastatic, inflammatory neutrophilic dermatoses that may occur in patients with derangements in myeloid cell proliferation.     

Acute generalized exanthematous pustulosis

A case of acute generalized exanthematous pustulosis (AGEP) is described. A brief review of AGEP is undertaken with emphasis on its differentiation from acute generalized pustular psoriasis. AGEP is also compared with and contrasted to acute febrile neutrophilic dermatosis, or ‘Sweet's syndrome’ and Sneddon-Wilkinson pustular dermatosis.     

Sweet's syndrome with subcutaneous involvement associated with pegfilgrastim treatment: first reported case

Neutrophilic panniculitis is an infrequent entity, considered by most authors as part of the ‘neutrophilic dermatosis’ spectrum. Few cases have been reported to be related with granulocyte colony‐stimulating factor (G‐CSF); we report a case of neutrophilic panniculitis and Sweet's syndrome lesions related with pegfilgrastim, a long‐acting G‐CSF. A 77‐year‐old woman with M2 acute myeloid leukemia was treated with chemotherapy as well as broad‐spectrum antibiotics and antifungal drugs because of febrile neutropenia. Ten days after a single dose of pegfilgrastim, she developed a limited number of purple plaques on the neck, left leg, both arms and several indurated and slightly mobile nodules on her forearms. Skin biopsy of a plaque showed a diffused dermal neutrophilic infiltrate with dermal edema. Biopsy of a nodule showed a lobular neutrophilic panniculitis without vasculitis. No foreign material was found in those biopsies. No organisms were detected in blood, urine or tissue cultures. She was started with prednisolone 40 mg once a day, with dramatic improvement within the next 2 days. This case is noteworthy for the simultaneous appearance of Sweet's syndrome and neutrophilic panniculitis and it is the first case of neutrophilic panniculitis associated with this drug, pegfilgrastim.