Stevens-Johnson syndrome with idiopathic thrombocytopenic purpura treated with dexamethasone pulse therapy

Stevens-Johnson syndrome (SJS) is a severe, episodic, acute, mucocutaneous hypersensitivity reaction often caused by drugs. We herewith report a case of SJS with idiopathic thrombocytopenic purpura (ITP) that did not respond to daily oral prednisolone therapy. When treated with dexamethasone pulse therapy, the response was found to be very good. Therefore, we concluded that dexamethasone pulse therapy can be a good and an effective alternative therapy for treatment of such patients. However, to establish its role, further trials in more patients are needed.     

高球蛋白血症性紫癜并发原发性干燥综合征一例

患者，女，47岁。双下肢反复出现瘀点、瘀斑4个月，劳累、不良情绪刺激后加重。实验室检查球蛋白增高、血沉增快、类风湿因子强阳性、轻度贫血、血小板正常；抗SSA、SSB抗体2+，Schirmer试验+，唾液腺ECT：唾液腺自主排泌功能降低。唇腺病理活检符合干燥综合征。结合患者临床表现，诊断为高球蛋白血症性紫癜合并原发性干燥综合征。使用糖皮质激素联合羟氯喹、乙酰半胱氨酸、白芍总苷等口服并规律减量， 随访2年病情稳定无复发。     

Purpura Fulminans Associated with Streptococcus pneumoniae Septicemia in an Asplenic Pediatric Patient

Purpura fulminans is a rapidly progressive syndrome of small-vessel thrombosis and hemorrhagic necrosis of the skin accompanied by disseminated intravascular coagulation. We describe a case of Streptococcus pneumoniae septicemia in an asplenic 5-year-old boy on oral tacrolimus, with a past medical history of multivisceral organ transplantation and subsequent development of purpura fulminans on his chest and distal extremities. The acute infectious form of purpura fulminans is usually caused by gram-negative bacteria. Cases secondary to gram-positive encapsulated bacteria usually occur when individuals are immuno-suppressed or have anatomic or functional asplenia. Our patient had both, which likely increased his susceptibility, and he responded well to antimicrobial therapy in addition to prophylactic coverage in the setting of his immunosuppression. We review the literature for similar cases due to S. pneumoniae in the pediatric population and discuss the etiology and treatment of purpura fulminans.     

The skin in disseminated intravascular coagulation

The cutaneous manifestations of disseminated intravascular coagulation (DIC) present in thirty-three of forty-five patients included petechiae, purpura, palpable variants of both, acral cyanosis, haemorrhagic bullae, purpura fulminans, subcutaneous dissecting haematomata, or bleeding from wound or venipuncture sites. In 47% of cases cutaneous lesions were the presenting sign. Coagulation tests were usually abnormal in a pattern diagnostic of DIC. However when initial coagulation-test observations were equivocal, punch biopsy of the skin yielded fibrin thrombi and thus was helpful in the early diagnosis of DIC, later confirmed by serial coagulation studies. Recognition of the types of skin lesions associated with DIC permits prompt diagnosis and institution of appropriate therapy.     

Antiphospholipid antibody syndrome (Hughes' syndrome)

ABSTRACT: A wide variety of dermatologic manifestations have been described in the antiphospholipid syndrome (APS). The most common is livedo reticularis, which is associated not only with cerebrovascular events but also with systemic hypertension and heart valve abnormalities. Its mechanism remains unclear and no treatment is effective. Lower limb ulcers may be related to deep vein or skin vessel thrombosis with clinical features of livedoid vasculitis. The latter frequently persist despite antiplatelet therapy, thus requiring anticoagulation. The pseudovasculitis lesions are frequently misdiagnosed if skin biopsies are not performed, especially in systemic lupus erythematosus-related APS. Antiplatelet therapy is prescribed in the absence of large vessel thrombosis. Widespread cutaneous necrosis and digital gangrenes may be life threatening. They require full anticoagulation. When these lesions appear concomitantly to multiple vascular occlusions in the setting of "catastrophic" APS, steroids and therapy which can achieve a prompt reduction of antiphospholipid antibodies titers should probably be added to anticoagulation.     

Acute Hemorrhagic Edema of Infancy: A Troubling Cutaneous Presentation with a Self‐Limiting Course

Acute hemorrhagic edema of infancy (AHEI) is an unusual form of leukocytoclastic vasculitis with dramatic distinguishing skin lesions that occurs in infants ages 4 to 24 months old. The disease presents with skin eruptions that usually start with large (1–5 cm), symmetrically distributed, hemorrhagic lesions in a characteristic cockade pattern. The lesions are typically located on the lower extremities, face (in particular the ears, cheeks, and eyelids), and gluteal area. Fever may accompany skin eruptions. Clinical presentation at onset requires clinical and laboratory examination to distinguish it from more serious diseases and other vasculitis. The main differential diagnosis of AHEI is Henoch‐Schönlein purpura. AHEI is generally a self‐limiting disease, so a conservative approach should be considered. Topical or systemic corticosteroid therapy has been reported to be beneficial, as well as antihistamines and dapsone, although AHEI usually resolves completely with or without treatment. We report two cases of AHEI and an update of the literature.     

Churg-Strauss syndrome with renal involvement: a case report

We report a case of Churg-Strauss syndrome with renal involvement. A 51-year-old woman was admitted to our hospital in April of 1999 complaining of abdominal pain and severe diarrhea followed by multiple purpura on both legs. A diagnosis of Churg-Strauss syndrome was made on the basis of the presence of preceding asthma, eosinophilia, increased IgE, and the histological findings of a biopsy taken from the purpura. Creatinine clearance level at admission was low (43.3 ml/min) and urinalysis revealed proteinuria and microscopic hematuria. These abnormalities were improved with systemic steroid therapy. Renal involvement seems to be more common than previously reported in Churg-Strauss syndrome.     

Relapsing Henoch-Schönlein purpura in an adult patient associated with hepatitis B virus infection

Henoch‐Schölein purpura is usually a disease of children presenting with arthralgia, abdominal pain, renal involvement, and palpable purpura. Viral and bacterial infections may have a role in its etiology. We present a 32‐year‐old male patient with recurrent Henoch‐Schölein purpura in association with a chronic hepatitis B infection of ten years duration. The patient had received lamuvudine and interferon‐α for the treatment of hepatitis B infection for a year. The skin lesions disappeared with the treatment of the hepatitis B infection. Four months after discontinuation of the therapy, the purpuric papules reappeared with reactivation of the hepatitis B infection. Although rarely reported, hepatitis B virus infection should be considered in patients with Henoch‐Schölein purpura.     

Atypical purpura fulminans with benign monoclonal gammopathy

A 67-year-old woman with a history of thrombophlebitis had been taking warfarin sodium for 1 1/2 years when she developed multiple skin lesions resembling warfarin-induced skin necrosis or purpura fulminans. Despite discontinuing the warfarin and administering prednisone, the lesions increased in size. Disseminated intravascular coagulation (DIC) was found and resolved with heparin sodium therapy, and her skin lesions healed. This patient was believed to have an atypical form of purpura fulminans rather than warfarin-induced skin necrosis because of the duration of warfarin therapy and the dramatic resolution with heparin. A monoclonal (IgG) gammopathy was found, which stabilized as the skin lesions resolved, and fulfilled the criteria for a benign (asymptomatic) monoclonal gammopathy.     

SAPHO综合征的皮肤表现及其进展

SAPHO综合征是一类表现为滑膜炎、痤疮、脓疱病、骨肥厚、骨炎的综合征,为一种少见的累及骨、关节、皮肤的慢性炎症性疾病.SAPHO综合征的皮肤表现多样化,主要包括掌跖脓疱病、严重痤疮、寻常性银屑病、毛囊闭锁三联征等.引起SAPHO综合征皮肤表现的原因尚不明确,研究认为,可能与活化Toll样受体2通路、P2X7-白细胞介素1β轴调节异常相关.SAPHO综合征的病情不易控制,治疗上传统使用非甾体类抗炎药作为一线用药;糖皮质激素、双磷酸盐、抗风湿药物等通常为二线用药.也有研究表明,使用肿瘤坏死因子α拮抗剂能够改善病情,但其疗效尚存争议.近期研究发现,白细胞介素17A拮抗剂苏金单抗治疗掌跖脓疱病可取得良好疗效.正确认识其皮肤表现对于SAPHO综合征的早期诊断、治疗以及对机制的深入研究均有重要意义.     

复合性局部疼痛综合征

复合性局部疼痛综合征临床上分为两型:CRPS Ⅰ和CRPSⅡ.前者无明确的神经受损史,后者有明确的神经受损史,该病突出的症状是疼痛,并伴有皮肤颜色改变、皮温异常及汗液分泌功能的异常.该病治疗较困难,目的是减轻患者的疼痛和改善患者的肢体功能.概述其临床表现、发病机制、诊断和鉴别诊断以及治疗.

Abstract：

Complex regional pain syndrome (CRPS) is a rare condition. Two types of CRPS have been recognized: CRPS Ⅰ which occurs without a definite nerve lesion, and CRPS Ⅱ with the presence of definite nerve lesions. The prominent symptom of this disease is pain, and the pain is accompanied by skin color changes, abnormal skin temperature and sudomotor activity. This paper presents the clinical manifestation, pathogenesis, diagnosis, differential diagnosis and therapy of this entity.     

Acquired cutis laxa secondary to Sweet syndrome in a child (Marshall syndrome): A rare case report

Sweet syndrome is rare in the pediatric population and usually responds well to treatment, resolving without sequelae. Marshall syndrome is a rare pediatric skin disease characterized by loss of elastic tissue (cutis laxa) secondary to acquired, localized neutrophilic dermatitis without any internal organ involvement. Only few cases of Marshall syndrome (acquired cutis laxa type II) have been reported. Systemic steroids and dapsone show excellent results in Sweet syndrome. Although there is no satisfactory treatment for cutis laxa, dapsone can be used in the acute phase for control of swelling.     

Clinical Analysis of Recurrent Hypergammaglobulinemic Purpura Associated with Sjögren Syndrome

Recurrent purpuric lesions are occasionally seen in patients with Sjögren syndrome. Hypergammaglobulinemia is one of the underlying precipitating factors of this condition. Clinical and histopathological analyses were performed on 5 cases of hypergammaglobulinemic purpura associated with Sjögren's syndrome, and the effects of immunomodulatory therapy were evaluated with regards to these conditions. Three out of 5 cases were successfully treated with oral gold compound (Auranofin) and one case with a low dose of cyclophosphamide. Episodic purpura subsided two months after initiation of therapy with improved serum IgG levels. Salivary flow and serum amylase levels also improved in some cases. Immunomodulatory therapy may be useful in managing recurrent purpura based on hypergammaglobulinemia associated with Sjögren syndrome.     

Nonbacterial purpura fulminans and severe autoimmune acquired protein S deficiency associated with human herpesvirus-6 active replication

Nonbacterial purpura fulminans (PF) is rare, usually follows viral infection in young children, and is characterized by specific coagulation disorders, requiring specific therapy. Following a transient rash, a 2-year-old previously healthy girl developed PF without haemodynamic impairment. Laboratory data revealed disseminated intravascular coagulation and a severe transient protein S deficiency. Antiprotein S autoantibodies and active human herpesvirus-6 (HHV6) replication were demonstrated. Purpuric skin lesions spread very rapidly despite broad-spectrum antibiotics and right leg amputation. Plasmapheresis and intravenous immunoglobulins gave complete clinical recovery and normalization of protein S level within 10 days, with progressive clearance of antiprotein S autoantibodies. Transient severe protein S deficiencies have previously been reported in patients with nonbacterial PF, usually after varicella infection. This is the first documented case of PF after HHV6 infection.     

葡萄球菌烫伤样皮肤综合征16例临床分析

葡萄球菌烫伤样皮肤综合征（ＳＳＳＳ）是一种少见而严重的金黄色葡萄球菌引起的感染性疾病，主要发生于５岁以下的婴幼。报首幼儿及儿童病例共１６例，均有典型的临床皮肤症状，经早期应用抗生素治疗，１６例患儿均获痊俞。本文就ＳＳＳＳ的病因、临床表现、病理、鉴别诊断及治疗作了讨论。     

Management of leukocytoclastic vasculitis

Leukocytoclastic vasculitis (LcV) is the most common form of vasculitis of the skin and usually results from deposition of immune complexes at the vessel wall. It presents in different forms and in association with different diseases. When IgA is the dominant immunoglobulin in immune complexes, systemic involvement is likely in both children and adults (Henoch-Sch?nlein purpura--HSP). LcV due to IgG- or IgM-containing immune complexes has less systemic involvement and a better prognosis than HSP. Other forms of LcV include cryoglobulinaemic, urticarial and ANCA-associated LcV as well as LcV associated with vasculopathy and coagulopathy in SCLE/SLE or in bacteraemia/sepsis. The aim of diagnostic guidelines is to determine the specific type and systemic involvement of LcV and to identify an underlying cause. Basic work-up should encompass history of drug intake and of preceding infections, biopsy with immunofluorescence, differential blood count, urine analysis and throat swabs. Therapy of immune complex LcV often does not require aggressive therapy due to a usually favourable course. It includes avoidance or treatment of eliciting agents and use of compression stockings to reduce purpura. There are no large prospective randomized controlled studies. Corticosteroids are indicated when there are signs of incipient skin necrosis. In chronic or relapsing LcV we suggest colchicine as a first-line and dapsone as a second-line therapy. Corticosteroids may reduce the incidence of severe renal insufficiency in children according to some studies, but there is no study showing such an effect in adults. Severe systemic vasculitis requires immunosuppressive strategies.     

嗜酸粒细胞增多综合征的治疗进展

嗜酸粒细胞增多综合征是病因不明,以骨髓、外周血和组织中嗜酸粒细胞增多为特点,并累及多器官的一组疾病,仅累及皮肤者称为嗜酸粒细胞增多性皮炎.传统药物如糖皮质激素、羟基脲、环孢素等均为非特异性作用药物,长期服用不良反应明显.近年发现,该病与嗜酸系融合基因或异常T淋巴细胞克隆有关,酪氨酸激酶抑制剂、干扰素α、白介素-5单克隆抗体等对该病有特异性疗效.     

Autoerythrocyte sensitization syndrome with vertigo and hemilateral auditory impairment

Autoerythrocyte sensitization syndrome (AES) is characterized by recurrent, painful purpura or ecchymosis. Testing for the reappearance of lesions after injection of the patient's own erythrocytes is usually useful for the diagnosis of AES, but the significance of this test is still controversial. As the lesions often appear in patients with psychiatric disorders, mental factors such as depression and stress are considered to be involved in the occurrence and exacerbation of AES. We report a 28-year-old woman who presented recurrent episodes of painful purpura with vertigo and hemilateral auditory impairment after difficulties at her workplace. After the diagnosis of AES, she was referred for psychiatric counselling, after which the symptoms disappeared. These findings suggest that treatment for psychological disorders is important in patients with AES.     

Segmental stiff skin syndrome treated with secukinumab

Segmental stiff skin syndrome is a rare fibrosing scleroderma-like disorder characterized by progressive indurations of the skin leading to joint contractures, decreased mobility, and pain. Treatment options are limited; we report a patient that showed improvement with anti-IL17 biologic therapy.     

MPO-ANCA- and IgA-positive systemic vasculitis: a possibly overlapping syndrome of microscopic polyangiitis and Henoch-Schoenlein purpura

Microscopic polyangiitis (MPA) can be distinguished from Henoch-Schoenlein purpura (HSP) based on the presence of renal-pulmonary complications, myeloperoxidase-antineutrophil cytoplasmic antibody (MPO-ANCA) seropositivity and pauci-immune glomerulonephritis; these characteristics of MPA are generally not found in HSP. Here, we present a unique case with MPA and HSP overlapping syndrome. A 74-year-old man presented with a skin rash accompanied by intracranial bleeding, acute renal failure and pulmonary hemorrhage resulting in a fatal outcome. An autopsy revealed the marked formation of crescentic glomerulonephritis, diffuse pulmonary alveolar hemorrhage and focal cerebral bleeding. Histological features showing both dermal small-vessel vasculitis and cutaneous polyarteritis nodosa-like arteritis suggested MPA rather than HSP, in which the dermal small vessels are exclusively affected. Meanwhile, capillary immunoglobulin (Ig)A deposits in the skin and glomeruli suggested HSP.
As MPA and HSP overlapping syndrome characterized by the synergistic effect of MPO-ANCA and the IgA immune complex may result in a fatal outcome, aggressive immunosuppressive therapy should be initiated as early as possible.