Childhood bullous pemphigoid: a case report and 10-year follow up

Bullous pemphigoid (BP) is a very rare acquired immunbullous disease in children and infants. We report the case of a 15-month-old boy referred to our service with multiple tense bullae located predominantly on the trunk, neck and proximal portion of the limbs. Palms, soles and oral mucosa were also affected. Histopathologic and immunopathologic features were characterized with bullous pemphigoid. The patient responded well to systemic steroids, with improvement of his condition. After a 10-year follow up the patient was no longer suffering from the disease.     

红皮病性大疱性类天疱疮1例

 患者女,78岁。因“躯干、四肢红斑、鳞屑伴瘙痒5年,加重3年余”入院,入院后1周大腿及腹部出现紧张性水疱。住院第6天、第9天血清抗BP180抗体均阳性,IgE>2 500 U/mL。皮损组织病理可见表皮下水疱,真皮浅层大量淋巴细胞为主的浸润。直接免疫荧光示:基底膜带IgG和C3线状沉积,IgA阴性。诊断：红皮病性大疱性类天疱疮。给予甲泼尼龙40 mg静脉滴注1天1次,治疗1周,病情好转出院。1年后随访,患者泼尼松片减量至15 mg口服1天1次,躯干四肢可见轻度红斑,无水疱、糜烂和瘙痒     

Childhood bullous pemphigoid: report of three cases

Bullous pemphigoid (BP) is a disorder that rarely occurs in children. We hereby describe three cases of childhood BP aged 2-4 months, which are among the youngest reported in the literature. BP was confirmed by histopathology, direct and indirect immunofluorescence with salt-split skin test and immunoblotting. These patients were successfully treated with systemic corticosteroids with a complete clinical remission.     

药物诱发的大疱性类天疱疮一例

患者,女,20岁。全身皮肤红斑水疱伴瘙痒1个月。1个月前患者因口服“氨酚黄那敏颗粒”后全身出现红斑水疱。皮肤专科查体：上唇、颈部、胸背、腋下、外阴及双大腿内侧见红斑,其上多发黄豆至蚕豆大小的水疱、大疱,疱壁紧张,部分水疱破溃,上附淡黄色痂,尼氏征(-),部分区域可见虹膜样改变,口腔黏膜、舌部受累,可见散在糜烂、水疱。实验室检查、组织病理、直接免疫荧光结合盐裂试验,诊断为药物诱发的大疱性类天疱疮。经甲泼尼龙等治疗1个月后痊愈停药。     

Childhood bullous pemphigoid – report of a case with dermal fluorescence on salt-split skin

Summary Bullous pemphigoid (BP) is an acquired bullous disorder which predominantly affects the elderly. It is rare in children, and may be clinically indistinguishable from other immunobullous disorders. As routine histology may be non-specific, a definitive diagnosis of childhood BP usually depends on the results of direct and indirect immunofluorescence investigations.
We report a 5-year-old girl who developed bullous pemphigoid, associated with atypical immunofluorescence findings. Indirect immunofluorescence on split-skin showed a pure dermal pattern of IgG binding. This is usually suggestive of epidennolysis bullosa acquisit, but Western immunoblotting was positive with epidermal extracts, confirming a diagnosis of BP. Dermal binding on split-skin occurs in about 5% of adult cases of BP, and has not been reported previously in childhood BP.     

利妥昔单抗联合糖皮质激素治疗难治性大疱性类天疱疮一例

患者,男,35岁。四肢红斑、丘疹、水疱1个月。行皮肤组织病理、直接、间接免疫荧光及盐裂试验,符合大疱性类天疱疮诊断。予系统运用糖皮质激素,环磷酰胺冲击,联合吗替麦考酚酯、沙利度胺、静注人免疫球蛋白等治疗,疗效欠佳,改用利妥昔单抗联合糖皮质激素治疗后,病情好转。     

利福平对糖皮质激素治疗大疱性类天疱疮的干扰

报告1例应用利福平导致对糖皮质激素治疗产生抵抗的大疱性类天疱疮.患者女,44岁.全身出现红斑、水疱2年余,复发1个月余.结合临床表现、组织病理检查及免疫荧光检查诊断为大疱性类天疱疮.患者入院前因疑为结核性胸膜炎,给予利福平抗结核治疗.入院后给予泼尼松60 mg/d及环孢素、环磷酰胺、氨苯砜治疗,泼尼松剂量逐渐加至180 mg/d,仍不能控制病情,而在停用利福平之后,病情在5 d内得到缓解.     

眼周局限性大疱性类天疱疮一例

患者,女,12岁.眼周反复红斑、水疱4个月,伴瘙痒.皮损组织病理示:表皮下水疱,真皮浅层淋巴细胞、嗜酸粒细胞、中性粒细胞等炎性细胞浸润.直接免疫荧光(DIF):表皮基底膜IgG、C3阳性线状沉积.ELISA检测:血清抗BP230抗体2.6 U/mL,抗BP180抗体阴性.诊断:局限性大疱性类天疱疮.予以甲泼尼龙、氨苯砜...     

Childhood cicatricial pemphigoid with linear IgA deposits: a case report

A 16-year-old boy with a bullous eruption from the age of thirteen, also presented symptoms of scarring conjunctivitis and involvement of oral mucosal membranes. Linear IgA deposits were demonstrated in the basement membrane zone in skin and conjunctiva. The eruptions improved during therapy with aldesulfonsodium, but complete remission was not obtained. Gluten-free diet did not significantly influence the activity of the disease. The described patient seems to fit in a recently described entity of chronic bullous diseases.     

婴儿湿疹合并大疱性类天疱疮1例

1岁以内的婴儿大疱性类天疱疮国外仅有10余例报告,国内未见报告,婴儿湿疹合并大疱性类大疱疮至今国内外均未见报告.文中报告首例婴儿湿疹合并大疱性类天疱疮患者,用丙种球蛋白治疗有效,该例婴儿湿疹合并大疱性类天疱疮的发病可能与疫苗接种有关.     

利格列汀相关的BP230型大疱性类天疱疮一例国内首报

患者女,66岁,因头皮、颈部水疱1个月就诊。既往有2型糖尿病病史6年,于起疹前6个月开始口服二肽基肽酶4抑制剂利格列汀5 mg每日1次。皮肤科检查：头皮散在绿豆至花生米大小水疱,部分水疱破溃,可见渗出、结痂;左侧颈部一鸽蛋大小大疱及2个绿豆大小水疱,疱壁紧张,疱液清亮,基底无明显红斑,尼氏征阴性。酶联免疫吸附试验示抗B...     

Hypereosinophilic syndrome as prodrome of bullous pemphigoid:report of a case

Persistent hypereosinophilia, cardiac involvement and a recurrent erythematous-papular pruritic eruption histologically characterized by eosinophilic spongiosis are described in a 77-year-old man. This condition, suggestive of "idiopathic" hypereosinophilic syndrome, represented the prodromic phase of atypical bullous pemphigoid, which manifested 7 months later.     

Childhood bullous pemphigoid successfully treated with diaminodiphenyl sulfone

Bullous pemphigoid (BP) is an acquired autoimmune blistering disease which predominantly affects the elderly. It is rare in children and infants. We reported a 14-year-old girl presenting with a month history of relapsing tense bullae on the face and extremities. Histopathological examination of the lesional skin revealed a subepidermal bulla with infiltration of eosinophils, neutrophils, and lymphocytes. Direct immunofluorescence showed linear deposits of IgM and C3 at the basement membrane zone. Indirect immunofluorescence using normal human skin sections as a substrate detected IgG anti-basement membrane zone antibodies in the patient's serum and that using 1M NaCl split skin sections showed that the patient's antibodies bound to the epidermal side of the split skin. Immunoblot analysis using normal human epidermal extracts demonstrated the presence of autoantibodies against the 230-kDa BP antigen. Furthermore, the patient's serum reacted with the recombinant protein of the NC16a domain of the 180-kDa BP antigen by immunoblot analysis and enzyme-linked immunosorbent assay. Our patient showed significant improvement of the skin lesions with systemic administration of diaminodiphenyl sulfone.     

儿童线状IgA大疱性皮病

报告1例儿童线状IgA大疱性皮病,以口周、下腹部、外阴、臀部、大腿内侧及四肢多数水疱为主要临床表现、病理见表皮下疱,疱内有大量中性粒细胞、红细胞和少量嗜酸性粒细胞.直接免疫荧光见基底膜带有线状IgA沉积.     

Childhood localized vulvar pemphigoid is a true variant of bullous pemphigoid.

BACKGROUND--Childhood localized vulvar pemphigoid has been recently reported in four girls. A fifth child with this proposed rare variant of bullous pemphigoid is described. Moreover, findings in the various immunopathologic studies we performed establish this entity as a true morphologic variant of bullous pemphigoid. OBSERVATIONS--In situ deposits of IgG in this patient's epidermal basement membrane zone localized to the epidermal side of 1 mol/L of saline-split skin. Moreover, the patient had circulating IgG autoantibodies that bound the epidermal side of 1 mol/L of saline-split skin in indirect immunofluorescence microscopy and immunoprecipitated the 230-kd bullous pemphigoid antigen from biosynthetically radiolabeled human keratinocyte extracts. These laboratory findings are identical to those documented in patients with the generalized "classic" form of bullous pemphigoid. CONCLUSIONS--This study demonstrates that a child with clinical, histopathologic, and immunopathologic features of localized vulvar pemphigoid had circulating autoantibodies that identify a specific keratinocyte antigen, the bullous pemphigoid antigen, which may serve as a molecular marker for this disease.