Bullöse Skabies und durch Skabiesbefall getriggertes bullöses Pemphigoid

Two patients were admitted to our hospital with tense blisters on an erythematous base, typical for bullous pemphigoid. In both patients an infestation with Sarcoptes scabiei was diagnosed by dermatoscopy as well as histological examination. In one patient the clinical diagnosis of bullous pemphigoid could be confirmed by immunofluorescence microscopy, histopathology and a clinical relapse of bullous pemphigoid without scabies infestation. In the other patient no evidence for an autoantibody-mediated autoimmune blistering disease was found.We postulate that bullous scabies could develop after long persistency of the parasites leading to a specific immune response with activation of T helper type 2 (Th2) cells causing high levels of the cytokine interleukin 5 and then consecutively eosinophilia. Secretion of proteolytic enzymes near the basal membrane zone might explain the development of intraepidermal, often suprabasal blisters. In contrast, in the first patient the scabies infestation might have triggered a flare up of the underlying autoimmune disease. Comparison of our two patients demonstrates two entities: bullous pemphigoid triggered by scabies as a Koebner phenomenon and a bullous subtype of scabies mimicking bullous pemphigoid.Therefore both, scabies infestation triggering bullous pemphigoid and bullous pemphigoid-like scabies should be included in the differential diagnosis of vesicles, tense blisters and erythema, especially at an early clinical stage.     

A case of crusted scabies with a bullous pemphigoid-like eruption and nail involvement

We report a case of a 71-year-old man infected at a nursing home who developed a bullous pemphigoid-like eruption with nail involvement. He was diagnosed by his family doctor as suffering from eczema and was treated with topical corticosteroids, then blisters started appearing. He was next diagnosed as suffering from bullous pemphigoid and treated with oral prednisolone, which worsened his condition. He was finally diagnosed as having crusted scabies with bullous pemphigoid-like eruptions and nail involvement at our clinic. He was then prescribed oral ivermectin (two doses of 12 mg ivermectin with a 1-week interval) and topical lindane (1%gamma-BHC in petrolatum) for scabies with 5% salicylic acid in plastibase as an additional treatment for the crusted lesions on his soles. He showed remarkable improvement in 2 weeks, and his nails showed complete recovery after 7 weeks of occlusive dressing treatment with 1%gamma-BHC. One and a half years later, the patient showed no sign of a recurrence of scabies. The histology of a blister taken from this patient was similar to that of bullous pemphigoid. Direct immunofluorescence showed immunoglobulin (Ig)G and C3 deposition at the dermoepidermal junction similar to that of bullous pemphigoid, but indirect immunofluorescence was negative. The bullous symptoms of this patient were considered to be due to the scabies, because the patient recovered completely after receiving treatment for scabies. Indirect immunofluorescent study is important to distinguish between scabies with blister formation and true bullous pemphigoid.     

Scabies presenting with bullous pemphigoid-like lesions

A wide range of clinical manifestations may be seen in scabies, from classic pruritic papules and burrows to secondary features such as impetigo. Bullus lesions are a less frequent. Twenty cases of scabies presenting with bullae have been reported so far in the medical literature. Differentiating this subtype of scabies from the immunobullous disease bullus pemphigoid is a diagnostic challenge. A 42-year-old man was referred to our dermatology outpatient clinic with 3-month history of severe pruritus and tense blisters affecting mainly the lower trunk, arms and legs. An initial biopsy was suggestive for bullous pemphigoid. Close physical examination revealed small excoriated papules and a few burrows on borders of the hands and wrists. Skin scraping of the lesions on wrists was positive for Sarcoptes scabiei. Another biopsy specimen from a recent blister revealed subepidermal bullae with fibrin and inflammatory cells, particularly eosinophils. Direct immunofluorescence exam was negative. The patient was treated with lindane lotion followed by crotamiton cream with near complete resolution of the lesions. Scabies must be considered in patients presenting with recent onset of unexplained pruritic bullous lesions. Biopsy and immunofluorescence studies together with skin scrapings for Sarcoptes scabiei could help to differentiate these cases from bullous pemphigoid. Antiscabietic treatment results in resolution of bullous lesions in the affected patients.     

Bullous scabies mimicking bullous pemphigoid

Scabies is an infestation caused by Sarcoptes scabiei and characterised by polymorphous lesions that may include burrows, papules, nodules, excoriation and crusts. Vesicular and bullous lesions are rather rare. Several diseases may be confused with scabies. We report a case of bullous scabies which, on the basis of the clinical and histopathological picture, mimicked bullous pemphigoid. Direct and indirect immunofluorescence were negative. Bullae recurred and persisted despite systemic corticosteroids. The patient was successfully treated with 5% permethrin and remained disease free for up to 12 months of follow-up.     

IgA bullous pemphigoid: a distinct blistering disorder

We report a patient with an eccrine carcinoma who developed localized blistering which clinically resembled pemphigoid, histologically showed subepidermal blistering with features of both dermatitis herpetiformis and bullous pemphigoid, responded to dapsone and exhibited linear IgA deposition on direct immunofluorescence. The nosological position of patients with linear IgA deposition and subepidermal blistering is not clear. A review of the literature reveals that in adults linear IgA deposition may occur in three separate situations: dermatitis herpetiformis, bullous pemphigoid and a third condition of which our case is an example which is best termed IgA bullous pemphigoid. This condition is distinguished from cases of dermatitis herpetiformis with linear IgA by the clinical features and the site of IgA deposition on immunoelectronmicroscopy. It is distinguished from cases of bullous pemphigoid with linear IgA by the absence of circulating IgG antibasement membrane zone antibody, the therapeutic response to dapsone and the frequent occurrence of circulating IgA antibasement membrane zone antibody. IgA bullous pemphigoid has not previously been reported with a carcinoma but the association lends further support to the concept that this eruption represents a variant of pemphigoid.     

放射性皮炎继发大疱性类天疱疮一例

【摘要】 目的 报告放射性皮炎继发大疱性类天疱疮1例,探讨大疱性类天疱疮与放射性皮炎的相关发病机制。病例内容 患者女,82岁,左乳腺癌切除术后10年,术后放疗处出现放射性皮炎。半月前于放射性皮炎位置开始出现浮肿性红斑,水疱,大疱,尼氏征阴性,并伴有瘙痒,随后于背部,下肢出现二处水疱。组织病理为表皮下疱,免疫病理显示基底膜带IgG和C3沉积,血清中存在针对基底膜带成分的自身抗体。予米诺环素加烟酰胺治疗两周后病情控制满意。结论 放射性皮炎继发大疱性类天疱疮国际上偶见报道,有学者认为局部放疗能改变基底膜的性质,使自身抗原暴露,引起自身免疫反应;并能影响金属基质蛋白酶和血管内皮生长因子的表达,故认为大疱性类天疱疮有可能是放疗的一个潜在副作用。     

A study of benign chronic bullous dermatosis of childhood and comparison with dermatitis herpetiformis and bullous pemphigoid occurring in childhood

Eighteen patients with benign chronic bullous dermatosis of childhood were studied and the findings compared with those of dermatitis herpetiformis (twenty-two cases) and bullous pemphigoid (five cases) beginning in childhood. The patients with benign chronic bullous dermatosis of childhood had a moderately pruritic bullous eruption with maximal involvement of the pelvic and perioral regions which tended to occur at an earlier age than either dermatitis herpetiformis or bullous pemphigoid. In contrast to dermatitis herpetiformis one-third of the cases with benign chronic bullous dermaiosis of childhood went into remission. Evidence of coeliac disease was only found in the dermatitis herpetiformis group. Surprisingly both diseases shared HLA-B8. A linear BMZ band of IgA was detected on direct immunofluorescence in all but one of the cases with benign chronic bullous dermatosis of childhood and circulating antibodies were detectable in two-thirds. Routine histopathology was of little value in distinguishing between benign chronic bullous dermaiosis of childhood and dermatitis herpetiformis or bullous pemphigoid. Several paradoxes have yet to be explained before it can be determined whether benign chronic bullous dermatosis of childhood is a variant of dermatitis herpetiformis or linear IgA disease.     

Bullous scabies

Bullous scabies is a rare presentation usually occurring in patients who are elderly. Clinical, histologic, and immunofluorescent findings mimic bullous pemphigoid. We report a case of bullous scabies in a 4-year-old boy and review the literature.     

Sarna ampollosa con respuesta terapéutica a la ivermectina

Bullous scabies is a rare disease that is usually diagnosed in elderly patients. The clinical, histological, and immunological findings are identical to bullous pemphigoid. In a review of the literature, we found reports of 24 cases. We present a new case of bullous scabies in a 72-year-old man. The lesions responded to treatment with oral ivermectin.     

Polymorphic pemphigoid. Is it still worth using this terminology?

The term polymorphic pemphigoid has been used in the literature as a variant of bullous pemphigoid. But this term is imprecise and now obsolete, since patients with linear IgA dermatosis have been reported under this terminology. The patients who develop an atypical subepidermal bullous disease with clinical and histological features of both bullous pemphigoid and dermatitis herpetiformis may actually be classified into three groups: (1) vesicular variant of bullous pemphigoid; (2) linear IgA dermatosis, and (3) mixed subepidermal bullous disease.     

Defining urticarial dermatitis: a subset of dermal hypersensitivity reaction pattern

BACKGROUND: Urticarial dermatitis may represent a useful term for a subset of a reaction pattern designated most commonly as dermal hypersensitivity by pathologists. The term is not commonly used, and requires definition to determine whether it is clinically relevant. OBJECTIVES: To define urticarial dermatitis and distinguish it from other urticarial reaction patterns and to review the frequency with which dermatologists can recognize clinical settings that match the biopsy findings of urticarial dermatitis. DESIGN: Retrospective analysis of clinical and/or histological diagnosis of urticarial dermatitis, applying strict histological criteria in a center using urticarial dermatitis as a diagnostic term in 190 archived reports. SETTING: Tertiary referral dermatopathology service reporting for dermatological practices in Sydney, Australia. MAIN OUTCOME MEASURES: The correlation between clinical and histological diagnoses of urticarial dermatitis and alternate diagnoses was analyzed. The frequency of positive immunofluorescence findings for bullous pemphigoid was determined in a subset of patients with urticarial dermatitis in whom this test was ordered to exclude prodromal bullous pemphigoid. RESULTS: Urticarial dermatitis was the histological diagnosis in at least 1 biopsy result in 148 patients, and matched the provisional clinical diagnosis in 49 (33.1%) patients. Urticarial dermatitis was the only diagnosis provided in 21 patients. The main alternate clinical diagnoses provided were early bullous pemphigoid or dermatitis herpetiformis (47 patients [31.8%]), dermatitis (39 patients [26.4%]), drug reaction (35 patients [23.6%]), urticarial vasculitis (24 patients [16.2%]), and urticaria (12 patients [8.1%]). In 91 patients with a clinical diagnosis of urticarial dermatitis, the histological diagnosis in at least 1 biopsy result was matched in 49 patients (53.8%); other histological diagnoses included dermatitis (21 patients [23.1%]), papular urticaria (12 patients [13.2%]), drug reaction (6 patients [6.6%]), and urticaria (3 patients [3.3%]). Review of 38 direct immunofluorescent results for prodromal bullous pemphigoid and a biopsy finding of urticarial dermatitis revealed only 3 positive results (7.9%). CONCLUSIONS: Urticarial dermatitis seems to be a useful histological and clinical term for a subset of the dermal hypersensitivity reaction pattern. Although the clinical presentation is not restricted to a specific entity, eczema and drug reactions seem to be the most frequent clinical associations; and in a subset of patients, urticarial dermatitis remains as a recognizable reaction pattern. Urticarial dermatitis without eosinophilic spongiosis is not a reliable indicator for bullous pemphigoid, because the findings of immunofluorescence are often negative.     

Histology of linear IgA disease, dermatitis herpetiformis, and bullous pemphigoid

The histologic appearances of cutaneous biopsy specimens from 30 patients with linear IgA disease with a continuous band of IgA along the basement membrane, four patients with a linear pattern of granular IgA along the basement membrane, 26 patients with dermatitis herpetiformis who had IgA in the papillary dermis, and 23 patients with bullous pemphigoid who had IgG and/or C3 along the basement membrane were compared. Those with linear and granular IgA and dermatitis herpetiformis differed from those with bullous pemphigoid in five respects. Multiple microabscesses and fibrin at tips of papillae and leukocytoclasis were less common in bullous pemphigoid, whereas a dense infiltrate of eosinophils in and below bullae and a linear infiltrate of eosinophils along the basement membrane were more common in bullous pemphigoid. Also, multilocular bullae and acantholysis were more common in dermatitis herpetiformis than in bullous pemphigoid. Linear IgA disease differed from dermatitis herpetiformis in two respects. Acantholysis and fibrin at the tips of papillae and leukocytoclasis were more common in dermatitis herpetiformis. The specimens from patients with granular IgA did not differ significantly from those with linear IgA or dermatitis herpetiformis. The appearances of biopsy specimens of patch tests with potassium iodide taken from 11 patients with dermatitis herpetiformis and linear or granular IgA disease were similar to those taken from spontaneous lesions.     

Bullous eruption associated with scabies: evidence for scabetic induction of true bullous pemphigoid

Some patients with scabies develop bullae concomitantly with, or subsequently after, the occurrence of scabetic lesions. Although several immunofluorescence studies have demonstrated immunoglobulin deposition in the basement membrane zone of bullous lesions, it remained unclear whether these antibodies are directed to bullous pemphigoid antigens. We clearly show that two scabetic patients with bullous eruptions had circulating antibodies against BP180 and/or BP230 as determined by Western blotting analysis. This is the first report to demonstrate that at least some of the bullous eruptions occurring in scabetics are true bullous pemphigoid.     

Linear arrangement of neutrophils along the Basal layer in bullous pemphigoid: a unique histological finding

Bullous pemphigoid is an inflammatory autoimmune subepidermal bullous disease with distinct immunohistological features. We report an unusual case of a 59-year-old woman with a bullous eruption whose lesional skin biopsy showed a subepidermal blister with a linear arrangement of neutrophils, mimicking linear IgA bullous dermatosis. However, direct immunofluorescence studies demonstrated IgG and C3 linear deposition along the basement membrane zone, compatible with bullous pemphigoid. We suggest that bullous pemphigoid should therefore be considered in the differential diagnosis of neutrophil-rich subepidermal bullous diseases along with dermatitis herpetiformis and linear IgA.     

A subepidermal blistering disease with histopathological features of dermatitis herpetiformis and immunofluorescence characteristcs of bullous pemphigoid: a novel subepidermal blistering disease or a variant of bullous pemphigoid?

A 64-year-old man presented with a bullous eruption which clinically and histopathologically resembled dermatitis herpetiformis. However, direct immunofluorescence analysis showed IgG deposits at the basement membrane zone, indicating a relationship with bullous pemphigoid or epidermolysis bullosa acquisita. Indirect immunofluorescence studies on salt-split skin showed binding of IgG mainly on the dermal side of the blister. Immunoblot analysis revealed a novel 200 kDa dermal antigen that could be associated with a major pathogen in this blistering a disease. The histopathological similarity to dermatitis herpetiformis and the immunofluorescence findings indicating bullous pemphigoid or epidermolysis bullosa acquisita seem typical of a distinct subepidermal blistering disease characterized by this 200 kDa antigen. However, the pathogenetic role of autoantibodies against this antigen should be further elucidated before confirming whether this case represents a novel subepidermal blistering disease or a special variant of bullous pemphigoid.     

Dyshidrosiform bullous pemphigoid: trigger factors

This report presents 2 patients with a rare form of bullous pemphigoid on (heir palms and soles. To date, at least 16 cases of dyshidrosiform pemphigoid have been reported. The first case was accompanied by a bacterial fool infection, the second by contact dermatitis. The possibility that dyshidrosiform bullous pemphigoid was induced by these trigger factors is discussed.     

Complement activation in bullous skin diseases.

Pemphigus, bullous pemphigoid, cicatricial pemphigoid, dermatitis herpetiformis, and herpes gestationis are members of the chronic vesiculobullous skin diseases of man. The complement system, including both the classical and alternative pathways, may be important in the pathogenesis of these diseases. In pemphigus, early complement components (C1, C4, and C2) appear to be activated in addition to later components (C3 and C5), suggestive of classical pathway activation. Participation of properdin in addition to early complement components suggests local activation of both complement pathways in bullous pemphigoid and cicatricial pemphigoid. Herpes gestationis and dermatitis herpetiformis may be bullous skin diseases entirely mediated by the alternate or properdin pathway. The specific immunopathologic findings in these diseases are discussed.     

Junctional blisters in acquired bullous disorders of the dermal-epidermal junction zone: role of the lamina lucida as the mechanical locus minoris resistentiae

The level of cleavage was determined in a variety of acquired bullous diseases of the dermal-epidermal junction zone (bullous pemphigoid, dermatitis herpetiformis, porphyria cutanea tarda and epidermolysis bullosa acquisita). We used an indirect immunofluorescence technique to examine the basal membrane zone with anti-type IV collagen and anti-laminin antisera and bullous pemphigoid sera. The majority of blisters examined proved to be junctional, including those from disorders hitherto considered to be dermolytic. Dermolytic cleavage was encountered only sporadically in microvesicles of dermatitis herpetiformis, in one small vesicle and in one out of five large blisters of porphyria cutanea tarda and in a large lesion of epidermolysis bullosa acquisita. We conclude that in acquired bullous disorders of the dermal-epidermal junction zone the preferential site of split formation is the lamina lucida which appears to act as a locus minoris resistentiae; dermolytic split formation of substantial extent occurs only when the sublaminal fibrillar apparatus is mechanically compromised.     

Adult linear IgA bullous dermatosis: a polymorphic disorder

We report on two patients with unusual forms of adult linear IgA bullous dermatosis. One was a middle-aged woman who had targetoid lesions and bullae on her trunk and extremities. This patient first presented with lesions that clinically resembled erythema multiforme, but these evolved into a widespread eruption with bulging, elongated bullae. Examination of a biopsy specimen showed changes compatible with dermatitis herpetiformis and bullous pemphigoid. Findings on immunofluorescence studies showed deposition of linear IgA at the basement membrane zone. The second patient was an elderly woman with intensely pruritic vesicles whom we classified as having vesicular pemphigoid, until the linear IgA band on direct immunofluorescent test results became the predominant immunofluorescent finding. These cases are reported because of their unusual clinical presentations. The mechanism for the targetoid lesions in the first patient is discussed.     

Amoxicillin-induced bullous pemphigoid in a child

The Authors describe the case of a 6-year old female child who presented a bullous dermatitis following the administration of amoxicillin, with clinical, histopathological and immunological features typical of childhood bullous pemphigoid. The peculiar aspects of drug-induced bullous pemphigoid are therefore reviewed, with particular reference to the hypotheses behind the relative induction mechanism.