Cases Of Bullous Pemphigoid Research Articles

Epidermal interleukin-8 and its receptor CXCR2 in drug-induced toxic epidermal necrolysis

In drug-induced toxic epidermal necrolysis (TEN), the epidermal destruction is associated with a slight to moderate lymphomonocytic cell infiltrate. Interleukin (IL)-8, which is a keratinocyte-derived pro-inflammatory cytokine, might be involved in this process. The IL-8 receptor CXCR2 has also been shown to be overexpressed in some epidermal disorders. IL-8 concentration was measured by ELISA in both serum and blister fluid from 10 patients with TEN. Data were compared with similar dosages performed in 15 cases of second-degree burn and 7 cases of bullous pemphigoid (BP). CXCR2 expression on keratinocytes was studied using immunohistochemistry on skin biopsies performed in TEN bullous lesions and clinically uninvolved skin of the same patients. IL-8 was significantly overexpressed in TEN blister fluid compared with TEN serum (P = 0.0015). However, no difference was found in IL-8 concentrations present in blister fluid of TEN, second-degree burn and BP. CXCR2 was moderately expressed in the epidermis of some TEN blisters, but was never expressed in clinically uninvolved skin. CXCR2 expression was not found in the follicular epidermal root sheaths of patients with TEN. These results indicate that abundant IL-8 appears to be locally produced in TEN epidermis, but this overexpression is not disease-specific. Because of the paucity of the inflammatory infiltrate in TEN, it is unlikely that IL-8 induces epidermal destruction through its chemotactic activity. Moreover, the complete absence of neutrophils in TEN lesions indicates that the major chemotactic effect of IL-8 on neutrophils is not operative in TEN skin. This implies that IL-8 activates different functions according to the local environment. CXCR2 expression on TEN keratinocytes is expressed on some necrotic keratinocytes, consistent with a discrete IL-8 proapoptotic activity. The lack of CXCR2 expression in the follicular root sheaths argues against a role for IL-8 in TEN epidermal repair.

Penfigóide bolhoso no lactente

O penfigóide bolhoso é doença que comumente acomete pessoas idosas. Desde o primeiro caso na infância, confirmado por imunofluorescência em 1970, cerca de 50 casos foram relatados e poucos ocorreram em crianças com menos de um ano de idade. Apresentase caso de penfigóide bolhoso em lactente de quatro meses de idade e discutem-se as características clínicas e imunopatológicas dessa rara bulose da infância.

A case of bullous pemphigoid and figurate erythema in association with metastatic spread of carcinoma.

SUMMARY Clinical and laboratory findings in a case of bullous pemphigoid developing during the course of metastatic spread of rectal adenocarcinoma are presented. In addition a transient figurate erythema occurred. The possibility of a causal relationship between bullous pemphigoid and malignant disease is discussed.

Une pemphigoïde bulleuse épargnant un lymphœdème acquis

Bullous pemphigoid is an autoimmune disease, common in the elderly and generally of symmetrical and systemic localization. We report a case with sparing of the lower limb and acquired lymphedema secondary to lymph node surgery. A 74-year-old woman was hospitalized for a bullous eruption. The left lower limb was completely spared and was unaffected by pruritus. Acquired lymphedema was seen in this limb secondary to lymph node surgery. Standard histopathology tests confirmed the diagnosis of bullous pemphigoid with subepidermal blistering, while a direct immunofluorescence antibody test showed linear binding of IgG and C3 throughout the basement membrane. Western blotting revealed anti-BPAg2 antibodies. Skin biopsy on the lymphedema spared by the disease revealed no inflammatory infiltrate in the dermis. However, linear binding of anti-IgG and anti-C3 autoantibodies was observed. Other cases of localized bullous pemphigoid appearing on body areas treated by UV or radiotherapy have been reported. Cases of bullous pemphigoid with predilection for areas of lymphedema have also been previously described: the hypothesis has been advanced of reduced lymphatic flow, with increased antigen-antibody contact enabling better binding. Our case is original and, given the protective nature of this lymphedema, suggests two hypotheses. There could be deterioration of local cellular immunity, with decreased activation of T lymphocytes. They could also be impairment of nervous conduction, as suggested by the absence of pruritus, with partial or total inhibition of neurogenic inflammation.

Laryngeal involvement in a patient with bullous pemphigoid

Laryngeal involvement in a patient with bullous pemphigoid

Clinicoepidemiological features and course of 43 cases of bullous pemphigoid in Kuwait

The clinicoepidemiological characteristics and course of bullous pemphigoid (BP) have not been described in populations from the Arabian Gulf. Hypothesis. Ethnic and regional variations can influence the clinical behaviour and course of autoimmune diseases. In this study, 43 patients with BP, registered in our autoimmune bullous diseases (ABD) clinic over a span of 14 years, were studied to determine the clinicoepidemiological features and course of the disease in our region. BP was observed to be the second commonest ABD in our clinic (27%), with a minimum estimated incidence of 2.6 cases/million/year among the referral population. The largest proportion (93%) of the patients was of Arab ethnicity and the female to male ratio was 5.1 :1. Mean +/- SD age at diagnosis was 65.20 +/- 18.80 years. Most of the patients (96%) had moderate to severe disease, and mucosal involvement was seen in 37% of the patients. Systemic steroids (prednisolone 20-60 mg daily) alone or in combination with azathioprine, intravenous immunoglobulins, tetracyclines, mycophenolate mofetil or dapsone were used to treat theses cases. At the last follow-up, 32% of patients were in complete remission and off treatment. The first-year mortality was 30%. Old age and poor general medical condition were the significant risk factors (P < 0.05) contributing to the mortality. The study highlights the regional variations of BP and thus a need to uncover the ethnic, genetic and geographical influences, if any, responsible for these variations.

A Case of Bullous Pemphigoid Arising in Juvenile Hyaline Fibromatosis with Oral Squamous Cell Carcinoma

We experienced a case of bullous pemphigoid (BP) arising in juvenile hyaline fibromatosis (JHF) associated with oral squamous cell carcinoma (SCC). JHF is a very rare disease inherited in an autosomal recessive fashion. Skin-colored, elastic hard nodules up to 10 cm in size were scattered mainly on the trunk and scalp. Gingival hypertrophy and an ill-defined mass with an irregular surface on the hard palate were noticed. A skin biopsy and a gingival biopsy confirmed the diagnosis of JFH and oral SCC, respectively. The patient was receiving anti-cancer drugs for the treatment of oral SCC and the treatment was successful. In the meantime, bean to nail sized bullas were noticed on the hands and feet. Histological examination, immunofluorescence method and ELISA confirmed the diagnosis of BP. The pathophysiological mechanisms are discussed in the present case.

A case of bullous pemphigoid associated with autoantibodies targeting antigenic sites other than the NC16a domain of BP180

Bullous pemphigoid (BP) is associated with autoantibodies to the 180-kDa BP antigen (BP180), and the antigenic site exists on noncollagenous 16a (NC16a) domain of BP180. We now report a male BP patient whose IgG autoantibodies did not react against the NC16a domain of BP180 by either immunoblotting or ELISA, whereas they did react with BP180 extracted from normal human keratinocytes. Anti-BP180 cicatricial pemphigoid was ruled out due to the lack of conjunctival mucosal involvement and the absence of scarring in the oral cavity. Our findings indicate that there is an antigenic reactive region other than NC16a on the extracellular domain of BP180. There have been few reports describing detailed clinical features of BP caused by autoantibodies targeting antigenic sites other than the NC16a domain. We conclude that it is difficult to differentiate their clinical features from those associated with autoantibodies targeting the NC16a domain of BP180.

Pemphigoïde du sujet jeune: Étude rétrospective de 74 cas

Bullous pemphigoid usually affects elderly people. Only a few isolated cases among people younger than 65 years have been reported.Describe the clinical and biological characteristics of patients younger than 60 years suffering from bullous pemphigoid, compare them with the usual characteristics known among elderly people and search for potential pathological associations.Retrospective, national, multicenter study. Clinical, biological and histological characteristics were recorded with a standardised questionnaire as well as treatments and associated pathologies.Seventy-four cases of bullous pemphigoid diagnosed between June 1970 and March 2002 were analyzed. Mean age at the beginning of the disease was 46 +/- 11.6 years. Further explorations by indirect immunofluorescence of separated skin and/or immuno-electron microscopy and/or immunoblotting were performed for 42 patients (56.8 p. 100). Clinical characteristics among this restricted population were comparable to those found among the 32 other cases. Compared to usual data on bullous pemphigoid in elderly people, we observed a greater proportion of extensive form of disease (75 p. 100), a more frequent head and neck involvement (39.2 p. 100) and an overexpression of anti-BP180 autoantibodies (48 p. 100). Neoplasm was notified for 7 patients (9.5 p. 100), 18 (24.3 p. 100) suffered from a pathology of the basement membrane zone (6 psoriasis, 6 atopic dermatitis and 6 lichen) and 13 from neurological disease, among which 4 were bedridden. Fourty-six patients (62.2 p. 100) received drugs for the long term (mean 2.12 +/- 2.43), 4 patients were treated by PUVAtherapy and 2 by radiotherapy.Our results suggest that bullous pemphigoid among young people is more severe and more active than the usual form in the elderly. This particular form could be the result of a higher expression of anti-BP180 autoantibodies, which are considered as a marker of poor prognosis in this disease. We also found a high frequency of pathological associations and physical treatment, all responsible for damage to the basement membrane zone, which can involve auto-immunization against hemidesmosome components.

Localized bullous pemphigoid overlying a fistula for hemodialysis

Two cases of bullous pemphigoid exclusively limited to the skin overlying a hemodialysis fistula in patients with chronic renal failure are reported. This is an unusual association, but should be taken into account in the differential diagnosis of bullous disorders among patients on hemodialysis. We hypothesize about the pathogenesis of this process.

A Case of Bullous Pemphigoid Successfully Treated by Plasmapheresis: Assessment of the Change in Titers of Circulating Antibodies by Immunoblotting and Enzyme‐Linked Immunosorbent Assay

We report a case of bullous pemphigoid successfully treated with double filtration plasmapheresis. The changes in titers of circulating autoantibodies were assessed by immunoblotting and enzyme-linked immunosorbent assay (ELISA) using a recombinant protein of the non-collagenous 16a (NC16a) domain of the 180 kDa bullous pemphigoid antigen (BP180). The ELISA was shown to be more sensitive in detecting disease-specific antoantibodies in the bullous pemphigoid sera. The reduction of titers of circulating autoantibodies in the sera correlated well with the decrease in the disease activity in both the first and second rounds of plasmapheresis treatment in this case.

Mycophenolate mofetil in autoimmune and inflammatory skin disorders

Mycophenolate mofetil (MMF) has been widely used as an immunosuppressant in organ transplantation. MMF has recently been added to therapeutic regimens for skin disorders. Expanding the use of MMF in dermatology, we describe additional patients with autoimmune and inflammatory skin diseases, including 4 cases of pemphigus vulgaris, 1 case of pemphigus foliaceus, 1 case of perineal and metastatic cutaneous Crohn’s disease, 1 case of bullous pemphigoid and psoriasis, and 1 case of psoriasis. Most of these patients had refractory disease or had developed significant side effects to conventional therapy, including azathioprine, methotrexate, prednisone, cyclosporine, acitretin, PUVA, UVB, and tacrolimus. MMF was effective and well tolerated in all these patients. The dosages of MMF ranged from 500 mg twice daily (for psoriasis and Crohn’s disease) to 1250 mg twice daily (for 3 of 4 patients with pemphigus vulgaris). MMF is an effective and relatively safe immunosuppressant in autoimmune and inflammatory skin diseases. (J Am Acad Dermatol 1999;40:265-8.)

Analysis of desquamative gingivitis using direct immunofluorescence in conjunction with histology.

Chronic desquamative gingivitis (DG) is a common condition often first recognized by periodontists. DG is characterized clinically by erythema with epithelial desquamation, ulceration, and/or the presence of vesiculobullous lesions of the free and attached gingiva. However, DG is actually a clinical manifestation of several different disease processes, particularly lichen planus and benign mucous membrane pemphigoid. Correct diagnosis in DG is critical since proper treatment and follow-up will depend on which disease is involved. However, the diseases that cause DG frequently present diagnostic problems because their lesions often resemble each other clinically and routine histological examination sometimes cannot differentiate between them. Thus, immunohistology, particularly immunofluorescence, is increasingly being used with routine histology to more accurately diagnose DG diseases. This article reviews our experience over the past 10 years in the diagnosis of 72 cases of DG using direct immunofluorescent (DIF) in conjunction with histology and clinical evaluation. Of the 72 DG cases in this study, 30 cases were diagnosed as erosive lichen planus or lichenoid mucositis; 29 cases were diagnosed as benign mucous membrane pemphigoid; 2 cases each of linear IgA disease and pemphigus vulgaris were diagnosed; there was 1 case of bullous pemphigoid; and 1 suspected case of paraneoplastic pemphigus. Even with DIF analysis, 7 cases could not be definitively assigned a particular cause. DIF analysis is not only proving very useful for differential diagnosis, but also adds insight into possible pathogenic mechanisms of DG.

Bullous Pemphigoid in an HB Virus Carrier: Interaction between Corticosteroids and HB Virus

A case of bullous pemphigoid (BP) in a 47-year-old female hepatitis B virus (HBV) carrier is reported. Disseminated bullae covered the patient's entire body including the hands and feet. BP was diagnosed on the basis of pathological and immunological observations examining immunofluorescence and cross reactions to both bullous pemphigoid antigen (BPAG) 1 and BPAG 2. Although oral prednisolone (80 mg daily) rapidly healed the eruption, liver function failed to respond, resulting in the patient's death. This liver dysfunction was attributed to the HBV infection. Use of the polymerase chain reaction showed that HBV in this case had transformed into its precore mutation form during the therapy. Standard tests on first examination did not detect HBV in this patient, but later detailed analysis suggested that she had been an HBV carrier since birth. Such carriers sometimes test negative for HBs antigen, when only a little HBV is present in the liver. Great care should also be exercised when using high doses of corticosteroids to treat bullous diseases in HBV carriers, because these drugs may aggravate HBV infections and cause liver failure.

Bullous pemphigoid associated with internal malignancies: identification of 180-kDa antigen by western immunoblotting.

We report three cases of bullous pemphigoid associated with internal malignancies. Two cases were associated with gastric cancer and one with rectal cancer. Immunoblot analysis, using epidermal extract of normal human skin, revealed that these patients' sera reacted only with bullous pemphigoid antigen with a molecular weight of 180 kDa (BP180). The relationship of BP180 to malignant tumours is discussed.

Drugs Associated With Bullous Pemphigoid

Bullous pemphigoid is the most frequent autoimmune blistering disease. It occurs in the elderly. The cause of this disease is unknown, but cases of bullous pemphigoid have been occasionally attributed to drug therapy. We conducted a multicenter prospective case-control study looking at the drugs used on a long-term basis before the onset of the disease in 116 incident cases of bullous pemphigoid and 216 control patients with malignant or benign skin tumors. Case patients and control patients received many drugs on a long-term basis (mean +/-SD, 4.4+/-3.2 and 4.4+/-2.7 years respectively). Two classes of drugs, neuroleptics and diuretics, were used more frequently by case patients than control patients. Neuroleptics were used by 15.5% of case patients and 8.3% of control patients (adjusted odds ratios; 1.9; 95% confidence interval, 0.95 to 3.8). Diuretics were used by 36.2% of case patients and 24.5% of control patients (adjusted odds ratios, 1.8; 95% confidence interval, 1.1 to 2.9). Among neuroleptics, no specific drug emerged. The association with diuretics was only linked to aldosterone antagonists, which were taken by 12.9% of case patients and 4.6% of control patients (adjusted odds ratios, 3.1; 95% confidence interval, 1.4 to 7.1). These results suggest that some drug therapies may be a risk factor for bullous pemphigoid. The cause of this association should be further investigated.

Chronic herpes gestationis and antiphospholipid antibody syndrome successfully treated with cyclophosphamide

Herpes gestationis shares many features with other bullous diseases, especially bullous pemphigoid. Treatments of benefit in bullous pemphigoid may also be effective for herpes gestationis. Cyclophosphamide, azathioprine, and other immunosuppressive agents have been used successfully in refractory cases of bullous pemphigoid. We describe a patient with severe and persistent herpes gestationis in the postpartum period and the antiphospholipid antibody syndrome, both of which were refractory to high-dose corticosteroid therapy. Treatment with pulsed-dose intravenous cyclophosphamide produced an excellent clinical response.

A case of generalized pustular psoriasis followed by bullous disease: and atypical case of bullous pemphigoid or a novel bullous disease?

We describe a 31-year-old Japanese woman with generalized pustular psoriasis treated with PUVA who subsequently developed a bullous disease. Throughout the disease course, there was no phase of psoriasis vulgaris. Although several reports describe coexistence of psoriasis vulgaris and bullous disease such as bullous periphigoid, coexistence of generalized pustular psoriasis without any phase of psoriasis vulgaris and bullous disease is rare. As for the bullous disease, direct immunofluorescence study showed IgG and C3 deposition along the basement membrane zone. Indirect immunofluorescence disclosed IgG antibasement membrane zone antibodies. Indirect immunofluorescence on 1 mol/l sodium chloride-split skin demonstrated linear IgG staining almost exclusively on the dermal side of the split. Western immunoblot analysis revealed that the antibody was directed to neither epidermolysis bullosa acquisita antigen nor bullous pemphigoid antigens. Considering the unusual clinical course, we suspect the possibility of a novel autoimmune blistering disease.

A case of generalized pustular psoriasis followed by bullous disease: and atypical case of bullous pemphigoid or a novel bullous disease?

A case of generalized pustular psoriasis followed by bullous disease: and atypical case of bullous pemphigoid or a novel bullous disease?

Bullous pemphigoid-a report of 86 cases from Taiwan

We reviewed 86 cases of bullous pemphigoid and the results were compared with those reported in the literature. Seventy-eight per cent of the patients developed generalized blisters and 22% had localized blisters, including three cases of dyshidrosiform pemphigoid and one case of pretibial pemphigoid. Oral mucosal involvement was noticed in 12.8% of the patients. Fifteen per cent of the patients had internal malignancies but the incidence was not significantly different from the control group. Direct immunofluorescence in our series showed a high positive rate of 98.8%. Indirect immunofluorescence was positive in 48.1% of the 54 patients in whom this was carried out. Peripheral blood eosinophilia was observed in 22.1% of the patients. Prednisolone alone or in combination with immunosuppressive agents was the mainstay of treatment. Treatment side-effects was observed in 33% of the patients. Thirty per cent of the patients had a complete remission after a mean follow-up period of 26.9 months. Bullous pemphigoid (BP) is a chronic debilitating autoimmune blistering disease. It is characterized clinically by generalized tense bullae and histologically by subepidermal blisters. Immunofluorescence is crucial in the diagnosis and shows linear deposits of C3 and/or IgG at the basement membrane zone (BMZ). Information regarding this disease in Chinese patients is quite limited. In this study, 86 patients with BP were reviewed. The clinical and histological features, immunofluorescence, modes of therapy and outcome were studied.