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1.
Article | IMSEAR | ID: sea-223158

ABSTRACT

Background: Information on bullous pemphigoid in an Indian context is scarce. Aim: To report clinico-demographic profile, associated comorbidities and prescription pattern of bullous pemphigoid patients in India. Methods: This was a retrospective study, where past records of all bullous pemphigoid patients diagnosed and treated between November 2013 and October 2019 were accessed and analysed. Patients having a compatible clinical presentation with either histopathological and/or direct immunofluorescence evidence of bullous pemphigoid were included. Results: There were 96 bullous pemphigoid patients, with a male: female ratio of 1.6:1. The mean age at diagnosis was 62.5 ± 2.2 years, with mean duration of illness 27.5 ± 4.5 months before presentation. Comorbidities were present in 80 (83%) patients, with type 2 diabetes mellitus (38.5%), hypertension (36.4%) and neurological illness (16.7%) being the commonest ones. Clinically, blisters were the predominant presentation in 81 (84.4%) patients. The majority (87.5%) of patients showed a predominant eosinophilic infiltrate on histopathology. Direct immunofluorescence revealed immunoglobulin G deposits with complement C3 in 77 (80.2%) cases. The majority of patients (77.1%) were treated with oral prednisolone, either alone (11.5%) or in combination (65.6%) with other topical and systemic agents. Topical steroids were used in 29.1%, azathioprine in 28%, dapsone in 16.7% and omalizumab in 6.2% of patients. Limitations: The study is retrospective. Immunofluorescence on salt split skin, direct immunofluorescence serration pattern analysis, and immunoblotting were not performed. Hence, there is a possibility that a few included cases were suffering from other subepidermal autoimmune bullous diseases like epidermolysis bullosa acquisita or anti-p200 pemphigoid. Conclusion: Bullous pemphigoid patients in this study had a younger age of onset and showed male preponderance. Comorbidities like type 2 diabetes, hypertension and neurological disorders were freq

2.
Article | IMSEAR | ID: sea-223132

ABSTRACT

Background: Bullous pemphigoid is the most common subepidermal autoimmune blistering disease. Till now, the reported prognostic factors in bullous pemphigoid vary considerably. Aims: The purpose of this study was to determine the overall survival rate and prognostic factors in bullous pemphigoid. Methods: We conducted a retrospective cohort study on newly diagnosed bullous pemphigoid patients between July 2001 and November 2019 in a referral unit for autoimmune blistering skin diseases in Romania. Results: One hundred forty-eight patients were included in the study. The Kaplan–Meier overall survival rates at 1, 3, 5 and 10 years were respectively 74.2% (95% confidence interval, 67.5–81.6%), 53.4% (45.7–62.2%), 43.6% (35.9–53%) and 31.3% (23.5–41.7%). The median follow-up among survivors was 48 months (interquartile range: 11–150). Ninety (60.8%) patients died during the follow-up period; of them, 38 (42.2%) had active disease at the time of death. Advanced age, neurological diseases, valvular heart disease, malignancies, use of statins, skin infections and extensive cutaneous involvement were linked to poorer outcomes, while the use of topical corticosteroids was associated with increased overall survival. Limitations: This study lacks a control cohort to validate the obtained results. It was conducted in a retrospective manner in a single centre. In addition, indirect immunofluorescence microscopy was not performed in all patients. Conclusion: Beyond ageing and neurological comorbidities, the prognosis of bullous pemphigoid patients was significantly influenced by the presence of skin infections, valvular heart disease, use of statins and extensive cutaneous involvement. Topical corticosteroid treatment was associated with increased survival in these patients

3.
Indian J Pathol Microbiol ; 2023 Mar; 66(1): 70-74
Article | IMSEAR | ID: sea-223388

ABSTRACT

Context: Bullous pemphigoid (BP) and “Pemphigus diseases” (PD) can have overlapping clinical manifestations and accurate distinction is crucial for appropriate management. Aims: The study aimed at analyzing the utility of simple hematological markers of systemic inflammation like neutrophil-to-lymphocyte ratio (NLR), neutrophil-to-eosinophil ratio (NER), and platelet-to-lymphocyte ratio (PLR) in clinical decision making in the setting of clinical differentials of BP and PD in a particular case. Methods: This single-centre based retrospective observational analytical study included adult subjects newly diagnosed to have BP (n=66) or PD (n=53), confirmed with direct immune-fluorescence testing, over a period of six years. Blood counts performed using Coulter™ hematology analyser, at the time of their initial presentation, were retrieved from the hospital medical records, and the leucocyte ratios were calculated.Statistical Analysis: The data were compared between the two groups, using Mann–Whitney U test and chi-square test /Fisher's exact test. ROC curve analysis was performed to estimate cut-off values. Results: The BP group had a significantly higher NLR, total leukocyte counts (TLC), absolute eosinophil counts (AEC), and absolute lymphocyte counts (ALC), and lower NER values compared to the PD group (P < 0.05). Areas under ROC for NLR, NER, TLC, AEC, and ALC were between 0.5 and 0.7. NLR ? 7, AEC ? 2055/cumm, and TLC ? 15,000/cumm had a specificity of 90.6, 100, and 100% respectively for identifying BP patients out of the two groups, but with a low sensitivity of 22.7, 21, and 22.7%, respectively. Conclusions: NLR can be a valuable diagnostic adjunct in subtyping autoimmune bullous disorders, albeit in a small proportion of cases.

4.
Rev. cuba. med. mil ; 52(1)mar. 2023.
Article in Spanish | LILACS-Express | LILACS | ID: biblio-1521984

ABSTRACT

Introducción: El penfigoide ampolloso es una dermatosis vesicular ampollosa autoinmunitaria subepidérmica, asociada a la formación de autoanticuerpos que reconocen autoantígenos en la zona de la membrana basal. El tratamiento inmunomodulador con corticoides es la primera línea en el control de la enfermedad. Objetivo: Presentar el caso clínico de un paciente con diagnóstico de penfigoide ampolloso de origen idiopático. Caso clínico: Paciente masculino de 81 años con lesiones ampollosas dolorosas a la palpación de contenido serohemático, tamaño variado, bordes regulares y base eritematosa a nivel generalizado. La evaluación inicial sugiere diagnóstico de penfigoide ampolloso e infección bacteriana activa de las lesiones en la piel. Se solicitan exámenes de laboratorio e imágenes diagnósticas, se descartan etiologías infecciosas, autoinmunes o neoplásicas asociadas, se inicia tratamiento con corticosteroides intravenosos con adecuada evolución clínica. Finalmente, no se identifican enfermedades asociadas a las lesiones ampollosas del paciente. Conclusión: El penfigoide ampolloso es una entidad poco frecuente, con una elevada tasa de mortalidad si se realiza un diagnóstico y tratamiento tardío. Reconocer las principales manifestaciones y variantes clínicas de esta enfermedad permite un oportuno enfoque diagnóstico y terapéutico, este último basado en el control de la respuesta inflamatoria contra la piel y otros órganos.


Introduction: Bullous pemphigoid is a subepidermal autoimmune bullous vesicular dermatosis associated with the formation of autoantibodies that confirm autoantigens in the basement membrane area. Immunomodulatory treatment with corticosteroids is the first line in the control of the disease. Objective: To present the clinical case of a patient diagnosed with a bullous pemphigoid of idiopathic origin. Clinical case: 81-year-old male patient with blistering lesions that are painful on palpation with serohematic content, varied in size, regular borders and a generalized erythematous base; that the initial evaluation suggests a diagnosis of bullous pemphigoid and active bacterial infection of the skin. Laboratory tests and diagnostic images are requested, infectious, autoimmune or associated neoplastic etiologies are ruled out, treatment with intravenous corticosteroids is started with adequate clinical evolution. Finally, no diseases associated with the patient's blistering lesions were identified. Conclusion: Bullous pemphigoid is a rare entity, with a high mortality rate if a late diagnosis and treatment is performed. Recognizing the main manifestations and clinical variants of this disease allows for a timely diagnostic and therapeutic approach, the latter based on the control of the inflammatory response against the skin and other organs.

5.
An. bras. dermatol ; 98(1): 13-16, Jan.-Feb. 2023. tab, graf
Article in English | LILACS-Express | LILACS | ID: biblio-1429641

ABSTRACT

Abstract Background Significant association between bullous pemphigoid (BP) and stroke has been reported. This study aimed to evaluate the level of anti-BP180 antibody in stroke patients to explore the relationship between BP and stroke in their pathogenesis. Methods We collected serum samples from stroke patients and matched controls between February 2019 and June 2020. The anti-BP180 antibody levels were measured by enzyme-linked immunosorbent assay (ELISA). Results A total of 1183 stroke patients including 970 with cerebral infarction (CI), 192 with intracerebral hemorrhage (ICH), 21 with CI and ICH, and 855 controls were enrolled in this study. Anti-BP180 autoantibody values were significantly higher in stroke patients than in controls (p < 0.001). Anti-BP180 autoantibody-positive rates were 12.51% (148) in stroke patients and 4.68% (40) in controls (p < 0.001, OR = 2.65). In anti-BP180 autoantibody-positive subjects, the values were significantly higher in stroke patients than in controls (p < 0.001). However, only 10 (6.76%) stroke patients and 3 (7.5%) controls had high values (> 100 RU/mL) (p = 0.87). Stratified analysis showed that anti-BP180 antibody positive rates were independent of age, sex, and stroke subtypes in the stroke group. Positive rates in patients with both CI and ICH were nearly two times higher than those in patients with either CI or ICH alone (p = 0.11, OR = 1.94). Study limitations This study had a limited sample size and lacked quantitative criteria for stroke severity. Conclusions Anti-BP180 antibody values and positive rates were higher in stroke patients than in controls, suggesting that stroke patients may have higher of developing BP.

6.
Article | IMSEAR | ID: sea-222232

ABSTRACT

Scabies is an ectoparasitic infection caused by Sarcoptes scabiei var. hominis in humans. Accurate figures of the prevalence of scabies are difficult to obtain, it seems to affect around 100�0 million people worldwide. Scabies is usually transmitted by close intimate physical contacts, such as the sharing of a bed or prolonged hand-holding. Scabies mites survive for 24� h outside of human bodies. Itching is the most obvious manifestation of scabies, which is usually sparing the face in adult classic scabies. The onset occurs 3�weeks after acquiring the infection. Bullous pemphigoid-like scabies is one of the atypical and rare forms of scabies that may be overlooked and mismanaged, where only 44 cases worldwide with this mode of presentation were reported. Here, we reported a series of three cases of bullous pemphigoid-like scabies, which presented with nocturnal itching and vesiculobullous lesions that are to the best of our knowledge the first cases reported in our country.

7.
Chinese Journal of Dermatology ; (12): 156-158, 2022.
Article in Chinese | WPRIM | ID: wpr-933516

ABSTRACT

A 58-year-old female patient presented with painful and itchy skin lesions on the head, neck, chest and back for 20 days, and was admitted to the hospital in February 2010. The skin lesions manifested as superficial erosions on an erythematous base with positive Nikolsky′s sign. Histopathological examination showed fissures above the granular and spinous cell layers, and scattered dyskeratotic cells. Direct immunofluorescence (DIF) assay revealed IgG and C3 deposits between spinous cells. The patient was initially diagnosed with pemphigus foliaceus. After the treatment with triamcinolone (24 mg/d) , tripterygium glycosides (60 mg/d) , nicotinamide (300 mg/d) and tetracycline (2 g/d) , skin lesions gradually subsided. In June 2017, the patient was readmitted due to itchy skin lesions on the head, neck, chest, back and hands for 15 days, which manifested as tense bullae on an erythematous base with negative Nikolsky′s sign. Histopathological examination showed subepidermal blisters and infiltration of eosinophils and lymphocytes in the blisters and superficial dermis, DIF assay revealed the deposition of IgG and C3 along the basement membrane zone, and enzyme-linked immunosorbent assay showed positive results for anti-BP180 antibody but negative results for anti-Dsg1 and Dsg3 antibodies. Then, a diagnosis of bullous pemphigoid was confirmed. The patient was treated with oral triamcinolone (12 mg/d) , nicotinamide (300 mg/d) and tetracycline (2 g/d) , and the lesions rapidly subsided.

8.
Article | IMSEAR | ID: sea-212482

ABSTRACT

The unique thing about this case is that it is the first reported case of bullous pemphigoid in the elderly that has been clinically diagnosed with histologic findings highly suggestive of this blistering disorder although an immunofluorescence could not be done due to unavailability in the centre. Patient was managed successfully and discharged home with improvement on subsequent hospital visits. This case report shows how diagnosis of an immunobullous disease was made and managed in a resource poor setting. A descriptive summary of 65-year old black Nigerian woman with bullous pemphigoid covering history, physical examination and management. The main diagnosis was bullous pemphigoid in a recently diagnosed type 2 diabetic. Patient was placed on corticosteroids as well as immunosuppressive agents and diabetes was managed with subcutaneous insulin and oral hypoglycemics with appropriate wound care. Patient showed remarkable improvement after management and was discharged home with subsequent follow up in the clinics. Bullous Pemphigoid (BP) can be diagnosed clinically with a high index of suspicion with the aid of skin biopsy and histology, and can be managed successfully even in a resource poor centre where immunofluorescence facilities are lacking.

9.
Malaysian Journal of Medicine and Health Sciences ; : 127-130, 2020.
Article in English | WPRIM | ID: wpr-876828

ABSTRACT

@#Bullous pemphigoid (BP) is a chronic and the most frequent immune-mediated subepidermal blistering disorder which mainly affects elderly individuals. The autoantibodies produced following T-cell dysregulation are directed against BP180 (BPAg2) and BP230 (BPAg1), hemidesmosomal proteins located in the basement membrane zone (BMZ) of the epidermis. BP may present with polymorphic dermatological features including non-bullous manifestations and blisters. Therefore, a wide range of differential diagnoses such as eczema, urticaria, pemphigus and the differentials for subepidermal blister with eosinophils such as epidermolysis bullosa acquisita (EBA) and bullous drug eruptions should be considered in such cases. The associations of solid organ internal malignancies and BP are quite rare and vary between studies. Here, we present a case of paraneoplastic bullous pemphigoid (PNBP) in a patient with underlying renal cell carcinoma who was initially diagnosed with worsening hand-foot syndrome (HFS) which has led to withdrawal of his oral chemotherapy treatment.

10.
The Malaysian Journal of Pathology ; : 445-448, 2020.
Article in English | WPRIM | ID: wpr-876039

ABSTRACT

@#The author reports two cases of Bullous pemphigoid (BP) with neurofibroma (NF)-like histopathological change. The two patients without neurofibromatosis type 1 (NF1) presented with several bullae on their trunk. Based on the results of positivity for anti-BP180 antibody, direct immunofluorescence, and histopathological findings, they were diagnosed with BP. Histologically, another lesion in the dermis, which was composed of spindle cells with wavy nuclei, collagen fibers, and mast cells, was located close to the bulla. Immunohistochemically, the spindle cells were diffusely positive for S-100 protein and CD34, and weakly positive for epithelial membrane antigen in certain foci. These findings were considered to be “NF-like” histopathological change. This is the first two cases of BP with NF-like histopathological change in patients without NF1.

11.
Article | IMSEAR | ID: sea-200224

ABSTRACT

Bullous pemphigoid is an acquired autoimmune disease characterized by subepidermal vesicles and bullae. The etiology is mostly idiopathic with the highest occurrence in elderly patients. However, it is now well-accepted that bp has been triggered by or associated with drug therapy. Over 50 agents have been implicated as a cause of Drug-induced bullous pemphigoid, including diuretics, ace inhibitors, and antibiotics. We present a case of Bullous pemphigoid in a 75 year old male probably induced by furosemide. A 75 year old male was admitted to the dermatology department of KIMS hospital, Bengaluru. Presented with multiple tense bullae and vesicles over both upper limbs, forearm and few collapsed bullae and vesicles over the extensor aspect of forearm. Patient had a past history of myocardial infarction and undergone coronary artery bypass grafting for the same and treated with multiple medications. Among the treatment given injection furosemide was the one of the drug, after which he developed lesions and also presented with fluid filled bullae. A diagnosis of bullous pemphigoid was made based on clinical history and was treated with prednisolone, halobetasol and antibiotics. The lesions improved significantly with the above management and patient recovered enough to be discharged from the hospital after 5 days. Severe and serious reactions such as bullous pemphigoid can be caused by used drugs like furosemide.

12.
Rev. chil. dermatol ; 35(2): 72-75, 2019. ilus
Article in English | LILACS | ID: biblio-1103486

ABSTRACT

El penfigoide ampollar en niños es extremadamen-te infrecuente. La mayoría de los casos ocurre en adultos mayores, resultando fundamental conside-rar que en medicina todo es posible y esta enferme-dad igualmente podría presentarse en niños, por lo que se debe prestar especial atención a sus manifes-taciones clínicas, realizar los exámenes apropiados para descartar diagnósticos diferenciales y de esta forma, iniciar un tratamiento eficaz en el momen-to preciso. A continuación, presentamos un caso clínico de penfigoide ampollar que ocurrió en un paciente pediátrico atendido en nuestro hospital universitario.


Bullous pemphigoid in children is extremely in-frequent. Most of them predominate in elderly persons, resulting fundamental to consider that in medicine everything could be possible, and this disease could occur in children, so we must pay special attention to its clinical manifestations, to take the appropriate exams to rule out differen-tial diagnoses and in this way perform an effective treatment at the precise moment. Following, we present a clinical case of bullous pemphigoid that occurred in a pediatric patient attended at our university hospital.


Subject(s)
Humans , Female , Child , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Prednisone/therapeutic use , Diagnosis, Differential
13.
Korean Journal of Dermatology ; : 569-570, 2019.
Article in English | WPRIM | ID: wpr-786268

ABSTRACT

No abstract available.


Subject(s)
Pemphigoid, Bullous , Pemphigus
14.
Chinese Journal of Integrated Traditional and Western Medicine in Intensive and Critical Care ; (6): 125-126, 2019.
Article in Chinese | WPRIM | ID: wpr-754518

ABSTRACT

Bullous pemphigoid is a chronic obstinate dermatological disease. Hormone is the main therapeutic method, but the disease course is rather long, relapse is frequently seen and difficult to be radically cured; many complications may occur such as pulmonary infection, etc. From traditional Chinese medicine (TCM) basic principal points of view "lung governing skin and hair" and "strengthen the body resistance to eliminate pathogenic factors", the author explored the TCM therapy of bullous pemphigoid. By using clearing away lung heat and invigorating lung qi as the main principles supplemented by invigorating spleen and kidney, eliminating phlegm and blood stasis for treatment of such disease, relatively satisfactory therapeutic results were obtained.

15.
Acta Academiae Medicinae Sinicae ; (6): 685-689, 2019.
Article in Chinese | WPRIM | ID: wpr-775975

ABSTRACT

Bullous pemphigoid(BP)is an autoimmune blister disease caused by antibodies to the basement membrane zone.It usually exhibits tense bullae in skin and mucous membrane.BP patients can suffer from thrombosis due to hypercoagulation and/or hemorrhage due to the presence of anti-coagulation factors,which may be explained by the eosinophils,antibodies to the coagulation factors,and fibrinolytic system hyperactivity or inhibition.


Subject(s)
Humans , Autoantibodies , Blister , Blood Coagulation Disorders , Blood Coagulation Factors , Eosinophils , Hemorrhage , Mucous Membrane , Pathology , Pemphigoid, Bullous , Skin , Pathology , Thrombosis
16.
Geriatr., Gerontol. Aging (Online) ; 12(2): 102-104, abr.-jun.2018.
Article in English | LILACS | ID: biblio-914973

ABSTRACT

OBJECTIVES: This study aims to analyze data on the epidemiology, treatment and course of bullous pemphigoid in 50 patients and compare findings to the data already available in the literature. METHODS: Data were collected retrospectively through medical records and analyzed statistically. A review of the literature was conducted using articles indexed in the MEDLINE (via PubMed) database. RESULTS: The mean age at diagnosis was 71.1 years. Comorbidities were observed in almost all cases, and the association between bullous pemphigoid and neurological diseases was present in 18% of patients, in agreement with recent data in the literature. CONCLUSION: Care of comorbidities, especially neurological diseases, which increase the mortality of patients with bullous pemphigoid, is thus essential


OBJETIVOS: Este estudo tem por objetivo analisar dados epidemiológicos, de tratamento e evolução de 50 pacientes com diagnóstico de penfigoide bolhoso e comparar aos dados já existentes na literatura. MÉTODOS: Os dados foram coletados retrospectivamente por meio de prontuários médicos e analisados estatisticamente. Foi realizada revisão da literatura mediante artigos indexados na base de dados MEDLINE (via PubMed). RESULTADOS: A média de idade ao diagnóstico foi de 71,1 anos. Comorbidades foram observadas em quase a totalidade dos casos, e a associação entre penfigoide bolhoso e doenças neurológicas esteve presente em 18% dos pacientes, em concordância com dados recentes da literatura. CONCLUSÃO: Ressaltamos a atenção às comorbidades, sobretudo doenças neurológicas, que aumentam a mortalidade dos pacientes com penfigóide bolhoso


Subject(s)
Humans , Male , Female , Adult , Middle Aged , Aged , Young Adult , Pemphigoid, Bullous/epidemiology , Comorbidity , Retrospective Studies , Pemphigoid, Bullous/drug therapy
17.
Annals of Dermatology ; : 468-472, 2018.
Article in English | WPRIM | ID: wpr-716496

ABSTRACT

Patients with chronic lymphocytic leukemia (CLL) rarely exhibit an exaggerated insect bite-like reaction without a history of an arthropod bite. We report a case of an insect bite-like reaction in a 74-year old man with CLL. The patient presented with a 2-year history of recurrent itchy erythematous patches and blisters on the whole body. He had been diagnosed with CLL 2 years ago, and the skin lesions developed 1 month after remission. The result of a skin biopsy was consistent with insect bite. Immunohistochemical staining of the infiltrated cells showed positive reactions for CD3, CD5 and negative for CD20, CD23. Direct and indirect immunofluorescence revealed negative results. The patient was treated with oral prednisolone and dapsone, under the diagnosis of CLL-associated insect bite-like reaction, and showed marked improvement. Dermatologist should be aware of insect bite-like reaction associated with CLL as a distinct disease entity that is similar to insect bite or bullous pemphigoid.


Subject(s)
Humans , Arthropods , Biopsy , Blister , Dapsone , Diagnosis , Fluorescent Antibody Technique, Indirect , Insect Bites and Stings , Insecta , Leukemia, Lymphocytic, Chronic, B-Cell , Pemphigoid, Bullous , Prednisolone , Skin
18.
Annals of Dermatology ; : 544-549, 2018.
Article in English | WPRIM | ID: wpr-717770

ABSTRACT

BACKGROUND: Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by tissue-bound and circulating autoantibodies directed against BP180 and/or BP230 antigens. Various inflammatory cells are involved in the development of blister in BP. OBJECTIVE: The aim of this study was to evaluate the correlation between peripheral leukocyte counts and BP severity. METHODS: We retrospectively included 60 patients with BP, who had not been treated with systemic steroid at the time of blood sampling. The patients were classified into two groups, those with admission history (admission group) and those without admission history (non-admission group). Disease severity was evaluated using three parameters: admission history, initial steroid dosage, and modified version of a pemphigus scoring system. We evaluated the correlation between peripheral leukocyte counts and disease severity measured by the three parameters. RESULTS: The admission group showed a significant increase in disease severity measured by initial steroid dosage and severity score compared with the non-admission group. Additionally, the admission group had increased total leukocyte, eosinophil, and neutrophil counts. In the correlation study, the peripheral eosinophil and neutrophil counts showed positive correlation with BP severity evaluated by both initial steroid dosage and the pemphigus scoring system. CONCLUSION: Peripheral eosinophil and neutrophil counts can be used as a marker in predicting disease severity in patients with BP.


Subject(s)
Humans , Autoantibodies , Blister , Eosinophils , Leukocyte Count , Leukocytes , Neutrophils , Pemphigoid, Bullous , Pemphigus , Retrospective Studies , Statistics as Topic
19.
Rev. chil. dermatol ; 34(1): 32-35, 2018. ilus
Article in Spanish | LILACS | ID: biblio-965818

ABSTRACT

El Penfigoide nodular es una variante clínica poco frecuente de penfigoide buloso. Corresponde a una dermatosis ampollar subepidérmica, crónica, autoinmune, caracterizada por auto anticuerpos contra antígenos específicos de hemidesmosomas en la unión dermo-epidérmica. Su incidencia es desconocida. La etiopatogenia aún no es entendida del todo. Se presenta clínicamente como una superposición de características de pénfigo buloso y prurigo nodular. El diagnóstico se basa en hallazgos clínicos e inmunopatológicos. La histopatología con inmunofluorescencia directa es el gold standard para el diagnóstico. El manejo es difícil, tiene mala respuesta a corticoides potentes locales, siendo necesario el uso de corticoides sistémicos y diferentes inmunosupresores solos o combinados junto a antihistamínicos para el manejo de prurito intenso. Se presenta un caso de pénfigo nodular, donde destaca su buena respuesta a terapia combinada con metotrexato y luz UVB de banda angosta.


Pemphigoid Nodularis is a rare clinical variant of bullous pemphigoid. It is considered an autoimmune, chronic, subepidermal blistering dermatosis, characterized by antibodies against hemidesmosome-specific antigens at the dermo-epidermal junction. Its incidence is unknown and its etiopathogenetic not fully understood. Clinically, it presents with overlapping features of bullous pemphigoid and prurigo nodularis. The diagnosis is based on clinical and immunopathological findings, being the histopathological study with immunofluorescence the gold standard. The management is difficult; since it has a poor response to local potent corticosteroids, requiring the use of systemic corticosteroids and different immunosuppressants alone or combined with antihistamines for the intense pruritus. We present a case of nodularis pemphigoid, highlighting the good response to the combination of methotrexate and phototherapy with narrow band UVB.


Subject(s)
Humans , Female , Aged , Ultraviolet Therapy/methods , Pemphigoid, Bullous/therapy , Biopsy , Enzyme-Linked Immunosorbent Assay , Methotrexate/therapeutic use , Pemphigoid, Bullous/pathology , Combined Modality Therapy
20.
Korean Journal of Dermatology ; : 120-123, 2017.
Article in Korean | WPRIM | ID: wpr-208037

ABSTRACT

Bullous pemphigoid is a rare nonhematologic autoimmune complication of chronic lymphocytic leukemia. There have been roughly 10 cases worldwide since 1974, and searches through Korean dermatological literature revealed no reported cases. A 72-year-old man had been diagnosed with chronic lymphocytic leukemia and treated with chemotherapy for 7 months. After that, he was in complete remission, and the chemotherapy was discontinued consequently. One month later, multiple erythematous blisters, papules, and crusts developed on his trunk and both extremities. Histopathologic examination and immunofluorescence of the tense blister revealed a bullous pemphigoid. We present this rare case as the first documented case report of bullous pemphigoid following chronic lymphocytic leukemia in the Korean dermatological literature.


Subject(s)
Aged , Humans , Blister , Drug Therapy , Extremities , Fluorescent Antibody Technique , Leukemia, Lymphocytic, Chronic, B-Cell , Pemphigoid, Bullous , Transcutaneous Electric Nerve Stimulation
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