Bullous pemphigoid associated with milia, increased serum IgE, autoantibodies against desmogleins, and refractory treatment in a young patient

Abstract: Bullous pemphigoid is a blistering autoimmune disease characterized by two hemidesmosomal proteins (anti-BP180 and 230). Pemphigus, by contrast, is characterized by two autoantibodies (anti-desmoglein 1 and 3). Coexistence of autoantibodies of bullous pemphigoid and pemphigus in a patient is rare. A 25-year-old male patient was admitted to our hospital, reporting a 3-month history of multiple papules, vesicles, and erosions over an extensive erythema on the entire body. Laboratory tests showed high levels of serum IgE, anti-BP180 antibodies, and anti-desmoglein 1 and 3. Histopathologic and immunopathologic features were characterized by bullous pemphigoid. No improvement was seen with systemic corticosteroid therapy, however, pulse corticosteriod therapy combined with methylprednisolone, immunosuppressants, immunomodulators, and plasmapheresis led to the recovery of his condition with numerous milia.

Association between HLA-DQB1[asterisk]03:01 and Bullous pemphigoid in Iranian patients

A common Human Leukocyte Antigen [HLA] class II allele, DQ beta 1[asterisk]03:01, seems to be associated with Bullous pemphigoid [BP] in Caucasians whereas previous studies in other ethnic groups showed other HLA class II alleles as genetic predisposing factors for BP. To investigate the association of HLA class II alleles and haplotypes with BP in Iranian population. Methods: Fifty patients with Bullous pemphigoid and 180 geographically matched, healthy individuals as control group enrolled into this study. HLA typing of class II [DR and DQ alleles] was carried out using polymerase chain reaction based on sequence-specific primers method. Class II DQA1 and DQB1 typing showed a significantly higher frequency of HLA-DQA1[asterisk]05:01 [45% vs. 33%, p=0.03], HLA-DQB1[asterisk]03:01 [36% vs. 23.6%, p=0.02] and HLA-DQB1[asterisk]04:01 [4% vs. 1.6%, p=0.04] in the BP patients compared with controls. For DRB1 allele frequencies, there were no significant disease associations. The frequency of DRB1[asterisk]08:01/DQA1[asterisk]05:01/DQB1[asterisk]03:01 [3% vs. 0%, p=0.02] haplotype showed an increase among patients compared with controls. Our data suggest that Iranian patients with BP present the same genetic predisposition linked to HLA-DQB1[asterisk]03:01 previously reported in Caucasians

Pemphigoid gestationis: clinical and laboratory evaluation

INTRODUCTION: Pemphigoid gestationis, also known as herpes gestationis, is a rare autoimmune blistering disease associated with pregnancy. It usually occurs during the second or third trimester, but it may be present at any stage of pregnancy or the puerperium. The clinical, histologic, and immunopathological features of pemphigoid gestationis are similar to those of the pemphigoid group of disorders. METHODS: We hereby report seven patients who were diagnosed with pemphigoid gestationis and followed at the Autoimmune Blistering Disease Clinic in the Department of Dermatology of the University of Sao Paulo Medical School between 1996 and 2008. DISCUSSION: Demographic and clinical findings, such as median age, sites of involvement, and gestational age of onset of our patients, coincide with those described in previous reports. The majority of patients (85 percent) exhibited complement C3 or C3 and immunoglobulin G (IgG) deposition along the basement membrane zone (BMZ) on immunofluorescence. Herpes gestationis factor (HG) factor was postitive in four out of six patients (67 percent), and three out of five patients recognized the bullous pemphigoid recombinant antigen (BP180) by ELISA. CONCLUSION: This study revealed a good outcome of the newborns from pemphigoid gestationis affected mothers, based on the absence of pemphigoid gestationis cutaneous lesions, mean birth weight, and normal Apgar scores and gestational age at birth.

Dermatoses bolhosas auto-imunes / Autoimmune bullous dermatoses

Dermatoses bolhosas autoimunes são doenças cuja manifestação cutânea primária e fundamental consiste em vesículas e bolhas. Classificam-se conforme a localização da bolha, em intraepidérmica e subepidérmica. Os pacientes produzem autoanticorpos contra estruturas específicas da pele detectáveis por técnicas de imunofluorescência, immunobloting e Elisa. Os recentes avanços da biologia molecular e celular têm permitido conhecer esses autoantígenos, contra os quais os pacientes se sensibilizam e que estão localizados na epiderme ou na junção dermoepidérmica. São doenças de baixa incidência, porém de elevada morbidade e por vezes letais. O objetivo deste trabalho é revisar e descrever os progressos nos conhecimentos de quatro doenças vésico-bolhosas autoimunes: pênfigo foliáceo endêmico (fogo selvagem), pênfigo vulgar, penfigóide bolhoso e dermatite herpetiforme.

Autoimmune bullous dermatoses are diseases in which blisters and vesicles are the primary and fundamental types of skin lesion. Their classification is based on the location of the blister: intraepidermal and subepidermal. Patients produce autoantibodies against self-specific structures of the skin detectable by immunofluorescence techniques, immunoblotting and ELISA. Recent advances in molecular and cellular biology have brought to knowledge these self-antigens, against which patients are sensitized, and which are found in epidermis or in the dermo-epidermal junction. These are low incidence, but high morbidity diseases that may be fatal. The aim of this article is to review and describe the progress of four autoimmune vesiculobullous disorders: endemic pemphigus foliaceous (wild fire), pemphigus vulgaris, bullous pemphigoid and dermatitis herpetiformis.

Inmunofluorescencia de las enfermedades ampollas autoinmunes / inmunofluorescence in bullous autoimmune diseases

Las enfermedades ampollosas adquiridas autoinmunes representan un grupo heterogéneo de entidades caracterizadas por la formación de ampollas producidas por alteraciones inmunológicas. Debido al gran interés e importancia de estas enfermedades, es muy necesario conocer las técnicas de inmunofluorescenca así como su interpretación para su clasificación y diagnóstico. El propósito de esta revisión es recordar algunos de los signos clínicos, hallazgos histológicos y de inmunofluorescencia de las enfermedades ampollosas autoinmunes

Avances en la estructura y función de la membrana basal / Advances in basement membrane structure and function

La zona de membrana basal es una estructura compleja. Está constituída por la membrana plasmática de la célula basal, lámina lúcida, lámina densa y región de anclaje; cada una de ellas posee diversos componentes moleculares. Sus principales funciones biológicas son actuar como soporte y barrera mecánica, permite la fijación dermoepidérmica, regular la filtración y permeabilidad y guiar la regeneración tisular

Penfigoide cicatrizal localizado (variedad Brunsting-Perry) / Localized cicatricial pemphigoid (Brunsting-Perry)

Se presenta un paciente de sexo masculino de 74 años con diagnóstico clínico e inmunopatológico de penfigoide cicatrizal localizado, cuyas lesiones excedieron a la localización clásicamente descripta (nuca y dorso a nivel de la cintura escapular). Fue tratado inicialmente con sulfonas y luego con deflazacort, manteniéndose en la actualidad libre de lesiones. Se revisan las características clínicas, patológicas e inmunológicas de la entidad

Imunidade celular em pênfigo folíaceo Sul-Americano / Cellular immunity. Pemphigus foliaceus in South America

No presente trabalho é avaliada a imune reposta celular "in vivo" e "in vitro" em trinta pacientes com Pênfigo foliáceo Sul-americano, que foram realizadas com auxílio dos testes de sensibilidade cutânea ao PPD, tricofitina, oidiomicina, TBK, extrato total de pele humana e fitohemaglutinina pura; das provas de inibiçäo de migraçäo dos leucócitos com extrato total de pele humana penfigosa e normal e da avaliaçäo quantitativa dos linfócitos no sangue periférico e da análise das áreas timo-dependentes e independestes em cortes histológicos de linfonodos

Epidermolise bolhosa adquirida (Penfigoide dermolitico) / Acquired epidermolysis bullosa (dermolytic pemphigoid)

E estudado um caso de epidermolise bolhosa adquirida (Penfigoide bolhoso) em um homem adulto com insucesso terapeutico. E feita uma revisao da entidade e estudadas as relacoes com o penfigoide bolhoso o e penfigoide cicatricial. Discute-se a autonomia da entidade e destaca-se, sobretudo, como de valor diagnostico o nivel da clivagem-sublamina densa

Penfigoide ampollar y artritis reumatoidea / Bullous penphigoid and rheumatoid arthritis

La asociación de artritis reumatoidea con pemfigoide ampollar es muy infrecuente. Reportamos el caso de un paciente portador de A.R. que, luego de 30 años de evolución, se le asocian lesiones de penfigoide ampollar. Este nuevo aporte de la literatura es el primero en la Argentina y tratamos de demostrar que no es una asociación fortuita sino que es una verdadera conjunción de dos fenómenos autoimmunes