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 TherapyABSTRACT
Abstract: Dyshidrosiform pemphigoid is an acquired autoimmune variant of bullous pemphigoid with persistent vesicobullous eruptions localized on the palms or soles, or both. It generally occurs in the elderly and is rarely reported in childhood. Hereby, we describe the first case of dyshidrosiform pemphigoid in a 12-year-old child, which was limited to the dorsal hands and treated successfully with dapsone (diaminodiphenyl sulfone). Along with this report, we also review the clinical features of various types of dyshidrosiform pemphigoid.
Subject(s)
Humans , Male , Child , Pemphigoid, Bullous/diagnosis , Hand , Hand Dermatoses/diagnosis , Pemphigoid, Bullous/pathology , Pemphigoid, Bullous/drug therapy , Dapsone/therapeutic use , Hand Dermatoses/pathology , Hand Dermatoses/drug therapy , Anti-Infective Agents/therapeutic useABSTRACT
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.
Subject(s)
Humans , Male , Adult , Immunoglobulin E/blood , Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/pathology , Desmogleins/immunology , Keratosis/immunology , Keratosis/pathology , Skin/pathology , Autoantibodies/blood , Autoantigens/blood , Biopsy , Methylprednisolone/therapeutic use , Pemphigoid, Bullous/drug therapy , Non-Fibrillar Collagens/blood , Pressure Ulcer/pathology , Glucocorticoids/therapeutic use , Keratosis/drug therapyABSTRACT
El pénfigo herpetiforme es una variante clínica infrecuente del pénfigo, que presenta similitud clínica con la dermatitis herpetiforme, con presencia histológica de acantólisis y de ampolla intrapidermica. Se presenta el caso de un paciente de 61 años, con lesiones vesiculares pruriginosas, de 3 a 8 mm un, de conformación arciforme y herpetiforme, localizadas en el tórax y las extremidades. La histopatología y la inmunofluorescencia directa fueron compatibles con pénfigo. El paciente tuvo mejoría luego del tratamiento con inmunoglobulina intravenosa.
The pemphigus herpetiformis is a rare clinical variant of pemphigus, which presents clinical similarity with dermatitis herpetiformis, but with the histological presence of acantholysis and intraepidermal blister. We present the case of a 61 year old male patient with pruritic vesicular lesions of 3-8 mm of acute and herpetiform configuration over his thorax and extremities. The histopathology and direct immunofluorescence were consistent with pemphigus. The patient had improvement after treatment with intravenous immunoglobulin.
Subject(s)
Humans , Male , Aged , Pemphigoid, Bullous , Pemphigoid, Bullous/pathologyABSTRACT
Bullous pemphigoid is an autoimmune subepidermal blistering dermatosis that is uncommon in childhood. We report a case of a female infant, 3 months old, which presented clinical and laboratory data for the confirmatory diagnosis of bullous pemphigoid. The authors used immunohistochemical staining for collagen type IV that allowed the differentiation of bullous pemphigoid from other subepidermal bullous diseases. Opportunely we review the clinical, immunological, therapeutic and prognostic features of this pathology in children.
O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, incomum na infância. Relatamos um caso de lactente feminina, com 3 meses de idade, que apresentou dados clínicos e laboratoriais confirmatórios para o diagnóstico de penfigoide bolhoso. Os autores utilizaram a coloração de imuno-histoquímica para o colágeno tipo IV que permitiu a diferenciação do penfigoide bolhoso de outras buloses subepidérmicas. Oportunamente, revisamos as características clínicas, imunológicas, terapêuticas e prognósticas da patologia na criança.
Subject(s)
Female , Humans , Infant , Pemphigoid, Bullous/pathology , Blister/drug therapy , Blister/pathology , Collagen Type I/analysis , Diagnosis, Differential , Immunohistochemistry , Pemphigoid, Bullous/drug therapy , Skin/pathology , Treatment OutcomeABSTRACT
El penfigoide ampollar (PA) es una enfermedad infrecuente, de curso crónico y benigno, que aparece en personas de edad avanzada y se caracteriza por la presencia de ampollas subepidérmicas.En términos generales, el diagnóstico de las enfermedades ampollares se basa en las manifestaciones clínicas, los hallazgos histopatológicos y la inmunofluorescencia directa. Si bien la ausencia de alguno de estos métodos puede dificultar el mismo, una adecuada correlación clínico-patológica permite, en la mayoría de los casos, arribar al diagnóstico y realizar el tratamiento apropiado. El PA puede ser causado por fármacos y produce cuadros clínicos similares al PA idiopático. A continuación se presentan dos casos con diagnóstico de penfigoide ampollar por fármacos.
Bullous Pemphigoid is a chronic, infrequent benign disease of the elderly, characterized by the presenceof subepidermal bullae. Diagnosis is based on clinical, histopathological and direct immunofluorescencefindings. Though the absence of any of them hampers the diagnosis, a correct clinico-pathologic correlation is necessary to make the appropriate treatment. Drug induced-BullousPemphigoid presents with identical clinical features as those of the Idiopathic Bullous Pemphigoid.We present two patients with drug-induced Bullous Pemphigoid.
Subject(s)
Humans , Male , Aged , Drug Hypersensitivity/etiology , Pemphigoid, Bullous/chemically induced , Pemphigoid, Bullous/pathology , Ampicillin/adverse effects , Omeprazole/adverse effects , Skin/pathology , Sulbactam/adverse effectsABSTRACT
O penfigoide bolhoso é uma dermatose bolhosa autoimune subepidérmica, mais comumente observada na população idosa (acima dos 70 anos). Autoanticorpos são formados contra antígenos específicos da zona de membrana basal: BP180 e BP230 (proteínas do hemidesmossomo). Apresentamos três casos de penfigoide bolhoso, em adultos com menos de 50 anos de idade, destacan do as peculiaridades clínicas na faixa etária mais jovem.
Bullous pemphigoid is an autoimmune subepidermal bullous dermatosis more commonly observed in the elderly (over 70 years old). Autoantibodies are produced for specific antigens of the epidermal basement membrane zone: BP 180 and BP 230 (hemidesmosome proteins). We report three cases of bullous pemphigoid in adults younger than 50 years old, discussing the clinical characteristics of the disease in younger patients.
Subject(s)
Adult , Female , Humans , Middle Aged , Pemphigoid, Bullous/pathology , Age Factors , Dapsone/therapeutic use , Pemphigoid, Bullous/drug therapy , Prednisone/therapeutic useABSTRACT
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.
Subject(s)
Humans , Autoimmune Diseases/immunology , Autoimmune Diseases/pathology , Skin Diseases, Vesiculobullous/immunology , Skin Diseases, Vesiculobullous/pathology , Autoantibodies/immunology , Autoantigens/immunology , Autoimmune Diseases/therapy , Dermatitis Herpetiformis/immunology , Dermatitis Herpetiformis/pathology , Dermatitis Herpetiformis/therapy , Enzyme-Linked Immunosorbent Assay , Fluorescent Antibody Technique , Immunoblotting , Pemphigoid, Bullous/immunology , Pemphigoid, Bullous/pathology , Pemphigoid, Bullous/therapy , Pemphigus/immunology , Pemphigus/pathology , Pemphigus/therapy , Skin Diseases, Vesiculobullous/therapyABSTRACT
Apresenta-se caso de epidermólise bolhosa adquirida inflamatória. Paciente do sexo masculino, 53 anos, há seis meses com erupção vesicobolhosa pruriginosa sobre base eritematosa no tronco, axilas e membros. O exame anatomopatológico mostrou bolha subepidérmica com neutrófilos. A imunofluorescência direta revelou depósitos lineares de IgG, IgA, IgM e C3 na zona da membrana basal, sendo a imunofluorescência indireta e o Salt Split Skin indireto negativos. Anticorpos antinucleares não reagentes. Houve melhora do quadro com prednisona e cicatrização de algumas lesões com formação de milia. Trata-se de apresentação rara de epidermólise bolhosa adquirida, com lesões iniciais predominantemente inflamatórias.
We report a case of an inflammatory variant of epidermolysis bullosa acquisita in a 53-year-old male, with itching blistering eruption on the trunk, armpits and limbs for six months. The skin biopsy specimen showed subepidermal blister with neutrophils. Direct immunofluorescence revealed linear depositions of IgG, IgA, IgM and C3 at the basement membrane; indirect immunofluorescence and salt Split Skin were negative. Antinuclear antibodies were also negative. Improvement of the blisters followed treatment with systemic corticotherapy and some lesions healed with milia. This is a rare presentation of epidermolysis bullosa acquisita, with inflammatory lesions at first.
Subject(s)
Humans , Male , Middle Aged , Epidermolysis Bullosa Acquisita/pathology , Pemphigoid, Bullous/pathology , Skin/pathology , Anti-Inflammatory Agents/therapeutic use , Biopsy , Diagnosis, Differential , Epidermolysis Bullosa Acquisita/drug therapy , Pemphigoid, Bullous/drug therapy , Prednisone/therapeutic useABSTRACT
Os autores fazem uma revisäo da literatura sobre essa entidade e relatam 3 casos ocorridos no HU-UFJF no período de 1992 e 1998 colocando em evidência as principais características clínicas encontradas.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , Epidermolysis Bullosa/pathology , Brazil , Dermatitis Herpetiformis/pathology , Epidermolysis Bullosa Acquisita/pathology , Pemphigoid, Bullous/pathology , Pemphigus/pathology , Porphyria Cutanea Tarda/pathology , Retrospective StudiesSubject(s)
Humans , Male , Aged , Pemphigoid, Bullous/pathology , Pemphigoid, Bullous/diagnosis , Pruritus/etiologyABSTRACT
El penfigoide ampollar es una enfermedad adquirida que usualmente afecta a adultos.Es rara en niños y clínicamente puede ser indistinguible de otras enfermedades ampollares.Presentamos un caso de penfigoide ampollar en una niña de 3 meses de vida con ampollas generalizadas
Subject(s)
Infant , Pemphigoid, Bullous/history , Pemphigoid, Bullous/pathology , Pemphigoid, Bullous/therapy , Skin DiseasesSubject(s)
Humans , Adult , Dermatitis, Atopic/etiology , Dermatitis, Contact/etiology , Eczema, Dyshidrotic , Keratoderma, Palmoplantar , Nickel/adverse effects , Pruritus/etiology , Eczema, Dyshidrotic/classification , Eczema, Dyshidrotic/diet therapy , Eczema, Dyshidrotic/etiology , Eczema, Dyshidrotic/physiopathology , Sweat Glands/physiology , Hyperhidrosis/physiopathology , Pemphigoid, Bullous/pathology , Piroxicam/adverse effects , Stress, Psychological , SeasonsABSTRACT
Se realizó una revisión del tema "Penfigoide ampollar", analizando ubicación nosológica, epidemiología, clínica, variantes, laboratorio, inmunohistopatología, diagnóstico diferencial, pronóstico y tratamiento
Subject(s)
Humans , Male , Female , Adolescent , Aged , Diagnosis, Differential , Eosinophilia/etiology , Pemphigoid, Bullous/diagnosis , Azathioprine/administration & dosage , Azathioprine/adverse effects , Azathioprine/therapeutic use , Cyclophosphamide/administration & dosage , Cyclophosphamide/therapeutic use , Cyclosporine/therapeutic use , Dapsone/therapeutic use , Immunoglobulin E/blood , Niacinamide/administration & dosage , Niacinamide/therapeutic use , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/pathology , Tetracycline/administration & dosage , Tetracycline/therapeutic useABSTRACT
Os autores descrevem um caso de penfigóide bolhoso em paciente idosa. Revisam a literatura e chamam atençäo para dificuldade diagnóstica do mesmo
Subject(s)
Humans , Female , Pemphigoid, Bullous , Aged, 80 and over , Biopsy , Fluorescent Antibody Technique , Pemphigoid, Bullous/diagnosis , Pemphigoid, Bullous/drug therapy , Pemphigoid, Bullous/pathology , Prednisone/therapeutic use , Terfenadine/therapeutic useABSTRACT
Diversas enfermedades cutáneas se caracterizan por alteraciones localizadas preferentemente en la zona de membrana basal. Estas dermatosis se han hereditarias y adquiridas. Se pueden encontrar cambios ultraestructurales en la membrana basal, muchos de los cuales serían causados por fenómenos autoinmunes. Múltiples investigaciones han permitido aclarar la morfogénesis de las lesiones clínicamente visibles que afectan esta zona en particular. En la presente revisión se realiza una puesta al día de la última información de la literatura sobre estas interesantes enfermedades