Penfigoide gestacional. Reporte de caso

El penfigoide gestacional, antes conocido como herpes gestacional, es una dermatosis rara, ampollosa, autoinmune y específica del embarazo. Aunque la etiología no se conoce del todo, la mayoría de los pacientes desarrollan anticuerpos contra una proteína hemidesmosomal transmembrana de 180 kDa. Los síntomas que definen la enfermedad son el picor intenso y las lesiones urticariales que evolucionan hacia ampollas dolorosas. Las lesiones comienzan en la región periumbilical en el 90% de los casos y se extienden rápidamente a otras zonas del cuerpo, a excepción de la cabeza y las mucosas. Es más frecuente en el segundo o tercer trimestre. En las muestras de biopsia de piel, la tinción de inmunofluorescencia directa valida el diagnóstico. Los corticoides tópicos pueden ser utilizados para tratar los síntomas leves, mientras que los corticoides orales y los antihistamínicos deben usarse para tratar los casos graves. Se presenta un caso de penfigoide gestacional.

Pemphigoid gestationis, formerly known as herpes gestationis, is a rare, blistering, autoimmune, pregnancy-specific dermatosis. Although the etiology is not fully understood, most patients develop antibodies against 180 kDa transmembrane hemidesmosomal protein. The defining symptoms of the disease are intense itching and urticarial lesions that evolve into painful blisters. Lesions begin in the periumbilical region in 90% of cases and spread rapidly to other areas of the body, except for the head and mucous membranes. It is most frequent in the second or third trimester. In skin biopsy specimens, direct immunofluorescence staining validates the diagnosis. Topical corticosteroids can be used to treat mild symptoms, while oral corticosteroids and antihistamines should be used to treat severe cases. A case of pemphigoid gestationis is presented.

Penfigoide Gestacional / Pemphigoid Gestationis

Resumen 22. Las enfermedades ampollares de tipo penfigoide,se caracterizan por la presencia de ampollas de origen autoinmune.El Penfigoide gestacional(PG), es parte de este grupo de enfermedades y se define por la presencia de una erupción ampollar,intensamente pruriginosa de comienzo agudo, generalmente en el segundo trimestre del embarazo o posparto inmediato, en ocasiones puede haber compromiso fetal y asociarse con otras enfermedades de origen autoinmune.

Abstract 26. Pemphigoid-type bullous diseases are characterized by the presence of blisters of autoimmune origin.Gestational Pemphigoid (PG) is part of this group of diseases and is defined by the presence of an intensely pruritic bullous eruption of acute onset, usually in the second trimester of pregnancy or immediate postpartum period, there may be fetal compromise and associated with other diseases of autoimmune origin.

Penfigoide gestacional en el tercer trimestre del embarazo / Pemphigoid gestationis in the third trimester

RESUMEN Introducción: el penfigoide gestacional es una enfermedad ampollar infrecuente que se caracteriza por una erupción urticariana, intensamente pruriginosa en tronco y extremidades, que aparece al final del embarazo y después del parto. Evoluciona con rapidez hacia una dermatosis vesiculoampollar de tipo penfigoide ampollar, dado que tienen muchas similitudes clínicas, histológicas e inmunopatológicas. Objetivos: presentar las características clínicas de una paciente gestante con penfigoide gestacional. Presentación de caso: gestante con antecedentes prenatales de embarazo anterior normal sin lesiones de piel y un embarazo actual de 32 semanas. Fue atendida en la consulta de Dermatología del Policlínico Universitario Raúl Sánchez donde se evidenció un cuadro cutáneo polimorfo caracterizado por lesiones de piel en forma de pequeñas placas múltiples, redondeadas de aspecto urticariano y lesiones eritemato-papulosas infiltradas, que se encontraron localizadas en abdomen, miembros inferiores y muy pruriginosas. Los elementos clínicos y el estudio histopatológico confirmaron el diagnóstico de penfigoide gestacional. Recibió tratamiento médico con mejoría clínica evidente. Conclusiones: se presentó un caso clínico conocido, pero de aparición escasa, por lo que existen problemas para su diagnóstico y debe tenerse presente por la similitud con otras dermatosis de piel en gestantes. Presenta riesgo de morbilidad para el recién nacido, aunque se preserva la salud materna a pesar de su etiología autoinmune. El adecuado diagnóstico de esta enfermedad permitirá un seguimiento y tratamiento médico adecuado por el equipo multidisciplinario responsable para lograr una adecuada calidad de vida y mejor indicador materno infantil.

ABSTRACT Introduction: pemphigoid gestationis is a rare blistering disease characterized by an intensely itchy urticarial rash on the trunk and extremities that appears at the end of pregnancy and after delivery. It rapidly evolves into vesiculo ampullary dermatosis of the pemphigoid blistering type, since they have many clinical, histological and immune-pathological similarities. Objectives: to present the clinical characteristics of a pregnant patient with pemphigoid gestationis. Case Report: a pregnant woman with a prenatal history of a normal previous pregnancy; without skin lesions and a current 32-week pregnancy is presented. She was attended in the Dermatology Clinic at Raul Sanchez University Polyclinic where a polymorphic skin condition was evidenced by skin lesions in the form of small multiple, rounded plates of urticarial aspect and erythematous-papular filtered lesions, which were located in the abdomen, lower limbs and very pruritic rash. Clinical elements and histopathological study confirmed the diagnosis of pemphigoid gestationis. She received medical treatment achieving evident clinical progress. Conclusions: a known clinical case was presented but of scarce incidence, therefore there are problems for its diagnosis and it must be taken into account due to the similarity with other skin dermatoses in pregnant women. It presents a morbidity risk for the newborn, maternal health is preserved in spite of its autoimmune etiology. The adequate diagnosis of this entity will allow a follow up along with an adequate medical treatment by a responsible multidisciplinary team, achieving an adequate quality of life and a better maternal-infant indicator.

BP180-related autoimmune blistering diseases / 中华皮肤科杂志

BP180-related autoimmune blistering diseases include bullous pemphigoid,lichen planus pemphigoides,linear IgA bullous dermatosis,pemphigoid gestationis and cicatricial pemphigoid.There are multiple autoantibody-reactive sites on the extracellular region of BP180.Current studies show that there is heterogeneity in the autoimmune blistering disease-related target sites on BP 180,and different clinical manifestations of the same disease are related to the heterogeneity of target sites.However,further studies and analysis are still needed for the mechanism of the heterogeneity.

Clinical study of skin changes in low and high risk pregnant women

Abstract: BACKGROUND: During pregnancy there is immunological, metabolic, endocrine and vascular changes responsible for physiological and pathological skin changes. OBJECTIVES: determine the prevalence of specific physiological changes and pregnancy, comparing the period of gestation of their appearances and compare type of prenatal care as the skin changes. METHODS: A cross-sectional study with 905 pregnant women. RESULTS: The prevalence of physiological skin changes was 88.95% and the most common was pigment. The prevalence of specific dermatoses was 8.72% and atopic eruption was the most common. CONCLUSION: Physiological changes were seen more in the 3rd quarter, as well as the specific dermatoses. No statistical difference in prenatal low risk compared to high risk was observed, whereas the cutaneous physiological changes and specific pregnancy dermatoses.

A Case of Pemphigoid Gestationis with Involvement of the Face / 대한피부과학회지

Pemphigoid gestationis, also known as herpes gestationis, is a rare blistering disease associated with pregnancy, caused by autoantibody to the basement membrane component. It occurs in the second or third trimester of pregnancy or directly after delivery, as erythematous, urticarial papules, and plaques on the whole body, which aggravate to vesicles and bullae with severe pruritus. Histopathologically, subepidermal blisters and eosinophilic spongiosis are shown, and linear deposition of C3 in the dermoepidermal junction is seen on direct immunofluorescence assay. We present the case of 35-year-old woman who was diagnosed with pemphigoid gestationis, with the involvement of the uncommon site of the face.

Immunofluorescence testing in the diagnosis of autoimmune blistering diseases: overview of 10-year experience

BACKGROUND: Immunofluorescence testing is an important tool for diagnosing blistering diseases. OBJECTIVE: To characterize the immunofluorescence findings in patients diagnosed with autoimmune blistering skin diseases. METHODS: We retrospectively analyzed immunofluorescence results encompassing a 10-year period. RESULTS: 421 patients were included and divided into 2 groups: group 1- intraepidermal blistering diseases (n=277) and 2- subepidermal blistering diseases (n=144). For group 1, positive DIF findings demonstrated: predominance of IgG intercellular staining (ICS) and C3 for pemphigus foliaceus-PF (94% and 73% respectively), pemphigus vulgaris-PV (91.5%-79.5%) and paraneoplastic pemphigus-PNP (66%-33%); ICS IgA in 100% of IgA pemphigus cases, and IgG deposits in the basement membrane zone (BMZ) along with ICS in one Hailey-Hailey patient. The IIF findings revealed mean titers of 1:2.560 for PV and 1:1.280 for PF. For paraneoplastic pemphigus, IIF was positive in 2 out of 3 cases with rat bladder substrate. In group 2, positive DIF findings included multiple deposits at basement membrane zone for epidermolysis bullosa acquisita-EBA (C3-89%,IgG-79%,IgA-47%,IgM-21%) mucous membrane pemphigoid-MMP (C3,IgG,IgA,IgM-80%) and bullous pemphigoid-BP (C3-91%,IgG-39%,IgA-11%,IgM-6%), and IgA at basement membrane zone for IgA linear disease (99%) and dermatitis herpetiformis-DH (dermal papillae in 84.6%). For lichen planus pemphigoides, there was C3 (100%) and IgG (50%) deposition at basement membrane zone. indirect immunofluorescence positive findings revealed basement membrane zone IgG deposits in 46% of BP patients, 50% for EBA, 15% for IgA linear dermatosis and 50% for LPP. Indirect immunofluorescence positive results were higher for BP and EBA with Salt-Split skin substrate. CONCLUSION: Our results confirmed the importance of immunofluorescence assays in diagnosing autoimmune blistering diseases, and higher sensitivity for indirect ...

Purification and identification of anti-BP180 NC16A antibodies from the sera of patients with bullous pemphigoid or herpes gestationis / 中华皮肤科杂志

Objective To develop a method to purify and identify anti-BP180 NC16A antibodies from the sera of patients with bullous pemphigoid (BP) or herpes gestationis.Methods The GST/NC16A fusion protein was expressed in a prokaryotic expression vector pGEX-2TBP180NC16A,and then crosslinked to glutathione sepharose beads.Anti-BP180 NC16A antibodies were isolated from the sera of 3 patients with BP and 2 patients with herpes gestationis by affinity chromatography,and analyzed by immunofluorescence,Western blot and enzyme linked immunosorbent assay (ELISA).Results The GST/NC16A fusion protein with a relative molecular mass of 37 000 was successfully expressed by the prokaryotic vector pGEX-2TBP180NC16A.Purified anti-BP180 NC16A antibodies were obtained from the sera of patients by the affinity chromatography,and ELISA revealed that the concentration of anti-BP180 NC16A was 2.4 mg/ml.The purified antibody could bind to the basement membrane zone of human skin,suggesting a strong biological activity of the antibodies.Western blot showed a single band corresponding to the expected molecular mass for anti-BP180 NC16A antibodies,indicating a high purity of the isolated antibodies.Conclusion The anti-BP180 NC16A antibodies purified by microbead-based affinity chromatography from the sera of patients with BP or herpes gestationis are highly active and specific.

Penfigoide gestacional "Herpes gestationis": revisión a partir de un caso clínico / Gestational pemphigoid "Herpes gestationis": review from a clinical case

La embarazada es susceptible a cambios en la piel y fanéreos que pueden ser fisiológicos como patológicos. El reconocimiento de estas entidades es fundamental para un correcto manejo. La clasificación y nomenclatura de las dermatosis del embarazo ha sido controversial y confusa, principalmente dado el pobre conocimiento que se tiene sobre el origen de estas entidades. El objetivo de esta revisión es informar sobre el conocimiento actual del penfigoide gestacional a partir de un caso clínico, centrándose en su diagnóstico y tratamiento como patología multidiscilpinaria.

The pregnant woman is susceptible to both physiologic and pathologic changes of the skin and appendages. Recognition of these entities is important for appropriate management. The classification and nomenclature have been controversial and confusing, mainly because of the poor knowledge that we have regarding the origin of this entities. The purpose of this review is to contribute to the current knowledge of pemphigoid gestationis, based on a case-report its diagnosis and treatment as a multidisciplinary pathology.

Dermatoses of Pregnancy: Clues to Diagnosis, Fetal Risk and Therapy

The specific dermatoses of pregnancy represent a heterogeneous group of pruritic skin diseases that have been recently reclassified and include pemphigoid (herpes) gestationis, polymorphic eruption of pregnancy (syn. pruritic urticarial papules and plaques of pregnancy), intrahepatic cholestasis of pregnancy, and atopic eruption of pregnancy. They are associated with severe pruritus that should never be neglected in pregnancy but always lead to an exact work-up of the patient. Clinical characteristics, in particular timing of onset, morphology and localization of skin lesions are crucial for diagnosis which, in case of pemphigoid gestationis and intrahepatic cholestasis of pregnancy, will be confirmed by specific immunofluorescence and laboratory findings. While polymorphic and atopic eruptions of pregnancy are distressing only to the mother because of pruritus, pemphigoid gestationis may be associated with prematurity and small-for-date babies and intrahepatic cholestasis of pregnancy poses an increased risk for fetal distress, prematurity, and stillbirth. Corticosteroids and antihistamines control pemphigoid gestationis, polymorphic and atopic eruptions of pregnancy; intrahepatic cholestasis of pregnancy, in contrast, should be treated with ursodeoxycholic acid. This review will focus on the new classification of pregnancy dermatoses, discuss them in detail, and present a practical algorithm to facilitate the management of the pregnant patient with skin lesions.

Penfigoide del embarazo: a propósito de un caso clínico y revisión de la literatura / Pemphigoid gestationis: a case report and literature review

El penfigoide del embarazo (PE), denominado también penfigoide gestacional (PG) o herpes gestationis (HG), es una enfermedad ampollar autoinmunitaria poco frecuente asociada al embarazo. Usualmente ocurre durante o tercer trimestre de la gestación, pero puede presentarse en cualquier etapa de ésta e incluso en el puerperio. La enfermedad tiene un inicio súbito, con placas eritematosas anulares urticariales sobre la que se desarrollan ampollas, las que progresan a vesículas y bulas intensamente pruriginosas. Presentamos el caso clínico de una embarazada con una erupción papuloampollar cuya clínica e histopatología fueron concordante con penfigoide del embarazo.

Pemphigoid of pregnancy also called pemphigoid gestationis (PG) or herpes gestationis (HG) is a rare autoinmune bullous disease associated with pregnancy. It usually occurs during second or third trimester of gestation but can occur at any stage of it and even in the postpartum period. The disease has a sudden onset, with erythematous annular urticarial plaques on which blister develop, envolving into vesicles and bullae intensely itchy. We report the case of a pregnant woman with a rash of papules and blisters whose clinical and histopathology were consistent with pemphigoid gestationis.