ABSTRACT
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.
Subject(s)
Adult , Female , Humans , Pregnancy , Basement Membrane , Blister , Eosinophils , Fluorescent Antibody Technique, Direct , Pemphigoid Gestationis , Pemphigoid, Bullous , Pregnancy Trimester, Third , PruritusABSTRACT
RESUMEN Durante el embarazo ocurren numerosos cambios inmunológicos, metabólicos, endocrinos y vasculares que pueden propiciar la susceptibilidad de la mujer embarazada a presentar cambios en la piel y sus anexos. El penfigoide gestacional es una enfermedad rara, autoinmune, caracterizada por una dermatosis ampollosa, muy pruriginosa, con probable afectación del producto. Se describe el caso en una paciente con penfigoide gestacional, confirmado histológicamente y se discuten su evolución, las complicaciones observadas en el recién nacido, además de realizar una revisión en la literatura.
ABSTRACT Many inmunologic changes occur During pregnancy occur, metabolic, endocrine and vascular that may promote susceptibility of pregnant women to have changes in the skin and its annexes. Gestational pemphigoid is a rare, autoimmune bullous dermatosis characterized by very itchy, with probable involvement of the product. We describe a patient with Gestational pemphigoid, histological confirmation and we discuss its evolution, the complications in the newborn, in addition to a review in the literature.
ABSTRACT
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.
Subject(s)
Humans , Female , Pregnancy , Adolescent , Pemphigoid Gestationis/diagnosis , Pemphigoid Gestationis/drug therapy , Clobetasol/therapeutic use , Prednisone/therapeutic use , Chlorpheniramine/therapeutic useABSTRACT
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.
Subject(s)
Humans , Adolescent , Female , Pregnancy , Pemphigoid Gestationis/diagnosis , Pemphigoid Gestationis/therapy , Diagnosis, Differential , Pregnancy Outcome , Pemphigoid Gestationis/immunology , Pemphigoid Gestationis/pathologyABSTRACT
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.
Subject(s)
Adult , Female , Humans , Infant, Newborn , Pregnancy , Young Adult , Pemphigoid Gestationis/pathology , /analysis , Immunoglobulin G/analysis , Pregnancy Outcome , Pemphigoid Gestationis/drug therapy , Pemphigoid Gestationis/immunology , Pemphigoid, Bullous/immunology , Pruritus/pathology , Urticaria/pathology , Young AdultABSTRACT
Herpes gestationis is a rare pregnancy-associated autoimmune bullous disease resembling bullous pemphigoid in both clinical and immunohistopathological aspects. Generally it may be improved spontaneously or easily controlled by topical or low-dose corticosteroid. Very rarely, however, severe and long-standing cases that did not respond to high-dose corticosteoid therapy were reported. We present a case of an unusual severe herpes gestationis in a 26-year-old woman that was resistant to high-dose corticosteroid and/or dapsone therapy. But the lesions responded dramatically to low-dose cyclosporine.