Penfigoide gestacional y erupción ampollar en el recién nacido / Bullous pemphygoid and blisters in the newborn

El penfigoide gestacional es una dermatosis rara, que se presenta durante el embarazo. Se caracteriza por una respuesta autoinmune contra las proteínas de los hemidesmosomas, que genera un clivaje entre la epidermis y la dermis tanto de la piel como de las mucosas. Clínicamente, presenta prurito intenso, placas y pápulas eritematosas, que evolucionan a apollas con distribución en el abdomen y los miembros. Como complicaciones, en el feto puede generar parto prematuro y bajo peso para la edad gestacional, con alto riesgo de mortalidad. (AU)

Gestational pemphygoid is a rare, autoimmune dermatosis that occurs during pregnancy. It is characterized by an autoimmune response against hemidesmosome proteins, generating a cleavage between the epidermis and the dermis in the skin and mucous membranes. Clinically it presents with intense pruritus, plaques and erythematous papules that evolve to blisters that are distributed mainly in the abdomen and limbs. The complications are preterm birth and low weight for gestational age, with high risk of mortality. (AU)

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.

Case for diagnosis

Pemphigoid gestationis is a rare, autoimmune blistering dermatosis of pregnancy. No increase in fetal or maternal mortality has been demonstrated, but a greater prevalence of premature and small-for-gestationalage babies has been reported. Topical and systemic corticosteroids and antihistamines are the manstay of treatment. The authors report a case of a 27-year-old woman at 28-weeks gestation with sudden onset of pruriginous vesicles and blisters in the abdomen and limbs. Systemic corticosteroids were introduced and maintained throughout gestation to prevent flares and tapered after the birth of a healthy child.

Intravenous Immunoglobulin Therapy for Persistent Pemphigoid Gestationis with Steroid Induced Iatrogenic Cushing's Syndrome

No abstract available.

Penfigoide gestacional, revisión bibliográfica y caso clínico / Gestational pemphigoid, literature review and case report

Las dermatosis específicas del embarazo son un grupo heterogéneo de desórdenes pruriginosos que se presentan hacia el tercer trimestre del embarazo y/o postparto inmediato. Estas incluyen al, pengifoide gestacional, la colestasis gestacional, la psoriasis pustulosa del embarazo las pápulas, placas urticarianas pruriginosas del embarazo y las foliculitis pruriginosas del embarazo...

Frequency of specific dermatoses of pregnancy in a tertiary care hospital

To assess the frequency of specific dermatoses of pregnancy in a tertiary care hospital. This cross sectional study was conducted on patients attending outpatient department of Dermatology, civil hospital, Karachi referred from the outpatient department of gynecology and obstetrics spanning, 1[st] May 2006 till 31[st] August 2006. Pregnant females of different age groups, both primigravida and multigravida fulfilling the selection criteria were enrolled. After an informed consent, selected patients were subjected to a detailed history and clinical examination. Diagnosis of the diseases was made clinically and relevant investigations were carried out where required. 100 patients, comprising 32 primigravida [32%] and 68 multigravida [68%], diagnosed as suffering from specific dermatoses of pregnancy completed the study. Minimum age of presentation was 20 years and maximum 44 years the mean age being 32 +/- 6.8 years. Prurigo of pregnancy [96%] was the most common dermatoses seen followed by pruritic folliculitis of pregnancy [2%], herpes gestationis [1%] and polymorphic eruption of pregnancy [1%]. Amongst 96 patients [96%] suffering from prurigo of pregnancy, there were 28 primigravida [29%] and 68 multigravida [71%]. Both the primigravida with pruritic folliculitis of pregnancy presented one each in the 2[nd] and 3[rd] trimester. Patients suffering from herpes gestationis and polymorphic eruption of pregnancy [PUPP] were primigravida presenting in 2[nd] trimester. Specific dermatoses of pregnancy can present in any trimester. Prurigo of pregnancy remains the most common disorder followed by pruritic folliculitis of pregnancy.

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.

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.

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.

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.

Clinical and Epidemiological Studies of Patients with Autoimmune Bullous Diseases / 대한피부과학회지

BACKGROUND: Autoimmune bullous diseases (ABDs) are a rare significant group of dermatoses that pose great challenges to the dermatologist. So far, few epidemiological surveys for the whole spectrum of ABDs have been completed in Korea, though many epidemiological survey have focused on single ABDs. OBJECTIVE: The aim of this study was to evaluate the incidence and clinical findings of patients in the Chonnam Province of Korea with ABDs over a 5 year period. METHODS: A retrospective analysis was conducted on 52 ABDs patients from 2002 to 2006. ABDs were diagnosed chiefly by histopathologic findings, immunofluoresence (IF), and immunoblotting with epidermal and dermal human foreskin extract. For the exact diagnosis of ABDs, several additional diagnostic methods such as indirect IF with salt-split normal skin, immunoblotting (IB) with normal keratinocyte cells, enzyme-linked immunosorbent assay (ELISA) with recombinant proteins, immunoprecipitaion (IP) were also performed. RESULTS: Out of 52 patients diagnosed with ABDs during the study period, pemphigus vulgaris was observed to be the commonest ABD (38.5%) followed by bullous pemphigoid (29.8%), pemphigus foliaceus (17.8%), epidermolysis bullosa acquisita (5.8%), paraneoplastic pemphigus, pemphigoid gestationis (3.8%), and linear IgA bullous dermatoses (1.9%). CONCLUSION: This study showed the incidence of the ABDs in Chonnam-Gwangju province area were very low (52 cases over 5 years) and was stationary over this period. Pemphigus vulgaris and bullous pemphigoid were common diseases among many ABDs. The incidence of ABDs in Korea as a whole needs to be investigated.

Dermatoses específicas da gravidez / Specific dermatosis of the pregnancy

Os autores abordam as dermatoses específicas da gestação, fazendo uma revisão sobre suas características clínicas, critérios diagnósticos e opções terapêuticas.

[Pemphgoid gestations: a report of 30 cases]

Pemphigoid gestationis is an acute autoimmune subepidermal bullous dermatosis which occurs during pregnancy and post partum. It is a rare disease which incidence vary from 1/1700 to 1/60000 pregnancy. It occurs in young woman aged between 20 to 40 years. The authors try throughout a retrospective study of 30 cases of pemphigoid gestationis to drave the epidemiological and clinical profile and the immunopathological characteristics of this affection

Pele na gestacão / Skin in pregnancy

As manifestacões cutâneas durante a gravidez correspondem a alteracões fisiológicas, dermatoses próprias do período gestacional ou doencas pré-existentes. O penfigóide gestacional, a erupcão polimorfa da gravidez, a foliculite pruriginosa, o prurigo da gravidez e o prurido gravídico são considerados dermatoses próprias da gestacão. Uma forma de psoríase pustulosa grave, o impetigo herpetiforme, está descrito na gestacão. As lesões cutâneas da artrite psoriásica, do lúpus eritematoso, da dermatopolimiosite e os pênfigos podem piorar na gravidez. O eritema nodoso e o eritema nodoso hanseniano podem ser desencadeados pela gravidez. O tratamento deve considerar os riscos e benefícios para a mãe e o concepto.

Dermatoses específicas da gravidez / Specific dermatoses of pregnancy

Durante as últimas décadas, grande número de artigos trouxeram novas acepções sobre classificação, patogênese, tratamento, prognóstico e riscos fetais associados a dermatoses específicas da gravidez. O conhecimento sobre as dermatoses específicas da gravidez é muito importante para os obstetras que geralmente fazem o diagnóstico das afecções cutâneas durante o pré-natal. O objetivo desta revisão é sistematizar a análise da literatura baseada em evidências quanto às dermatoses específicas da gravidez

Diagnóstico diferencial de enfermedades ampollares en el embarazo: a proposito de dos casos clínicos / Differential diagnosis of blistering disease during pregnancy: apropos of two clinical cases

Durante el embarazo se producen algunas enfermedades ampollares, dentro de las cuales las más frecuente es el herpes gestationis o penfigoide del embarazo. Sin embargo, es importante tener en cuenta otros diagnósticos diferenciales. En el presente artículo se revisan, a propósito de dos casos clínicos, los conocimientos actuales sobre el herpes gestationis y sus diagnósticos diferenciales.

Herpes gestacional / Herpes gestationis

El Herpes Gestacional, también denominado Penfigoide Gestacional, forma parte de las dermatosis específicas del embarazo. Este artículo pretende revisar los conocimientos actuales sobre esta patología.

[Pemphigoid gestationis: a pregnancy- related pathology underestimated by obstetricians]

Pemphigoid gestations [herpes gestationis] is an autoimmune bullous dermotosis occurring during pregnancy or in the post-partum period. The interaction of this rare pathology with pregnancy is underestimated by obstetricians. Authors report one case of pemphigoid gestations diagnosed in a pregnant woman at term. After a literature review clinical aspects of this dermatosis and especially its interactions, with pregnancy are assessed