Retraso de crecimiento intrauterino precoz y severo junto con manifestación atípica de herpes gestacional / Early and severe intruterine growth restriction and atypical manifestations of herpes gestationis

El herpes gestationis (HG), o penfigoide gestacional (PG), es una rara pero grave enfermedad, que puede afectar al curso del embarazo y a su resultado perinatal. Se piensa que tiene una posible etiología inmunológica. Presenta una serie de características clínicas y anatomopatológicas específicas que se describen en este artículo

Pemphigoid gestationis, or herpes gestationis, is an uncommon and severe disease that can affect the course of pregnancy and perinatal outcome. Its etiology is thought to be autoimmune. The specific clinical and histological features are described

Repercusión del herpes gestationis en los estados materno y fetal / Effect of herpes gestationis on maternal and fetal health

Objetivo: Estudiar la repercusión del herpes gestationis en los estados materno y fetal. Sujetos y métodos: La base de este estudio la constituyen 2 pacientes controladas durante los meses de marzo a octubre de 2003 por el Servicio de Ginecología y Obstetricia del Hospital Universitario Son Dureta de Palma de Mallorca. Ambas fueron fotografiadas en diferentes momentos de su evolución y se les realizaron biopsias de las lesiones que confirmaron el diagnóstico de herpes gestationis. Los controles obstétricos realizados no difieren de los habituales durante una gestación de curso normal. Resultados: La evolución de las pacientes fue favorable tras el uso de corticoterapia. Respecto a las complicaciones fetales descritas, observamos un caso de crecimiento intrauterino retardado y otro de prematuridad. El carácter autoinmune de la enfermedad y su predisposición hereditaria quedan reflejados por el hallazgo de antígenos de histocompatibilidad específicos en una de nuestras pacientes. Conclusiones: El herpes gestationis no es una entidad grave siempre y cuando realicemos un diagnóstico y tratamiento correctos

Objective: To analyze the effect of herpes gestationis on maternal and fetal health. Subjects and methods: Two patients were studied from March to October, 2003 by the Obstetrics and Gynecology Service of Son Dureta University Hospital of Palma, Majorca. Both patients were photographed at various times during the course of the disease. The diagnosis of herpes gestationis was confirmed by biopsy. Their obstetric management did not differ from that in normal pregnancy. Results: Outcome in these patients was favorable after steroid therapy. Regarding fetal complications, low intrauterine growth was observed in one fetus and prematurity in the other. The autoimmune character of the disease and hereditary predisposition were reflected in the finding of specific histocompatibility antigens in one of the two patients. Conclusion: Herpes gestationis is not a serious disease if a correct diagnosis is made and appropriate treatment provided

A case of chronic herpes gestationis: persistent disease or conversion to bullous pemphigoid?

We report the case of a 38-year-old woman with herpes gestationis (HG) persistent for 26 months postpartum with typical erythematous-edematous grouped lesions associated with vesicles, blisters, and scaled crusts on most parts of her body. Despite high doses of oral prednisolone, azathioprine, and dapsone, and a trial of 5 plasmaphereses, the disease has persisted to date. Histopathologic examination of lesional skin showed subepidermal blisters, focal basal cell necrosis, and a dermal inflammatory infiltrate including many eosinophils. Direct immunofluorescence showed linear C3 staining in the basement membrane zone and the complement fixation test demonstrated circulating antibasement membrane zone antibodies at a low titer (1:80). HLA typing demonstrated an A2, A24, B35, B52, DR4,5, DR13,15, DRW52,53 phenotype. We present this case as chronic persistent HG and discuss the differential diagnosis between chronic persistent HG and HG evolving to bullous pemphigoid, together with a careful examination of similar cases reported in literature.

Herpes gestationis may present itself as a paraneoplastic syndrome of choriocarcinoma-a case report.

BACKGROUND: Herpes gestationis (HG) is a rare, recurrent, pruritic, vesicobullous dermatosis occurring predominantly in pregnancy and seldom in early puerperium. Reports of the association of HG with choriocarcinoma are extremely rare and this case highlights such a possible link. CASE: This case focuses on the late postpartal manifestation of HG being associated with metastatic high-risk choriocarcinoma. Direct immunofluorescence was used to verify the diagnosis of HG. Symptomatic therapy with corticosteroids and antihistamines alleviated the pruritic symptoms associated with HG, but only the recurrent course of chemotherapy for the choriocarcinoma resulted in complete disappearance of all signs and symptoms of the HG. CONCLUSION: The time course of this case highlights once again the possible association of HG as a paraneoplastic syndrome of choriocarcinoma.

Dermatoses of pregnancy.

Several reported dermatoses of pregnancy have not survived the scrutiny of time and thus including them in current classification schemes does not serve any useful purpose. This review resolves issues in the existing conflicting literature.

[Neurologic manifestations in a child of a mother with gestational herpes]. / Manifestations neurologiques chez un enfant de mère atteinte d'herpès gestationis.

BACKGROUND: Herpes gestationis in the neonate is usually associated with an increased risk of premature birth and/or low birth weight for gestational age (GA) and sometimes skin lesions. Neurologic manifestations are nos described in these babies. CASE REPORT: A boy was born at 35 weeks of GA from a mother who developed skin eruption typical of herpes gestationis. His weight was 2320 g and his height was 46 cm. He had transient respiratory distress syndrome and was given antibiotics due to suspected group B streptococcus infection. He developed on day 3 skin vesiculous eruption which disappeared within 3 days and neurologic manifestations: hypertonia and hyperkinesis, abnormal EEG. The CSF was normal. The manifestations spontaneously disappeared within 5 days. The herpes gestationis factor was present in both mother and infant. CONCLUSION: A relationship between the maternal herpes gestationis and neonatal neurologic manifestations is possible; there was no other known causes for the transient neurological disease.

Chronic herpes gestationis and antiphospholipid antibody syndrome successfully treated with cyclophosphamide.

Herpes gestationis shares many features with other bullous diseases, especially bullous pemphigoid. Treatments of benefit in bullous pemphigoid may also be effective for herpes gestationis. Cyclophosphamide, azathioprine, and other immunosuppressive agents have been used successfully in refractory cases of bullous pemphigoid. We describe a patient with severe and persistent herpes gestationis in the postpartum period and the antiphospholipid antibody syndrome, both of which were refractory to high-dose corticosteroid therapy. Treatment with pulsed-dose intravenous cyclophosphamide produced an excellent clinical response.

New observations on pemphigoid 'herpes' gestationis.

Pemphigoid gestationis (PG) is a rare auto-immune bullous disease of pregnancy that is characterized by linear deposition of C3 along the basal membrane zone of lesional, perilesional and uninvolved skin when examined by direct immunofluorescence. Circulating antibodies of IgG1 class are also present. The aberrant expression of MHC class II molecules in the placenta appears to be important in triggering the immune response in the lamina lucida of the basement membrane zone in the placenta which then cross-reacts with the skin. PG appears to be unique in that it produces both basement membrane zone antibodies and anti-HLA antibodies. Chronic placental insufficiency can occur so that there is a clear tendency for premature deliveries and small-for-date babies. Secondary auto-immune disorders are significantly increased in PG and appear to be related to the HLA antigens DR3 and DR4. Finally abnormalities of complement polymorphism have recently been described.

Secondary autoimmune diseases in herpes gestationis (pemphigoid gestationis).

BACKGROUND: Herpes gestationis (HG) is an autoimmune disease of the skin that occurs exclusively in association with pregnancy (or trophoblastic disease). It is associated with the HLA-DR3 and -DR4 antigens that are also associated with several other autoimmune diseases. HG has previously been reported in association with Graves' disease. OBJECTIVE: Our purpose was to determine the frequency of other autoimmune disease(s) in patients with a history of HG. METHODS: Seventy-five patients with a history of HG were studied for the frequency of other autoimmune diseases. RESULTS: We found an increased frequency of Graves' disease in patients with a history of HG. Those with HG have an increased risk for the development of other autoantibodies. There is an increased frequency of autoimmune diseases in the family members of patients with HG. CONCLUSION: Secondary autoimmune disease in HG is unusual, but does occur. The most frequent is Graves' disease.

Fetal risks in herpes gestationis.

BACKGROUND: The potential risks to the fetus in herpes gestationis have long been a controversial subject, but because of the rarity of the disease, have only occasionally been studied. OBJECTIVE: The purpose of this study was to determine the incidence of fetal complications in herpes gestationis. METHODS: We collected and analyzed the obstetric histories of 74 patients and compared their involved pregnancies with their uninvolved pregnancies. RESULTS: There was an obvious tendency for premature delivery associated with herpes gestations. A slight tendency toward small-for-gestational-age newborns associated with herpes gestationis was confirmed, but, perhaps surprisingly, no increase in spontaneous abortions or stillbirths was noted. The use of systemic steroids did not appear to influence risk. CONCLUSION: Herpes gestationis is associated with an increase in prematurity and small-for-gestational-age infants.

Epidural analgesia for a parturient with herpes gestationis.

A 23-yr-old parturient with herpes gestationis spontaneously delivered a normal healthy infant under epidural analgesia. She received five injections of bupivacaine 0.5 per cent over a ten-hour period. There was no infection at the lumbar region, even though her body was covered with vesicles and bullae including the face and neck. Eight months after delivery the patient still has a vesicular eruption which occurs mainly during her menses.

Herpes gestationis.

A case of clinically typical herpes gestationis is presented. The diagnosis was confirmed by light microscopy and immunofluorescence studies. The symptoms subsided at delivery, but recurred during subsequent oral contraceptive medication. The newborn infant was affected temporarily. The treatment, the fetal risk and the implications of the diagnosis on future pregnancies and contraceptive therapy are discussed.