Cases reported "Pemphigoid Gestationis"

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1/37. The time course of the change in antibody titres in herpes gestationis.

    The time course of the change in antibody titres was examined postpartum after treatment in two patients with herpes gestationis. The first patient, a 29-year-old woman seen first in the 32nd week of her first pregnancy, had an exudative erythema, and developed an itchy erythema with small tense vesicles on the trunk and legs after delivery in the 40th week of pregnancy. The second patient, a 28-year-old woman seen first in the 28th week of her first pregnancy, had an itchy exudative erythema, small tense vesicles and crusts on the legs. After a Caesarean section in the 40th week of pregnancy performed because of cardiac complications in the fetus, the skin lesions extended to the trunk and extremities. Direct immunofluorescence revealed linear depositions of IgG and C3 at the basement membrane zone (BMZ) and indirect immunofluorescence was positive at the epidermal side of the BMZ in 1 mol/L NaCl-split skin in both cases. In patient 1, prednisolone, 20 mg/day, administered 4 months after delivery, gave rapid improvement (within 1 week) of the skin lesions; in patient 2, minocycline, 200 mg/day, administered 2 weeks after delivery, gave improvement within 2 weeks. immunoblotting against epidermal extracts revealed the presence of antibodies directed to the 180 kDa bullous pemphigoid antigen in both sera. Indirect immunofluorescence and immunoblot were positive for at least 2 months in patient 1 and for 5 months in patient 2 after disappearance of the skin lesions. ( info)

2/37. Severe subepidermal blistering disorder with features of bullous pemphigoid and herpes gestationis.

    Herpes gestationis (HG) and bullous pemphigoid (BP) are blistering disorders with similar features, including urticarial lesions that progress to blisters and immunodeposition of C3 in a linear pattern at the basement membrane zone. Among their differences, HG is distinguished by its association with pregnancy, the puerperium, or hormonal perturbation. We describe the immunopathologic findings and clinical course in a multiparous woman with a severe blistering eruption. The patient was not pregnant. Malignancy evaluation was negative, and hormonal testing was normal. Histologic examination demonstrated a subepidermal bulla with eosinophils. Direct immunofluorescence showed C3 in a strong linear band at the dermal-epidermal junction. Indirect immunofluorescence demonstrated circulating IgG and IgG3 antibodies to basement membrane zone (epidermal component on salt-split skin), and complement-fixing IgG. immunoprecipitation demonstrated antibodies to a 180-kd keratinocyte protein antigen. By clinical definition, this patient has BP. However, her disease presentation demonstrated features of both BP and HG. ( info)

3/37. 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. ( info)

4/37. 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. ( info)

5/37. Herpes gestationis.

    Herpes gestationis, also known as pemphigoid gestationis, is a rare autoimmune disease of pregnancy. It is characterized by itching and skin lesions. The disease causes prominently maternal discomfort but fetal and neonatal complications have been reported. There are only scattered reports of cutaneous neonatal herpes gestationis in the literature; however, the frequency and severity of fetal illness are still debated. We describe 2 cases of herpes gestationis diagnosed and managed at the King Khalid University Hospital, Riyadh, Kingdom of saudi arabia. ( info)

6/37. pemphigoid gestationis: treatment with immunoapheresis.

    BACKGROUND: Pemphigoid (herpes) gestationis is a rare blistering disease of pregnancy. Topical and systemic corticosteroids and antihistamines are usually sufficient for treatment. CASE REPORT: A patient suffering from severe, therapy-resistant pemphigoid gestationis was successfully treated with the additional use of six sessions of immunoapheresis. Systemic corticosteroids could be tapered and serum autoantibody levels to bullous pemphigoid antigen 2 (BPAG2 = BP180) significantly dropped during this treatment. CONCLUSION: Immunoapheresis represents a helpful therapeutic option with few side effects for severe cases of pemphigoid gestationis, unresponsive to conventional treatment. ( info)

7/37. A case of gestational diabetes arising following treatment with glucocorticosteroids for pemphigoid gestationis.

    A case of pemphigoid gestationis, a rare autoimmune disease unique to pregnancy, is described. To control the skin lesions systemic corticosteroids were required. The patient developed gestational diabetes mellitus. The pregnancy continued to 37 weeks and a live male infant was delivered. There was no evidence of macroscopic changes in the placenta. The skin lesions resolved in the postpartum period. ( info)

8/37. pemphigoid gestationis: report of a case with umbilical artery Doppler assessment.

    BACKGROUND: pemphigoid gestationis is a rare autoimmune disorder of pregnancy characterized clinically by a pruritic, papular rash and in some cases intrauterine growth restriction and premature delivery. The growth disorder is secondary to antibody deposition in the placental bed, with resultant placental insufficiency. The appropriate fetal assessment required during these gestations remains uncertain. The use of serial Doppler velocimetry of the umbilical arteries has not been reported to date in this disorder. CASE: A woman had pemphigoid and progressively decreasing umbilical artery end-diastolic flow over a period of weeks without documented fetal growth restriction. Delivery was finally prompted by reversal of end-diastolic flow. CONCLUSION: We suggest that parturients with this condition undergo frequent umbilical artery Doppler studies to document end-diastolic velocity even without the ultrasound finding of intrauterine growth restriction. ( info)

9/37. Don't do anything rash!

    BACKGROUND: Peripartum and postpartum dermatologic lesions may or may not be an indicator of morbidity or mortality for either the fetus or mother. CASE: Postpartum dermatologic lesions occurred in a 41-year-old multipara, 1 week after a repeat cesarean delivery for severe intrauterine growth restriction and nonreassuring fetal testing. The diagnosis, using histopathologic techniques, and the pharmacologic management are discussed. CONCLUSION: This case raises intriguing questions regarding prompt diagnosis, treatment, and the counseling of patients with dermatologic lesions with respect to current and future pregnancy outcomes. ( info)

10/37. pemphigus gestationis (herpes gestationis): a case report.

    pemphigus gestationis is a rare disease, probably of immunologic origin, that causes the placenta to absorb immunoglobulins and deposit them in its basement membrane. The resulting placental insufficiency leads to increased morbidity and mortality among the offspring of such patients. A case of pemphigus gestationis is presented, and the importance of early diagnosis is discussed. ( info)
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