Presentation
The clinical presentation of PG is polymorphic with patients initially presenting with erythematous papules, plaques or targetoid lesions followed by appearance of small vesicles and bullae on normal skin or on top of urticarial plaques. [edoj.org.eg]
Tense bullae are present on the arms of this otherwise healthy 32-year-old primigravida woman. [emedicine.medscape.com]
Patients typically present in the second or third trimester, although nearly 25% of patients present in the postpartum period. [gynecology-obstetrics.imedpub.com]
It can present with palmo-plantar lesions, but the mucous membranes are generally spared. [clinmedjournals.org]
Clinical description Pemphigoid gestationis (PG) typically presents during the second or third trimesters, although onset during the first trimester and postpartum is possible. Pruritus may remain the only symptom. [orpha.net]
Entire Body System
- Fever
Approximately five out of 10,000 pregnant women have varicella (chickenpox). 7 It causes fever and a disseminated pruritic, vesicular, umbilicated rash with crusted lesions. [aafp.org]
Her husband recently had an upper respiratory infection and her son recently had a fever and a rash. The patient has never been vaccinated nor has anyone else in the family. She has a history of cold sores and chickenpox. [step2.medbullets.com]
Treatment includes medication for fever (see above, anti-inflammatory drugs) and taking plenty of fluids. [emedicinehealth.com]
Generalized symptoms such as fever and malaise may develop, and lymph nodes in the groin may enlarge. Lesions may persist in this stage for a week or more, and complete healing may take four to six weeks. [britannica.com]
- Anemia
Patients with pemphigoid gestationis have a higher relative prevalence of other autoimmune diseases, including Hashimoto thyroiditis, Graves disease, and pernicious anemia, which are also associated with HLA-DR3 and DR-4 haplotypes Patient Education Mothers [emedicine.medscape.com]
[…] pregnancies Mechanism and pathophysiology autoimmune bullous dermatosis pathophysiology antibodies against hemidesmosomal proteins undetermined trigger Associated conditions other autoimmune diseases such as Hashimoto's thyroiditis Grave's disease pernicious anemia [medbullets.com]
[…] spontaneously within days or weeks Blisters in small percentage of infants May develop or recur in gestational trophoblastic disease Molar pregnancy (hydatidiform mole) Choriocarcinoma Autoimmune-associated diseases Graves disease most common Pernicious anemia [arupconsult.com]
- Swelling
Appearance Herpes Gestationis often starts with extremely itchy, inflamed hives or tiny swelling that occur round the belly button. It doesn’t take too long for the rash to spread to other parts of the body after the initial appearance. [whatisherpes.net]
This response is manifested by redness, itching, swelling, and blister formation. Diagnosis Pemphigoid gestationis is usually diagnosed by taking skin biopsies of different areas of the rash and normal-appearing skin. [verywell.com]
Often the glands in the groin swell up. In both sexes urination can be painful while the blisters are present. There may be a fever. Women sometimes get a slight discharge. [netdoctor.co.uk]
- Sepsis
We report a case of intrauterine HSV type 1 infection with atypical cutaneous manifestations, misdiagnosed initially as neonatal sepsis. [jcnonweb.com]
puerperal sepsis (O85) when the reason for maternal care is that the disease is known or suspected to have affected the fetus (O35-O36) Use Additional code (Chapter 1), to identify specific infectious or parasitic disease ICD-10-CM Diagnosis Code [icd10data.com]
Bacterial infections were considered Staphylococcus aureus pyoderma or staphylococcal scalded skin syndrome, pustules with listeriosis, bullae with Group B streptococci sepsis, hemorrhagic ulcerative lesions with congenital syphilis, and congenital candidiasis [academic.oup.com]
Early administration of aciclovir has been successful in treating HSV hepatitis. 5, 7 Liver transplantation is another treatment option, 11 but the risk of recurrent HSV hepatitis with overwhelming viral dissemination and concurrent enterococcal sepsis [mja.com.au]
かんぴしゅ) 4 Hereditary angioedema with C1 esterase inhibitor deficiency C1エステラーゼを伴う遺伝性血管性浮腫 インヒビター欠乏症 4 Herpes dermatitis ヘルペス皮膚炎 4 Herpes gestationis 妊娠性疱疹 4 Herpes oesophagitis ヘルペス食道炎 4 Herpes ophthalmic ヘルペス 眼科 4 Herpes pharyngitis ヘルペス咽頭炎 4 Herpes sepsis [ikenori.com]
- Malaise
A 24-year-old woman's first pregnancy was uneventful until the sixth month when mild malaise and a highly pruritic abdominal rash occurred. A biopsy and direct immunofluorescence confirmed the suspected diagnosis of herpes gestationis. [patientcareonline.com]
Generalized symptoms such as fever and malaise may develop, and lymph nodes in the groin may enlarge. Lesions may persist in this stage for a week or more, and complete healing may take four to six weeks. [britannica.com]
|| doi: 10.5694/j.1326-5377.2002.tb04565.x Published online: 17 June 2002 Clinical Record A 30-year-old woman in the 30th week of pregnancy was admitted with a two-day history of fever, malaise, dysuria, frequent micturition and mild lower abdominal [mja.com.au]
Patients feel unwell and may complain of fatigue, malaise, fevers, chills, nausea, vomiting or diarrhea. Pustular psoriasis of pregnancy tends to remit upon delivery but occasionally flares postpartum. [cancertherapyadvisor.com]
Systemic symptoms are severe and include malaise, fever, anorexia, nausea, vomiting, diarrhea, and tetany [ 68,69 ]. Leukocytosis and elevated erythrocyte sedimentation rate are common. [uptodate.com]
Skin
- Blister
The goals of therapy (ie, control pruritus, suppress extensive blistering but not totally eliminate blister formation) should be discussed with the patient prior to treatment. [emedicine.medscape.com]
Therefore, there may be some factors other than antibodies to BP AG2, which may contribute to blistering in PG. [ncbi.nlm.nih.gov]
Histopathology of skin showing the presence of subepidermal blister. (10X, H & E) Figure 2b. Blister cavity filled with eosinophils (white arrows). (40X, H & E) Figure 3. [jpgo.org]
It was originally called herpes gestationis because of the blistering appearance, although it is not associated with the herpes virus. Diagnosis of PG becomes clear when skin lesions progress to tense blisters during the second or third trimester. [aarda.org]
- Eruptions
Differential diagnosis Differential diagnosis includes atopic eruption of pregnancy, polymorphic eruption of pregnancy, and intrahepatic cholestasis of pregnancy, herpes virus infection, urticaria, drug hypersensitivity reactions, contact dermatitis and [orpha.net]
Despite the increased sensitivity of immunoelectron microscopy all cases of polymorphic eruption of pregnancy were completely negative. [ncbi.nlm.nih.gov]
[…] her·pes ges·ta·tio·'nis a polymorphous, bullous eruption, more common on the extremities and abdomen than on the upper trunk, with the appearance of pemphigoid or dermatitis herpetiformis; beginning in the second or third trimester, flaring about the [medical-dictionary.thefreedictionary.com]
Patient had similar but less severe eruption in the last trimester of her third pregnancy, one year back, and the baby died 3 days after a preterm vaginal delivery. [edoj.org.eg]
Polymorphic eruption of pregnancy morphology: coalescing urticarial papules on the abdomen. Fig. 10. Polymorphic eruption of pregnancy morphology: urticarial plaques on the lateral surface of the upper thigh. Fig. 11. [glowm.com]
- Pruritus
Intense pruritus is the consistent hallmark of PG, while the clinical course is variable. [edoj.org.eg]
To sum up, rare as it is, gestational pemphigoid should be considered in every case of pruritus and vesiculobullous rash in a pregnant or postpartum patient. [dovepress.com]
The condition involves mainly the skin, and results in bulous lesions with intense pruritus, which can sometimes be very difficult to deal with. [scielo.conicyt.cl]
It is characterized by maternal pruritus affecting the palms and soles, and increased rates of preterm labor and adverse fetal outcomes. 4, 5 There are no dermatologic features, except for excoriation from the pruritus. [aafp.org]
- Skin Disease
A rare pregnancy-associated autoimmune skin disease that is characterised by an itchy rash that develops into blisters. It is most common during the second and third trimesters of pregnancy. [ebi.ac.uk]
Black Journal of the American Academy of Dermatology 1992 BACKGROUND Herpes gestationis (HG) is an autoimmune disease of the skin that occurs exclusively in association with pregnancy (or… (More) [semanticscholar.org]
Abstract Autoantibodies associated with herpes gestationis (HG), a pregnancy-associated autoimmune skin disease, target the hemidesmosomal protein BP180. [ncbi.nlm.nih.gov]
[…] her·pes ges·ta·ti·o·nis ( hĕr'pēz jes-tā'shē-ō'nis ) A polymorphous, bullous eruption beginning in the second or third trimester of pregnancy, flaring about the time of delivery, and subsequently resolving. herpes gestationis An uncommon skin disease [medical-dictionary.thefreedictionary.com]
Pemphigoid gestationis is a rare pregnancy-associated autoimmune skin disease that is characterised by an itchy rash that develops into blisters. It is most common during the second and... [medicaldialogues.in]
- Erythema
In toxic erythema of pregnancy prominent striae were frequently present. [ncbi.nlm.nih.gov]
Herpes gestationis can be confused with erythema multiforme. [patientcareonline.com]
A 27-year-old Japanese female in the 36 th week of her first pregnancy had a pruritic infiltrative erythema on the extremities. [ijdvl.com]
Urogenital
- Cesarean Section
She indicated that these lesions started appearing 2 days following the delivery of her fourth child by a cesarean section. [jpgo.org]
Can you be a surrogate if you’ve had C-sections? Having a previous cesarean-section will not automatically disqualify you from becoming a surrogate. [surrogate.com]
If tests show the virus to be active or you have a herpes genital lesion close to your delivery date, your doctor may recommend having a cesarean section instead of a vaginal birth. [webmd.com]
If the patient has any genital lesions or prodromal symptoms at the onset of labor, then she will require delivery by cesarean section to reduce the likelihood of vertical transmission to the neonate, which can be fatal. 1-2 Gestational pemphigoid, or [mdmag.com]
Neurologic
- Headache
An initial infection starts after an incubation period of 3-7 days, after which fever, headaches, fatigue and muscle pains can occur. [americanskin.org]
Episodes of flushing, myalgia, headaches, nausea and vomiting, tachycardia, and a slight increase in blood pressure have been reported in most cases between 30 and 60minutes after starting infusion.3 Anaphylaxis is very rare and has mainly occurred in [actasdermo.org]
Possible side-effects of aciclovir include skin rash, light sensitivity, headache, dizziness, tummy upset and tirednesss. [netdoctor.co.uk]
[…] gestational diabetes reported that the patient was successfully treated with IVIG, and symptoms improved within 4 weeks of therapy.10 IVIG has also been used successfully as a steroid-sparing agent in antepartum PG.26 The most common side effects are headaches [emjreviews.com]
The first set symptoms are: Itching sensations Burning sensations Tingling sensations Sores/Blisters Lesions Discharge – vaginal Painful urination Swollen lymph nodes in the groin area Flu-like symptoms – fever, headaches, muscle aches Some of these symptoms [herpeshelper.net]
- Seizure
発作 8 Seizure anoxic 無酸素発作 8 Seizure cluster 発作の群れ 8 Seizure like phenomena 発作的な現象 8 Seizure prophylaxis 発作の予防 8 Sensation of foreign body 異物感 8 Septic embolus 敗血症性塞栓 8 Septic pulmonary embolism 敗血症性肺塞栓症 8 Severe acute respiratory syndrome 重症急性呼吸器症候群 [ikenori.com]
In patients with pustular psoriasis of pregnancy it is essential to closely monitor electrolytes, especially calcium, as hypocalcemia can lead to delirium, seizures and tetany. [cancertherapyadvisor.com]
Meningo-encephalitis Features of meningo-encephalitis include: isolated or part of disseminated disease presents with: encephalopathy (mean 11 days of age) seizures are common and often intractable absent gag-reflex is a particular feature. [bettersafercare.vic.gov.au]
Transmission typically occurs during labor and delivery and may cause death, neurologic deficits, blindness, seizures, and learning disabilities ( 37, 38 ). [msphere.asm.org]
Hypocalcemia may be present, possibly related to hypoparathyroidism [ 68 ] and can lead to tetany, delirium, and seizures. Albuminuria, hypoalbuminemia, pyuria, and hematuria occasionally occur. [uptodate.com]
Workup
Biopsy and immunohistology are both used in the workup of the disease. [arupconsult.com]
Sections Pemphigoid Gestationis Overview Practice Essentials Pathophysiology Epidemiology Prognosis Patient Education Show All Presentation History Physical Examination Show All DDx Workup Laboratory Studies Histologic Findings Show All Treatment Medication [emedicine.medscape.com]
HLA Type
- HLA-B8
In herpes gestationis, immunofluorescence studies were consistently positive, there was a high frequency of HLA-B8 and an association with autoimmune thyrotoxicosis. Toxic erythema of pregnancy did not share these immunological features. [ncbi.nlm.nih.gov]
An additional laboratory finding in patients with HG is their increased frequency of the HLA-B8 and HLA-DR3 haplotypes as well as the HLA-DR3, -DR4 paired haplotype. [pubmed.ncbi.nlm.nih.gov]
- HLA-A1
Immunogenetic studies reveal an increase in HLA antigens DR3 or DR4, and curiously, nearly 50% of patients have the simultaneous presence of both.22 The extended haplotype HLA-A1, B8, DR3 is known to be in linkage disequilibrium with a deletion of C4A [accessmedicine.mhmedical.com]
Immunogenetic studies reveal an increase in HLA antigens DR3 or DR4, and curiously, nearly 50 percent of patients have the simultaneous presence of both. 21 The extended haplotype HLA-A1, B8, DR3 is known to be in linkage disequilibrium with a deletion [dermaamin.com]
Treatment
Treatment The main goal of the treatment is to reduce pruritus and prevent the formation of new blisters. The treatment strategy depends on the severity of the affected skin lesions and whether our patient is pregnant or postpartum. [dovepress.com]
We report this case of pemphigoid gestationis in view of the importance of early diagnosis and treatment for prevention of fetal risk. [omicsonline.org]
Treatment is challenging in that the disease is extremely rare to allow for controlled studies and most of the treatment options are based on case reports and clinical experience. [scienceopen.com]
View Figure 2 Figure 3: (a) Affection of the anterior thorax; (b) Post-treatment. View Figure 3 Figure 4: (a) Affection of the lower extremities; (b) Post-treatment. [clinmedjournals.org]
[…] by rituximab. ( 27606493 ) Tourte M....Descamps V. 2016 17 Successful treatment of a severe early onset case of pemphigoid gestationis with intravenous immunoglobulin in a twin pregnancy conceived with in vitro fertilisation in a primigravida. ( 27922278 [malacards.org]
Prognosis
A new assessment of incidence, diagnosis,and fetal prognosis. Am J Obstet Gynecol. 1969;104:39-45. 4. Holmes RC, Black MM. The fetal prognosis in pemphigoid gestationis(herpes gestationis). Br J Dermatol. 1984;110:67-72. 5. Shornick JK. [patientcareonline.com]
The incidence, classification, aetiology, natural history, diagnosis, prognosis and therapy of the disease are briefly discussed. [ncbi.nlm.nih.gov]
Prognosis The disease is typically self-limiting; however, onset in the first or second trimester and presence of blisters is associated with adverse pregnancy outcomes. [orpha.net]
Prognosis of the fetus is usually good, but the rate of premature births and small-for-date babies rises. [wikidoc.org]
Etiology
Etiology and pathophysiology are not established, but an immunological basis is implicated by the frequent finding of C 3 along the basement membrane zone of perilesional skin, the presence of herpes gestationis factor, increased incidence of HLA-DR3 [jhu.pure.elsevier.com]
Its etiology is unknown, but it is considered as an autoimmune-mediated dermatosis closely related to the pemphigoid group. [ncbi.nlm.nih.gov]
The etiology of herpes gestationis is uncertain. [popline.org]
Epidemiology
Patient Population: Prevalence and Epidemiology Knee // Shoulder & Elbow // Hip // Spine // Foot & Ankle // Hand & Wrist Fragility Fractures: Diagnosis and Treatment Shoulder & Elbow The Characteristics of Surgeons Performing Total Shoulder Arthroplasty [amjorthopedics.com]
Summary Epidemiology The estimated incidence ranges between 1/3,000-60,000 pregnancies. [orpha.net]
Epidemiology PG is very rare and affects only about 1 person in 50,000-2 million pregnancies (depending on HLA type prevalence).[3] The association with HLA-DR3 and HLA-DR4 haplotypes is reflected in a higher incidence in Caucasians.[4] Presentation[4 [patient.info]
Epidemiology Frequency United States In the United States, pemphigoid gestationis has an estimated prevalence of 1 case in 50,000 pregnancies. [3] International Findings from European studies suggest that pemphigoid gestationis has an overall incidence [emedicine.medscape.com]
Pathophysiology
Etiology and pathophysiology are not established, but an immunological basis is implicated by the frequent finding of C 3 along the basement membrane zone of perilesional skin, the presence of herpes gestationis factor, increased incidence of HLA-DR3 [jhu.pure.elsevier.com]
However, because no control bullous pemphigoid sera showed this reactivity, it may suggest a different pathophysiology between herpes gestationis and bullous pemphigoid. [ncbi.nlm.nih.gov]
Thus, the pathophysiology of these latter two conditions is considered in more detail in the following article. [doi.org]
[…] autoimmune bullous dermatosis pathophysiology antibodies against hemidesmosomal proteins undetermined trigger Associated conditions other autoimmune diseases such as Hashimoto's thyroiditis Grave's disease pernicious anemia Prognosis increased incidence [medbullets.com]
Prevention
We report this case of pemphigoid gestationis in view of the importance of early diagnosis and treatment for prevention of fetal risk. [omicsonline.org]
[…] relief of symptoms and the prevention of secondary infection. [ozarkderm.com]
Fortunately, treatment with systemic corticosteroids used in PG does not prevent breastfeeding.5 Second-generation antihistamines are recommended to control pruritus. [dovepress.com]
Anyone who works in a high-risk setting and is exposed to blood should get a vaccine against hepatitis B to prevent infection. [webmd.com]
I have genital herpes – how can I prevent my baby from getting the virus? Firstly, make sure you tell your health care provider that you have genital herpes. Antiviral medication may be offered in late pregnancy to prevent or reduce recurrence. [pregnancyinfo.ca]