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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 8  |  Issue : 3  |  Page : 147-151

Pemphigoid gestationis in a resource-limited setting


1 Department of Obstetrics and Gynaecology, Faculty of Clinical Sciences, College of Medicine, ABU, Zaria, Nigeria
2 Department of Internal Medicine, Faculty of Clinical Sciences, College of Medicine, ABU, Zaria, Nigeria

Date of Web Publication27-Sep-2019

Correspondence Address:
Dr. Fadimatu Bakari
Department of Obstetrics and Gynaecology, Faculty of Clinical Sciences, College of Medicine, ABU, Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_15_19

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  Abstract 


Pemphigoid gestationis is a rare autoimmune vesicobullous dermatosis that is unique to pregnancy. It commonly occurs during the second half of pregnancy, particularly the 3rd trimester. In general, it presents with intense, itchy erythematous papules and plaques that often spares the palms and soles. These lesions culminate to form intense bullae that often become secondarily infected if neglected. Vertical transmission of maternal antibodies in utero can occur and the infants could present with similar lesions for few weeks postdelivery. In resource-rich setting, the diagnosis is often confirmed with the direct immuno-fluorescence study, but in a resource-limited setting, a high index of suspicion is required to make a clinical diagnosis and commence prompt therapy to optimize maternal and fetal outcomes. We report a case of a 32-year-old grand multipara who presented at 34 weeks gestation with a 12-week history of infected skin lesions characteristic of pemphigoid gestationis. She was commenced on steroid therapy and antibiotics based on the clinical diagnosis. She went into preterm spontaneous labor at 36 weeks and delivered a boy baby who manifested with similar lesions at 2 weeks of live.

Keywords: Neonatal pemphigoid, pemphigoid gestationis, resource-limited setting


How to cite this article:
Bakari F, Dogbanya G, Muhammad TT, Umaru-Sule H, Sulayman HU, Avidime S, Adesiyun AG. Pemphigoid gestationis in a resource-limited setting. Arch Int Surg 2018;8:147-51

How to cite this URL:
Bakari F, Dogbanya G, Muhammad TT, Umaru-Sule H, Sulayman HU, Avidime S, Adesiyun AG. Pemphigoid gestationis in a resource-limited setting. Arch Int Surg [serial online] 2018 [cited 2019 Oct 19];8:147-51. Available from: http://www.archintsurg.org/text.asp?2018/8/3/147/268124




  Introduction Top


This rare pregnancy-associated autoimmune dermatosis occurs more in Caucasian's/Asians than in Negros and has an incidence that ranges between 1:2000 and 1:5000 depending on the prevalence of the HLA-haplotypes DR3 and DR4.[1] Multiparous women in their second- or third-trimester of pregnancy are commonly affected, but onset in the first trimester or postpartum period has also been reported.[2],[3] Recurrences in subsequent pregnancies are common and are usually more severe and with an earlier onset.[4] Generally, it presents with intense itchy erythematous papules and plaques that often spares the palms and soles. These lesions culminate to form intense bullae that often become secondarily infected if neglected. Vertical transmission of maternal antibodies in utero can occur and the infants could present with similar lesions for few weeks postdelivery. In resource-rich setting, the diagnosis is often confirmed with direct immunofluorescence (DIF) study, but in a resource-limited setting, a high index of suspicion is required to make a clinical diagnosis and commence prompt therapy to optimize maternal and fetal outcomes.

The aim of this case report is to raise awareness on the clinical presentation of this disease among clinicians, particularly obstetrician and general practitioners on the need for a high index of suspicion to prevent severe and widespread disease in the mother and adverse neonatal outcomes such as preterm delivery and neonatal pemphigoid gestationis.


  Case Report Top


Mrs. HS was a 32 year-old G8P6+1 3 Alive, whose estimated gestational age at presentation was 34 weeks. She presented with a 12-week history of generalized skin lesions that started when her pregnancy was about 22 weeks. The lesions started around her umbilicus as itchy reddish papules, which progressed to become tense vesicles. These vesicular lesions progressed to form blisters and bullae, which coalesced in areas to form ulcers. These lesions subsequently spread over 4 weeks to involve the whole of her trunk, her upper and lower limbs, the neck, and the face. The mucosal lining of her oral cavity and the genital regions were spared. Her soles and palms were also not involved. She developed a fever with chills and rigors at the onset of pussy discharge from the ulcers. These necessitated her to seek help from a health worker and the National Tuberculosis and Leprosy center, Saye, Zaria, Nigeria where she was commenced on medications and then subsequently referred to our hospital for expert management.

The index pregnancy was booked and had remained uneventful until the onset of rashes at 22 weeks gestation. All previous pregnancies were booked and supervised, but all her deliveries were at home except for the first delivery which was conducted in the hospital. She lost three children to childhood illnesses. She had no history of similar skin lesions in her previous pregnancies.

She attained menarche at 15 with normal menstrual flow and duration. Her past medical history was unremarkable, and she had no known drug allergy.

She was the first of three wives in a polygamous setting. She neither smoked cigarette nor ingested alcohol.

General physical examination revealed a young anxious woman who was ill-looking, febrile (37.8°C), and pale. She had generalized erythematous papules, blisters, and ulcers with areas of skin desquamation on her face, anterior and posterior trunk, and the limbs. Some of the lesions were secondarily infected and were discharging serosanguineous and pussy effluent [Figure 1], [Figure 2], [Figure 3], [Figure 4].
Figure 1: Extensive skin lesion on the neck and upper arm at presentation

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Figure 2: Extensive skin lesion on the back of the patient at presentation

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Figure 3: Extensive skin lesion on the chest and abdomen at presentation

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Figure 4: Extensive skin lesion on the legs at presentation

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Her pulse rate was 85 beats/min and her blood pressure was 110/80 mmHg. The symphysis-fundal height was 33 cm, which corresponded with her estimated gestational age. The fetal lie was longitudinal, and the presentation was cephalic. She had no uterine contractions, and fetal heart rate was regular. Pelvic examination revealed a cervix that was closed and uneffaced.

She was comanaged with the dermatologist, and a clinical diagnosis of pemphigoid gestationis was made. Blood samples were taken for investigations. She had a biophysical profile done, and all parameters were within the normal limits except for gross body movement. The ultrasound gestational age was 35 weeks. Her packed cell volume was 27%, and serum electrolytes and liver function test were within the normal limits. Skin biopsy of lesions was taken for histology but was not accepted in the laboratory because the payment was not done. Direct immunofluorescence assay was considered, but the facility for such an investigation was not available in our center. She was prescribed with 15 mg oral prednisolone daily, topical agents, including 1% hydrocortisone cream and dermazine cream. She was also prescribed a higher dose of hematinics. Prednisolone tablet was increased to 40 mg daily by the dermatologist after the 1st week. She was given empirical antibiotic for the infected skin lesions. By the 2nd week on admission, there were minimal new lesions, and the earlier lesions were desquamating and drying up [Figure 5].
Figure 5: Second week on admission

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At 36 weeks 5 days precisely, she went into spontaneous labor and had an uneventful spontaneous preterm delivery of a live male infant that weighed 2.7 kg with the good Apgar score. She did very well after delivery and was discharged home 48 h later with steroid therapy. Her baby developed similar but milder form skin lesions on the limbs and neck few days after delivery [Figure 6]. She was then seen at the postnatal clinic 2 weeks postpartum.
Figure 6: Mild skin lesion on the baby at 2 weeks

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The dose of prednisolone was reduced to 20 mg daily after delivery for 2 weeks, and then it was further tapered by 5 mg weekly before finally discontinuing at 2 months postpartum.


  Discussion Top


This rare autoimmune dermatosis of pregnancy is an important cause of intense unrelenting itching during pregnancy that is capable of causing maternal distress and limiting daily activities. If neglected, the blisters and bullae eruptions could become secondarily infected, leading to painful ulcers. Mrs. HS presented with these skin lesions at 22 weeks gestation which conforms to the most common time of presentation of the disease, but she only sought for medical care when the lesions became infected and painful 12 weeks after onset of symptoms.

The etiopathogenesis of the disease results from the binding of circulating auto-antibodies to the basement membrane at the dermoepidermal junction. This triggers an immune response by neutrophils and esinophils, leading to subepidermal vesicles and blistering of skin and mucus membrane.[5] The trigger for autoantibody production still remains elusive but is thought to result from recognition of placental tissue as antigenic leading to the immune response by cross-reaction with proteins in the maternal skin.[6]

Pemphigoid gestationis initially presents with intense erythematous urticarial patches and plaques, which are generally periumbilical.[7] These lesions progress to form tense vesicles and blisters. The rash spreads peripherally to involve the whole body but typically sparing the face, palms, and soles.[8] Mrs. HS was admitted with similar presentation except her face was not spared. This could be due to her late presentation that led to the widespread and severity of her disease condition. Periods of remission and flare-up can occur late in pregnancy and in the immediate postpartum period, respectively. This is in response to fluctuations in sex hormones. The disease is usually self-limiting. After delivery, these lesions usually resolve spontaneously within weeks to months.[5],[9]

Several methods have been employed in the diagnosis of pemphigoid gestationis. These methods can be used either singly or in combination depending on the available resources. These methods include clinical evaluation, histological findings, DIF or indirect immunofluorescence assay, enzyme-linked immunosorbent assay, and C4d immunochemistry can all be used to diagnose PG. However, DIF is the most commonly used method.[5] The diagnosis of pemphigoid gestationis in Mrs. HS was based on her clinical symptoms and signs. We had no available resources to do DIF assay, which is the most commonly used method in making a diagnosis. Scraping of skin lesion was taken for histological analysis, but the sample was not accepted in the laboratory because of lack of payment.

The main aim of therapy in women with pemphigoid gestationis is to relieve pruritus and prevent progression of the rashes to blisters and bullae formation. Therapy may also play a role in preventing preterm labor. Neonatal pemphigoid gestationis may also be prevented by decreasing the rate of placental transmission of maternal antibodies.[10] Remission of the disease was noticed after 1 week of commencing steroids in our patients. She had minimal new lesions and the old lesions were desquamating with no flare-up of lesions noticed [Figure 7] and [Figure 8].
Figure 7: Desquamating skin lesions on the face at one week following commencement of therapy

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Figure 8: Desquamating skin lesion

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She had spontaneous preterm labor close to term and was delivered of a live male baby that weighed 2.7 kg. The baby manifested with similar but transient skin lesion that resolved spontaneously after 2 weeks of live. Her skin lesions had completely healed by the 4th month postpartum. They were both followed up closely until 6 months postdelivery. She was properly counseled on her condition and the likelihood of it reoccurring in her subsequent pregnancies. She was discharged from the postnatal clinic and referred to our family planning unit for contraception.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Ambros-Rudolph CM. Dermatoses of pregnancy – Clues to diagnosis, fetal risk and therapy. Ann Dermatol 2011;23:265-75.  Back to cited text no. 1
    
2.
Ambros-Rudolph CM, Mullegger RR, Vaughan-Jones SA, Kerl H, Black MM. The specific dermatoses of pregnancy revisited and reclassified: results of a retrospective two-center study on 505 pregnant patients. J Am Acad Dermatol 2006;54:395-404.  Back to cited text no. 2
    
3.
Al-Saif F, Elisa A, Al-Homidy A, Al-Ageel A, Al-Mubarak M. Retrospective analysis of pemphigoid gestationis in 32 Saudi patients – Clinicopathological features and a literature review. J Reprod Immunol 2016;116:42-5.  Back to cited text no. 3
    
4.
Hallaji Z, Mortazavi H, Ashtari S, Nikoo A, Abdollahi M, Nasimi M. Pemphigoid gestationis: Clinical and histologic features of twenty-three patients. Int J Womens Dermatol 2017;3:86-90.  Back to cited text no. 4
    
5.
Sävervall C, Sand FL, Thomsen SF. Pemphigoid gestationis: Current perspectives. Clin Cosmet Investig Dermatol 2017;10:441-9.  Back to cited text no. 5
    
6.
Roth MM. Pregnancy dermatoses: Diagnosis, management, and controversies. Am J Clin Dermatol 2011;12:25-41.  Back to cited text no. 6
    
7.
Jenkins RE, Hern S, Black MM. Clinical features and management of 87 patients with pemphigoid gestationis. Clin Exp Dermatol 1999;24:255-9.  Back to cited text no. 7
    
8.
Bergman H, Melamed N, Koren G. Pruritus in pregnancy: Treatment of dermatoses unique to pregnancy. Can Fam Physician 2013;59:1290-4.  Back to cited text no. 8
    
9.
Vaughan Jones SA, Hern S, Nelson-Piercy C, Seed PT, Black MM. A prospective study of 200 women with dermatoses of pregnancy correlating clinical findings with hormonal and immunopathological profiles. Br J Dermatol 1999;141:71-81.  Back to cited text no. 9
    
10.
Şenturk S, Dilek N, Tekin YB, Çolak S, Gundogdu B, Güvendag Guven ES. Pemphigoid gestationis in a third trimester pregnancy. Case Rep Obstetr Gynecol 2014;1:4.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]



 

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