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Case for diagnosis.
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MedLine Citation:
PMID:  24626668     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
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.
Authors:
Rita Cabral; Vera Teixeira; Ana Brinca; Barbara Fernandes; José Pedro Reis
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Anais brasileiros de dermatologia     Volume:  89     ISSN:  1806-4841     ISO Abbreviation:  An Bras Dermatol     Publication Date:    2014 Jan-Feb
Date Detail:
Created Date:  2014-03-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0067662     Medline TA:  An Bras Dermatol     Country:  Brazil    
Other Details:
Languages:  eng     Pagination:  167-8     Citation Subset:  IM    
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Full Text
Journal Information
Journal ID (nlm-ta): An Bras Dermatol
Journal ID (iso-abbrev): An Bras Dermatol
Journal ID (publisher-id): An Bras Dermatol
ISSN: 0365-0596
ISSN: 1806-4841
Publisher: Sociedade Brasileira de Dermatologia
Article Information
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®2013 by Anais Brasileiros de Dermatologia
open-access:
Received Day: 18 Month: 1 Year: 2013
Accepted Day: 03 Month: 4 Year: 2013
Print publication date: Season: Jan-Feb Year: 2014
Volume: 89 Issue: 1
First Page: 167 Last Page: 168
PubMed Id: 24626668
ID: 3938374
DOI: 10.1590/abd1806-4841.20142456

Case for diagnosis*
Rita Cabral1
Vera Teixeira1
Ana Brinca2
Barbara Fernandes3
José Pedro Reis4
1 MD, Resident in the Dermatology Department, Coimbra University Hospital Center- Coimbra, Portugal.
2 MD, Resident in the Dermatology Department, Coimbra University Hospital Center- Coimbra, Portugal.
3 Graduate in the Dermatology Department, Portuguese Oncology Institute of Coimbra - Coimbra, Portugal.
4 Graduate in the Dermatology Department, Coimbra University Hospital Center - Coimbra, Portugal.
Correspondence: MAILING ADDRESS: Ana Rita Gomes da Cruz Rodrigues Cabral, Praceta Mota Pinto, 3000-075 Coimbra, Portugal. E-mail: ritaca@portugalmail.com

CASE REPORT

A 27-year-old woman at 28-weeks gestation presented with a widespread, pruritic eruption of macular, confluent lesions with tense vesicles and some blisters in the arms and thighs (Figure 1). The lesions initially presented at 26 weeks of gestation on the legs and spread to the abdomen, arms, and back. Past medical history included one prior abortion due to sicklecell disease, without any history of similar symptoms. The patient had been previously treated with methylprednisolone cream and oral cetirizine, with persistence of the skin lesions. A cutaneous biopsy was performed in lesional skin, showing the presence of multiple vesicles in the dermal-epidermal junction, filled by serosity and eosinophils. In the underlying dermis, a marked edema outlined a dermal-epidermal detachment, with a dense inflammatory infiltrate (predominantly with eosinophils) extending to the dermis (Figure 2). Direct immunofluorescence in perilesional noninvolved skin showed linear deposists of C3 at the basement-membrane zone (Figure 3).


DISCUSSION

Pemphigoid gestationis is a rare, autoimmune blistering dermatosis of pregnancy, with an incidence ranging up to 1:50.000~60.000 pregnancies depending on the prevalence of the HLA-haplotypes DR3 and DR4.1,2 PG typically develops in the second or third trimester of pregnancy, with an abrupt onset, but may appear any time during pregnancy or even in the immediate postpartum period. Severe pruritus is followed by the appearance of erythematous, urticarial papules and plaques that progress to tense vesicles and blisters. The lesions usually arise on the abdomen, often involving the umbilicus, and spread centrifugally, sparing face, palms, soles and mucous membranes (< 20% cases). Flares have been observed at or immediately after delivery1 , pre-menses and with the use of oral contraceptives (25% of patients).3-5

The criteria for the diagnosis for PG include an appropriate clinical presentation and specific histologic findings of a subepidermal blistering process and a linear C3 deposition along the basement membrane in direct immunofluorescence, with or without deposition of immunoglobulin G (20-25% of cases).

Treatment depends on the severity of the disease and aims to prevent blister formation and control pruritus. Mild cases may be treated with topical corticosteroids and oral antihistamines.1,4 Potent topical glucocorticoids, oral corticosteroids (prednisone 0.5~1 mg/kg/day), and oral antihistamines are reserved for more serious cases.6

In our case, clinical suspicion of PG was confirmed by histological and direct immunofluorescence findings and systemic treatment with methylprednisolone (0,5mg/Kg/day) was initiated during the pregnancy with gradual clinical improvement, despite a relapse after a first attempt to reduce the dosage, resulting in extension of the systemic treatment until delivery and 6 weeks after. A healthy, asymptomatic male infant was born without cutaneous lesions. To date the patient has not reported a flare with her menses. The present case corroborates the importance of a timely clinical and histopathological diagnosis of PG, thus preventing or minimizing the risk of adverse effects for the fetus. An interdisciplinary approach is also of crucial importance for the benefit of the pregnant woman and her pregnancy, and also for the infant, as well as during the postpartum period.7


Notes

*Work performed at the Coimbra University Hospital Center - Coimbra, Portugal.

Financial funding: None

Conflict of interest: None

REFERENCES
1. Ambros-Rudolph CM. Dermatoses of Pregnancy - Clues to Diagnosis, Fetal Risk and TherapyAnn DermatolYear: 20112326527521909194
2. Semkova K,Black M. Pemphigoid gestationis: current insights into pathogenesis and treatmentEur J Obstet Gynecol Reprod BiolYear: 200914513814419520487
3. Ambros-Rudolph CM,Müllegger 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 patientsJ Am Acad DermatolYear: 20065439540416488288
4. Jenkins RE,Hern S,Black MM. Clinical features and management of 87 patients with pemphigoid gestationisClin Exp DermatolYear: 19992425525910457123
5. Amato L,Mei S,Gallerani I,Moretti S,Fabbri P. A case of chronic herpes gestationis: persistent disease or conversion to bullous pemphigoid?J Am Acad DermatolYear: 20034930230712894083
6. Chi CC,Wang SH,Charles-Holmes R,Ambros-Rudolph C,Powell J,Jenkins R,et al. Pemphigoid gestationis: early onset and blister formation are associated with adverse pregnancy outcomesBr J DermatolYear: 20091601222122819298272
7. Alves GF,Nogueira LSC,Varella TCN. Dermatology and PregnancyAn Bras DermatolYear: 200580179186

Figures

[Figure ID: f01]
FIGURE 1 

Macular, confluent, pruritic lesions in the abdomen and arms of a 26 weeks pregnant woman



[Figure ID: f02]
FIGURE 2 

Cutaneous biopsy in lesional skin revealed a marked edema that outlined a dermal-epidermal detachment, with a dense inflammatory infiltrate (predominantly with eosinophils) extending to the dermis



[Figure ID: f03]
FIGURE 3 

Direct immunofluorescence in perilesional noninvolved skin showed linear deposists of C3 at the basement-membrane zone



Article Categories:
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Keywords: Pregnancy, Pruritus, Skin diseases, vesiculobullous.

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