Hindawi Publishing Corporation
Case Reports in Obstetrics and Gynecology
Volume 2013, Article ID 267268, 5 pages
http://dx.doi.org/10.1155/2013/267268

Case Report
Pemphigoid Gestationis after Spontaneous Expulsion ofa Massive Complete Hydatidiform Mole
Naoki Matsumoto,1,2 Marie Osada,1 Kou Kaneko,3 Ken Ohara,4 Daito Noguchi,1Haruhiko Udagawa,1 Nagazumi Suzuki,1 Chieko Matsumoto,1 and Sachio Takahashi1
1Department of Obstetrics and Gynecology, Fukaya Red Cross Hospital, 5-8-1 Kamishibachonishi,Fukaya City, Saitama 366-0052, Japan2
Department of Obstetrics and Gynecology, Tatedebari Sato Hospital, 96 Wakamatsucho, Takasaki City, Gunma 370-0836, Japan3
Department of Pathology, Fukaya Red Cross Hospital, 5-8-1 Kamishibachonishi, Fukaya City, Saitama 366-0052, Japan
4
Department of Obstetrics and Gynecology, Saitama Medical Center, Saitama Medical University,
1981 Kamoda, Kawagoe City, Saitama 350-8550, Japan
Correspondence should be addressed to Naoki Matsumoto; research@matsumotoc.org
Received 18 May 2013; Accepted 5 August 2013
Academic Editors: C.-C. Liang and E. ShalevCopyright © 2013 Naoki Matsumoto et al. This is an open access article distributed under the Creative Commons Attribution
License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly
cited.
Pemphigoid gestationis (PG) is a rare, perinatal, autoimmune, and blistering dermatosis. Only few cases of PG involving
hydatidiform moles have been reported. Complete hydatidiform moles are usually evacuated by dilatation and curettage. We reporta patient with a massive complete hydatidiform mole that underwent spontaneous expulsion; she subsequently developed PG. A19-year-old unmarried nulligravid woman was referred to our hospital following excessive vaginal bleeding after an uncertain
amenorrheal period. The patient presented with preshock vital signs, severe anemia, and a positive urine pregnancy test. Imaging
examinations revealed a massive intrauterine mass (19 × 15 × 10 cm), suggesting a complete hydatidiform mole. She was hospitalized
and treated with blood transfusion. Sixteen hours after hospitalization, the massive molar mass underwent spontaneous expulsion
and bleeding ceased. Three days after the expulsion, she developed pruritic skin lesions including papules, erythemas, and bullae,
which spread over her entire body. Skin biopsy revealed PG and subepidermal blister formation and linear complement C3
deposition along the basement membrane zone, and the serum anti-BP180 antibody level was found to be high on measurement.
She was effectively treated with 50 mg/day of oral prednisolone. Her skin lesions disappeared, leaving pigmentation.

1. Introduction
Recently, in Japan, almost all pregnancies, irrespective of normal or abnormal, are examined during the early gestationalweeks. When a hydatidiform mole in the uterus is suspectedfollowing an imaging examination, such as ultrasonography,
it is usually evacuated by dilatation and curettage (D&C) for
histopathological diagnosis and treatment. However, massive
moles are occasionally difficult to remove by D&C.
Pemphigoid gestationis (PG), which has previously beencalled “herpes gestationis,” is a rare, autoimmune, selflimiting, and blistering dermatosis associated with pregnancy[1]. Its incidence is considered to be 1 in 50,000 to 60,000
pregnancies [2]. Hydatidiform moles occur in about 1 in 1,200

to 1,500 pregnancies [3, 4]. Therefore, PG in hydatidiform
mole patients is considered extremely rare.
We report a patient who presented with a massive
complete hydatidiform mole that underwent spontaneous
expulsion; she subsequently developed PG. We report asummary of our patient from obstetrical and gynecologicalaspects.

2. Case Presentation
The patient was a 19-year-old unmarried nulligravid Japanese
woman with no relevant past history. Her sexual partner wasa 40-year-old Japanese man. On September 23, 2007, she wasreferred to the emergency care unit of Fukaya Red Cross

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Case Reports in Obstetrics and Gynecology

(a)

(b)

(c)

(d)

Figure 1: Images of the complete hydatidiform mole. (a) Transvaginal ultrasonography (sagittal). (b) The magnetic resonance image (T2weighted, sagittal) shows a massive intrauterine mass (19 × 15 × 10 cm) with many small vesicles, no normal gestational sac, and no fetus. (c)The macroscopic image of the expelled mole that has a classical bunch of grapes appearance. (d) The microscopic image (hematoxylin andeosin staining) shows the edematous and swollen villi with circumferential trophoblastic proliferation (as the arrow indicates).
Table 1: Abnormal values of clinical laboratory tests.
Hospital day

Events

1stOn admission

Laboratory tests and their values
Hb 3.7 g/dL
WBC 22,100/mL (Neu 62.6%, Eo 4.3%, Ly 26.8%)
Serum creatinine 1.7 mg/dL, blood urea nitrogen 31 mg/dL

3rd

After blood transfusion and expulsion of the moleHb 6.9 g/dL
Serum hCG 80,700 mIU/mL

13th

After diagnosis of PG and before oral prednisolone
administration

WBC 20,600/mL (Neu 68.4%, Eo 14.4%, Ly 12.5%)
Serum immunoglobulin E 3970 U/mL
Anti-BP180-NC16a antibody index 360†

Eo: eosinophils; Hb: hemoglobin; hCG: human chorionic gonadotropin; Ly: lymphocytes; Neu: neutrophils; PG: pemphigoid gestationis; WBC: white blood
cell count.
†The normal limit of this index is <9.

Hospital, Saitama, Japan. Her chief complaint was excessive
vaginal bleeding after an uncertain period of amenorrhea. She
presented with preshock vital signs: a clear conscious state,
no fever, a pulse rate of 124 bpm, and a blood pressure of112/62 mmHg. Her hemoglobin concentration was 3.7 g/dL
and a qualitative urine human chorionic gonadotropin (hCG)
test was positive. Both ultrasonography (Figure 1(a)) andmagnetic resonance imaging (Figure 1(b)) showed a massive
intrauterine mass (19 × 15 × 10 cm) with many small vesicles,

no normal gestational sac, and no fetus; these featuresstrongly suggested the diagnosis of a complete hydatidiform
mole. Other abnormal values of clinical laboratory tests areshown in Table 1.
She was immediately hospitalized and treated with fluid
infusion and blood transfusion. Furthermore, it was considered that D&C is necessary. However, the D&C posed arisk of more bleeding and perforation because the uterus
was extremely enlarged and its muscular wall was very thin.

Case Reports in Obstetrics and Gynecology

3(a)

(b)

(c)

(d)

Figure 2: Macroscopic skin lesions including many tense bullae and edematous erythemas. (a) On the hands. (b) On the femurs. (c) On theneck. (d) On the abdomen.

Hence, we were reluctant to immediately perform a D&C.
Her vital signs were stable, but vaginal bleeding continuedintermittently. Therefore, we considered that we should carefully attempt a suction D&C using drugs that would prompt
uterine contractions. Sixteen hours after hospitalization, thebleeding suddenly increased, followed by spontaneous expulsion of a large mass. The uterus immediately contracted tothe size of an orange, reducing further bleeding. Macroscopic(Figure 1(c)) and microscopic (Figure 1(d)) examinations of
the mass confirmed the diagnosis of a complete hydatidiform
mole. She underwent suction D&C after the spontaneousexpulsion to ensure complete evacuation of the mole. Her
mild renal dysfunction had been gradually improving without proteinuria and oliguria.
Three days after expulsion of the mole, she developed
pruritic skin lesions. The lesions included papules and erythemas, which appeared on the dorsum of her hands andthe anterior part of the chest at first. She was initially treatedwith an oral antihistamine and a local corticosteroid, but thetreatment was not effective. Some of the lesions soon transformed into vesicles and bullae, which subsequently spreadover her entire body (Figures 2(a)–2(d)). We further decided
to consult dermatologists. A skin biopsy revealed subepidermal blister formation (Figure 3(a)). Linear complement
C3 deposition was observed along the basement membranezone on direct immunofluorescence analysis (Figure 3(b)).
Additional laboratory tests were performed to assess the dermatosis (Table 1). Eosinophilia appeared after the eruption.

Her serum immunoglobulin E level and anti-BP180-NC16a
antibody index (Mesacup BP180 Test, Medical & Biological
Laboratories Co., LTD) [5, 6] were very high (Table 1).
Therefore, the skin lesions were diagnosed as PG. She wastransferred to the Jichi Medical University Hospital, Tochigi,Japan, because of geographical reasons. She further received
30 mg/day (0.6 mg/kg/day) oral prednisolone; however, neweruptions occurred after a few days. Therefore, the dose wasincreased to 50 mg/day (1 mg/kg/day), which proved to bevery effective. Prednisolone treatment was gradually reduced
and stopped after 10 weeks.
The patient’s serum hCG level propitiously decreased
and became undetectable 12 weeks after expulsion of themole. Sixteen weeks after expulsion, the hCG level remained
undetectable and the skin lesions had disappeared without
recurrence but leaving pigmentation. However, she did notvisit the hospital after the above-mentioned consecutive
treatments, so no information regarding her further course
was available.

3. Discussion
We have shown that spontaneous expulsion of a large molar
mass does occur. In Japan, recently, almost all pregnancies
are examined in the early gestational weeks. A suspectedhydatidiform mole will usually be evacuated by D&C as
early as possible [3]. No randomized controlled studies exist
on the method of evacuation of molar diseases. However,4

Case Reports in Obstetrics and Gynecology

(a)

(b)

Figure 3: Biopsy specimens of the skin lesions. (a) The hematoxylin and eosin staining shows subepidermal blister formation (as the starindicates) with cellular infiltration composed mainly of lymphocytes with numerous eosinophils. (b) Direct immunofluorescence shows linear
complement C3 deposition (as the arrow indicates) along the basement membrane zone.
Table 2: Review of the literatures. Our patients and 4 previously reported molar cases which developed pemphigoid gestationis.
YearAuthors

Patient’s age (y)Previous gestations

DIF used for diagnosis

The first skin lesions were seen

1950
1974

Tillman [9]Dupont [10]†42
—

2—No
—

7 days after abortion—

1975
Yasue [11]1981 Tindall et al. [12]

53
28

45Yes
Yes

Before D&C
3 days after D&C

2013

190Yes3 days after spontaneous expulsion and D&C

This report

D&C: dilatation and curettage; DIF: direct immunofluorescence.
All cases were complete hydatidiform moles.
†We could not obtain details of the literature.

D&C is considered to be the preferred treatment for theevacuation of moles; moreover, suction D&C is preferred oversharp D&C [7, 8]. Medical induction of labor with oxytocin
or prostaglandins is not recommended [8]. In our patient,
we were apprehensive about performing a D&C because
the risk of bleeding and perforation was considered veryhigh. Fortunately, the massive mole underwent spontaneousexpulsion, and further bleeding was prevented. Because ofthis unanticipated spontaneous event, we could avoid thehigh risk D&C.
We have discussed here the practical, diagnostic, andtherapeutic aspects of PG. PG is a perinatal, autoimmune,pruritic, and vesiculobullous skin disorder, pathophysiologically resembling bullous pemphigoid. PG also occurs
in moles [9–12] and choriocarcinomas [13, 14]. PG should
be differentiated from several perinatal pruritic dermatoses
such as pruritic urticarial papules and plaques of pregnancy.
The three chief symptoms required for diagnosis of PG
are as follows: tense subepidermal blisters, complement C3
and/or immunoglobulin G deposition along the basement
membrane zone on direct immunofluorescence, and serumanti-BP180-NC16 antigen [2]. In the treatment of PG, oralcorticosteroids are standard [2]. Prednisolone is usually
started at a dose of 20–40 mg/day, and higher doses may benecessary in severe cases [1].
We report an extremely rare case, in which the patientpresented with both complete hydatidiform mole and PG.

Four previous cases in which PG coincided with a hydatidiform mole have been reported [9–12]. A summary of thesecases and our patient is presented in Table 2. The incidence of
molar diseases is rare and that of PG is even rarer; therefore,
patients with both of these conditions are extremely rare.
Prompt and precise diagnosis of PG remains challenging for
obstetricians and gynecologists; however, we hope that thisreport will help them to diagnose and treat similar cases.

Conflict of Interests
This study received no funding support, and all authors
declare no conflict of interests associated with this report.

Acknowledgments
A summary of this report was presented at the 65th Academic
Conference of the Japan Society of Obstetrics and Gynecology, Sapporo City, Hokkaido, Japan, 2013. The patient in thisreport was previously presented by Dr. Yuka Takatsuka andher colleagues, Department of Dermatology, Jichi MedicalUniversity, Tochigi, Japan. In their Letter to the Editor[15], they presented the patient’s dermatological results anddemonstrated the difference in T-cell profile on the lesions ofPG and the complete hydatidiform mole in the same patient.
The authors would like to thank them for their appropriate

Case Reports in Obstetrics and Gynecology
dermatological therapy for the patient and for providing
pictures (Figures 3(a) and 3(b)) for their report.

References[1] K. Semkova and M. Black, “Pemphigoid gestationis: current
insights into pathogenesis and treatment,” European Journal ofObstetrics Gynecology and Reproductive Biology, vol. 145, no. 2,pp. 138–144, 2009.
[2] J. Lipozenčić, S. Ljubojevic, and Z. Bukvić-Mokos, “Pemphigoidgestationis,” Clinics in Dermatology, vol. 30, no. 1, pp. 51–55,
2012.
[3] Japanese Society of Obstetrics and Gynecology and TheJapanese Society of Pathology, Eds., The General Rules For Clinical and Pathological Management of Trophoblastic Disease,
Kaneharashuppan, Tokyo, Japan, 3rd edition, 2011, (Japanese).
[4] R. S. Berkowits and D. P. Goldstein, “Gestational trophoblasticdiseases,” in Principals and Practice of Gynecologic Oncology,
W. J. Hoskins, C. A. Perez, and R. C. Young, Eds., pp. 1117–1137, Lippincott Williams & Wilkins, Philadelphia, Pa, USA, 3rdedition, 2000.
[5] K. Matsumura, M. Amagai, T. Nishikawa, and T. Hashimoto,“The majority of bullous pemphigoid and herpes gestationis
serum samples react with the NC16a domain of the 180kDa bullous pemphigoid antigen,” Archives of DermatologicalResearch, vol. 288, no. 9, pp. 507–509, 1996.
[6] M. Kobayashi, M. Amagai, K. Kuroda-Kinoshita et al., “BP180ELISA using bacterial recombinant NC16a protein as a diagnostic and monitoring tool for bullous pemphigoid,” Journal of
Dermatological Science, vol. 30, no. 3, pp. 224–232, 2002.
[7] J. B. Schlaerth, C. P. Morrow, F. J. Montz, and G. d’Ablaing,“Initial management of hydatidiform mole,” American Journalof Obstetrics and Gynecology, vol. 158, no. 6, part 1, pp. 1299–1306, 1988.
[8] J. T. Soper, D. G. Mutch, and J. C. Schink, “Diagnosis andtreatment of gestational trophoblastic disease: ACOG Practice
Bulletin No. 53,” Gynecologic Oncology, vol. 93, no. 3, pp. 575–585, 2004.
[9] W. G. Tillman, “Herpes gestationis with hydatidiform mole andchorion epithelioma,” British medical journal, vol. 1, no. 4668, p.
1471, 1950.
[10] C. Dupont, “Herpes gestationis with hydatidiform mole,” Transactions of the St. John’s Hospital Dermatological Society, vol. 60,no. 1, p. 103, 1974.
[11] T. Yasue, “On the immumopathologic and serologic findings ina case of bullous dermatosis with hydatidiform mole. Herpesgestationis? (AUTHOR’S TRANSL),” Nippon Hifuka GakkaiZasshi, vol. 85, no. 4, pp. 251–260, 1975.
[12] J. G. Tindall, T. H. Rea, I. Shulman, and F. P. Quismorio Jr.,
“Herpes gestationis in association with a hydatidiform mole.
Immunopathologic studies,” Archives of Dermatology, vol. 117,
no. 8, pp. 510–512, 1981.
[13] L. Slazinski and S. Degefu, “Herpes gestationis associated withchoriocarcinoma,” Archives of Dermatology, vol. 118, no. 6, pp.
425–428, 1982.
[14] S. Djahansouzi, C. Nestle-Kraemling, P. Dall, H. G. Bender, andB. Hanstein, “Herpes gestationis may present itself as a paraneoplastic syndrome of choriocarcinoma—a case report,” Gynecologic Oncology, vol. 89, no. 2, pp. 334–337, 2003.
[15] Y. Takatsuka, M. Komine, and M. Ohtsuki, “Pemphigoid gestationis with a complete hydatidiform mole,” Journal of Dermatology, vol. 39, no. 5, pp. 474–476, 2012.

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Case Reports in Obstetrics and Gynecology
Volume 2013, Article ID 267268, 5 pages
http://dx.doi.org/10.1155/2013/267268

Case Report
Pemphigoid Gestationis after Spontaneous Expulsion of a Massive
Complete Hydatidiform Moleᕧ኱࡞ወ⫾ࢆ⮬↛ፔฟࡋࡓᚋ࡟ዷፎᛶ⑁⑈ࢆⓎ⑕ࡋࡓ඲⬊≧ወ⫾ᯇᮏ┤ᶞ㸪㛗⏣ࡲࡾ⤮㸪වᏊ⪔㸪኱ཎ೺㸪㔝ཱྀ኱ᩯᏱ⏣ᕝ἞ᙪ㸪㕥ᮌỌ⣧㸪ᯇᮏᬛᜨᏊ㸪㧗ᶫᖾ⏨᪥ᮏㄒせ⣙
࠙⫼ᬒࠚዷፎᛶ⑁⑈㸦PG㸧࡜ࡣ㸪ዷፎ࡟㛵㐃ࡋ࡚Ⓨ⑕ࡍࡿࡲࢀ࡞⮬ᕫච␿ᛶ⓶⭵⑌ᝈ࡛࠶ࡾ
Ỉ⑁ᙧᡂࢆ୺ᚩ࡜ࡍࡿࠋ⬊≧ወ⫾࡟㛵㐃ࡋࡓ PG ࡢሗ࿌ࡣᑡ࡞࠸ࠋ㏆ᖺ࡛ࡣ඲⬊≧ወ⫾ࡣᏊᐑෆ⭷ᥙ∐⾡࡟ࡼࡾ㝖ཤࡉࢀࡿࡇ࡜ࡀ࡯࡜ࢇ࡝࡛࠶ࡿࠋ௒ᅇ㸪ᕧ኱࡞඲⬊≧ወ⫾ࢆ⮬↛ፔฟࡋ㸪ࡉࡽ࡟ᘬࡁ⥆࠸࡚ PG ࢆⓎ⑕ࡋࡓ⑕౛ࢆ⤒㦂ࡋࡓࡢ࡛ሗ࿌ࡍࡿࠋ
࠙⑕౛ࠚ19 ṓዪᛶࠋᮍ፧㸪ᮍ⤒ዷࠋ᭱⤊᭶⤒୙ヲࠋ↓᭶⤒ᚋࡢከ㔞ᛶჾฟ⾑ࢆ୺ッ࡟ᙜ㝔ࢆᩆᛴึデࠋព㆑Ύ᫂ࡔࡀࣉࣞࢩࣙࢵࢡࡢ≧ែࠋHb 3.7 g/dLࠋዷፎ཯ᛂ㝧ᛶࠋ࢚ࢥ࣮࣭MRI ࡛ࡣ㸪Ꮚᐑ⭍ෆ࡟↓ᩘࡢᑠᄞ⬊ࢆకࡗࡓ 19×15×10 cm ࡢ඘ᐇᛶ⭘⒗ീࢆㄆࡵ㸪ᕧ኱࡞඲⬊≧ወ⫾ࢆ♧၀ࡋࡓࠋධ㝔࡜ࡋ㍺⾑࣭⿵ᾮ⟶⌮ࢆ㛤ጞࠋධ㝔ࡢ 16 ᫬㛫ᚋ㸪ฟ⾑ࡢቑ㔞࡜࡜ࡶ࡟࡟୍ሢࡢ඲⬊≧ወ⫾ࢆ⮬↛ፔฟࡋࡓࠋࡑࡢᚋ㸪Ꮚᐑࡣ⮬↛཰⦰ࡋṆ⾑ࡋࡓࠋወ⫾ፔฟࡢ 3 ᪥ᚋ㸪ᥙ⑛ࢆక࠺⣚ᩬ࣭Ỉ⑁ࢆㄆࡵ㸪ࡑࢀࡽࡣ඲㌟࡟ᗈࡀࡗ࡚࠸ࡗࡓࠋ⓶⭵⏕᳨࡛ࡣ⾲⓶ୗỈ⑁ᙧᡂࢆㄆࡵ㸪ࡲࡓ⺯ගᢠయ┤᥋ἲ࡟࡚⾲⓶ᇶᗏᒙ࡟⿵య C3 ࡢ⥺≧ỿ╔ࢆㄆࡵࡓࠋ⾑Ύ୰ᢠ
BP180 ᢠయ㝧ᛶࠋ௨ୖࡢᡤぢ࠿ࡽ PG ࡜デ᩿ࡋ㸪ࣉࣞࢻࢽࢰࣟࣥෆ᭹ 1 mg/kg/day ࢆ㛤ጞࠋྠ
἞⒪ࡣዌຌࡋ㸪ࡑࡢᚋ₞ῶࡋ࡞ࡀࡽᢞ୚ᮇ㛫 10 㐌㛫࡛⤊஢ࠋPG ࡣⰍ⣲ỿ╔ࢆṧࡋࡓࡀ἞⒵ࡋࡓࠋ⾑୰ hCG ್ࡣ㡰ㄪ࡟పୗࡋ㸪ወ⫾ፔฟᚋ 12 㐌᳨࡛ฟ୙⬟࡜࡞ࡗࡓࠋወ⫾ፔฟᚋ 16 㐌ࡲ࡛ࡢࣇ࢛࣮ࣟ࢔ࢵࣉ࡛ࡣ hCG ࡢ෌ୖ᪼ࡸ PG ࡢ෌⇞ࡣㄆࡵ࡞࠿ࡗࡓࠋ