Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma
Pemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma.The patient...
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| Format: | Article |
| Language: | English |
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Elsevier
2020-05-01
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| Series: | Gynecologic Oncology Reports |
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| Online Access: | http://www.sciencedirect.com/science/article/pii/S2352578919301237 |
| _version_ | 1818159648698531840 |
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| author | Bijan Morshedi Kari Ring |
| author_facet | Bijan Morshedi Kari Ring |
| author_sort | Bijan Morshedi |
| collection | DOAJ |
| description | Pemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma.The patient is a 54-year-old Chinese female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her chest in February 2018. Given high suspicion for primary immunobullous disease despite negative immunofluorescence and lack of subepidermal split on initial biopsy, a repeat biopsy was performed from the right thigh showing positive intraepidermal “net-like” staining for C3 and IgG, but was negative for IgA, IgM, and fibrinogen. IgG antibodies against desmoglein 1 were elevated at 280u (reference range <18), but none resulted against desmoglein 3, consistent with pemphigus foliaceus. This patient’s PNP was resistant to treatment with azathioprine, dapsone, mupirocin cream, or betamethasone ointment, but responded to prednisone and rituximab per lymphoma protocol at 375 mg/m2 weekly for one month in December 2018.In February 2019, the patient had 2–3 episodes of postmenopausal vaginal bleeding and subsequent hysteroscopy with dilation and curettage revealed an undifferentiated uterine sarcoma. The patient underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, and bilateral pelvic lymph node sampling. After surgical staging, she noted significant improvement in her baseline skin lesions and has had no new lesions since surgery. Repeat desmoglein antibodies showed anti-Dsg1 antibodies of 32u (reference range <18) and anti-Dsg3 antibodies of 1u (reference range <19), as compared to the anti-Dsg1 antibodies of 280u in June 2018. She has since completed 4 cycles of adjuvant gemcitabine and docetaxel for her stage IIB undifferentiated uterine sarcoma with no recurrence of the pemphigus lesions. |
| first_indexed | 2024-12-11T15:49:20Z |
| format | Article |
| id | doaj.art-dde1bdb4878a404294984ddc71a69cc5 |
| institution | Directory Open Access Journal |
| issn | 2352-5789 |
| language | English |
| last_indexed | 2024-12-11T15:49:20Z |
| publishDate | 2020-05-01 |
| publisher | Elsevier |
| record_format | Article |
| series | Gynecologic Oncology Reports |
| spelling | doaj.art-dde1bdb4878a404294984ddc71a69cc52022-12-22T00:59:37ZengElsevierGynecologic Oncology Reports2352-57892020-05-0132Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcomaBijan Morshedi0Kari Ring1Corresponding author.; University of Virginia Health System, 1215 Lee St, Charlottesville, VA 22903, United StatesUniversity of Virginia Health System, 1215 Lee St, Charlottesville, VA 22903, United StatesPemphigus is a group of autoimmune intraepidermal blistering diseases caused by immunoglobulins directed against keratinocyte cell surface components. In this case report, we identify a non-classical paraneoplastic pemphigus (PNP) foliaceous related to an undifferentiated uterine sarcoma.The patient is a 54-year-old Chinese female with a past medical history of arthritis who presented with worsening fatigue in November 2017 and an itchy, blistering, erythematous annular plaque that first appeared on her chest in February 2018. Given high suspicion for primary immunobullous disease despite negative immunofluorescence and lack of subepidermal split on initial biopsy, a repeat biopsy was performed from the right thigh showing positive intraepidermal “net-like” staining for C3 and IgG, but was negative for IgA, IgM, and fibrinogen. IgG antibodies against desmoglein 1 were elevated at 280u (reference range <18), but none resulted against desmoglein 3, consistent with pemphigus foliaceus. This patient’s PNP was resistant to treatment with azathioprine, dapsone, mupirocin cream, or betamethasone ointment, but responded to prednisone and rituximab per lymphoma protocol at 375 mg/m2 weekly for one month in December 2018.In February 2019, the patient had 2–3 episodes of postmenopausal vaginal bleeding and subsequent hysteroscopy with dilation and curettage revealed an undifferentiated uterine sarcoma. The patient underwent an exploratory laparotomy, total abdominal hysterectomy, bilateral salpingo-oophorectomy, and bilateral pelvic lymph node sampling. After surgical staging, she noted significant improvement in her baseline skin lesions and has had no new lesions since surgery. Repeat desmoglein antibodies showed anti-Dsg1 antibodies of 32u (reference range <18) and anti-Dsg3 antibodies of 1u (reference range <19), as compared to the anti-Dsg1 antibodies of 280u in June 2018. She has since completed 4 cycles of adjuvant gemcitabine and docetaxel for her stage IIB undifferentiated uterine sarcoma with no recurrence of the pemphigus lesions.http://www.sciencedirect.com/science/article/pii/S2352578919301237Paraneoplastic pemphigusUndifferentiated uterine sarcomaUterine cancer pemphigusParaneoplastic syndrome |
| spellingShingle | Bijan Morshedi Kari Ring Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma Gynecologic Oncology Reports Paraneoplastic pemphigus Undifferentiated uterine sarcoma Uterine cancer pemphigus Paraneoplastic syndrome |
| title | Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma |
| title_full | Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma |
| title_fullStr | Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma |
| title_full_unstemmed | Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma |
| title_short | Cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma |
| title_sort | cutaneous paraneoplastic pemphigus syndrome associated with undifferentiated uterine sarcoma |
| topic | Paraneoplastic pemphigus Undifferentiated uterine sarcoma Uterine cancer pemphigus Paraneoplastic syndrome |
| url | http://www.sciencedirect.com/science/article/pii/S2352578919301237 |
| work_keys_str_mv | AT bijanmorshedi cutaneousparaneoplasticpemphigussyndromeassociatedwithundifferentiateduterinesarcoma AT kariring cutaneousparaneoplasticpemphigussyndromeassociatedwithundifferentiateduterinesarcoma |