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A Case of Paraneoplastic Pemphigus As a Preceding Manifestation of Underlying Follicular Lymphoma Treated with R-CHOP

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A Case of Paraneoplastic Pemphigus As a Preceding Manifestation of Underlying Follicular Lymphoma Treated with R-CHOP Paraneoplastic Pemphigus Treated with Rituximab pISSN 1013-9087ㆍeISSN 2005-3894 Ann Dermatol Vol. 33, No. 3, 2021 https://doi.org/10.5021/ad.2021.33.3.271 CASE REPORT A Case of Paraneoplastic Pemphigus as a Preceding Manifestation of Underlying Follicular Lymphoma Treated with R-CHOP Minsu Kim, Ji Yun Lee1, Soo-Chan Kim2, Jung-Im Na Departments of Dermatology and 1Internal Medicine, Seoul National University Bundang Hospital, Seongnam, 2Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea Paraneoplastic pemphigus is a rare, life-threatening disorder INTRODUCTION associated with an underlying neoplasm, which presents with painful stomatitis and polymorphous skin lesions. Suc- Paraneoplastic pemphigus (PNP) is an autoimmune bul- cessful diagnosis of paraneoplastic pemphigus can lead to lous disorder associated with an underlying neoplasm. the diagnosis and treatment of the underlying malignancy. The pathogenesis of PNP involves the production of auto- However, involvement of the respiratory system is typically antibodies against desmoglein and plakin proteins1. PNP unresponsive to treatment. Herein, we report the case of a is commonly misdiagnosed because it presents with vari- 44-year-old female diagnosed with paraneoplastic pemphi- ous clinical manifestations and often resembles other cuta- gus with underlying follicular lymphoma treated with a che- neous diseases. The histopathologic findings of PNP are motherapy regimen including rituximab. Her skin lesions also nonspecific and include interface dermatitis and li- and underlying lymphoma responded to treatment, but bron- chenoid dermatitis. The detection of autoantibodies against chiolitis obliterans continued to progress and resulted in fatal plakin proteins using indirect immunofluorescence (IIF) or respiratory failure. (Ann Dermatol 33(3) 271∼274, 2021) immunoblot shows high sensitivity and specificity for the diagnosis of PNP2. -Keywords- There is no standardized treatment guideline for PNP. Sys- Bronchiolitis obliterans, Follicular lymphoma, Paraneoplastic temic steroids are typically used as the first-line treatment, pemphigus, Rituximab and rituximab could be considered as a secondary treat- ment option. Although treatment of the underlying neo- plasm does not always lead to the treatment of PNP, the prognosis of PNP is determined by the nature of the un- derlying neoplasm1. PNP has a high mortality rate, and bronchiolitis obliterans (BO), which results from the in- Received November 26, 2019, Revised January 2, 2020, Accepted for publi- volvement of the respiratory system, is a common cause of cation January 9, 2020 death. Corresponding author: Jung-Im Na, Department of Dermatology, Seoul Na- tional University Bundang Hospital, 82 Gumi-ro 173beon-gil, Bundang-gu, Seongnam 13620, Korea. Tel: 82-31-787-7314, Fax: 82-31-787-4058, E-mail: CASE REPORT [email protected] ORCID: https://orcid.org/0000-0002-5717-2490 A 44-year-old female visited the emergency room due to This is an Open Access article distributed under the terms of the Creative a painful oral ulcer, which had developed 6 weeks prior. Commons Attribution Non-Commercial License (http://creativecommons. She had previously been diagnosed with aphthous stoma- org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work titis in the otolaryngology department and received sys- is properly cited. temic steroid treatment, but the oral lesion gradually pro- Copyright © The Korean Dermatological Association and The Korean gressed. She also complained of an erythematous mac- Society for Investigative Dermatology ulopapular rash covering her whole body and conjunc- Vol. 33, No. 3, 2021 271 M Kim, et al Fig. 1. Clinical images taken on the initial visit. Painful stomatitis (A) and a maculopapular eruption cover- ing the whole body (B). tival injection accompanied by fever, diarrhea, and weight loss (Fig. 1). The maculopapular eruption rapidly progres- sed to ulcerative patches, especially on her palms. She de- nied any medication history. Leukocytosis and eosino- philia were noted, but all other laboratory tests were with- in the normal ranges. Stevens-Johnson syndrome (SJS) with mucosal involvement was suspected, so she admitted to the internal medicine department for systemic steroid treat- ment. The skin lesion slightly improved after 1.5 mg/kg of meth- ylprednisolone therapy, but the stomatitis was unrespon- sive. The skin rashes recurred when the dose of methyl- prednisolone was reduced to below 1 mg/kg, and did not respond to cyclosporine or azathioprine. A skin biopsy was performed for further evaluation, and it showed inter- face dermatitis and subepidermal blister formation with Fig. 2. Histopathological examination showed subepidermal basal vacuolization. Dyskeratotic cells were detected in blister formation with interface dermatitis. Dyskeratotic cells the basal layer (Fig. 2). Direct immunofluorescence (DIF) were present in the basal layer and vacuolization was observed was performed twice, and showed a negative result and (H&E, ×100). linear deposits of C3 at the dermoepidermal junction, respectively. Antibodies against desmoglein 1 and 3 were However, the patient developed BO and invasive pulmo- not detected by enzyme-linked immunosorbent assay. IIF nary aspergillosis, and died from respiratory failure 20 was conducted, and immunoglobulin G deposition was months after the initial manifestation of PNP. detected on the normal human skin, mouse bladder epi- thelium and mouse lung epithelium (Fig. 3). A diagnosis of DISCUSSION PNP was made based on the IIF results. Chest and abdomen computed tomography were per- Anhalt et al.3 first described PNP as a rare autoimmune formed to detect the underlying neoplasm, and enlarge- blistering disease characterized by painful stomatitis and a ment of the axillary, supraclavicular, and retroperitoneal polymorphous cutaneous eruption accompanied by the lymph nodes was observed. Surgical excisional biopsy of production of autoantibodies. The associated neoplasms are the right inguinal lymph node was consistent with fol- mainly lymphoproliferative disorders. In contrast to other licular lymphoma. Due to bone marrow involvement, the autoimmune blistering diseases, PNP-associated autoanti- patient was diagnosed with stage IV follicular lymphoma. bodies invade not only stratified squamous epithelium but The patient received chemotherapy including rituximab, also other types of epithelium including the transitional cyclophosphamide, doxorubicin, vincristine, and predniso- epithelium of the bladder. When IIF is performed, these lone. Complete remission of the lymphoma was achieved autoantibodies react with rat bladder epithelium, so this after six cycles of chemotherapy, confirmed by positron diagnostic test can be used to distinguish PNP from other emission tomography. The stomatitis and skin rash were blistering diseases. also dramatically improved and well-maintained with The diagnosis of PNP is challenging due to its rare preva- low-dose steroid therapy (5 mg/day) for 8 months (Fig. 4). lence and various cutaneous manifestations. Given its 272 Ann Dermatol Paraneoplastic Pemphigus Treated with Rituximab Fig. 3. Indirect immunofluorescence showed positive results on (A) nor- mal human skin (1:10), (B) mouse bladder epithelium (1:40), and (C) mouse lung epithelium (1:10). of the underlying neoplasm. If the underlying tumor is be- nign or localized, PNP shows good prognosis and early re- section of the tumor can lead to a good outcome5. How- ever, treatment of the underlying neoplasm does not al- ways lead to the improvement of PNP1. Systemic steroids are typically used as the first-line treatment for PNP. Recently, several cases of PNP successfully treated with rituximab have been reported6-8. Rituximab is thought to act by depleting CD20-expressing B cells that produce au- toantibodies9. Our patient exhibited clinical remission of the underlying Fig. 4. The stomatitis (A) and skin eruption (B) entered clinical follicular lymphoma and cutaneous, mucosal involvement remission after rituximab treatment. of PNP after rituximab treatment. However even after re- mission, BO progressed and the patient died from respira- mucosal involvement, PNP is frequently misdiagnosed as tory failure. The exact mechanism underlying respiratory Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/ tract involvement remains unclear. Autoantibodies may re- TEN). Although the prevalence of SJS/TEN-like PNP is low- act with plakin proteins on the respiratory epithelium, and er than that of SJS/TEN, its mortality is much higher4. In destruction of the bronchial epithelium may lead to irre- some cases, PNP is diagnosed before the detection of an versible airway obstruction1. BO is resistant to conven- underlying malignancy, so early diagnostic work-up of the tional treatment for PNP, and lung transplantation is con- underlying neoplasm is necessary for treatment. Therefore, sidered the only effective treatment. A previous case re- the differential diagnosis of SJS and SJS/TEN-like PNP is port demonstrated that rituximab unsuccessfully treated important, and PNP should be considered in patients with BO, though evidence for the effectiveness of rituximab an atypical clinical course of SJS/TEN. against PNP-related BO is still lacking7. In this case, al- Anhalt et al.3 proposed five diagnostic criteria for PNP, in- though rituximab successfully treated
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