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    Paraneoplastic pemphigus associated with chronic lymphocytic leukemia: A case report.
    Medicine (Baltimore) 2017 Feb;96(8):e6184
    Department of Hematology, The First Affiliated Hospital of Chongqing University of Medical Sciences, Chongqing, China.
    Rationale: Paraneoplastic pemphigus (PNP) is an autoimmune syndrome associated with neoplasms. The treatment approach principally includes suppressing the immunity, but its therapeutic effect is not satisfying.

    Patient Concerns: We report a case of paraneoplastic pemphigus linked to chronic lymphocytic leukemia in a 63-year-old man. Read More

    Paraneoplastic pemphigus associated with Castleman disease: Progression from mucous to mucocutaneous lesions with epitope-spreading phenomena.
    Br J Dermatol 2017 Feb 18. Epub 2017 Feb 18.
    Departments of Dermatology, Kindai University Faculty of Medicine, Osaka-Sayama, Japan.
    Paraneoplastic pemphigus (PNP) is a frequently fatal autoimmune blistering disease of the skin and mucous membranes.(1) PNP is commonly associated with malignant neoplasms or haematological disorders like Castleman disease (CD). The eruptions may resemble those seen in various other conditions such as lichen planus (LP), graft-versus-host disease, erythema multiforme (EM), bullous pemphigoid and pemphigus vulgaris (PV). Read More

    Non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans.
    J Dermatol 2016 Dec 3. Epub 2016 Dec 3.
    Department of Dermatology, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan.
    Bronchiolitis obliterans is a small-airway obstructive lung disease for which immunologically mediated pathogenesis is supposed. Frequent association of bronchiolitis obliterans with paraneoplastic pemphigus is well known, but its association with other autoimmune bullous diseases has not been reported except for a case of anti-laminin-332-type mucous membrane pemphigoid in a patient with chronic graft-versus-host disease. We report a case of non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans in a patient without transplantation. Read More

    Pemphigus: Pathogenesis to Treatment.
    R I Med J (2013) 2016 Dec 1;99(12):28-31. Epub 2016 Dec 1.
    Assistant Professor, Department of Dermatology, The Warren Alpert Medical School of Brown University.
    Pemphigus vulgaris (PV), pemphigus foliaceus (PF), and paraneoplastic pemphigus (PNP) are a group of rare and fatal blistering diseases involving autoantibodies that target desmosomal proteins. The pathogenesis of pemphigus involves the production of activated B-cells and IgG with stimulation by IL-4 by T-helper 2 cells. Clinically these diseases present most often with epidermal erosions of the mucosae and skin caused by rapid rupturing of flaccid bullae. Read More

    Paraneoplastic Pemphigus and Autoimmune Blistering Diseases Associated with Neoplasm: Characteristics, Diagnosis, Associated Neoplasms, Proposed Pathogenesis, Treatment.
    Am J Clin Dermatol 2017 Feb;18(1):105-126
    Department of Dermatology, University of Illinois College of Medicine, UIC-Derm, MC624, 808 S. Wood Street, R380, Chicago, IL, 60612, USA.
    Autoimmune paraneoplastic and neoplasm-associated skin syndromes are characterized by autoimmune-mediated cutaneous lesions in the presence of a neoplasm. The identification of these syndromes provides information about the underlying tumor, systemic symptoms, and debilitating complications. The recognition of these syndromes is particularly helpful in cases of skin lesions presenting as the first sign of the malignancy, and the underlying malignancy can be treated in a timely manner. Read More

    Autoimmune Blistering Diseases in the Elderly: Clinical Presentations and Management.
    Drugs Aging 2016 Oct;33(10):711-723
    Department of Dermatology, St. George Hospital, Gray St, Kogarah, Sydney, 2217, NSW, Australia.
    Elderly patients are more susceptible to the development of autoimmune blistering disorders such as bullous pemphigoid, mucous membrane pemphigoid, epidermolysis bullosa acquisita, and paraneoplastic pemphigus. This article focuses on the clinical aspects of the aforementioned autoimmune blistering diseases and highlights the important factors involved in treating elderly patients. It is essential for clinicians to offer individualized treatment plans for these patients to optimize outcomes, as elderly patients often have multiple co-morbidities, polypharmacy, and suboptimal socioeconomic status that can adversely influence adequate compliance. Read More

    Paraneoplastic pemphigus: a short review.
    Clin Cosmet Investig Dermatol 2016 23;9:291-295. Epub 2016 Sep 23.
    Department of Dermatology, Icahn School of Medicine at Mount Sinai, New York, NY, USA.
    Paraneoplastic pemphigus (PNP) is a fatal autoimmune blistering disease associated with an underlying malignancy. It is a newly recognized blistering disease, which was first recognized in 1990 by Dr Anhalt who described an atypical pemphigus with associated neoplasia. In 2001, Nguyen proposed the term paraneoplastic autoimmune multiorgan syndrome because of the recognition that the condition affects multiple organ systems. Read More

    High-dose-rate intraluminal brachytherapy for paraneoplastic autoimmune multiorgan syndrome.
    Exp Ther Med 2016 Sep 15;12(3):1435-1439. Epub 2016 Jun 15.
    Institute of Clinical Medicine of Chonbuk National University-Biomedical Research Institute, Chonbuk National University Hospital, Jeonju, Jeollabuk-do 561-712, Republic of Korea; Department of Obstetrics and Gynecology, Chonbuk National University Hospital, Jeonju, Jeollabuk-do 561-712, Republic of Korea.
    Paraneoplastic autoimmune multiorgan syndrome (PAMS), also known as paraneoplasic pemphigus, involves the skin, internal organs and mucosa. PAMS-associated mortality may occur as a result of autoantibody formation against internal tumors and their infiltration into organs other than the skin lesions that characterize PAMS. The most common symptoms of PAMS include pain associated with continuous oral ulceration and resistance to pharmacological treatment. Read More

    Ocular Onset of Paraneoplastic Pemphigus Presenting as Hyperemic Conjunctivitis and Massive Bilateral Eyelid Ulceration: A Case Report and Literature Review.
    Ocul Immunol Inflamm 2016 Aug 18:1-4. Epub 2016 Aug 18.
    a Department of Ophthalmology , Humanitas Clinical and Research Institute , Rozzano , Milan , Italy.
    Introduction: Paraneoplastic clinical signs are characterized by a large and heterogeneous variety of manifestations due to several possible underlying neoplasms. Paraneoplastic pemphigus (PNP) is a particular paraneoplastic variety that usually primarily affects the dermic and/or oral mucosa and is characterized by a high rate of mortality (90%). Therefore, it is important to recognize its possible signs early. Read More

    Paraneoplastic pemphigus and myasthenia gravis, associated with inflammatory pseudotumor-like follicular dendritic cell sarcoma: response to rituximab.
    Clin Case Rep 2016 Aug 15;4(8):797-9. Epub 2016 Jul 15.
    Department of Neurology the First Bethune Hospital Jilin University Changchun Jilin Province China.
    Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms. The disease is most commonly of lymphoproliferative origin and presents high mortality. We describe a patient with PNP and myasthenia gravis associated with inflammatory pseudotumor-like follicular dendritic cell sarcoma, as well as the response to rituximab. Read More

    Extremities of the N-terminus of envoplakin and C-terminus of its linker subdomain are major epitopes of paraneoplastic pemphigus.
    J Dermatol Sci 2016 Oct 23;84(1):24-29. Epub 2016 Jun 23.
    Department of Dermatology, Peking University First Hospital, Beijing 100034, China; Beijing Key Laboratory of Molecular Diagnosis of Dermatoses, Beijing 100034, China. Electronic address:
    Background: Autoantibodies against N-terminal domains and linker subdomains of envoplakin (EVPL) and periplakin (PPL) were frequently detected in sera of paraneoplastic pemphigus(PNP) patients.

    Objectives: To further investigate finer epitopes of EVPL and PPL, and evaluate their associations with clinical aspects of PNP.

    Methods: We produced 12 overlapping truncated fragments of these regions in Escherichia coli, and measured their reactivities to sera of 65 PNP patients and 50 healthy volunteers by enzyme-linked immunosorbent assays (ELISA). Read More

    Recurrent corneal melting in the paraneoplastic pemphigus associated with Castleman's disease.
    BMC Ophthalmol 2016 Jul 12;16:106. Epub 2016 Jul 12.
    Department of Ophthalmology, Sun Yat-sen Memorial Hospital of Sun Yat-sen University, Guangzhou, 510120, China.
    Background: The ocular presentation of Castleman's disease (CD)-associated paraneoplastic pemphigus (PNP) has rarely been reported. In this report, we describe a young patient with CD-associated PNP who had recurrent corneal ulceration in addition to cicatrizing conjunctivitis.

    Case Presentation: We describe a case of 23-year-old male with mucocutaneous erosion and conjunctival injection and erosion who was found to have PNP. Read More

    Paraneoplastic pemphigus as a presentation of acute myeloid leukemia: Early diagnosis and remission.
    Hematol Oncol Stem Cell Ther 2016 Jun 23. Epub 2016 Jun 23.
    Department of Hematology and Cellular Therapy, Western Pennsylvania Cancer Institute, Pittsburgh, PA, USA.
    Skin lesions are frequently encountered in clinical practice which can be a presentation of systemic diseases not excluding an occult malignancy. Commonly reported paraneoplastic dermatologic manifestations include acanthosis nigricans, dermatomyositis, erythroderma, hypertrophic osteoarthropathy, Sweet syndrome, and paraneoplastic pemphigus (PNP). PNP is a rare autoimmune mucocutaneous disease characterized by severe stomatitis, polymorphic skin eruptions, and associated underlying neoplasms most commonly non-Hodgkin's lymphoma, chronic lymphocytic leukemia, and Castleman disease. Read More

    [A case of retroperitoneal Castleman's disease with paraneoplastic pemphigus].
    Zhong Nan Da Xue Xue Bao Yi Xue Ban 2016 May;41(5):548-52
    Department of General Surgery, Xiangya Hospital, Central South University, Changsha 410008, China.
    Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis, which is caused by potential neoplasm, especially the Castleman's disease. Castleman's disease associated with paraneoplastic pemphigus is misdiagnosed frequently and easily in clinical practices. Furthermore, it is reported that the mortality rate for this disease is very high. Read More

    Paraneoplastic Pemphigus Associated with Follicular Dendritic Cell Tumor in the Mediastinum.
    Case Rep Dermatol Med 2016 11;2016:6901539. Epub 2016 Apr 11.
    Ida B Scudder Cancer Centre, Radiation Oncology Unit 1, Christian Medical College, Ida Scudder Road, Vellore, Tamil Nadu 632004, India.
    Paraneoplastic Pemphigus (PNP) is an autoimmune bullous disease characterized by severe stomatitis, polymorphous skin eruptions, and underlying neoplasms. Diagnosis of cutaneous paraneoplastic disorders requires high index of suspicion. We describe a patient with PNP associated with follicular dendritic cell (FDC) tumor in the mediastinum, a rare neoplasm originating from follicular dendritic cells. Read More

    Castleman's Disease Presenting as Localized Abdominal Mass and Paraneoplastic Pemphigus.
    J Clin Imaging Sci 2016 26;6. Epub 2016 Feb 26.
    Department of Urology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
    Castleman's disease is a rare, benign lymphoproliferative disorder of unknown origin. Paraneoplastic pemphigus is a common association which presents as oral mucosal ulcerations. Abdominal and retroperitoneal Castleman's disease present either as a localized disease or as a systemic disease. Read More

    Case of paraneoplastic pemphigus with immunoglobulin (Ig)G and IgA antibodies to various antigens.
    J Dermatol 2016 Aug 11;43(8):944-6. Epub 2016 Mar 11.
    Department of Dermatology, Nippon Medical School, Tokyo, Japan.
    A 63-year-old Japanese man with non-Hodgkin B-cell lymphoma presented with erythematous skin lesions on his entire body, with oral, ocular and anal mucosal lesions. The patient was diagnosed with paraneoplastic pemphigus. Immunofluorescence showed both immunoglobulin (Ig)G and IgA antibodies to keratinocyte cell surfaces. Read More

    Autoimmune bullous diseases with skin and eye involvement: Cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica.
    Clin Dermatol 2016 Mar-Apr;34(2):205-13. Epub 2015 Dec 19.
    Department of Medicine (Dermatology), Vanderbilt University School of Medicine, Nashville, TN; Department of Pediatrics, Vanderbilt University School of Medicine, Nashville, TN.
    Autoimmune blistering diseases are a heterogeneous group of disorders that mostly affect the skin and mucous membranes. Occasionally, other organ systems may be involved, depending on the unique pathophysiology of each disease. Cicatricial pemphigoid, pemphigus vulgaris, and paraneoplastic pemphigus are distinct entities, but all have the potential to have cutaneous and ocular involvement. Read More

    Gastric Cancer and Paraneoplastic Pemphigus.
    Indian J Surg 2015 Dec 5;77(6):545-6. Epub 2015 Jul 5.
    Endoscopy Unit, RIPAS Hospital, Bandar Seri Begawan, BA 1710 Brunei.
    Paraneoplastic pemphigus is a relatively rare but significant acquired autoimmune mucocutaneous disorder that is characterised by diffuse erythema, painful blistering and sores of the skin and mucus membranes. The underlying pathogenesis is believed to be triggered by altered immune system in response to underlying neoplasm. The manifestations can predate, occur at the same time or after the diagnosis of cancer. Read More

    Epiplakin Is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients.
    J Invest Dermatol 2016 Feb;136(2):399-408
    Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan. Electronic address:
    All plakin family proteins are known to be autoantigens in paraneoplastic pemphigus (PNP). In this study, we first examined whether PNP sera also react with epiplakin, another plakin protein, by various immunological methods using 48 Japanese PNP sera. Immunofluorescence confirmed that cultured keratinocytes expressed epiplakin. Read More

    Childhood Pemphigus Foliaceus with Exclusive Immunoglobulin G Autoantibodies to Desmocollins.
    Pediatr Dermatol 2016 Jan-Feb;33(1):e10-3
    Department of Dermatology, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel.
    Pemphigus refers to a group of potentially fatal blistering skin diseases that are often due to the deleterious effects of autoantibodies directed against desmosomal antigens. Although desmogleins have been mainly implicated as autoantigens in pemphigus, a steadily growing body of evidence suggests that other desmosomal proteins may be causally involved as well. Antibodies directed against desmocollin-3 have been shown to play a direct role in the pathogenesis of several types of pemphigus. Read More

    Paraneoplastic pemphigus as a first sign of metastatic retroperitoneal inflammatory myofibroblastic tumor: (18)F-FDG PET/CT findings.
    Rev Esp Med Nucl Imagen Mol 2016 Jul-Aug;35(4):260-2. Epub 2015 Dec 28.
    Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India. Electronic address:
    A 30-year-old female presented with a 3-month history of erosive stomatitis and bullous lesions, along with recurrent episodes of abdominal pain. She was found to have a retroperitoneal lump in left lumbar region. Skin biopsy revealed bullous disorder. Read More

    Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma: report of a case and review of literature.
    Int J Clin Exp Pathol 2015 1;8(10):11983-94. Epub 2015 Oct 1.
    Department of Pathology, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University Guangzhou 510120, China.
    Follicular dendritic cell sarcoma (FDCS) is a rare tumor associated with paraneoplastic pemphigus. It is Blame drenchs auxiliary cell tumor which is derived from the peripheral lymphoid tissues. Throughout the world, several patients of paraneoplastic pemphigus associated follicular dendritic cell sarcoma were reported in the literature, but mostly originated from the neck lymph nodes, and extranodal origin of follicular dendritic sarcoma was rarely reported. Read More

    Bullous diseases: Kids are not just little people.
    Clin Dermatol 2015 Nov-Dec;33(6):644-56. Epub 2015 Sep 14.
    Departments of Dermatology & Pediatrics, Columbia University College of Physicians and Surgeons, 161 Fort Washington Avenue, 12th Floor, New York, NY, 10032, USA. Electronic address:
    Bullous diseases may be rare; however, this does not preclude the clinician from being familiar with their manifestations and treatment. After ruling out infection, genetically inherited blistering diseases are more likely to be the cause of blistering or erosions in the neonatal period, whereas immunobullous diseases are more common in adults. Published literature on immunobullous disorders reflects information gleaned from case reports and open-label case series; prospective studies and evidence-based treatments are limited. Read More

    Paraneoplastic pemphigus associated with fatal bronchiolitis obliterans and intractable mucosal erosions: Treatment with cyclosporin in addition to steroid, rituximab and intravenous immunoglobulin.
    J Dermatol 2016 Apr 28;43(4):419-22. Epub 2015 Oct 28.
    Department of Dermatology, Ehime University Graduate School of Medicine, Ehime, Japan.
    Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease that presents as severe mucosal erosions and variable cutaneous lesions and is primarily associated with hematologically malignant or benign diseases. A 59-year-old Japanese woman presented with oral, ocular and vaginal mucosal erosions and erythema as well as blistering on her trunk and limbs. She developed bronchiolitis obliterans; lymphadenopathy in the cervical, subclavian, para-aortic and intraperitoneal regions; and splenomegaly. Read More

    Rituximab Monotherapy and Rituximab-Containing Chemotherapy Were Effective for Paraneoplastic Pemphigus Accompanying Follicular Lymphoma, but not for Subsequent Bronchiolitis Obliterans.
    J Clin Exp Hematop 2015 ;55(2):83-8
    Department of Hematology, Rheumatology, and Infectious Disease, Kumamoto University Graduate School of Medicine.
    A 60-year-old male patient suffered from mild exertional dyspnea, wheezing, and systemic blisters. He was diagnosed with paraneoplastic pemphigus (PNP) with follicular lymphoma in the pancreas head and pelvic cavity. He was first treated with eight cycles of rituximab; his blisters and erosions gradually improved and highly elevated levels of auto-antibodies related to PNP gradually decreased to normal levels. Read More

    Epiplakin is a Paraneoplastic Pemphigus Autoantigen and Related to Bronchiolitis Obliterans in Japanese Patients.
    J Invest Dermatol 2015 Oct 19. Epub 2015 Oct 19.
    Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, Fukuoka, Japan.
    All plakin family proteins are known to be autoantigens in paraneoplastic pemphigus (PNP). In this study, we first examined whether PNP sera also react with epiplakin, another plakin protein, by various immunological methods using 48 Japanese PNP sera. Immunofluorescence confirmed that cultured keratinocytes expressed epiplakin. Read More

    Evaluation of a Newly Available ELISA for Envoplakin Autoantibodies for the Diagnosis of Paraneoplastic Pemphigus.
    J Drugs Dermatol 2015 Oct;14(10):1103-6
    Importance: Paraneoplastic pemphigus (PNP) is routinely diagnosed by the presence of autoantibodies for desmoplakin by indirect immunofluorescence (IIF) on rat bladder epithelium (RBE). IIF on RBE has recently been found to be positive in select cases of other blistering disorders. A new ELISA that detects envoplakin autoantibodies has recently been developed for the diagnosis of PNP. Read More

    Clinical and immunological findings in 104 cases of paraneoplastic pemphigus.
    Br J Dermatol 2015 Dec 14;173(6):1447-52. Epub 2015 Nov 14.
    Department of Dermatology, Kurume University School of Medicine, and Kurume University Institute of Cutaneous Cell Biology, 67 Asahimachi, Kurume, Fukuoka, 830-0011, Japan.
    Background: Although there are many reports of sporadic patients with paraneoplastic pemphigus (PNP), only a few systematic studies on large cohorts of patients with PNP have been reported.

    Objectives: To analyse the clinical and immunological findings in a large cohort of patients with PNP.

    Methods: This retrospective study consisted of 104 patients with PNP. Read More

    Transplacental Transmission of Pemphigus Herpetiformis in the Setting of Maternal Lymphoma.
    Pediatr Dermatol 2015 Nov-Dec;32(6):e234-7. Epub 2015 Jul 27.
    Division of Pediatric Dermatology, University of Minnesota, Minneapolis, Minnesota.
    Pemphigus herpetiformis (PH) is characterized by grouped vesicular or papular pruritic lesions with histologic and immunopathologic features of pemphigus. PH can manifest at any age, and paraneoplastic cases have been reported. We describe a healthy boy born with acral crateriform erosions of the hands and feet whose 35-year-old mother had similar lesions. Read More

    Paraneoplastic Pemphigus: Autoimmune-Cancer Nexus in the Skin.
    Anticancer Agents Med Chem 2015 ;15(10):1215-23
    6078 Clinical and Translational Research Center, 875 Ellicot Street, Buffalo, NY 14203.
    Paraneoplastic pemphigus (PNP) is a debilitating and lethal adult and pediatric autoimmune blistering skin disease that occurs in the setting of a known or occult neoplasm. It is characterized by painful mucosal erosions that are often resistant to therapy. These clinical characteristics, in addition to positive immunohistochemistry findings make the diagnosis of PNP. Read More

    A middle-aged man presenting with unexplained mucosal erosions and progressive dyspnoea.
    BMJ Case Rep 2015 May 7;2015. Epub 2015 May 7.
    Division of Pulmonary, Allergy, Critical Care and Sleep Medicine, The Ohio State University Wexner Medical Center, Columbus, Ohio, USA.
    Paraneoplastic pemphigus (PNP) is a rare syndrome driven by antibodies (IgG) binding to desmogleins and other epidermal proteins leading to skin erosions. In rare instances, these same IgG proteins may also target the bronchial mucosa leading to an irreversible fibrotic reaction within the epithelium and subsequent obstructive lung disease. A 51-year-old man presented to the emergency department with 2-3-month history of dyspnoea as well as oral and genital ulcerations and inguinal lymphadenopathy. Read More

    Clinical and laboratory characterization of 114 cases of Castleman disease patients from a single centre: paraneoplastic pemphigus is an unfavourable prognostic factor.
    Br J Haematol 2015 Jun 30;169(6):834-42. Epub 2015 Mar 30.
    Department of Haematology, Peking University First Hospital, Beijing, China.
    This study retrospectively collected the clinical and laboratory data of 114 patients with Castleman disease (CD) from a single medical centre. Clinical classification identified 62 patients (54·4%) with unicentric Castleman disease and 52 (45·6%) with multi-centric Castleman disease. Pathological classification revealed 68 cases (59·6%) of hyaline vascular variant, 16 (14·1%) mixed cellular variant (Mix) and 30 (26·3%) plasmacytic variant. Read More

    Application of C4d Immunohistochemistry on Routinely Processed Tissue Sections for the Diagnosis of Autoimmune Bullous Dermatoses.
    Am J Dermatopathol 2016 Mar;38(3):186-8
    Departments of *Dermatology, and †Pathology, Edouard-Herriot Hospital, Lyon University, Lyon, France.
    The diagnosis of autoimmune bullous dermatoses relies greatly on direct immunofluorescence (DIF) examination performed on frozen tissue sections, showing deposits of immunoglobulins and/or C3 on specific cutaneous structures. However, frozen material is not always available for DIF; therefore, alternative techniques are needed in the diagnostic procedure. We therefore tested the usefulness of C4d immunohistochemistry on formalin-fixed, paraffin-embedded tissue sections for the diagnosis of bullous pemphigoid (BP) and pemphigus (P). Read More

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