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    Paraneoplastic pemphigus without antibodies to desmoglein 1 and 3.
    Dermatol Online J 2018 02 15;24(2). Epub 2018 Feb 15.
    Feinberg School of Medicine, Northwestern University, Chicago, Illinois,.
    Paraneoplastic pemphigus is a severe autoimmune blistering disease presenting in the setting of underlying malignancy. Paraneoplastic pemphigus is associated with diffuse painful stomatitis throughout the oral cavity with extension to the lips. The cutaneous findings are varied and have been described as lichenoid, pemphigoid, and targetoid lesions. Read More

    Blistering diseases in the mature patient.
    Clin Dermatol 2018 Mar - Apr;36(2):231-238. Epub 2017 Oct 3.
    Department of Dermatovenereology, University Hospital Center Zagreb and University of Zagreb School of Medicine, Zagreb, Croatia. Electronic address:
    Autoimmune blistering diseases (AIBD) are a group of chronic diseases affecting the skin and mucous membranes, with different presentation, clinical course, histologic and immunopathologic findings, and different therapeutic approach. Blisters develop as a result of autoantibodies directed against distinct adhesion structures within desmosomes or within the basement membrane zone. The most common AIBD that develops in the elderly is bullous pemphigoid (previously also named "pemphigoid senilis"), but mature patients can also present with other AIBD as mucous membrane pemphigoid, epidermolysis bullosa acquisita, paraneoplastic pemphigus, pemphigus vulgaris, pemphigus foliaceus, linear IgA dermatosis, and dermatitis herpetiformis. Read More

    Paraneoplastic Pemphigus Associated With Castleman Disease Detected by 18F-FDG PET/CT.
    Clin Nucl Med 2018 Jun;43(6):464-465
    A 36-year-old woman suffering from oral ulcerations, skin rash, and dyspnea was suspected of having paraneoplastic pemphigus. An F-FDG PET/CT was performed to detect the underlying neoplasms and revealed an F-FDG-avid mass in the lower right retroperitoneum. The mucocutaneous lesions and dyspnea were relieved after the tumor excision, and the pathology indicated Castleman disease. Read More

    Clinical, immunological profile and follow up of patients with pemphigus: A study from India.
    Indian J Dermatol Venereol Leprol 2018 Mar 12. Epub 2018 Mar 12.
    Department of Dermatology, Christian Medical College and Hospital, Vellore, Tamil Nadu, India.
    Background: Pemphigus has a protracted course and multiple factors influence its prognosis. The objective of this study was to describe the epidemiology and clinical profile of pemphigus patients and to study its influence on treatment end points.

    Methods: This was a retrospective chart review done in an Indian tertiary care hospital from December 1991 to December 2013. Read More

    Paraneoplastic Pemphigus Associated with B-cell Chronic Lymphocytic Leukemia Treated with Ibrutinib and Rituximab.
    Intern Med 2018 Mar 9. Epub 2018 Mar 9.
    Department of Hematology, National Cancer Center Hospital, Japan.
    Paraneoplastic pemphigus (PNP) is a severe autoimmune blistering disease associated with an underlying malignancy, and its prognosis is poor. We herein report the first patient with B-cell chronic lymphocytic leukemia/small lymphocytic lymphoma (B-CLL/SLL)-associated PNP successfully treated with the Bruton's tyrosine kinase inhibitor ibrutinib and rituximab. Although his PNP lesions did not improve with ibrutinib monotherapy, the combination of ibrutinib and rituximab was effective against B-CLL/SLL-associated PNP. Read More

    Oral pemphigus.
    G Ital Dermatol Venereol 2018 Jun 6;153(3):383-388. Epub 2018 Mar 6.
    Department of Surgery, Medicine, Dentistry, Transplantation Morphology, Oncology, and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy.
    The involvement of the oral mucosa in patients affected by pemphigus vulgaris (PV), paraneoplastic, IgA pemphigus, and in some cases iatrogenic pemphigus is common and often a prelude to skin lesions. Intraepidermal bullae are caused by acantholysis, induced by IgG autoantibodies directed against the desmosomes and the domain of numerous keratinocytes self-antigens desmogleins (namely cadherins), thus supporting the autoimmune nature of the disease. Apoptosis may contribute to the acantholysis. Read More

    Pemphigus group: overview, epidemiology, mortality, and comorbidities.
    Immunol Res 2018 Apr;66(2):255-270
    Department of Dermatology, Rambam Health Care Campus, POB 9602, 31096, Haifa, Israel.
    Pemphigus forms a group of rare autoimmune bullous diseases that affect the skin and mucous membranes. This group has a chronic course leading to high morbidity and mortality. It is characterized by the production of pathogenic autoantibodies directed against different proteins of the desmosome, leading histologically to intraepidermal cleavage, and clinically to vesicles and erosions on the epithelium of the mucous membranes and/or the skin. Read More

    Paraneoplastic Pemphigus in a 34-year-old.
    Dermatol Online J 2017 Dec 15;23(12). Epub 2017 Dec 15.
    New York University Health, New York.
    Paraneoplastic Pemphigus (PNP) is a rare and often fatal autoimmune mucocutaneous blistering disease associated with an underlying malignancy. It is thought to be caused by antibodies to tumor antigenscross-reacting with epithelial antigens, specifically desmosomal and hemidesmosomal antigens. There are at least five clinical morphologic variants of PNP, with the earliest and most consistent finding beingsevere stomatitis. Read More

    Rituximab and intravenous immunoglobulin as alternatives to long-term systemic corticosteroids in the treatment of pemphigus: a single center case series of 63 patients.
    Dermatol Online J 2018 Jan 19;23(12). Epub 2018 Jan 19.
    Department of Dermatology, University of Florida College of Medicine, Gainesville, Florida.
    Rituximab and intravenous immunoglobulin [IVIg] have recently emerged as effective treatments for pemphigus refractory to corticosteroids [CS]. This case series sought to compare the clinical, serologic,and adverse effects of CS, IVIg, and rituximab in patients with pemphigus. A retrospective review of 63 patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), or paraneoplastic pemphigus (PNP)was performed. Read More

    [Paraneoplasms of the skin].
    Internist (Berl) 2018 Feb;59(2):134-144
    Klinik und Poliklinik für Dermatologie und Allergologie, Klinikum der Universität München, Ludwig-Maximilians-Universität München, München, Deutschland.
    The cutaneous manifestations of malignancies include nonmalignant skin disorders that occur in association with malignancies (facultative paraneoplastic dermatoses) and skin disorders that are always associated with hematologic diseases or solid tumors (obligate paraneoplastic dermatoses). Paraneoplastic increase of growth factors or immunological reactions lead to a variety of inflammatory, hyperkeratotic or proliferative skin reactions. When paraneoplastic dermatoses develop before cancer is diagnosed, recognition of these skin diseases can accelerate both the diagnosis and treatment. Read More

    Paraneoplastic Pemphigus with Underlying Retroperitoneal Inflammatory Myofibroblastic Tumor: A Case Report and Review of the Literature.
    Indian Dermatol Online J 2017 Nov-Dec;8(6):478-481
    Department of Pharmacology, Islamic Azad University, Science and Research Branch, Tehran, Iran.
    Inflammatory myofibroblastic tumor (IMT) is a peculiar low-grade neoplasm of spindle cell fibroblasts and myofibroblasts in an inflammatory background. The lung is the most common site of involvement. Here, we report a case of paraneoplastic pemphigus (PNP)-associated with an extensive retroperitoneal IMT. Read More

    Paraneoplastic Pemphigus: Insight into the Autoimmune Pathogenesis, Clinical Features and Therapy.
    Int J Mol Sci 2017 Nov 26;18(12). Epub 2017 Nov 26.
    Dipartimento di Medicina Interna e Specialità Mediche, Dermatologia, Sapienza Università di Roma, Piazzale Aldo Moro, 5, 00185 Roma, Italy.
    Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by paraneoplastic pemphigus patients. The pathogenesis of paraneoplastic pemphigus is not yet completely understood, although some immunological aspects have been recently clarified. Read More

    Diagnosis of Castleman Disease.
    Hematol Oncol Clin North Am 2018 02;32(1):53-64
    Medical Oncology, Dana-Farber Cancer Institute, 450 Brookline Avenue, M230 Boston, MA 02215, USA; VA Boston Healthcare System, 1400 VFW Parkway, West Roxbury, MA, USA. Electronic address:
    Castleman disease (CD) is a rare and heterogenous group of disorders sharing in common an abnormal lymph node pathology. CD comprises distinct subtypes with different prognoses. Unicentric CD and multicentric CD are featured by specific systemic manifestations and may be associated with Kaposi sarcoma, non-Hodgkin and Hodgkin lymphoma, and POEMS syndrome. Read More

    Paraneoplastic pemphigus without detectable anti-plakin antibodies in a patient with non-Hodgkin lymphoma.
    Ann Hematol 2018 Mar 16;97(3):543-544. Epub 2017 Nov 16.
    Department of Dermatology, School of Medicine, University Clinic of Navarra, University of Navarra, PO Box 4209, Pamplona, Navarre, Spain.

    Spectrum of orocutaneous disease associations: Immune-mediated conditions.
    J Am Acad Dermatol 2017 Nov;77(5):795-806
    Division of Dermatology, Baylor University Medical Center, Dallas, Texas. Electronic address:
    There are a number of diseases that manifest both on the skin and the oral mucosa, and therefore the importance for dermatologists in clinical practice to be aware of these associations is paramount. In the following continuing medical education series, we outline orocutaneous disease associations with both immunologic and inflammatory etiologies. Read More

    Clinical Analysis of Unicentric Castleman's Disease with Paraneoplastic Pemphigus and Bronchiolitis Obliterans.
    Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2017 08;39(4):492-498
    Department of Hematology,PUMC Hospital, CAMS and PUMC,Beijing 100730,China.
    Objective To investigate the clinical features of unicentric Castleman's disease(UCD)with paraneoplastic pemphigus(PNP)and bronchiolitis obliterans(BO).Method Data of UCD patients with PNP and BO from Peking Union Medical College Hospital were retrospectively analyzed,along with literatures review. Results Totally 23 cases(11 males and 12 females)were enrolled. Read More

    Routine detection of serum antidesmocollin autoantibodies is only useful in patients with atypical pemphigus.
    Exp Dermatol 2017 12 29;26(12):1267-1270. Epub 2017 Oct 29.
    Lübeck Institute of Experimental Dermatology (LIED), Lübeck, Germany.
    Autoantibodies against the 3 desmocollin (Dsc; Dsc1-Dsc3) isoforms have been described in different pemphigus variants. Here, we developed state-of-the-art detection systems for serum anti-Dsc1, Dsc2 and Dsc1 IgG and IgA. These assays were applied in 5 different cohorts including pemphigus vulgaris (PV) patients with compatible direct immunofluorescence (IF) microscopy but no reactivity against desmogleins 1 and 3 (n = 24) and sera from patients with autoimmune blistering diseases with positive direct IF microscopy taken at the time of diagnosis (n = 749). Read More

    Paraneoplastic Pemphigus. A Life-Threatening Autoimmune Blistering Disease.
    Actas Dermosifiliogr 2017 Dec 8;108(10):902-910. Epub 2017 Aug 8.
    Servicio de Dermatología, Hospital General de México, Mexico.
    Paraneoplastic pemphigus (PNP), a subset of pemphigus, is a unique autoimmune blistering condition that can affect multiple organs other than the skin. It is a life-threatening disease associated with an underlying malignancy, most commonly of lymphoproliferative origin. The clinical picture may resemble pemphigus, pemphigoid, erythema multiforme, graft-versus-host disease, or lichen planus. Read More

    Achievement of the longest survival of paraneoplastic pemphigus with bronchiolitis obliterans associated with follicular lymphoma using R-CHOP chemotherapy.
    Int J Hematol 2017 Dec 8;106(6):852-859. Epub 2017 Aug 8.
    Department of Hematology, Japan Red Cross Fukui Hospital, 2-4-1 Tsukimi, Fukui, Fukui, 918-8501, Japan.
    Paraneoplastic pemphigus (PNP) is a rare, fatal, paraneoplastic autoimmune mucocutaneous blistering disease, commonly associated with lymphoproliferative disorders, including malignant lymphomas. Lymphoproliferative disorders associated with PNP are sometimes associated with a serious lung complication, bronchiolitis obliterans (BO). Due to its rarity, guidelines for the management of PNP have not been established. Read More

    Analysis of Serum Cytokine Profile in Pemphigus.
    Ann Dermatol 2017 Aug 21;29(4):438-445. Epub 2017 Jun 21.
    Department of Dermatology and Cutaneous Biology Research Institute, Gangnam Severance Hospital, Yonsei University College of Medicine, Seoul, Korea.
    Background: Pemphigus is a group of autoimmune blistering diseases affecting skin and mucous membranes. While pemphigus is an autoantibody mediated disease, the role of T cells and cytokines in the pathogenesis is being increasingly recognized.

    Objective: This study was conducted to observe alterations in the serum cytokine levels of patients with pemphigus vulgaris (PV), pemphigus foliaceous (PF), paraneoplastic pemphigus (PNP) and compare with bullous pemphigoid (BP) and healthy subjects. Read More

    PD-1 inhibitor-associated lichenoid inflammation with incidental suprabasilar acantholysis or vesiculation-Report of 4 cases.
    J Cutan Pathol 2017 Oct 29;44(10):851-856. Epub 2017 Aug 29.
    Department of Dermatology, Westmead Hospital, Sydney, Australia.
    Background: Immune checkpoint agents targeting programmed cell death-1 protein (PD1) or cytotoxic T-lymphocyte-associated protein-4 (CTLA-4) receptors are increasingly utilized in treatment of advanced malignancies. However, these immunotherapies are commonly associated with idiosyncratic cutaneous adverse reactions. Thus, recognition and awareness of these reactions are necessary. Read More

    A Case Report of Paraneoplastic Pemphigus Associated With Retroperitoneal Inflammatory Myofibroblastic Tumor.
    Acta Med Iran 2017 May;55(5):340-343
    Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
    Paraneoplastic pemphigus (PNP) is an autoimmune bullous disease associated with underlying neoplasms, both malignant and benign. The most constant clinical presentation of PNP is the presence of intractable stomatitis. Herein we present a 25-year-old male with a 3-month history of refractory stomatitis especially involving the lips and widespread vesiculobullous eruption on his trunk and extremities. Read More

    Evaluation of Autoimmune Bullous Diseases in Elderly Patients in Iran: A 10-Year Retrospective Study.
    Skinmed 2017 1;15(3):175-180. Epub 2017 Jun 1.
    Department of Dermatology, Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
    Autoimmune bullous diseases (ABDs) are uncommon but significant skin disorders with relatively high morbidity and mortality. Some surveys have been carried out to describe the spectrum of ABDs in a region, but this is the first that has focused on ABDs in elderly patients. This study was conducted to determine the clinicoepidemiologic features of ABDs in elderly patients. Read More

    Pemphigus.
    Nat Rev Dis Primers 2017 May 11;3:17026. Epub 2017 May 11.
    Department of Dermatology, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.
    Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. Read More

    Successful treatment with bendamustine and rituximab for paraneoplastic pemphigus.
    Ann Hematol 2017 Jul 26;96(7):1221-1222. Epub 2017 Apr 26.
    Division of Hematology, Department of Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.

    Is tocilizumab a potential therapeutic option for refractory unicentric Castleman disease?
    Hematol Oncol 2018 Feb 11;36(1):320-323. Epub 2017 Apr 11.
    Department of Haematology, University Hospitals of Bristol NHS Trust, Bristol, UK.
    Castleman disease is a rare lymphoproliferative disorder with 2 distinctly defined clinical forms. While multicentric Castleman disease (UCD) poses a potential therapeutic challenge, unicentric variant has historically been considered curable with surgical resection. Hence, little is known to guide management of patients with UCD, refractory to surgical resection and combination chemotherapy. Read More

    DRUG-INDUCED ORAL ULCERATIONS: CASE REPORT.
    Acta Clin Croat 2016 Jun;55(2):334-7
    A 70-year-old patient was admitted to the Department of Oral Medicine for multiple oral ulcerations on the left buccal mucosa, around 0.5 cm in diameter, as well as on the gingiva. Otherwise, the patient suffered from chronic lymphocytic leukemia, hypogammaglobulinemia, chronic renal insufficiency, with complete afunction of the right kidney, asthma, hypertension, gastritis and prostate hyperplasia. Read More

    Paraneoplastic Pemphigus Associated with a Malignant Thymoma: A Case of Persistent and Refractory Oral Ulcerations Following Thymectomy.
    Ann Dermatol 2017 Apr 24;29(2):219-222. Epub 2017 Mar 24.
    Department of Dermatology, Gangnam Severance Hospital, Cutaneous Biology Research Institute, Yonsei University College of Medicine, Seoul, Korea.
    Paraneoplastic pemphigus is a rare, life-threatening autoimmune mucocutaneous blistering disease associated with underlying neoplasia, commonly lymphoproliferative tumors. Herein we report a case of paraneoplastic pemphigus with a unique autoantibody profile associated with a malignant thymoma. A 56-year-old female patient presented with relapsing oral ulcerations accompanied by erythematous papules and patches on her extremities for 2 months. Read More

    Trichilemmal cystis in metastatic melanoma: a case report.
    Exp Oncol 2017 03;39(1):86-87
    Department of Dermatology, Department of Critical Care Medicine and Surgery, University of Florence, Firenze 50121, 50132, Italy.
    The malignant melanoma is a neoplasm associated with a wide variety of cutaneous paraneoplastic syndromes, as dermatomyositis, systemic sclerosis, paraneoplastic pemphigus. We describe a case of four multiple trichilemmal cystis arising on frontal region in the same patient with brain metastasis and unknown primary melanoma and discuss their relationship. Read More

    Successful use of Bruton's kinase inhibitor, ibrutinib, to control paraneoplastic pemphigus in a patient with paraneoplastic autoimmune multiorgan syndrome and chronic lymphocytic leukaemia.
    Australas J Dermatol 2017 Nov 13;58(4):e240-e242. Epub 2017 Mar 13.
    Department of Dermatology, Royal North Shore Hospital, Sydney, New South Wales, Australia.
    We present the case of a 51-year-old man who developed paraneoplastic pemphigus (PNP) in the context of chronic lymphocytic leukemia (CLL). His CLL was successfully controlled with ibrutinib. Concurrently, there was significant improvement of his PNP, suggesting that ibrutinib may be a very useful addition to the treatment options in this potentially life-threatening autoimmune disorder. Read More

    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

    Autoimmune Disease Manifestations in the Oral Cavity.
    Surg Pathol Clin 2017 Mar 28;10(1):57-88. Epub 2016 Dec 28.
    Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida, 1395 Center Drive, Gainesville, FL 32610, USA.
    Immune-related disorders of the oral cavity may occur as primary disease process, secondary to systemic disease or neoplasm, or as a reaction to medications and other agents. The entities represented within this group may vary significantly by severity, clinical presentation, microscopic presentation, and special testing results. The selected immune-related conditions of the oral cavity in this article are categorized and presented by their prototypical tissue reaction patterns: vesiculobullous, including acantholytic and subepithelial separation; psoriasiform; spongiotic; and lichenoid reaction patterns. Read More

    Non-paraneoplastic autoimmune subepidermal bullous disease associated with fatal bronchiolitis obliterans.
    J Dermatol 2017 Apr 3;44(4):461-464. 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

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