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Paraneoplastic Pemphigus in A Patient with Chronic Lymphocytic Leukemia: A Case Report
Paraneoplastic pemphigus (PNP) is a rare and autoimmune mucocutaneous disease associated with underlying neoplasia. The diagnosis of PNP is often difficult since skin manifestations maybe initially similar to other dermatological disorders. The case is here reported of an 84-year old male diagnosed with PNP associated with CLL, who initially presented with severe diffuse erythematous lesions in the oral mucosa and nasal mucosa, and vesicular-bullous lesions on the trunk and extremities. The patient died due to respiratory failure and hypotension caused by acute PTE.
Paraneoplastic pemphigus as the initial presentation of chronic lymphocytic leukemia
Annals of oncology : official journal of the European Society for Medical Oncology / ESMO, 2001
The case history of a 61-year-old male patient is described, who presented with severe stomatitis, conjunctivitis and leukocytosis. The diagnosis chronic lymphocytic leukemia (CLL) stage A (0) was made, for which no treatment was necessary. Progression of stomatitis and conjunctivitis and erythosquamous skin lesions with bullae and vesiculae formation developed. Under the diagnosis of bullous pemphigoid the patient was treated with corticosteroids. The histologic and immunofluorescence examination of a skin biopsy was compatible with this diagnosis, and antibodies to skin could not be detected in a first serum sample. Pseudomonas was cultured from all lesions, the corticosteroids were stopped and antibiotic treatment was started, without clear effect. Because of progression of skin lesions and debilitation, the patient finally declined all treatment and died five weeks after admission. Post-mortem examination showed enlarged lymphnodes in the cervical, aortal en iliacal areas, with ...
Paraneoplastic pemphigus associated with chronic lymphocytic leukaemia: Treatment with alemtuzumab
Leukemia Research, 2012
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. Diagnoses: At first, the patient was diagnosed with pityriasis rose caused by a viral infection. Biopsies for histology and immunofluorescence showed PNP, was treated with immunosuppressive and antiinfective therapy. Interventions: Immunosuppressive and antiinfective therapy were performed. Outcomes: The skin lesions of PNP were alleviated. However, the infections were aggravated and the disease progressed. The patient died of respiratory failure. Lessons: Treatment for PNP should be adapted to disease severity as early as possible. Antiinfection treatment should be timely and effective because infections are the most common complication that can lead to death.
Paraneoplastic pemphigus with a pemphigus vegetans-like plaque as the only cutaneous manifestation
Journal of the American Academy of Dermatology, 1998
Paraneoplastic pemphigus (PNP) is defined by 5 diagnostic criteria: (1) painful mucosal erosions and polymorphous skin eruption in the context of an occult or confirmed neoplasm; (2) histopathologic changes of keratinocyte necrosis, intraepidermal acantholysis, and vacuolar-interface dermatitis; (3) direct immunofluorescence (DIF) findings of intercellular IgG and complement and often linear or granular complement at the dermalepidermal junction (DEJ); (4) indirect immunofluorescence (IIF) testing that demonstrates circulating antibodies binding to simple, columnar, and transitional epithelia in addition to the typical pemphigus pattern of binding to skin and mucosa; (5) circulating autoantibodies that immunoprecipitate a high-molecular weight complex of polypep-tides from keratinocyte extracts weighing 250, 230, 210, 190, and 170 kd. Although refractory stomatitis is a consistent clinical feature, the cutaneous manifestations are characteristically variable. We describe a patient with PNP who presented with severe oral, laryngeal, and esophageal involvement. A pemphigus vegetans-like plaque was the only cutaneous manifestation.
Paraneoplastic Pemphigus Case Report
Paraneoplastic pemphigus is a rare autoimmune bullous dermatosis. It occurs more frequently around the age of 60 and is most commonly associated with lymphomas and leukaemia. This particular form of pemphigus has issues that are clinically, histopathologically and immunopathologically distinct from other forms of pemphigus. Severe mucosal erosions and a polymorph rash are the clinical manifestations. We report a case of paraneoplastic pemphigus diagnosed in the Clinical Hospital of Sibiu which allowed us to present a literature update of this subject. Keywords: pemphigus, chronic lymphocytic leukaemia, paraneoplastic Rezumat: Pemfigusul paraneoplazic este o dermatoză buloasă autoimună rar întâlnită. Apare mai frecvent în jurul vârstei de 60 de ani şi se asociază cel mai frecvent cu limfoame şi leucemii. Această formă particulară de pemfigus are aspecte clinice, histopatologice şi imunopatologice distincte de alte forme de pemfigus. Clinic se manifestă prin eroziuni severe ale mucoas...
Oral Manifestation of Paraneoplastic Pemphigus
Journal of Oral Medicine and Pain, 2019
Paraneoplastic pemphigus (PNP) is a rare and often fatal autoimmune blistering disease accompanied by both benign and malignant neoplasms. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by PNP patients. Oral ulcers are initial lesions in various autoimmune diseases like pemphigus, bullous pemphigoid, erythema multiforme, graft-versus-host, lichen planus, it does not improved despite of high-dose steroid therapy. We report a-35-year-old female who presented oral ulceration, lip crust and skin lesions. By doing several examinations, such as enzyme-linked immunosorbent assay, incisional biopsy with indirect immunofluorescence, she was diagnosed PNP with non-Hodgkin's lymphoma on pancreas.
Paraneoplastic pemphigus as a presentation of acute myeloid leukemia: Early diagnosis and remission
Hematology/Oncology and Stem Cell Therapy, 2016
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. PNP is characterized on histopathology as dyskeratotic epithelial cells with acantholysis with a typical immunofluorescence staining pattern of direct and/or indirect staining of intercellular, basement membrane, and dermoepidermal junction with immunoglobulin-G and C3. PNP has been described to have poor prognosis with a mortality range of 75-90% and a mean survival of less than 1 year. We describe a previously unreported case of PNP associated with acute myeloid leukemia (AML) where the patient presented with a nonhealing ulcer and hemorrhagic crusting on the face that did not respond to antimicrobials and steroids. Investigations revealed leukocytosis with peripherally circulating blasts. Skin biopsy revealed an evolving PNP and bone marrow biopsy confirmed evidence of AML. The patient underwent induction, consolidation, and then successful allogenic bone marrow transplantation with complete remission. The skin lesion, which was initially refractory to treatments, surprisingly resolved within 7 days of starting induction chemotherapy.
International Journal of Dermatology, 2005
Background Neoplasia-induced lichen planus is described as a cell-mediated reaction to unknown epithelial antigens. Paraneoplastic pemphigus (PNP), characterized by the presence of a specific array of autoantibodies, probably represents a different form of presentation of the same autoimmune syndrome where the mucocutaneous expression depends on the dominant pathologic mechanism.