In pemphigus, autoantibodies form against desmoglein. Paraneoplastic pemphigus paraneoplastic autoimmune multiorgan syndrome. Journal of the dermatologyamerican academy of volume 40 number 5 part 1 may 1999. We present a case of a 63year old male patient who was referred for surgical treatment of a lately diagnosed. The clinical presentation is quite varied with some eruptions resembling erythema multiforme, pemphigoid and graftversushost disease histology of. Pemphigus foliaceus is a rare autoimmune blistering disease which is characterised by superficial blisters, erosions and crusts on the skin. Paraneoplastic pemphigus is a multiorganic autoimmune disease, usually. Usually, oral, skin, and mucosal lesions are the earliest manifestations shown by.
This condition can occur at any age, but often strikes people in middle or older age. The blisters look like those in pemphigus foliaceus. Pnp international pemphigus pemphigoid foundation ippf. Retroperitoneal castlemans tumor and paraneoplastic. Pams, to denote the systemic nature of the syndrome is an autoimmune mucocutaneous blistering disease affecting adults or rarely children that generally heralds the presence of an underlying malignancy. Paraneoplastic pemphigus pnp is an often fatal paraneoplastic mucocutaneous blistering disease that is most commonly induced by lymphoproliferative disorders. Bronchiolitis obliterans may occur and can be fatal. This most serious form of pemphigus is paraneoplastic pemphigus.
Studies have shown that some populations may be at greater risk for certain types of pemphigus. It occurs in individuals who have concurrent cancers such as nonhodgkins lymphoma, chronic lymphocytic leukemia, and castleman disease. Paraneoplastic pemphigus is an autoimmune blistering disorder characterized by polymorphous skin lesions with features of both erythema multiforme and pemphigus vulgaris in association with internal neoplasms, especially nonhodgkins lymphoma. Case report adrenal castleman disease associated with. Paraneoplastic pemphigus pnp, a clinically and immunopathologically distinct mucocutaneous blistering dermatosis, is a severe form of autoimmune multiorgan syndrome generally associated with poor therapeutic outcome and high mortality. Paraneoplastic pemphigus is a lifethreatening disease intechopen. We present three new cases of paraneoplastic pemphigus associated with a malignant neoplasm and.
Paraneoplastic pemphigus paraneoplastic autoimmune multiorgan syndrome is a severely debilitating blistering disease affecting skin and mucous membranes in patients with malignancy, particularly hematologic malignancies. Paraneoplastic pemphigus pnp is a rare disease that demonstrates many of the clinical and laboratory findings of pemphigus vulgaris. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes unglued, a phenomenon called acantholysis. Media gallery direct immunofluorescence microscopy performed on epithelial biopsy specimen obtained from a patient with pemphigus vulgaris detects immunoglobulin g deposits at the. Paraneoplastic dermatoses comprise a heterogeneous group of noninherited skin conditions that manifest internal malignancy. Pemphigus is a group of rare autoimmune diseases that cause blistering of the skin and mucous membranes mouth, nose, throat, eyes, and genitals. Paraneoplastic pemphigus is a rare, severe blistering condition of the mucosal surfaces and skin associated with an underlying cancer. Hematologicrelated neoplasms or disorders were associated with 84% of the cases, with nonhodgkin lymphoma 38. Paraneoplastic pemphigus is a rare type of pemphigus. Most of these paraneoplastic dermatoses were diagnosed before 45. Pemphigus is the term used to describe a group of autoimmune diseases that cause the skin and mucous membranes to become blistered. Also known as paraneoplastic autoimmune multiorgan syndrome pams. Circulating autoantibodies of igg type target the desmosomal complexes, desmoglein 3 and 1. Paraneoplastic pemphigus pnp is an often fatal paraneoplastic mucocutaneous blistering disease that is most commonly induced by.
It is hypothesized that antigens associated with the tumor trigger an immune response resulting in blistering of the skin and mucous membranes while patients with malignant and benign tumors are both at risk, malignancy is associated with high mortality rates near 90%. Paraneoplastic autoimmune multiorgan syndrome pams is an alternative term used to refer to pnp. Paraneoplastic pemphigus pnp typically affects middleage patients but it can rarely occur in children. Pnppams can be defined and identified by a combination of the following features. Paraneoplastic pemphigus 23 cases known, preexisting neoplasm cases mucocutaneous symptoms before neoplasm detected.
Backgroundcastleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus. One variant, paraneoplastic pemphigus, can occur in patients who have malignant or benign tumors, most commonly nonhodgkin lymphoma. Paraneoplastic skin syndromes melissa piliang, md dermatology and anatomic pathology cleveland clinic. Introduction paraneoplastic pemphigus pnp is an autoimmune blistering syndrome with 5 welldescribed clinicopathologic phenotypes. Paraneoplastic pemphigus an overview sciencedirect topics. Paraneoplastic pemphigus is characterised by painful blisters and denuded areas of the mouth, lips, oesophagus and skin. Paraneoplastic pemphigus is a newly described autoimmune disease characterized by painful mucosal ulcerations and polymorphous skin lesions in association with an underlying neoplasm. Paraneoplastic pemphigus pnp was first described in 1990 by anhalt et al. Leger and colleagues studied the prognostic factors for overall survival in a series of patients with paraneoplastic pemphigus using univariate. Blisters and erosions are commonly observed and mimic those of pemphigus vulgaris, pemphigus foliaceus, or bullous pemphigoid, affecting any.
Paraneoplastic pemphigus pnp is a rare, autoimmunemediated. Paraneoplastic pemphigus pnp is a rare mucocutaneous autoimmune disease associated with neoplasm. Paraneoplastic conditions occur in association with malignancies cancer. Pv is the most common disease and represents this kind of potentially lifethreatening disease. Pemphigus vulgaris usually occurs in middleaged patients, affecting men and women in equal numbers. This iggmediated disease is initiated by an obvious or occult lymphoproliferative disorder in most cases. Transsternal complete thymoma resection achieved pemphigus regression. Paraneoplastic pemphigus sang eun lee, soochan kim department of dermatology and cutaneous biology research institute, yonsei university college of medicine, seoul, korea abstract paraneoplastic pemphigus pnp is a lifethreatening autoimmune mucocutaneous blistering disease associated with malignancy, particularly lymphoproliferative. A case of a 42yearold woman with paraneoplastic pemphigus as the first manifestation of thymoma is reported. Paraneoplastic pemphigus is a rare form of pemphigus associated with internal malignancies. Passive transfer of paraneoplastic pemphigus sera causes blistering in neonatal mice, proving that the antibodies present are pathogenic. Essentially, pemphigus can be divided into four major types. Neoplasias frequently related to paraneoplastic pemphigus include chronic. Paraneoplastic pemphigus pnp is a rare autoimmune blistering.
Paraneoplastic pemphigus pnp is an autoimmune disorder that is almost always linked to an underlying lymphoproliferative disorder. Paraneoplastic pemphigus pnp medigoo health medical. It is a newly recognized blistering disease, which was first recognized in 1990 by dr anhalt who described an atypical pemphigus with associated neoplasia. Although consensus guidelines have not been reached, four features are consistently found in the majority of pnp patients and are. Pemphigus is a group of organspecific autoimmune disorders, including pemphigus vulgaris pv, pemphigus foliaceus pf and paraneoplastic pemphigus pnp. This type of pemphigus is caused by an antibody called iga. This type of pemphigus is less common than pemphigus vulgaris in australia but in some parts of the.
Paraneoplastic pemphigus is a rare autoimmune skin disease that is always associated with a neoplasm. Description paraneoplastic pemphigus pnp pemphigus is a chronic autoimmune disorder that affects the skin and mucous membranes and causes the formation of blisters. Paraneoplastic pemphigus statpearls ncbi bookshelf. This causes blisters that slough off and turn into sores. Paraneoplastic pemphigus characteristically presents as painful oral and mucosal ulcerations, with concomitant skin involvement simulating pemphigus vulgaris or erythema multiforme. Castleman tumor is a distinct lymphoproliferative disorder of. Pdf paraneoplastic pemphigus is a newly recognized disease that occurs in some patients with lymphoproliferative neoplasms and. Castlemans disease is a rare lymphoproliferative syndrome. Paraneoplastic pemphigus pnp is a fatal autoimmune blistering disease associated with an underlying malignancy. It is the least common but most serious form of pemphigus, particularly considering the presence of the. They are a rare group of disorders that have an incidence of 210 cases per one million inhabitants in some areas of the world and a prevalence of 0.
Paraneoplastic pemphigus patients exhibit many different autoantibodies to proteins of the plakin family, an intracellular component of desmosomes and hemidesmosomes, including envoplakin 210kd, periplakin 190kd, bullous pemphigoid antigen i. Paraneoplastic pemphigus associated with castleman tumor. Paraneoplastic pemphigus pnp occurs in association with malignancies, and is characterised by painful blisters and denuded areas of the mouth, lips, oesophagus and skin. Paraneoplastic pemphigus typically presents with painful mucosal erosions and dusky. This file is licensed under the creative commons attributionshare alike 3. The clinical correlations between pemphigus and thymoma are presented. It is associated mainly with haematopoietic neoplasia and a unique autoantibody profile. An autoimmune vesiculobullous blistering disease characterized by intraepithelial blister formation. Castleman disease, adrenal tumor, paraneoplastic pemphigus, autoimmune disease, prognosis introduction castlemans disease cd, first described by castleman in 1956, is an uncommon and poor ly understood disorder of lymph node hyperpla sia with unknown etiology 1.
Associated malignancy may precede or occur in conjunction with mucocutaneous changes. Paraneoplastic pemphigus pnp is a rare and severe autoimmune blistering skin disease of humans. Nguyen et al categorized these subtypes as pemphiguslike, pemphigoidlike, erythema multiformelike, graftvshost. Lymphoma is the most commonly described associated malignancy but numerous other tumour associations have been described. The sarcomas associated with this are unusual retroperitoneal sarcomas that appear to be of lymph. In pemphigus vulgaris pv, the blister occurs in the deeper part of the epidermis, just above the basal layer, and in pemphigus foliaceus pf, also called superficial pemphigus. The pemphigus family is a rare group of immunobullous conditions affecting skin and or mucous membranes. Castleman tumor, a rare lymphoproliferative disorder, is one of the associated tumors in paraneoplastic pemphigus pnp. Paraneoplastic pemphigus pnp is an autoimmune disease that adversely affects the skin and mucous membranes. Desmoglein forms the glue that attaches adjacent epidermal cells via attachment points called desmosomes. Paraneoplastic pemphigus is rare, and, while the exact incidence is unknown, it is likely underreported.
Prognostic factors of paraneoplastic pemphigus dermatology. On the basis of their unique clinical, histologic, and immunofluorescence findings, readers should also be able to differentiate these new pemphigus variants from the common forms of pemphigus. Paraneoplastic pemphigus fitzpatricks dermatology, 9e. Symptoms typically include blisters in the oral cavity and esophagus as well as in the genital region and sometimes the lungs. This type can also cause small bumps with pus inside. Among human neoplasms thymomas are associated with highest frequency with paraneoplastic autoimmune diseases. Paraneoplastic pemphigus pnp 2 is an uncommon disorder that shares overlapping clinical features with sjs 3 and immunochemical features with pemphigus vulgaris. All reported patients with an associated malignant neoplasm have had a poor prognosis. You may do so in any reasonable manner, but not in.
Pemphigus vegetans causes thick sores in the groin and under the arms. The exact percentages vary as additional cases accrue, but the three most commonly associated neoplasms have always been nonhodgkins lymphoma, chronic lymphocytic leukemia, and castlemans disease. Fever, chills and other flulike symptoms may present as well. In 2010, 450 cases were reported in the literature. Individuals with certain types of cancer, such as lymphoma, are often diagnosed with. The clinical course of paraneoplastic pemphigus tends to be rapid and fatal despite immunosuppressive therapy. The disease can be occasionally associated with a paraneoplastic pemphigus pnp, an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin. Ings of paraneoplastic pemphigus associated with castle mans disease. Pemphigus genetic and rare diseases information center. Paraneoplastic pemphigus pnp is an autoimmune disorder stemming from an underlying tumor. Our patient lived almost 8 years after the onset of paraneoplastic pemphigus, the longest time that anyone with this disease is known to have survived. The three main categories of pemphigus are paraneoplastic pemphigus, pemphigus vulgaris and pemphigus foliaceus. Because this is a complex condition to diagnose, special testing is needed. The pemphigus family are rare autoimmune blistering diseases affecting skin andor mucous membranes.
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