The response to different therapeutic modalities is controversial, especially regarding the. Other than the lip, genital areas are often involved, plasma cell balanitis or vulvitis. Pdf plasmoacanthoma of oral cavity and plasma cell. Enlargement and chronic exposure of the mucous membrane on the lower lip becomes affected by the environment, leading to erosion, ulceration, crusting, and, occasionally, infection. Plasma cell gingivitis pcg is a rare inflammatory disease of the gums, which is defined by plasma cell infiltration into subepithelial tissue. For example, plasma cells will likely secrete igg3 antibodies if they matured in the presence of the cytokine interferongamma. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for immunoglobulin light chain, with kappa predominance. Germinal center cell differentiation into plasma cells 515 days after initial antigen exposure, a portion of the germinal center b cells will upregulate irf 4 critical for plasma cell differentiation irf4 knockout mice lack ig secreting plasma cells irf4 overexpression promotes plasma cell differentiation. Differential diagnosis should consider allergicirritant contact cheilitis, candidiasis, syphilis, queyrats erythroplasia, granulomatous cheilitis, plasmoacanthoma, plasmacytoma, squamous cell carcinoma and exfoliative or factitious. Plasma cell cheilitis is a very rare presentation of a condition which more. The response varies from case to case and the criteria for medication choice are empirics. In the usa, plasma cells myeloma corresponds to 1% of the total of all malignant tumors and to 10% to 15% of the hematopoietic neoplasms 8,9. Plasma cell cheilitis jama dermatology jama network.
The etiology is largely unknown, but it is thought to be due to a hypersensitivity. The treatment of exfoliative cheilitis represents a clinical challenge. Although the histological picture is shared with other diseases of the lips, plasma cell cheilitis is not associated with any known dermatoses. Thus, people with plasma cell disorders are often at higher risk of infections. These cells are derived from mesenchyme cells which give rise to other types of connective tissue as well. Plasma cell gingivitis pcg is a rare condition of the gingiva, characterized by in fi ltration of plasma cells. This simple presentation will highlight the key points in differentiating, diagnosing these orders. Plasma cells develop from b cells b lymphocytes, a type of white blood cell that normally produces.
Plasma cell cheilitis appears as well defined, infiltrated, dark red plaque with a superficial lacquerlike glazing. Plasma cell cheilitis is a rare, idiopathic, and benign inflammatory mucosal condition characterised by a dense plasma cell infiltrate within the mucosa. Plasma cell vulvitis the bc centre for vulvar health. The patient successfully had a full excision of one lesion and a spontaneous resolution of the. Vitiligo is a progressive depigmenting disorder characterized by the presence of white patches and hairs due to destruction of functional mela nocytes in the epidermis. We present a case of plasma cell cheilitis, with the differential diagnosis and options for treatment. Small bumps, called milia, can develop from blocked pores along the outer lips. Plasma cell stomatitis pcs, an uncommon condition, is characterized by massive and dense infiltration of plasma cells into the connective tissue. Occasionally, the lesion became increasingly swollen and painful and. Cheilitis glandularis is a rare inflammatory disorder of the lip. Plasmoacanthoma and plasma cell cheilitis are rare disorders of obscure etiology characterized by a plasma cell infiltrate an 80yearold woman presented with a verrucous, fleshy, skin colored plaque over lips, gingiva, and the palate and painful. Jan 01, 2004 abstract plasma cell cheilitis is a rare inflammatory disorder of the lip with a characteristic band. Dermoscopic features of plasma cell cheilitis and actinic. It is mainly characterized by swelling of the lip with hyperplasia of the salivary glands.
Clinically granulomatous cheilitis with plasma cells. Mproteins may consist of both heavy and light chains. Plasma cell cheilitis is a rare inflammatory disorder which shows a dense, bandlike infiltrate of plasma cells in the upper dermis. It presents clinically as a diffuse, erythematous and papillary lesion of the gingiva, which frequently bleeds, with minimal trauma 3.
Plasma cell cheilitis is an uncommon chronic inflammatory dermatitis that presents with flat to slightly elevated erosive. We report a patient who presented with oral features suggestive of malignancy. Plasmoacanthoma and plasma cell cheilitis are rare disorders of obscure etiology characterized by a plasma cell infiltrate. Cheilosis can be defined as a medical condition, in which the corners of the mouth becomes inflamed. Plasma cell cheilitis, journal of the european academy of.
Scottsdale, arizona rochester, minnesota jacksonville, florida. Plasma cell cheilitis is a very rare presentation of a condition which more usually occurs on the gingiva termed plasma cell gingivitis or sometimes the tongue. Although the etiology may be unknown, this is believed to be a hypersensitivity reaction in some cases. Plasma cells article about plasma cells by the free dictionary.
In the present case, removal of the gingival lesion by gingivectomy resulted in the reduction of lip swelling suggesting that the combined lesion of the. Background plasma cell cheilitis is an unusual benign plasma cell proliferative disease of an unknown etiology that typically presents on the lip. Plasma cell meningitis is an uncommon neurologic complication of multiple myeloma with a very poor prognosis median overall survival of 3 months. Oral inflammatory and ulcerative disease in small animals. An 80yearold woman presented with a verrucous, fleshy, skin colored plaque over lips, gingiva, and the palate and painful swallowing for over a period of 6 months. Feline plasma cell pododermatitis grove heights mn home. Plasma cell cheilitis, also known as plasma cell orificial mucositis is a benign inflammatory condition clinically characterized by erythematous plaque on lips that may be ulcerated. Plasma cell cheilitis is an extremely rare disease, characterized by erythematousviolaceous, ulcerated and asymptomatic plaques, which evolve slowly. The everincreasing number of abnormal plasma cells also invades and damages various tissues and organs, and the antibody produced by the clone of plasma cells can sometimes damage vital organs, especially the kidneys and bones. The patient successfully had a full excision of one lesion and a spontaneous. Overview of plasma cell disorders hematology and oncology. Case report a rare case of plasma cell gingivitis with.
The resulting group of genetically identical cells called a clone produces a large quantity of a single type of antibody immunoglobulin. Successful treatment of plasma cell cheilitis with topical. The histological characteristics include dermal infiltrate composed of mature plasmocytes. Jun 15, 2010 plasma cell gingivitis associated with cheilitis is a very rare condition, and only a few cases have been reported in the literature. Plasma cells are usually prominent, particularly beneath areas of ulceration. Request pdf plasma cell cheilitis, successfully treated with topical 0. Plasma cells are the effector cells dedicated to the production of a high amount of antibodies, while the immunological memory is maintained by memory b cells that are the effectors of rapid immunological response upon later exposure to the same antigen klein and dallafavera, 2008. Plasma cell gingivitis is a rare condition, appearing as generalized erythema redness and edema swelling of the attached gingiva, occasionally accompanied by cheilitis lip swelling or glossitis tongue swelling. Learn about the veterinary topic of oral inflammatory and ulcerative disease in small animals.
Plasma cell definition of plasma cell by medical dictionary. Plasma cell cheilitis pcc is a rare, idiopathic, and benign inflammatory mucosal condition characterised by dense polyclonal plasma cell infiltrates within the mucosa 1. It usually involves the lip vermilion and the vermilion border, but the surrounding skin and the oral mucosa may also be affected 1. Plasma cell cheilitis pcc is an inflammatory disorder of unknown etiology that affects the lip.
A dense collection of mature plasma cells predominated in the biopsy specimen from a mass on the lower lip of a 67yearold woman. Thus, in this case, as we assumed, the significant development of t cells may be a key for the presence of plasma cell cheilitis. This condition is also known as angular cheilitis, perleche or angular stomatitis. Bowens disease of the glans penis erythroplasia of. Find specific details on this topic and related topics from the msd vet manual. The disease formerly known as lymphocyticplasmacytic.
To raise awareness of plasma cell mucositis as a rare differential diagnosis for oral mucosal ulceration and its macroscopic similarity to malignancy. It usually presents as a circumscribed, flat to slightly raised, eroded plaque or patch, usually on the lower lip of elderly individuals 1. Takamasa ito, ken natsuga, shintaro tanimura, satoru aoyagi, hiroshi shimizu doi. Actinic cheilitis and bowens disease both need to be distinguished from pagetoid dyskeratosis, which has been found as an incidental histological. Plasma cell cheilitis presented as erosive edematous circumscribed patches or plaques affecting mainly the lower lip of elderly male patients. We present herein two cases of plasma cell cheilitis, in which topical tacrolimus showed beneficial effects, suggesting that this immunomodulatory agent is a promising option for plasma cell cheilitis. Cheilitis glandularis swelling and eversion of lower lip with patulous openings of the ducts of the mucous glands chronic, inflammatory. Plasma cell diseases include multiple myeloma, systemic lightchain al amyloidosis, and monoclonal immunoglobulin deposition disease. Department of dermatology, kangdong sacred heart hospital, hallym university college of medicine, seoul, korea. B kumar, r sharma, m rajagopalan, and b d radotra department of dermatology, postgraduate institute of medical education and research, chandigarh, india. Cheilosis pictures, symptoms, causes and treatment 2018. Plasma cell cheilitis, successfully treated with topical 0. Gingivitis, cheilitis, and glossitis have been described as a triad for plasma cell gingivostomatitis 6.
Plasma cell cheilitis pcc is a rare, idiopathic, inflammatory disease that occurs on the lip, with histological findings characteristically showing. Case report a rare thymoma case with seven paraneoplastic. Plasma cell neoplasms, clinicopathological characteristics. A plasma cell is a fully differentiated, mature lymphocyte in the b cell lineage. It is histologically characterized by plasma cell infiltrates into the mucosa. Plasma cell dyscrasias refer to a spectrum of disorders characterized by the monoclonal proliferation of lymphoplasmacytic cells in the bone marrow and, sometimes, tissue deposition of monoclonal immunoglobulins or their components table 211. A clinicopathological and immunohistochemical study of cases jin yong lee, kwang ho kim, 1 ji eun hahm, jae won ha, won joo kwon, 1 chul woo kim and sang seok kim. Plasma cell cheilitis is due to normal aging and can start as early as age 30. Overview of plasma cell disorders blood disorders msd. Plasma cell diseases are a type of blood cancer in which plasma cells become malignant and can cause damage to the bones, kidneys, heart, bone marrow and immune system, and as a result can make patients sick.
A patient with involvement of the lips and epiglottis associated with psoriasis and fissured tongue is described. Plasma cell cheilitis is an uncommon chronic inflammatory dermatitis that presents with flat to slightly elevated erosive erythematous plaques. Plasma cell disorders are of unknown etiology and are characterized by the disproportionate proliferation of one clone. Plasma cell gingivitis pcg, an infrequent benign inflammatory condition of unknown etiology, is a type of plasma cell orificial mucositis, which includes a wide spectrum of conditions. We present the case of a yearold girl who had pcg with an erythematous congestive plaque on the anterior maxillary gingiva for 4 years. Squamous cell carcinoma arising from plasma cell cheilitis. Oct 14, 2019 plasma cell lesions are the neoplastic clonal proliferation of plasma cells involving single bones plasmacytoma or multiple bones multiple myeloma.
Actinic cheilitis an overview sciencedirect topics. The response to different therapeutic modalities is controversial, especially regarding the effectiveness of corticosteroids. Plasma cell cheilitis is a rare, idiopathic mucosal condition, which has previously been given scant attention among oral and maxillofacial publications. Plasma cell myeloma multiple myeloma, myelomatosis, medullary plasmacytoma is a bone marrowbased, malignant disorder of postgerminal center bcells that is characterized by a clonal prol.
Plasma cell cheilitis commonly involves the lower lip of the elderly, presenting as a flat to raised, erythematous plaque with a dry, atrophic, shiny surface and varying degrees of fissuring. Since b cell maturation also involves somatic hypermutation a process completed before differentiation into a plasma cell, these antibodies frequently have a very high affinity for their antigen. All the formed elements are originally derived from a. Abstract plasma cell cheilitis is a rare inflammatory disorder of the lip with a characteristic band. An ovoid cell with an eccentric nucleus having chromatin arranged like a clock face or spokes of a wheel. Nov 23, 2009 this includes angular cheilitis, plasma cell cheilitis, actinic cheilitis cheilitis glandularis, cheilitis granulomatosa, exfoliative cheilitis and factitious cheilitis. As with most cell types, plasma cells can mutate to give rise to cancer. They begin when a single plasma cell multiplies excessively. This benign condition is characterized by gingival erythema and edema. Dermoscopic features of plasma cell cheilitis and actinic cheilitis takamasa ito, ken natsuga, shintaro tanimura, satoru aoyagi, hiroshi shimizu doi. The pathogenesis is unknown, but may be a hypersensitivity or an autoimmune process. Plasma cell disorders may be suspected because of clinical manifestations most often bone disease, renal failure, and low blood counts, or an incidental finding of elevated serum protein or proteinuria that leads to further evaluation with serum or urine protein electrophoresis. The cause is unknown, and there are no tests to aid in diagnosis.
Plasma cell vulvitis pcv is a rare chronic inflammatory skin condition that can affect the skin of the vulva. It is called plasma cell gingivitis where the gingiva gums are involved, plasma cell cheilitis, where the lips are involved, and other terms such as plasma cell orifacial mucositis, or plasma cell gingivostomatitis where several sites in the mouth are involved. Melkerssonrosenthal syndrome, plasma cell cheilitis, and nonvenereal sclerosing lymphangitis of the penis are. Histopathologically it is characterized by dense plasma cell infiltrates in a bandlike pattern in dermis, which corresponds to zoons plasma cell balanitis. The result is a corresponding increase in the serum level of its product, the monoclonal immunoglobulin protein mprotein. It is characterized histologically by a dense infiltrate of plasma cells with a variety of clinical features. Bone and extraosseous plasmacytomas comprise 3% to 5% of plasmatic cell neoplasms in the usa. Pdf plasma cell stomatitis associated with khat catha. Further examples of cheilitis glandularis were described by sutton,lo, i1 schamberg,g and others.
Attempts to identify specific benign or malignant processes failed, attempts to induce plasma cell infiltrations on mucosal and nonmucosal surfaces by allergic and irritant stimuli were not successful. Plasma cell cheilitis pcc is a rare sitespecific type of plasma cell mucositis reported in older adults, with higher prevalence in men. Overview of plasma cell disorders blood disorders merck. Plasma cell gingivitis12 is a rare condition,3 appearing as generalized erythema and edema of the attached gingiva, occasionally accompanied by cheilitis. Successful treatment of plasma cell cheilitis with topical tacrolimus. A type of white blood cell that produces and secretes antibodies. It is a benign noncancerous condition and is not due to an infection and can not be transmitted to other people. On the lips, the condition appears as sharply outlined, infiltrated, dark red. Bowens disease of the glans penis erythroplasia of queyrat. Plasma cell gingivitis which is also called atypical gingivitis or plasma cell gingivostomatitis, is a rare benign inflammatory condition of unknown aetiology.