Plasma cell cheilitis is an idiopathic benign inflammatory condition,characterized by dense plasma cell infiltrates in the lips and other mucosae close to body orifices. It has a higher rate of reoccurrence and needs a detailed and careful analysis of etiology. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive. Overview of plasma cell disorders hematology and oncology.
Clinical examination showed well demarcated erythematous erosive plaques on his lower lip that histology subsequently confirmed as pcc fig. Symptomatic patients generally have abundant atypical plasma cells but focal marrow involvement may. The reason that a biopsy may be done is because actinic cheilitis have a greater chance of transforming into a malignancy because they can be a precancerous condition for developing skin cancer. Takamasa ito, ken natsuga, shintaro tanimura, satoru aoyagi, hiroshi shimizu doi. B kumar, r sharma, m rajagopalan, and b d radotra department of dermatology, postgraduate institute of medical education and research, chandigarh, india. Cheilitis is an acute or chronic inflammation of the lips. Melkerson rosenthal syndrome cheilitis granulomatosa fissured tongue oral granulomas. Treatment with topical clobetasol propionate was effective.
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. Plasma cell gingivitis pcg is a rare inflammatory disease of the gums, which is defined by plasma cell infiltration into subepithelial tissue. Clinically granulomatous cheilitis with plasma cells. Plasma cell cheilitis is an unusual benign plas ma cell proliferative. Successful treatment of plasma cell cheilitis with topical tacrolimus. It usually involves the lip vermilion and the vermilion border, but the surrounding skin and the oral mucosa may also be affected. Learn about the veterinary topic of oral inflammatory and ulcerative disease in small animals. Plasma cell cheilitis and plasmoacanthoma perhaps represent a spectrum of oral plasma cell mucositis with plasmoacanthoma being an advanced version of the former. Plasma cell cheilitis, journal of the european academy of. Jan 01, 2004 abstract plasma cell cheilitis is a rare inflammatory disorder of the lip with a characteristic band. 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. Gingivitis, cheilitis, and glossitis have been described as a triad for plasma cell gingivostomatitis 6. It presents clinically as a diffuse, erythematous and papillary lesion of the gingiva, which frequently bleeds, with minimal trauma. This simple presentation will highlight the key points in differentiating, diagnosing these orders.
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 department of dermatology, kangdong sacred heart hospital, hallym university college of. Lossoffunction mutations in cast cause peeling skin. Differential diagnosis should consider allergicirritant contact cheilitis, candidiasis, syphilis, queyrats erythroplasia, granulomatous cheilitis, plasmoacanthoma, plasmacytoma, squamous cell carcinoma and exfoliative or factitious. Histopathology images of plasma cell myeloma by pathpedia. 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. Plasma cell gingivitis, which is also called atypical gingivitis or plasma cell gingivostomatitis, is a rare benign inflammatory condition of unknown aetiology. Histopathology also helps in differentiation of this entity from discoid lupus erythmetaosus, lichenoid dermatoses, plasma cell cheiltis, cheilitis glandularis, actinic cheilitis or neoplasia 7,8. Nonsecretory myeloma plasma cell cheilitis pcc is a rare, idiopathic, and benign inflammatory mucosal condition characterised by dense polyclonal plasma cell infiltrates within the mucosa 1. The response to different therapeutic modalities is controversial, especially regarding the. Actinic cheilitis pictures, treatment, symptoms, causes. Dec 16, 2011 plasma cell disorders is a difficult topic where most residents and students confuse with regarding to differentiating between various types of paraproteinemias or plasma cell dyscrasias. Plasma cell cheilitis is due to normal aging and can start as early as age 30.
Case report a rare case of plasma cell gingivitis with. Plasma cell gingivitis pgc is a rare disease of gingival tissues which is difficult to treat. To raise awareness of plasma cell mucositis as a rare differential diagnosis for oral mucosal ulceration and its macroscopic similarity to malignancy. 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. Unlike their precursors, plasma cells cannot act as antigen presenting cells because they no longer display mhc ii. Actinic cheilitis and bowens disease both need to be distinguished from pagetoid dyskeratosis, which has been found as an incidental histological.
The patient successfully had a full excision of one lesion and a spontaneous. A rare case of plasma cell gingivitis with cheilitis. 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. Find specific details on this topic and related topics from the merck vet manual. Plasma cell gingivitis associated with inflammatory chelitis. 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 swallowing for over a period of 6 months. Dermoscopic features of plasma cell cheilitis and actinic. Small bumps, called milia, can develop from blocked pores along the outer lips. Plasma cell disorders is a difficult topic where most residents and students confuse with regarding to differentiating between various types of paraproteinemias or plasma cell dyscrasias. Oct 14, 2019 plasma cell lesions are the neoplastic clonal proliferation of plasma cells involving single bones plasmacytoma or multiple bones multiple myeloma. 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. Histopathologically it is characterized by dense plasma cell infiltrates in a bandlike pattern in dermis, which corresponds to zoons plasma cell balanitis.
Oral inflammatory and ulcerative disease in small animals. Histopathology images of plasma cell leukemia primary by. The present case is of an 18yearold male patient suffering from gingival enlargement along with cheilitis. Primary plasma cell leukemia is a rare malignancy defined as the presence of more than 20% plasma cells in the blood of all white blood cells or greater than 2,000 plasma cells per micro liter in blood. Exfoliative cheilitis due to habitual lip biting and. Dermoscopic features of plasma cell cheilitis and actinic cheilitis takamasa ito, ken natsuga, shintaro tanimura, satoru aoyagi, hiroshi shimizu doi. This benign condition is characterized by gingival erythema and edema. 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. Since b cell maturation also involves somatic hypermutation a process completed before differentiation into a plasma cell, these antibodies frequently have a. A dense collection of mature plasma cells predominated in the biopsy specimen from a mass on the lower lip of a 67yearold woman. Plasma cell cheilitis presented as erosive edematous circumscribed patches or plaques affecting mainly the lower lip of elderly male patients. 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. Lip biopsy examination will show the upper dermis with a dense bandlike lichenoid infiltrate made up of mature plasma cells 16 19 20.
Plasma cell gingivitis associated with inflammatory. In orofacial granulomatosis, sections show oral mucosa with a sparse inflammatory infiltrate and mild oedema figure 1. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for immunoglobulin light chain, with kappa predominance. Department of dermatology, kangdong sacred heart hospital, hallym university college of medicine, seoul, korea. The patient successfully had a full excision of one lesion and a spontaneous resolution of the. Attempts to identify specific benign or malignant processes failed, attempts to induce plasma cell infiltrations on mucosal and nonmucosal surfaces by allergic and. Plasma cell cheilitis jama dermatology jama network. Plasmoacanthoma and plasma cell cheilitis are rare disorders of obscure etiology characterized by a plasma cell infiltrate. Clinically, the lesions need to be distinguished from plasma cell cheilitis, 58 cheilitis granulomatosis melkerssonrosenthal syndrome see p. The histology reveals a lymphocytic infiltration and, in some cases, the. Case report a rare case of plasma cell gingivitis with cheilitis. Plasma cell cheilitis mimicking a melkerssonrosenthal syndrome. For example, plasma cells will likely secrete igg3 antibodies if they matured in the presence of the cytokine interferongamma.
Successful treatment of plasma cell cheilitis with topical. In general, physicians and dentists know little about what causes oral tumor development and progression. Hypersensitivity reaction due to antigen is considered as primary etiological factor. Clinically granulomatous cheilitis with plasma cells sarkar s. Orofacial granulomatosis is also referred to as granulomatous cheilitis, and is characterised clinically by chronic relapsing swelling of the lips and oral mucosa.
Successful treatment of plasma cell cheilitis with topical calcineurin inhibitors. Plasma cell cheilitis pcc is an inflammatory disorder of unknown etiology that affects the lip. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for immunoglobulin light chain. Actinic cheilitis actinic cheilitis ac is a chronic inflammatory process that affects the lower lip in 95% of the cases. Although the etiology may be unknown, this is believed to be a hypersensitivity reaction in some cases. Report of two cases yuka hanami md, yoshikazu motoki md, toshiyuki yamamoto md dermatology online journal 17 2. Exfoliative cheilitis may resolve spontaneously, but if persistent, it is usually refractory to treatment and difficult to. 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. Lip biopsy should reveal evidence of granulomatous inflammation, edema, perivascular infiltrate of plasma cells and lymphocytes and dilatation of lymphatic. Calpastatin is an endogenous specific inhibitor of calpain, a calciumdependent cysteine protease. Plasmoacanthoma of oral cavity and plasma cell cheilitis. Plasma cell cheilitis is an uncommon chronic inflammatory dermatitis that presents with flat to slightly elevated erosive. Plasma cell cheilitis pcc is a rare, idiopathic, and benign. Abstract plasma cell cheilitis is a rare inflammatory disorder of the lip with a characteristic band.
Symptomatic patients generally have abundant atypical plasma cells but focal marrow involvement may yield only scattered but morphologically atypical plasma cells. Actinic cheilitis and squamous cell carcinoma of the lip. On the lips, the condition appears as sharply outlined, infiltrated, dark red. It is caused by chronic exposure to sunlight or artificial ultraviolet radiation. Dermatology department, pathology department, hospital geral santo antonio, oporto. The response to different therapeutic modalities is controversial, especially regarding the effectiveness of corticosteroids. Plasma cell gingivitis associated with cheilitis is a very rare condition, and only a few cases have been reported in the literature. Plasma cell cheilitis is a rare inflammatory disorder of the lip with a. Plasma cell cheilitis is a benign inflammatory disease that belongs to the. Plasma cell cheilitis pcc is a rare, idiopathic, and benign inflammatory mucosal condition characterised by dense polyclonal plasma cell infiltrates within the mucosa 1. We report a patient who presented with oral features suggestive of malignancy. The condition has been reported under a wide variety of names to affect the penis, vulva,lips, buccal mucosa, palate, gingiva, tongue, epiglottis. Squamous cell carcinoma of the lip is reported together with oral carcinomas in the brazilian official statistics. Dermoscopic features of plasma cell cheilitis and actinic cheilitis.
Actinic cheilitis is the main precancerous lesion of the lip. Overall, they account for 40% of the head and neck carcinomas. Histopathology of gingival tissue revealed dense infiltrate of plasma cell in subepithelial connective tissue with peculiar cart wheel appearance and that of lip showed chronic inflammatory infiltrate of lymphocytes without specific granulomas. Thus, the final diagnosis was plasma cell gingivitis with inflammatory cheilitis. Plasma cell lesions are the neoplastic clonal proliferation of plasma cells involving single bones plasmacytoma or multiple bones multiple myeloma. It is characterized histologically by a dense infiltrate of plasma cells with a variety of clinical features. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for.
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. Here we show that lossoffunction mutations in calpastatin cast are the genetic causes of an autosomalrecessive condition characterized by generalized peeling skin, leukonychia, acral punctate keratoses, cheilitis, and knuckle pads, which we propose to be given the acronym plack syndrome. 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. Plasma cell gingivitis pcg is a rare condition of the gingiva, characterized histopathologically by infiltration of plasma cells in connective tissue.
The cause is unknown, and there are no tests to aid in diagnosis. It usually presents as a circumscribed, flat to slightly raised, eroded plaque or patch, usually on the lower lip of elderly individuals 1. 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. Plasma cells are usually prominent, particularly beneath areas of ulceration. Common symptoms include erythema, dryness, scaling, fissuring, edema, itching, and burning.
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