Plasma Cell Cheilitis: A Clinicopathological and Immunohistochemical Study of 13 Cases.

BACKGROUND: Plasma cell cheilitis is an unusual benign plasma cell proliferative disease of an unknown etiology that typically presents on the lip. OBJECTIVE: The aim of this study was to investigate the clinicopathological characteristics of 13 cases of plasma cell cheilitis. METHODS: The present study investigated the clinical manifestations, treatment modalities, and outcome of 13 patients diagnosed with plasma cell cheilitis from 2011 to 2016 at Kangdong Sacred Heart Hospital and Hallym University Sacred Heart Hospital. Biopsy specimens of the all cases were evaluated using conventional hematoxylin and eosin staining with kappa and lambda immunoglobulin light chain immunohistochemistry. RESULTS: The age of the patients ranged from 39 to 86 years (mean, 64.7 years), with male predominance. Histopathologically, 61.5% and 38.5% of patients showed band-like and pan dermal plasmacytic infiltrates, respectively. Eosinophilic infiltration was noted in 69.2% of patients. All cases showed both kappa and lambda immunoglobulin light chain reactivities, and kappa predominance was confirmed in 9 patients (69.2%). A majority of the patients was treated with local therapy, such as intralesional steroid injection with topical tacrolimus. Among the 13 patients, plasma cell cheilitis completely resolved, partially resolved, and recurred in 3 (23.1%), 5 (38.5%), and 5 patients (38.5%), respectively. CONCLUSION: Plasma cell cheilitis presented as erosive edematous circumscribed patches or plaques affecting mainly the lower lip of elderly male patients. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for immunoglobulin light chain, with kappa predominance.

Ectodermal Dysplasia-Skin Fragility Syndrome: A Rare Case Report.

Ectodermal dysplasia/skin fragility syndrome (ED-SFS) is a newly described autosomal recessive disorder characterized by skin fragility and blistering, palmoplantar keratoderma, abnormal hair growth, nail dystrophy, and occasionally defective sweating. It results from mutations in the PKP1 gene encoding plakophilin 1 (PKP1), which is an important component of stratifying epithelial desmosomes and a nuclear component of many cell types. Only 12 cases of this rare genodermatosis have been reported so far. We present an unusual case of ED-SFS in a 12-year boy who was normal at birth but subsequently developed skin fragility, hair and nail deformities, abnormal dentition, palmoplantar keratoderma, and abnormal sweating but no systemic abnormality.

Plasma cell gingivitis associated with inflammatory chelitis: a report on a rare case.

BACKGROUND: Plasma cell gingivitis (PGC) is a rare disease of gingival tissues which is difficult to treat. It has a higher rate of reoccurrence and needs a detailed and careful analysis of etiology. Further, its association with chelitis is rare, only few cases have been reported and the condition with this presentation poses a diagnostic dilemma. CASE REPORT: This paper reports a 16 year old male with a complaint of bleeding and swelling of gingiva since 3 years. The gingival enlargement occurred on facial aspect of upper and lower anterior teeth involving attached gingival. He also presented with swelling of both lips which was recurrent and for the same duration of 3 years. DISCUSSION: Based on clinical features and histopathological findings, a diagnosis of plasma cell gingivitis with inflammatory chelitis was made. A detailed history of possible allergen exposure was taken and patch test was conducted to identify any such allergens. Other conditions were ruled out by blood investigation, detailed medical and drug history. CONCLUSION: A close collaboration between Periodontist and Dermatologist is essential to manage such a case.

Plasma Cell Cheilitis: A Clinicopathological and Immunohistochemical Study of 13 Cases

BACKGROUND: Plasma cell cheilitis is an unusual benign plasma cell proliferative disease of an unknown etiology that typically presents on the lip. OBJECTIVE: The aim of this study was to investigate the clinicopathological characteristics of 13 cases of plasma cell cheilitis. METHODS: The present study investigated the clinical manifestations, treatment modalities, and outcome of 13 patients diagnosed with plasma cell cheilitis from 2011 to 2016 at Kangdong Sacred Heart Hospital and Hallym University Sacred Heart Hospital. Biopsy specimens of the all cases were evaluated using conventional hematoxylin and eosin staining with kappa and lambda immunoglobulin light chain immunohistochemistry. RESULTS: The age of the patients ranged from 39 to 86 years (mean, 64.7 years), with male predominance. Histopathologically, 61.5% and 38.5% of patients showed band-like and pan dermal plasmacytic infiltrates, respectively. Eosinophilic infiltration was noted in 69.2% of patients. All cases showed both kappa and lambda immunoglobulin light chain reactivities, and kappa predominance was confirmed in 9 patients (69.2%). A majority of the patients was treated with local therapy, such as intralesional steroid injection with topical tacrolimus. Among the 13 patients, plasma cell cheilitis completely resolved, partially resolved, and recurred in 3 (23.1%), 5 (38.5%), and 5 patients (38.5%), respectively. CONCLUSION: Plasma cell cheilitis presented as erosive edematous circumscribed patches or plaques affecting mainly the lower lip of elderly male patients. The majority of histopathology cases showed characteristic plasma cell aggregation on the upper dermis that was immunopositive for immunoglobulin light chain, with kappa predominance.