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

Abstract

BackgroundPlasma cell cheilitis is an unusual benign plasma cell proliferative disease of an unknown etiology that typically presents on the lip.ObjectiveThe aim of this study was to investigate the clinicopathological characteristics of 13 cases of plasma cell cheilitis.MethodsThe 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.ResultsThe 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.ConclusionPlasma 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.

Plasma cell cheilitis: the diagnosis of a disorder mimicking lip cancer

“…Considering that PCC is rarely considered a differential diagnosis in the clinical routine, our provisional clinical diagnosis (actinic cheilitis or lip squamous cell carcinoma) was based on the most frequent ones for the clinical features of the patient. The histological diagnosis is based on the identification of a sheet-like infiltration of monomorphic mature plasma cells without anaplasia or prominent nucleoli, and Russel bodies are found occasionally 2 , 5 , 6. The clinicopathologic features of the current case were consistent with PCC.…”

“…One theory speculates that inflammatory cells such as T cells and macrophages affect the growth and differentiation of B cells 4 , 5 , 15 , 16. Another theory holds that PCC might be a response to exogenous factors such as trauma and solar damage 2 , 6. We assume that solar damage might have triggered PCC, considering our patient is a farmer who is constantly exposed to the sun without UV protection.…”

“…The lesion is presented as circumscribed erosive or erythematous plaques or patches on the labial mucosa, predominantly on the lower lip 1 , 3 , 4. Histopathologically, PCC consists of a proliferation of mature plasma cells distributed as a dense band-like subepithelial infiltrate 2 , 5 , 6. Additionally, the epithelium may present dyskeratosis, intercellular edema, erosion or ulceration, hyperkeratosis, and vacuolar degeneration in the conjunctival-epithelial junction 2 , 7.…”

“…Plasma cell cheilitis (PCC) is a rare site-specific type of plasma cell mucositis reported in older adults, with higher prevalence in men. 1 , 2 The lesion is presented as circumscribed erosive or erythematous plaques or patches on the labial mucosa, predominantly on the lower lip. 1 , 3 , 4 Histopathologically, PCC consists of a proliferation of mature plasma cells distributed as a dense band-like subepithelial infiltrate.…”

Criticisms and Controversies in the Diagnosis of Cheilitis

“…The clinical examination frequently leads to an initial misdiagnosis, which requires biopsy. Treatment include intralesional or systemic steroid and local application of immunomodulatory agents (e.g., tacrolimus, pimecrolimus) [8].…”