Case Letter

A Severe Presentation of Plasma Cell Cheilitis

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Plasma cell cheilitis (PCC) is an uncommon condition characterized by mature plasma cell infiltration of the dermis of the mucosal lip. The condition often presents as a red-brown patch or plaque on the lower lip in older individuals that can progress to erosions and edema. Diagnosis can be delayed because clinical findings are nonspecific and can mimic neoplastic, infectious, and inflammatory conditions. We describe a patient with PCC who presented to our institution via teledermatology. Findings were equivocal on 2 early biopsies until the presentation evolved to dramatic ulceration and necrosis, which prompted a third biopsy that was diagnostic for PCC. Empiric therapy with a class I topical corticosteroid was successful.


  • Plasma cell cheilitis (PCC) is a benign condition that affects the lower lip in older individuals, presenting as a nonspecific, red-brown patch or plaque that can progress slowly to erosions and edema.
  • Our patient with PCC experienced full resolution of symptoms with application of a class I topical corticosteroid.



Plasma cell cheilitis (PCC), also known as plasmocytosis circumorificialis and plasmocytosis mucosae,1 is a poorly understood, uncommon inflammatory condition characterized by dense infiltration of mature plasma cells in the mucosal dermis of the lip.2-5 The etiology of PCC is unknown but is thought to be a reactive immune process triggered by infection, mechanical friction, trauma, or solar damage.1,5,6

The most common presentation of PCC is a slowly evolving, red-brown patch or plaque on the lower lip in older individuals.2,3,5,7 Secondary changes with disease progression can include erosion, ulceration, fissures, edema, bleeding, or crusting.5 The diagnosis of PCC is challenging because it can mimic neoplastic, infectious, and inflammatory conditions.8,9

Treatment strategies for PCC described in the literature vary, as does therapeutic response. Resolution of PCC has been documented after systemic steroids, intralesional steroids, systemic griseofulvin, and topical calcineurin inhibitors, among other agents.6,7,10-16

We present the case of a patient with a lip lesion who ultimately was diagnosed with PCC after it progressed to an advanced necrotic stage.

Case Report

An 80-year-old male veteran of the Armed Services initially presented to our institution via teledermatology with redness and crusting of the lower lip (Figure 1). He had a history of myelodysplastic syndrome and anemia requiring iron transfusion. The process appeared to be consistent with actinic cheilitis vs squamous cell carcinoma. In-person dermatology consultation was recommended; however, the patient did not follow through with that appointment.

Figure 1. Ill-defined, red-brown patch of shallow erosions on the lower lip at the initial presentation.

Five months later, additional photographs of the lesion were taken by the patient's primary care physician and sent through teledermatology, revealing progression to an erythematous, yellow-crusted erosion (Figure 2). The medical record indicated that a punch biopsy performed by the patient’s primary care physician showed hyperkeratosis and fungal organisms on periodic acid–Schiff staining. He subsequently applied ketoconazole and terbinafine cream to the lower lip without improvement. Prompt in-person evaluation by dermatology was again recommended.

Figure 2. Well-defined, 2.0-cm, erythematous, yellow-crusted erosion on the right lower lip 5 months after the initial presentation.

Ten days later, the patient was seen in our dermatology clinic, at which point his condition had rapidly progressed. The lower lip displayed a 3.0×2.5-cm, yellow and black, crusted, ulcerated plaque (Figure 3). He reported severe burning and pain of the lip as well as spontaneous bleeding. He had lost approximately 10 pounds over the last month due to poor oral intake. A second punch biopsy showed benign mucosa with extensive ulceration and formation of full-thickness granulation tissue. No fungi or bacteria were identified.

Figure 3. After 10 days, the lesion progressed to a well-defined, 3.0×2.5-cm, yellow and black, crusted, ulcerated plaque on the right lower lip. Punch biopsy sites were selected at the margin of the plaque.

Consultation and Histologic Analysis
Dermatopathology was consulted and recommended a third punch biopsy for additional testing. A repeat biopsy demonstrated ulceration with lateral elements of retained epidermis and a dense submucosal chronic inflammatory infiltrate comprising plasma cells and lymphocytes (Figures 4 and 5). Immunohistochemical staining demonstrated a mixed inflammatory infiltrate with CD3+ T cells and CD20+ B cells. In situ hybridization studies demonstrated numerous lambda-positive and kappa-positive plasma cells without chain restriction. Periodic acid–Schiff with diastase and Grocott-Gomori methenamine-silver staining demonstrated no fungi. Findings were interpreted to be most consistent with a diagnosis of PCC.

Figure 4. A repeat biopsy demonstrated ulceration with lateral elements of retained epidermis and a dense submucosal chronic inflammatory infiltrate (H&E, original magnification ×2).

Figure 5. On higher magnification, the inflammatory infiltrate was noted to comprise plasma cells and lymphocytes (H&E, original magnification ×20).

Treatment and Follow-up
The patient was treated with clobetasol ointment 0.05% twice daily for 6 weeks and topical lidocaine as needed for pain. At 6-week follow-up, he displayed substantial improvement, with normal-appearing lips and complete resolution of symptoms.


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