Dear Editor,
A 40-year-old female presented with persistent swelling and redness of both upper and lower lips for the past 11 years. She had occasional pruritus over the lips. There was no history of lip licking, application of cosmetics, or use of any herbal tooth paste. She did not give history of gumming of the lips during early morning or any associated co-morbidities. She had symptoms suggestive of acid peptic disease. She had taken various modalities of treatments for this complaint, both systemic and topical, without much improvement. On examination, there was swelling and erythema diffusely involving both upper and lower lips, with hypopigmentation surrounding the lips [Figure 1]. Some areas showed vertical fissures and crusted erosions. On palpation, there was mild induration of the lips. There was no features of facial palsy or fissuring of the tongue. The inner aspect of the lips, other parts of oral mucosa, and gingiva were normal. General examination and systemic examination were normal.
Figure 1.
Edema, erythema, fissuring, and crusting of upper and lower lips
Blood hemogram, liver, and renal function tests and peripheral blood smear were normal. Bence Jones protein in urine was negative. Serum electrophoresis was normal. Venereal Disease Research Laboratory and Treponema Pallidium Hemagglutinationn Assay (VDRL and TPHA) were negative. Viral markers for HIV, hepatitis B and C were negative. ANA (Antinuclear Antibody) profile was negative. Ultrasound abdomen did not show any abnormality. Esophago-gastro-duodenoscopy for the acid peptic disease showed antral gastritis.
Histopathological examination of lip biopsy showed intense inflammation of superficial dermis with markedly predominant plasma cells [Figure 2]. Lymphocytes and eosinophils were also noted underneath the superficial dermis. Deep dermis showed congestion and mild perivascular lymphocytes.
Figure 2.
Upper and mid-dermis infiltrated with plasma cells (Red box) and lymphocytes (H&E, 400x)
Immunohistochemistry (IHC) showed polyclonal infiltrate of plasma cells with predominance of lambda positivity [Figure 3]. Histopathological and immunohistochemical features were consistent with plasma cell cheilitis (PCC). The patient was initially treated with griseofulvin but there was no response. She was then started on 40 mg prednisolone and gradually tapered over a period of one and half months and showed excellent response to oral prednisolone [Figure 4]. The patient is on regular follow-up for every three months for the past one year, the last follow-up being two months ago. She is under remission and there are no new lesions.
Figure 3.
Immunohistochemistry showing positive staining for plasma cells with lambda lineage (IHC, 400x)
Figure 4.
Excellent response following systemic prednisolone
Our patient presented with a chronic history of lip swelling and erythema. There was no history of topical applications or systemic illness. There were no features of facial palsy or fissuring of tongue (scrotal tongue). Skin biopsy showed the dermis to be infiltrated with plasma cells and immunocytochemistry showed plasma cell lineage. Correlating the clinical, histopathological, and immunohistochemical findings, the diagnosis of PCC was made.
PCC is a rare condition of unknown etiology. The exact etiopathogenesis in not clear. It is thought to be an immune meditative reactive process triggered by infections, trauma, mechanical friction, dental mouth washes, and solar damage.[1] It presents with asymptomatic chronic lip swelling sometimes mimicking angioedema. Angioedema is transient while PCC persists. Granulomatous cheilitis presents with lip swelling along with facial palsy and scrotal tongue (Melkersson-Rosenthal syndrome), and the histopathology shows granulomatous infiltrate and not plasma cells. Cheilitis glandularis presents with upper lip swelling and histopathology shows salivary gland hyperplasia. PCC is considered to be the counterpart of Zoon’s balanitis in the oral cavity.[2] It usually affects the lips in elderly women. The tongue, palate, gingiva, larynx, and epiglottis can also be involved.[3] There is no association of PCC with systemic disorders or multiple myeloma even though histopathology shows infiltration by plasma cells. There are no definite treatment modalities for PCC. Treatment reports are only anecdotal and there is no evidence regarding any specific treatment modality due to the rarity of the disease. Topical steroids, intralesional steroids, ciclosporine, tacrolimus, 308 nm excimer laser, and griseofulvin are the various treatment modalities mentioned in reports.[4,5] There has been a case report of 2% fusidic acid being effective for PCC probably due to the anti-inflammatory effect. There are no reports of systemic steroids being used in treatment for PCC. PCC should be considered in the differentials of lip swelling. We are reporting a rare case of PCC which responded well to systemic steroids.
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References
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