Dermatology Online Journal

A case of mucous membrane plasmacytosis successfully treated with cryotherapy
David James Najarian MD, Babar K Rao MD, Amy S Pappert MD
Dermatology Online Journal 14 (2): 6

Department of Dermatology, University of Medicine and Dentistry of New Jersey, Robert Wood Johnson Medical School. djn6u@yahoo.com

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Abstract

Mucous membrane plasmacytosis is a rare, often idiopathic, inflammatory disorder that frequently presents as an erythematous, velvety, or lobulated plaque on a mucosal surface. While mucous membrane plasmacytosis often runs a benign course, plaques are known to erode, ulcerate, and bleed. Moreover, according to a recent review of mucous membrane plasmacytosis, treatments of this disorder are inconsistently successful. We report a case of erosive, hemorrhagic mucous-membrane plasmacytosis of the lips treated successfully with cryotherapy. To the best of our knowledge, this case represents the second case of mucous membrane plasmacytosis successfully treated with cryotherapy. The long term response of our patient's condition to cryotherapy with no functional side effects may warrant further study of this technique for severe erosive mucous membrane plasmacytosis.

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Clinical synopsis

A 56-year-old male was referred to our clinic for a yellow crusted plaque on the lower lip for 2 weeks duration. He had a history of acquired immunodeficiency syndrome, a CD4+ T cell count of 12/mm³ (NL 623-1295), and a viral load of 279,000 copies/mL (NL less than 50). He also had a chronic, untreated hepatitis C virus infection. He was taking no medicine when he presented to us.

Physical examination revealed a 2 cm by 1 cm yellow crusted plaque on the lower lip. A bacterial culture of the plaque demonstrated abundant methicillin resistant Staphylococcus aureus (MRSA), and a viral culture of the plaque's eroded base was negative. A diagnosis of MRSA impetigo of the lip was made, and we prescribed linezolid 600 mg bid and mupirocin cream bid. In addition, although the viral culture was negative, we prescribed valacyclovir 1 gram twice daily for empiric treatment of labial herpes simplex virus (HSV).

Figure 1	Figure 2
Figure 1. Initial presentation of hemorrhagic, erosive, mucous membrane plasmacytosis Figure 2. Biopsy of the lip demonstrated a dense dermal infiltrate of plasma cells

Upon follow-up 2 weeks later the impetigo had completely resolved. However, painful, coagulated, hemorrhagic plaques were seen on both lips (Fig. 1). A repeat bacterial culture of the lip was negative and correlated with the clinical resolution of the impetigo. A biopsy of the lower lip was also performed. The epidermis was remarkable for lozenge-shaped keratinocytes and intercellular edema. There was no pseudoepitheliomatous hyperplasia, ballooning degeneration, reticular degeneration, nuclear inclusion bodies, or multinucleated keratinocytes. There was neither intra- nor sub-epidermal blistering. The dermis was remarkable for a dense infiltrate of mostly plasma cells (Fig. 2). Numerous dilated vessels with foci of extravasation were also observed. There were no neutrophilic abscesses. Direct immunofluoresence was unremarkable. Periodic acid schiff stain was negative for fungi. Immunoprecipitation demonstrated a slight increase of the κ to λ light chain ratio, but light chain restriction was not seen. Warthin-Starry stain failed to reveal Treponema pallidum, and repeated rapid plasma reagent tests were negative. At this point, clinical and histological features confirmed a diagnosis of hemorrhagic, erosive mucous membrane plasmacytosis (MMP).

A subset of cases of MMP may result from an allergic contact dermatitis, and a patch test to evaluate for this possibility was negative [1]. In addition, our patient's condition failed to resolve after he discontinued his use or consumption of toothpaste, mouthwashes, mints, and gums.

Figure 3	Figure 4
Figure 3. Coagulated, hemorrhagic plaques sealed the patient's lips together Figure 4. Appearance 2 days after treatment with cryotherapy

To treat his MMP we discontinued mupirocin cream and prescribed hydrocortisone butyrate 0.1 percent cream, 4 percent lidocaine cream, and petrolatum. Upon follow up 1 week later, his lips were sealed shut by coagulated, hemorrhagic crusts, and he was in severe distress (Fig. 3). We initiated a discussion of the risks and benefits of his therapeutic options, which have been described in a recent review of MMP (Table 1) [2]. The patient refused more potent topical glucocorticosteroids and refused glucocorticosteroid injections, but he agreed to a trial of cryotherapy, which has been reported once to induce remission of MMP [3]. (Figs. 4, 5, 6)

Figure 5	Figure 6
Figure 5. Appearance 2 weeks after treatment with cryotherapy Figure 6. Appearance 6 weeks after treatment with cryotherapy

His lips were unsealed with saline soaks. Then, after applying 4-percent lidocaine cream to the lips for 30 minutes, liquid nitrogen cryotherapy with the open-spray technique was performed on lip lesions with a freeze time of approximately 30 to 60 seconds (time elapsed between initial freeze and subsequent complete thaw). Hemostasis was then achieved with silver nitrate. Upon follow up 2 days later large crusts were seen on both lips, but the patient reported no bleeding and no pain, and he was able to eat easily (Fig. 4). By week 2 crusts fell off (Fig. 5), and by week 6 complete re-epithelialization with non-inflamed mucosa occurred (Fig. 6). On a followup visit 2 months after cryotherapy the patient complained of minor lip numbness but no other symptoms. Numbness had resolved at the 6 month followup visit.

Conclusion

We report a case of hemorrhagic, erosive, MMP of the lips; long-lasting remission was achieved with cryotherapy for a freeze time of approximately 30 to 60 seconds. Cryotherapy has been reported once to induce remission of MMP for 6 months, and the use of this modality also led to a 6-month remission for our patient [3]. The prolonged response of his condition to cryotherapy with no functional side effects may warrant further study of this technique for severe erosive MMP.

Acknowledgements: We are grateful to Melitza Suarez, M.D. for referring the patient to our service.

References

1. Sollecito TP, Greenberg MS. Plasma cell gingivitis. Report of two cases. Oral Surg Oral Med Oral Pathol 1992;73(6):690-3. PubMed

2. Bharti R, Smith DR. Mucous membrane plasmacytosis: a case report and review of the literature. Dermatol Online J 2003;9(5):15. PubMed

3. Morioka S, Nakajima S, Yaguchi H, Naito K, Iwahara K, Ogawa H. Vulvitis circumscripta plasmacellularis treated successfully with interferon alpha. J Am Acad Dermatol 1988;19(5 Pt 2):947-50. PubMed

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