Ciclosporin

Abstract

While ulcerative lichen planus is a common diagnosis when involving the mucosa, it is uncommonly found on the cutaneous surface. Cutaneous ulcerative lichen planus is usually found on the palmar or plantar surfaces (1,2) and has only rarely been described elsewhere (3-6). We describe a case of cutaneous ulcerative lichen planus involving the pretibia and exhibiting pathergy, which to our knowledge has not been previously reported. We also describe successful treatment with oral acitretin in conjunction with topical and intralesional corticosteroids.

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Case Report

A 50-year-old white female was referred to our clinic for a persistent ulcer on her right pretibial area 7 months following curettage and intralesional 5-flourouracil to treat a biopsyproven squamous cell carcinoma. The wound enlarged despite appropriate wound management, including outpatient therapy in a wound care center and compression therapy. Over the next several months, repeat biopsies were performed to exclude residual squamous cell carcinoma. She additionally received a course of doxycycline and rifampin to treat a culturepositive MRSA colonization. Despite these measures, the ulcer persisted, and wide excision for occult carcinoma and placement of a graft were considered. At this time, she was referred to our clinic for additional dermatologic consultation.

She presented with a 5 X 4 cm, partially epithelialized, irregularly shaped, shallow ulceration without undermined or indurated borders on the right pretibial leg. She had mild pitting edema of each leg below the knees. The rest of her cutaneous examination was unremarkable. Her past medical history was significant only for hypertension. Tissue cultures for acid fast bacteria, fungi, and routine bacteria were negative. Repeat biopsies at the ulcer borders for routine hematoxylin and eosin staining revealed a lichenoid dermatitis consistent with lichen planus. Ulcerative lichen planus was subsequently diagnosed.

Oral acitretin 25 mg daily was started after liver associated enzymes (LAEs) and triglycerides (TGs) were found to be normal. Intralesional injection of triamcinolone (10 mg/mL) was performed around the borders of the ulcer at the beginning of treatment. Additionally, she applied topical clobetasol ointment twice daily and continued general wound care practices including compression therapy. After two weeks of treatment, marked improvement was noted with complete reepithelialization in some areas. After four weeks of treatment, complete healing was noted with residual atrophy and post-inflammatory hyperpigmentation. LAEs and TGs remained within the normal range. Acitretin was then decreased to 25 mg every other day and tapered off over the next two months. At her three month follow up, she remained clear and no longer required any form of treatment.

Discussion

The diagnosis of ulcerative lichen planus can be easily missed, as it is an unlikely cause of persistent cutaneous ulcerations. In our case, the ulceration occurred postoperatively within a wound from curettage of a squamous cell carcinoma. The two most likely causes of persistant ulceration in such a case would include poor wound healing secondary to stasis dermatitis, which her clinical exam demonstrated, and residual carcinoma. She was adequately treated for poor wound healing and rebiopsied to rule out residual carcinoma. The differential diagnosis at presentation included recurrent squamous cell carcinoma, ulcerative lichen planus, deep fungal, mycobacterial, gram positive and negative pyodermas, and pyoderma gangrenosum.

Erosive or ulcerative lichen planus is commonly found on mucosal surfaces but has only rarely been seen on cutaneous surfaces. When found cutaneously, it usually involves the palms and/or soles, with associated nail destruction and sometimes scarring alopecia (1,2). Other variants reported include flexural presentations and those associated with polycythemia vera, rheumatoid arthritis, and sicca syndrome (3-6). Ours is the first case reported to occur on the extensor surface of an extremity and exhibit pathergy.

Whereas mucosal erosive lichen planus may respond to topical corticosteroid treatment, it can be refractory and warrant systemic treatment. Cutaneous ulcerative lichen, however, is notoriously difficult to treat by any method. Reported successful systemic therapies include cyclosporine, azathioprine, etretinate, thalidomide and dapsone (7-12).

In severe cases of papular cutaneous lichen planus, systemic retinoids are often chosen as steroid sparing agents or to avoid corticosteroids all together. Joshi et al. reported a 50-year old female with ulcerative lichen planus on the soles of 11 years' duration who experienced rapid and complete resolution within 3 months of instituting etretinate (8). But in another case reported by Fornasa, a 4-week trial of oral etretinate (unreported dose) and a 6-week trial with dapsone were both ineffective for plantar ulcerative lichen planus of 5 years' duration in a 72-year-old female (13). Interestingly, the ulcers had previously failed to respond to systemic steroids (unreported dose) for several months.

Retinoids exert numerous biologic effects through binding of nuclear retinoic acid and X receptors which act as nuclear transcription factors. Activated retinoid receptors regulate expression of various genes and proteins including growth factors, oncogenes, keratins, or transglutaminases, which in turn regulate processes such as epidermal differentiation (14).

We employed three treatment strategies with our patient. While the treatment success cannot be attributed to one strategy alone, we found the speed of resolution interesting, particularly in light of the reported difficulty in clearing cutaneous ulcerative lichen planus, and believe the addition of oral retinoids to topical or intralesional corticosteroids is an attractive choice of therapy.

References

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14. Peck GL. DiGiovanna JJ. The retinoids; in Freedberg IM. Eisen AZ. Wolff K. Austen KF. Goldsmith LA. Katz SI. Fitzpatrick TB (eds): Fitzpatrick's Dermatology in General Medicine, 5th ed. New York: McGraw-Hill, 1999; 2810-2811.

ROBERT L HENDERSON JR MD, PHILLIP M WILLIFORD MD, JOSEPH A MOLNAR MD PHD

ADDRESS FOR CORRESPONDENCE:

Phillip M Williford MD

Associate Professor of Dermatology

Director of Dermatologic Surgery

Wake Forest University Baptist Medical Center

Medical Center Boulevard

Winston-Salem. NC 27157-1071

Phone: (336) 716-3926

E-mail: pwillifo@wfubmc.edu

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