CLINICAL HISTORY:

Signs and symptoms:  Mild to moderate pain of the mouth and tongue for approximately five years.

Previous Treatment:  Semi-controlled with topicals (steroid and antifungal preparations).

Other information:  “Spots” appearing sporadically on the scalp. No history of bone marrow transplant, hematopoietic stem cell transplantation, transfusion of unirradiated blood products or solid organ transplantation.

PHYSICAL EXAM:

Mouth: Buccal and gingival mucosa with numerous violaceous plaques and erosive patches of erythema. Tongue with extensive thick-white violaceous plaques and erosive involvement.
Scalp: Multiple scattered erythematous patches with scale.
Genitalia: Labia with multiple reticulated plaques; sparing of the perineum and rectum.

LABORATORY TESTS:

DERMATOHISTOPATHOLOGY:

Mouth: The sections show irregular mucosal acanthosis. There is a dense
band-like lymphocytic infiltrate with focal hydropic degeneration and rare cytoid
bodies. Representational images courtesy of David Mehregan, MD.

DIFFERENTIAL DIAGNOSIS:

1.   Pemphigus vulgaris
2.   Discoid lupus erythematosus
3.   Graft vs. host disease (GVHD)
4.   Lichen Planus
5.   Psoriasis

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Oral lichen planus

DISCUSSION:

Oral lichen planus (OLP) can be a very frustrating disease for both the patient and dermatologist. Patients with symptomatic OLP often require intensive therapy to reduce the signs and symptoms of this painful and disabling inflammatory disease. Although controversial, patients with OLP may develop oral squamous cell carcinomas (OSCC). This patient subsequently developed OSCC five years after developing OLP.

While the pathogenesis of OLP is complex and not completely understood, extensive painful erosions can lead to a significant decrease in quality of life. OLP tends to be an unremitting disease, with reticulate (1/3 of cases), atrophic (1/5 of cases) and ulcerative forms. This patient demonstrates all three variants of OLP, as well as “vulvovaginal-gingival syndrome”, a distinct subset with concomitant genital involvement. Since many patients with OLP have “extra-oral” manifestations, a multidisciplinary medical team should routinely perform a thorough history and physical examination.

TREATMENT:

Actual treatment for this patient:

The principal aims of OLP therapy were the resolution of oral mucosal lesions and painful symptoms, as well as the maintenance of good oral hygiene. Topical corticosteroids, antimycotics and calcineurin inhibitors have been the mainstay of treatment. Other measures include routine clinical examinations and the elimination of smoking and alcohol to reduce the risk of OSCC.

Other Treatment options:

Given the age and health of the patient, more aggressive modalities were not viable. Currently, there is no curative treatment available for OLP; however, immunomodulation may provide potential benefit. OLP that is recalcitrant to topical corticosteroids may respond to topical tacrolimus. Other potential therapies for recalcitrant OLP include mycophenolate, systemic corticosteroids, retinoids, hydroxychloroquine, azathioprine, and dapsone.

REFERENCES:

1. Dissemond J. Oral lichen planus: an overview. J Dermatolog Treat. 2004;15:136-140.

2. Eisen D. The clincal features, malignant potential, and systemic associations of oral lichen planus: a study of 723 patients. J Am Acad Dermatol. 2002; 46:207-14.

3. Eisen D. The vulvovaginal-gingival syndrome of lichen planus. The clinical characteristics of 22 patients. Arch Dermatol. 1994;130:1379-82.

4. Eisen D. The evaluation of cutaneous, genital, scalp, nail, esophageal, and ocular involvement in patients with oral lichen planus. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999 Oct; 88:431-6.

5. Itin P, Surber C, Buchner S. Lack of effect after local treatment with a new cyclosporin formulation in recalcitrant erosive oral lichen planus. Dermatology. 1992;185:262-5.

Additional Comment:

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