A 38-year-old Filipino male presented with lichen planus-like plaques and dyspigmentation of the hands, lips, and oral mucosa. Eight months earlier he had received an allogeneic bone marrow transplant for the treatment of a hematologic malignancy. At the time of presentation, the patient was undergoing a slow tapering off from oral tacrolimus and prednisone. The onset of oral and cutaneous symptoms occurred de novo with no history suggestive of a preceding acute graft-versus-host reaction (i.e., gastrointestinal or hepatic dysfunction, acral erythema, erythroderma, or other erythematous eruptions).
The lichenoid form of chronic cutaneous graft-versus-host disease (GVHD) was suspected because of the morphology and pattern of the lesions in the setting of the patient’s chemotherapeutic history. The lesions were most pronounced on the trunk and distal extremities and consisted of asymptomatic violaceous, hyper- and hypo-pigmented plaques ( Figures 1 and 2 ). Following a typical course of chronic GVHD, in contrast with that of lichen planus, the patient’s nails remained uninvolved. Furthermore, there was no evidence of sclerodermoid change. As the pattern became more widespread, the patient developed exquisitely tender, lacy-white, and hypertrophic plaques on the buccal mucosa and tongue ( Figures 3 and 4 ). At the time of dermatologic consultation, the patient’s oral lesions were so painful that they prevented him from eating adequate meals.
Figure 1. Violaceous hyperpigmented macules on the palms.
Figure 2. Hypopigmented lesions on the lips.
Figure 3. Erosive plaque on the buccal mucous membranes.
Figure 4. Erosive plaque on the tongue.
Cutaneous punch biopsies were performed, and the results were consistent with the clinical presentation of chronic GVHD. A lichenoid pattern with pigment incontinence and lymphocyte tagging of apoptotic keratinocytes was observed.
The patient’s cutaneous symptoms improved with an increase in his dose of tacrolimus alone. However, his oral mucous membrane lesions remained. Increasing immunosuppression with oral prednisone was considered. However, in an attempt to reduce the risk of opportunistic infections associated with long-term use of systemic corticosteroids, a trial of localized intraoral ultraviolet light was proposed. Biweekly intraoral PUVA treatments were initiated ( Figure 5 ), and his symptoms improved rapidly. Twenty milligrams of methoxsalen (8-MOP) was given 1 hour prior to intraoral UVA using a standard dental composite curing light at an intensity of 0.5 J/cm2. The dose was cautiously increased, avoiding UV burns, to a maximum dose of 4.0 J/cm2.
Figure 5. Intraoral UVA treatment.
After 1 month of PUVA treatments (Figure 6), the oral lesions had greatly decreased in size, and the patient’s ability to tolerate opening his mouth to eat became normal. He continued UVA maintenance treatments and at nine months remained asymptomatic.
Figure 6. Only a small erosion is left after intraoral PUVA treatment.
Nathan S. Uebelhoer, DO, SkinCare Physicians, Chestnut Hill, Massachusetts Andrew Bookwater, DDS, Department of Dentistry, U.S. Naval Hospital, Okinawa, Japan
Jeffrey S. Dover, MD, FRCPC, SkinCare Physicians, Chestnut Hill, Massachusetts; Section of Dermatologic Surgery and Cutaneous Oncology, Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut; Department of Dermatology, Dartmouth Medical School, Hanover, New Hampshire
Kenneth A. Arndt, MD, SkinCare Physicians, Chestnut Hill, Massachusetts; Section of Dermatologic Surgery and Cutaneous Oncology, Department of Dermatology, Yale University School of Medicine, New Haven, Connecticut; Department of Dermatology, Dartmouth Medical School, Hanover, New Hampshire; Department of Dermatology, Harvard Medical School, Boston, Massachusetts
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