Chronic graft-versus-host disease presenting as glossitis

A 32-year-old man presented to the dermatology unit at the University Hospital, Bologna, Italy, with an eight-month history of glossitis, which restricted his oral intake partially [Figure 1]. Past medical history revealed an allogeneic bone marrow transplantation at the age of four years to treat Fanconi anemia. Oral examination showed a whitish hypertrophic plaque, partially ulcerated and with a rugged surface, on the left side of the dorsum of the tongue. Biopsy from the lesion revealed keratinized squamous cell epithelium, apoptosis of the basal cells, accompanied by a band-like lymphocytic infiltrate. Clinical and histologic findings were consistent with lichenoid chronic graft-versus-host disease.

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Figure 1:

Chronic graft-versus-host disease of the tongue: whitish elevated plaque with erosions and atrophic areas

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The patient was treated with a topical combination of triamcinolone, retinoic acid and clotrimazole, with only slight improvement at the nine-week follow-up.

Allogeneic bone marrow transplantation is currently performed world-wide to treat many hematological disorders, resulting in a dramatic increase in survival rates. Despite the fact that the patients may remain free of their original disease for several years, some long-term effects, including chronic graft-versus-host disease, can have a negative impact on the survivors’ quality of life. Graft-versus-host disease has been classically divided into the acute and chronic variants, based on the time of onset, using a cutoff of 100 days. The skin is one of the most frequently affected organs in chronic graft-versus-host disease but oral and anogenital mucosae may also be involved. It has been estimated that the prevalence of oral graft-versus-host disease ranges between 45-83%. The American Society for Bone Marrow Transplantation has defined diagnostic criteria and specific differential features for oral graft-versus-host disease. These criteria comprise lichen planus-like lesions, hyperkeratotic papules and plaques and limited oral opening from sclerosis (microstomia). Other findings include xerostomia, mucocele, mucosal atrophy, pseudomembranes and ulcerations.