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Sclerodermatous Graft-vs-Host Disease
Clinical and Pathological Study of 17 Patients
Pablo F. Peñas, MD;
María Jones-Caballero, MD;
Maximiliano Aragüés, MD;
Jesús Fernández-Herrera, MD;
Javier Fraga, MD;
Amaro García-Díez, MD
Arch Dermatol. 2002;138:924-934.
Objective To collect and review all cases of sclerodermatous chronic graft-vs-host
disease from January 1, 1982, through December 31, 2000.
Setting University hospital in Madrid, Spain.
Patients During the study period, 493 allogenic bone marrow transplantations
were performed. Sclerotic lesions developed in 17 patients.
Results Sclerotic lesions appeared after a mean of 529 days. Previously, 10
(59%) of 17 patients showed a leopard-skin eruption. Sclerosis was generalized
in 12 patients and localized in 5. Nine patients presented with rippling of
the skin and 8 with lichen sclerosus lesions. We found no anti–Scl-70
or anti-centromere antibodies. Results of histological analysis showed pandermal
or deep-dermal sclerosis, slight vacuolar degeneration of the basal cell layer,
and follicular damage with follicular plugs. In 6 (50%) of the 12 patients
with evaluable biopsy specimens, septal panniculitis was found. Squamous syringometaplasia
and mucin deposits were also detected. Treatment with high doses of prednisone
and azathioprine helped in 8 of 9 patients. In 12 patients, sclerosis disappeared
after 487 days.
Conclusions Leopard-skin eruption, follicular involvement, ripply skin, and lichen
sclerosus lesions have been described poorly or not at all in sclerodermatous
graft-vs-host disease. The presence of lichen sclerosus, morphea, septal fibrosis,
and fasciitis suggests that the sclerosis can start at and affect any level
of the skin. Treatment with prednisone and azathioprine seems to halt the
process. Most patients have a good prognosis with treatment. Although most
lesions disappear, small areas of fibrosis may remain that do not produce
any physical or functional impairment.
From the Departments of Dermatology (Drs Peñas, Jones-Caballero,
Aragüés, Fernández-Herrera, and García-Díez)
and Pathology (Dr Fraga), Hospital Universitario de la Princesa, and Ambulatorio
Hermanos Sangro (Dr Jones-Caballero), Madrid, Spain.
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ABSTRACT
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