A 40-year-old woman with trisomy 21 developed an acute eruption on the hands, legs, and face 57 days after her second pancreas transplant for type 1 diabetes. Initial pancreas transplant was 12 years prior, complicated by antibody-mediated rejection. Patient received her second pancreas from a deceased male donor. She had induction with thymoglobulin and oral corticosteroids. Three units of irradiated blood were given in the operating room. The patient had stable white blood cell counts following transplantation with no evidence of leukopenia. Two weeks prior to her eruption, she had elevated liver enzymes, which resolved after discontinuation of isoniazid, prescribed for latent tuberculosis, and fluconazole. On physical examination, there were scaly pink plaques in the periocular area, dorsum of the hands, and the lower legs. In addition, she had punctate firm hemorrhagic papules of the fingertips and palms (Figure 1). The patient also had mild cervical adenopathy. Two skin biopsies were performed on the different morphologies on the hands. Immunosuppression was not reduced or altered following the onset of the rash.
Histopathologic evaluation of both specimens revealed scattered apoptotic keratinocytes within the epidermis, with vacuolization at the dermal-epidermal junction. There was a dense superficial to middermal lymphocytic infiltrate, with some scattered eosinophils (Figure 2). Additional finding of hemorrhage was seen in specimen from the fingertip. Findings were consistent with lichenoid tissue reaction and thus raised concern for lichenoid GVHD (grade 2) versus lichenoid drug eruption. Fluorescence in situ hybridization (FISH) testing for chromosomes X and Y found the lymphocytes as XY, while the surrounding normal tissue was XX, indicating that the inflammatory infiltrate was primarily of male (donor) origin, confirming the diagnosis of GVHD. Chimerism analysis on the peripheral blood showed 0% donor DNA.
Patient was diagnosed with cutaneous GVHD and was treated with topical corticosteroids. The rash and cervical adenopathy promptly resolved. Investigation for other organ involvement was done with routine laboratory analysis, as well as EGD and colonoscopy. No other organ involvement was identified, and she remained stable with no progression of her disease.