Mycosis fungoides after solid-organ transplantation: report of 2 new cases.

Long-term survival after solid-organ transplantation is increasing because of recent advances, including new immunosuppressive regimens to avoid graft rejection. However, the resultant modification of the immune system is associated with an increased risk of several cancers. The most common are skin cancers, and lymphomas are second in frequency. Nevertheless, posttransplant primary cutaneous lymphomas (PCLs) are rare, and their incidence is not well known currently. From the files of the Nephrology and Cardiology Departments of University Hospital "12 de Octubre" of Madrid, we obtained clinical data from 1612 transplanted patients and only found 2 cases of posttransplant PCLs, both were T-cell PCL. We reviewed the clinical, histopathological, and immunohistochemical characteristics; both cases were T-cell posttransplant PCLs manifested clinically as mycosis fungoides. One was a 57-year-old woman who had received a cadaveric kidney transplant, and the other was a 60-year-old man with a heart transplant. Histology and immunohistochemistry were consistent with the features of mycosis fungoides when lesions were completely developed. Up to 20% of all organ transplant recipients will suffer some form of malignancy. Unlike general population, 70% of PCLs in transplant recipients are B cell in origin and frequently show positivity for Epstein-Barr virus markers; whereas only 30% are cutaneous T-cell lymphomas. Different pathogenic hypothesis including reduced immune surveillance, chronic antigenic stimulation by transplant grafts, and the direct oncogenic effects of immunosuppressive drugs have been suggested. Although cutaneous B-cell lymphomas are more common, dermatopathologists should be aware that cutaneous T-cell lymphomas may also appear.

Dermatitis espongiótica y enfermedad de injerto contra huésped / Spongiotic dermatitis and graft versus host disease

La enfermedad de injerto contra huésped (EICH) es una reacción inmunológica de los linfocitos T del donante frente a los tejidos del receptor. La EICH aguda incluye las manifestaciones que aparecen en los primeros 3 meses del trasplante y se encuadra dentro del patrón microscópico de las dermatitis de interfase o reacciones liquenoides. Presentamos el caso de un varón de 35 años de edad, que recibió un trasplante alogénico de médula ósea. Realizó profilaxis de EICH con ciclosporina y metotrexato. A los 50 días postrasplante presentó una erupción cutánea, con diagnóstico anatomopatológico de dermatitis espongiótica. Quince días más tarde presentó empeoramiento de la clínica cutánea y alteración del perfil hepático, y una nueva biopsia cutánea mostró una dermatitis de interfase altamente sugestiva de EICH. Con el diagnóstico de EICH aguda cutánea grado 3 y hepática grado 1 se aumentó el tratamiento inmunosupresor, lo cual indujo una mejoría progresiva de ambos cuadros. Puesto que no existen características clínicas ni histológicas que diferencien si una erupción en el período postrasplante se debe a EICH o a fármacos, algunos autores aconsejan considerar dicha erupción como una EICH e incrementar la inmunosupresión, ya que las consecuencias del retraso del tratamiento pueden ser muy graves (AU)

Lichenoid nail changes as sole external manifestation of graft vs. host disease.

A 56-year-old-man who had refractory anemia with an excess of blasts underwent an allogeneic peripheral blood stem cell transplantation (PBSCT) from his brother after preparation with melphalan and fludarabin. He received GvHD (graft-vs.-host disease) prophylaxis with cyclosporine from day -1 at a daily dose of 5 mg/kg of body weight. The daily dosage was tapered gradually from day +20. On post-PBSCT day 68 he developed acute cutaneous GvHD grade 3 and acute gastrointestinal GvHD grade 2-3, which was resolved with a daily dose of 1 mg/kg of body weight of prednisone. The patient was discharged in good clinical condition and without signs of GvHD, and he started tapering his immunosuppressive treatment. By day 160 he developed oral lichen planus-like changes, with several reticulate white lesions on the oral mucosa. A biopsy specimen was microscopically consistent with lichenoid GvHD (Fig. 1). By day 150 after PBSCT, when he was being treated with CsA 100 mg once daily and prednisone 10 mg once daily, his fingernails started to grow abnormally and gradually became dystrophic and painful. Two months later his toenails became similarly affected. Although affecting all finger and toe nails, the lesions were especially important in both thumbs. Physical examination revealed multiple findings on his nails (Fig. 2): thickening, fragility, onycholysis, longitudinal striations, and even pterygium. The micological cultures were negative. A biopsy specimen showed an sparse papillary dermis lymphoid infiltrate with focal exocytosis and presence of isolated multiple necrotic keratinocytes (Fig. 3). These findings were interpreted as a lichenoid GvHD with oral and nail involvement. The patient did not have other associated cutaneous lesions. He did not develop signs or symptoms consistent with hepatic GvHD. In May 2000 thalidomide was added to the immunosuppressive therapy, at a daily dose from 100 to 300 mg according to tolerance (constipation, sedation, ...). The lesions on the oral mucous showed a substantial improvement, but the nail changes remained more or less stable. Thalidomide was discontinued after 7 months because the patient displayed numbness and tingling in the hands and feet consistent with a peripheral neuropathy. Twenty days later he stopped taking thalidomide and the oral lichenoid lesions worsened, resulting in difficulty in eating. He also developed periungueal erythema, swelling and intense pain after minimal trauma. The daily dose of prednisone increased to 20-30 mg with moderate improvement. However, the dose could not be increased because of the secondary immunosuppressive effects. Twenty-three months post-PBSCT the patient remains with intense oral and nail lichenoid lesions.

Alteraciones liquenoides en la uña comoúnica manifestación externa de laenfermedad del injerto contra el huésped / Lichenoid nail changes as sole external manifestation of grafz vs. host disease

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