Localized scleroderma-like lesions after bone marrow transplantation in man

Localized scleroderma-like skin lesions which developed in two children, from 8 to 10 months after successful bone marrow transplantation for aplastic anaemia, showed histopathological features resembling those of scleroderma. This finding, like the animal models described in the literature, provides additional support for the auto-immune nature of scleroderma.     

Cytomegalovirus infection in the bone marrow transplant patient

Cytomegalovirus(CMV) infection is an important contributor to the morbidity and mortality associated with bone marrow transplantation(BMT). Infection may lead to CMV disease involving multiple organs such as pneumonia, gastroenteritis, retinitis, central nervus system involvement and others. CMV seropositivity is an important risk factor and approximately half of BMT recipients will develop clinically significant infection most commonly in the first 100 d post-transplant. The commonly used tests to diagnose CMV infection in these patients include the pp65 antigenemia test and the CMV DNA polymerase chain reaction(PCR) assay. Because of its greater sensitivity and lesser turnaround time, the CMV PCR is nowadays the preferred test and serves as a main guide for pre-emptive therapy. Methods of CMV prevention include use of blood products from seronegative donors or leukodepleted products. Prophylaxis or pre-emptive therapy strategies for CMV prevention may be used post-transplant with the latter becoming more common. The commonly used antivirals for pre-emptive therapy and CMV disease management include intravenous gancyclovir and foscarnet. The role of intravenous immunoglobulin, although used commonly in CMV pneumonia is not clear.     

Nail abnormalities and scleroderma-like lesions on the face associated with systemic amyloidosis

INTRODUCTION: Mucocutaneous involvement in systemic amyloidosis occurs in 29 to 40 p. 100 of cases. Nail abnormalities are infrequent in AL amyloidosis. We report an original case of AL amyloidosis associated with cutaneous and integument alterations and scleroderma-like infiltration of the face. CASE REPORT: A 73 year-old woman was hospitalized because of weight loss and asthenia. She had been treated 4 years earlier with chemotherapy for a IgG-type multiple myeloma with complete resolution of the underlying monoclonal gammapathy. Cutaneous examination showed nail dystrophy of all fingernails associated with scleroderma-like skin changes on the chin and lips. Histopathologic study of a chin biopsy confirmed the presence of amyloid deposits in the dermis. Laboratory data were normal, without signs of recurrence of multiple myeloma. DISCUSSION: We report an original case of a patient who developed two unusual cutaneous manifestations associated with AL amyloidosis. Moreover, there was no correlation between the severity of the cutaneous lesions and the extent of the underlying hematological disease.     

Eruptive lesions in a patient with bone marrow transplantation

A 41‐year‐old man with a diagnosis of chronic myeloid leukemia presented with several elevated, small, soft, yellow papules, disseminated over the face and upper trunk. The cutaneous lesions had developed 5 years after transplantation ( Fig. 1 ).

Figure 1 Open in figure viewer PowerPoint Sebaceous hyperplasia on the upper trunk presenting as yellowish, dome‐shaped, asymptomatic papules, 1–2 mm in diameter. The lesion is umbilicated, with individual lobules growing out from the center; lesions may occur individually or in groups     

Naevi in allogeneic bone marrow transplantation recipients: the effect of graft-versus-host disease on naevi

BACKGROUND: Non-melanoma skin carcinoma is more common in transplant recipients, probably because of immunosuppression. An increased risk of developing melanoma could be a late effect of transplantation. The number of naevi, which is a risk marker for melanoma, is increased in renal transplant recipients of all ages and may be related to immunosuppression. The risk of melanoma has been suspected to be particularly high after bone marrow transplantation. Cutaneous graft-versus-host disease (GVHD) might be an interesting model for the study of interactions between naevi and the immune system. OBJECTIVE: To assess whether aBMT exposes an individual to a particularly high risk of melanoma, using naevi as a surrogate measure of the risk. To improve our knowledge of the effect of the immune system on naevi, using GVHD as a model. METHODS: We carried out an epidemiological case-control study with two age-, sex- and hair colour-matched controls for each case. The results were analysed with analysis of variance, a general linear model analysis and multivariate analysis. RESULTS: The number of naevi was not significantly increased in aBMT patients, as compared with controls, although there was a significant excess on the palms and legs. In exploratory subgroup case-control comparisons and with the general linear model, patients who were conditioned with a combination of two alkylating drugs at high doses, and patients who had an aBMT before the age of 20 years tended to have a higher count of naevi (P = 0.002 and P = 0.06, respectively). Conversely, there was a trend in favour of a lower count of naevi in patients with diffuse skin lesions of chronic GVHD (P = 0.01). These data were corroborated by multivariate analysis, which showed that conditioning with high-dose chemotherapy, the absence of severe chronic cutaneous GVHD and a young age at transplantation were the main variables that independently predicted an excess of naevi. CONCLUSIONS: This study of aBMT patients confirms that chemotherapy stimulates naevus growth. It also suggests for the first time that diffuse lesions of chronic cutaneous GVHD are associated with a decreased number of naevi. Whether allo-immunity, chronic skin inflammation or the masking of naevi by pigmentation and fibrosis is responsible for the decreased number of naevi requires further investigation. With respect to the long-term risk of melanoma in aBMT recipients, our results support an increased risk particularly when aBMT is performed at a young age, and when conditioning is with high doses of alkylating drugs.     

Bone marrow transplant recipients and their compliance with sun-protection measures in Queensland,Australia

There is no current published work on the sun-protection practices of bone marrow transplant recipients. The burden of post-transplant skin malignancy is a growing public health concern. This paper evaluates the compliance of bone marrow transplant recipients with advice about sun protection through a cross-sectional observational study involving participants completing a questionnaire regarding sun protection. Whilst most bone marrow transplant patients recalled receiving education (94%) and understood why they required additional precautions (84%), half did not practice adequate photoprotection. Future research should explore barriers to non-compliance with sun-protection measures in bone marrow transplant patients so additional interventions can be more targeted within this population.     

Effect of selenium intake on the prevention of cutaneous epithelial lesions in organ transplant recipients

Organ graft recipients have a high rate of pre-malignant and malignant epithelial lesions. Selenium directly influences the number of Langer-hans cells. In several studies selenium has shown its role in preventing various carcinomas, it was worth investigating whether it could prevent skin cancer linked to human papilloma virus (HPV). A multicentre, randomised, placebo-controlled, parallel group study in 184 recent organ transplant recipients was undertaken. Patients were treated for 3 years with 200 mug/day selenium (91 patients) or a matching placebo (93 patients), and then monitored for 2 years. Occurrence rates of warts and various keratoses (main criterion) and of skin cancers (secondary criterion) were compared in the two groups, using Kaplan-Meyer analyses. There was no difference between the two groups for the main criterion (odds-ratio 1.09, p = 0.72) or the secondary criterion (odds-ratio 3.08; p = 0.15). When both arms were pooled, phenotype and age were not found to be discriminatory factors, whereas a previous history of an actinic keratosis significantly increased the risk of developing a skin cancer by 17.5%. Safety was good and similar in both groups. Selenium was not shown to prevent the occurrence of skin lesions linked to HPV. The occurrence of skin cancer was higher if there had been a previous actinic keratosis, highlighting the importance of early dermatological follow-up of the transplanted population. This was demonstrated by the high rate of epithelial lesions detected, which was more than twice the rate usually reported in the literature.     

Skin lesions in adult liver transplant recipients: a study of 100 consecutive patients

BACKGROUND: The surgical advances made in the area of organ transplantation along with the use of more efficacious immunosuppression have meant an increase in patient survival. This longer-living transplant population has started to exhibit cutaneous problems, some of which lead to an increased mortality while others lead to a decline in the quality of life. OBJECTIVES: The primary objective was to determine the different types of cutaneous lesions encountered in the adult liver transplant population. Secondary objectives were to determine the impact, if any, of the duration of transplant, the type of immunosuppression involved and the degree of sun exposure and skin phototype, on the skin cancers encountered in this transplanted population. METHODS: Two dermatologists examined 100 consecutive liver transplant recipients (LTRs) attending the transplant outpatient department. Skin examination included the face and whole body and lesions found were categorized into the following groups: cutaneous malignancies, squamoproliferative lesions, cutaneous infections and others that did not fall into any of these categories. RESULTS: The reasons for organ transplantation were numerous. The mean age at transplantation was 42.5 years. The average time since transplantation was 5.5 (range 0.75-16 years). Four patients developed skin cancers; among them there were a total of seven skin cancers (one squamous cell carcinoma, six basal cell carcinomas). Fungal infections accounted for 19% of all cutaneous infections seen, viral infections 2% and bacterial infections 5%. Triple-drug immunosuppressive therapy (ciclosporin A, azathioprine and prednisolone) was used in 35% of LTR patients, while dual therapy (tacrolimus and prednisolone) was used in 48% and monotherapy (tacrolimus) was used in 17% of LTRs. CONCLUSIONS: Immunosuppressive therapy is believed to be one of the most important risk factors in the development of skin cancer in solid organ transplant recipients. The relatively low prevalence of skin cancer in our liver transplant population may in part be explained by the relatively high percentage of recipients on dual and monotherapy (48% and 17% respectively), and the shorter duration of therapy. Our study suggests that although LTRs are at higher risk of developing nonmelanoma skin cancer than the general population, the risk is comparable with other solid organ transplant recipients.     

Multiple poromas in a bone marrow transplant recipient: A case report

A poroma is a benign epithelial neoplasm that most commonly presents as a solitary papule on the palm or sole. We report the case of a 25-year-old male, with a history of acute myelogenous leukemia, who developed multiple poromas on the feet. Poromatosis - the occurrence of multiple poromas - has been described in six adults and one child; it appears to be more prevalent in patients with a history of lymphoproliferative disorder or radiation exposure.     

Cutaneous squamoproliferative lesions in kidney transplant recipients: an investigation of specific Epstein-Barr virus expression

In a previous report we demonstrated Epstein-Barr virus expression in cutaneous squamous cell carcinomas from heart transplant recipients. In a comparative study, skin lesions from renal allograft recipients were investigated for the presence of Epstein-Barr virus. Thirty cutaneous squamoproliferative lesions from 10 kidney transplant recipients were examined for Epstein-Barr virus-specific gene expression. The techniques used for detection were polymerase chain reaction, in situ hybridization and immunohistochemistry. Epstein-Barr virus DNA was not detected by polymerase chain reaction in the neoplasias, and only single Epstein-Barr virus-positive carcinoma cells were shown by in situ hybridization in three cases of infiltrative squamous cell carcinomas. Immunohistochemistry for Epstein-Barr virus latent membrane protein 1 showed a negative result in all samples. These findings differ from our earlier investigations of cutaneous squamous cell carcinomas from heart transplant recipients where Epstein-Barr virus expressions were common. This may indicate that the part Epstein-Barr virus plays in the development of post-transplant, cutaneous squamoproliferative disorders is related to type of organ transplantation and/or grade of immunosuppression.     

Vaccinations in kidney transplant recipients: Clearing the muddy waters

Vaccine preventable diseases account for a significant proportion of morbidity and mortality in transplant recipients and cause adverse outcomes to the patient and allograft. Patients should be screened for vaccination history at the time of pre-transplant evaluation and vaccinated at least four weeks prior to transplantation. For non-immune patients, dead-vaccines can be administered starting at six months post-transplant. Live attenuated vaccines are contraindicated after transplant due to concern for infectious complications from the vaccine and every effort should be made to vaccinate prior to transplant.Since transplant recipients are on life-long immunosuppression, these patients may have lower rates of serological conversion, lower mean antibody titers and waning of protective immunity over shorter period as compared to general population. Recommendations regarding booster dose in kidney transplant recipients with sub-optimal serological response are lacking. Travel plans should be part of routine post-transplant assessment and pre-travel vaccines and counseling should be provided. More studies are needed on vaccination schedules, serological response, need for booster doses and safety of live attenuated vaccines in this special population.     

Soft fibroma-like lesions on the legs of a patient with Kaposi's sarcoma and lymphedema

The case of a patient who developed multiple soft fibroma-like lesions on his lower extremities affected by lymphedema and Kaposi's sarcoma is reported. To the best of our knowledge, the coexistence of these three pathologic processes is unusual and has never been described before in the literature.     

Localized polyartheritis nodosa in the lower limb with new bone formation

A 61-year-old male patient, with a burned-out ankylosing spondylitis, developed polyarteritis in the right lower limb, associated with periosteal new bone formation in tibia and fibula. An acute exacerbation may have been precipitated by an infection with yersinia. HLA type: A 28, 26, B5, 27, Cwl.