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J Fraga

Publications and source records attributed to J Fraga.

At least 37 records · Page 2Linked to original sources
PubMed2002

Skin necrosis from extravasation of vinorelbine.

Skin necrosis from intravenous infiltration of soft tissue is a rare but potentially devastating complication of intravenous therapy. Vinca alkaloids are among the intravenous drugs with the highest destructive power. We report two cases of skin necrosis from accidental extravasation of vinorelbine, a semisynthetic analogue of vinblastine, rarely described as being responsible for this event. Histopathologic study showed separation of the dermis from necrotic epidermis, associated with cytologic atypia, in both patients, and focal necrosis of eccrine glands in one of them. We consider that intravenous infusions of vinorelbine should be performed using the preventive measures and care applied for other chemotherapeutic agents with high potential for induction of skin necrosis due to extravasation.

Aged↗
PubMed2002

Massive scrotal calcinosis.

BACKGROUND: Scrotal calcinosis is a benign entity characterized by the appearance of calcific masses within the dermis of scrotal skin. Its pathogenesis has not been fully elucidated. OBJECTIVE: A 36-year-old man with massive scrotal calcinosis is described. Our aim was to perform a histologic examination of the surgical piece with subsequent closure of the defect achieving satisfactory cosmetic results. METHODS: Case report and literature review. RESULTS: Histologic studies of multiple nodules showed no epithelial lining and one calcified epidermal cyst. Subtotal excision of the scrotal wall was performed with excellent results. CONCLUSION: Even though the pathogenic mechanism of this entity is still unclear, our findings support the theory of dystrophic calcification of epidermoid cysts. Surgical treatment is the only definitive treatment. Subtotal excision of the scrotal wall in cases of massive calcinosis may be performed with good results.

Adult↗
PubMed2001

Late appearance of acute graft-vs-host disease after suspending or tapering immunosuppressive drugs.

BACKGROUND: Graft-vs-host disease (GVHD) is divided into acute and chronic phases based on time and clinical and histological features. The criterion of 100 days after transplantation for separating acute GVHD from chronic GVHD has been challenged on the following points: (1) the lichenoid rash of chronic GVHD may be observed as early as day 31 and acute GVHD may persist after day 100 in some cases, and (2) specific histological features do not consistently separate acute from chronic GVHD defined as the number of days after transplantation. However, the appearance of acute cutaneous GVHD after day 100 is not well established. OBSERVATIONS: Three patients developed a rash with clinical and histological features of acute GVHD between days 153 and 192 after allogeneic bone marrow transplantation or peripheral blood stem cell transplantation. In all patients, the late flare of acute GVHD occurred after tapering or suspending the immunosuppressive regimen with cyclosporine or corticosteroids, and was accompanied by stigmata of chronic GVHD in other target organs. CONCLUSIONS: The rash of acute GVHD may be observed as late as 192 days after transplantation, especially after tapering or suspending the immunosuppressive drugs, and should be considered in the differential diagnosis of late erythematous eruptions after transplantation.

Acute Disease↗
PubMed2001

Clinical and genetic heterogeneity in hereditary haemochromatosis: association between lymphocyte counts and expression of iron overload.

To identify a new marker of expression of disease, independent of HFE genotype in patients with hereditary haemochromatosis (HHC), the total peripheral blood lymphocyte counts were analysed according to iron status in two groups of subjects with HFE mutations. The groups consisted of 38 homozygotes for C282Y, and 107 heterozygotes for the C282Y or compound heterozygotes for C282Y and H63D. For control purposes, total lymphocyte counts and iron status were also examined in 20 index patients with African dietary iron overload, a condition not associated with HFE mutations, and in 144 members of their families and communities. Mean lymphocyte numbers were lower in C282Y homozygous HHC index subjects with cirrhosis and higher iron stores than in those without cirrhosis and with lower iron burdens [(1.65 +/- 0.43) x 10(6)/mL vs. (2.27 +/- 0.49) x 10(6)/mL; p = 0.008]. Similarly, mean lymphocyte counts were significantly lower in C282Y heterozygotes and C282Y/H63D compound heterozygotes with iron overload and increased serum ferritin concentrations compared to those with normal serum ferritin concentrations (p < 0.05). Statistically significant negative correlations were found, in males, between lymphocyte counts and the total body iron stores, either in C282Y homozygous HHC patients (p = 0.031 in a multiple regression model dependent on age) and in C282Y heterozygotes or C282Y/H63D compound heterozygotes with iron overload (p = 0.029 in a simple linear model). In contrast, lymphocyte counts increased with increasing serum ferritin concentrations among the index subjects with African iron overload (r = 0.324, not statistically significant) and among the members of their families and communities (r = 0.170, p = 0.042). These results suggest that a lower peripheral blood lymphocyte count is associated with a greater degree of iron loading in HFE haemochromatosis but not in African iron overload, and they support the notion that the lymphocyte count may serve as a marker of a non-HFE gene that influences the clinical expression of HFE haemochromatosis.

Adult↗
PubMed2001

Widespread biphasic amyloidosis: response to acitretin.

We report a patient who presented with an intensely pruritic rash, with hyperpigmentation of the whole back and multiple hyperpigmented and hyperkeratotic papules over his forearms and lower extremities. Histologically, he showed deposits of amyloid that reacted positively with monoclonal antibodies against cytokeratins. The lesions were resistant to antihistamines and corticosteroids. Treatment with oral acitretin was started and great improvement of the lesion was noted; the patient became asymptomatic. We review the literature on the effect of retinoids in cutaneous amyloidosis.

Acitretin↗
PubMed2001

Mucocutaneous presence of cytomegalovirus associated with human immunodeficiency virus infection: discussion regarding its pathogenetic role.

OBJECTIVES: To investigate the significance of cytomegalovirus (CMV) in mucocutaneous lesions in patients with human immunodeficiency virus (HIV), and to elucidate its pathogenetic role in lesions genesis. DESIGN: Retrospective (study 1) and prospective (studies 2 and 3) surveys. SETTING: Departments of Dermatology, Pathology, and Microbiology at a university hospital in Madrid, Spain. PATIENTS: Seventeen HIV-infected patients with CMV presenting any type of mucocutaneous lesions (study 1); 27 HIV-positive patients with mucocutaneous vesicles and/or ulcers of any type and location (study 2); and 12 severely immunosuppressed HIV-positive volunteers (study 3). INTERVENTIONS: Mucocutaneous biopsy specimens from the lesions (studies 1 and 2) and from nonlesional skin (study 3) were analyzed by light microscopy, immunohistochemical analysis, and microbiological analysis (standard viral culture and shell-vial technique). MAIN OUTCOME MEASURES: Clinical data; histologic, immunohistochemical, and microbiological findings. RESULTS: (1) Studies 1 and 2: Most of the lesions where CMV was found were ulcers localized mainly on perianal, genital, and perigenital areas, usually as part of polymicrobial infections, particularly herpes simplex and varicella-zoster virus infections. The finding of CMV was confirmed in all cases by light microscopy; microbiological analysis was rarely useful. The finding of mucocutaneous CMV inclusions allowed their early detection in extracutaneous locations. (2) Study 3: Cytomegalovirus was present on healthy skin of the perianal area in 3 patients, and on the forearm in 1 patient. CONCLUSION: Cytomegalovirus does not play any significant pathogenetic role at least in most of the cutaneous lesions where it is found.

AIDS-Related Opportunistic Infections↗
PubMed2000

Giant cell lichenoid dermatitis within herpes zoster scars in a bone marrow recipient.

Cutaneous lesions arising in herpes zoster (HZ) scars are rare. We report a 34-year-old woman with acute lymphoblastic leukemia underwent allogenic bone marrow transplant (BMT). Ten days after the BMT, she developed clusters of vesicles over the right neck, scapula, shoulder and chest. She was treated with intravenous acyclovir and foscarnet. One month after the vesiculous episode of HZ she showed 5 mm to 2 cm clustered flat violaceous lichenoid papules and confluent plaques within the HZ scars. Histopathologic examination revealed a inflammatory infiltrate present in the papillary dermis with granulomatous aggregated formed by histiocytes, multinucleated giant cells and lymphocytes. She was treated with topic steroids with significant improvement. Pathologic findings are similar to those of an unusual lichenoid reaction named "giant cell lichenoid dermatitis". We present the first reported case of giant cell lichenoid dermatitis at the sites of HZ scars.

Acyclovir↗
PubMed2000

Lichen planus with lesions on the palms and/or soles: prevalence and clinicopathological study of 36 patients.

According to the literature, palmoplantar lesions in lichen planus (LP) are uncommon, and do not usually have the classically described clinical morphology. The aim of this study was to evaluate the prevalence of palmoplantar lesions in LP and to describe the characteristic clinicopathological appearances of LP affecting palms and soles. Palmoplantar LP with accompanying skin involvement accounted for 26% of our cases. The presence of very pruriginous erythematous scaly and/or hyperkeratotic plaques, with well-defined edges, located on the internal plantar arch, without involvement of the fingertips, and which usually disappear in a few months, is characteristic of palmoplantar LP. Histopathological examination shows the characteristic features of LP.

Adolescent↗
PubMed2000

Lupus erythematosus-like lesions in a carrier of X-linked chronic granulomatous disease.

Chronic granulomatous disease (CGD) is an inherited immunodeficiency disease. Carrier status of CGD has been reported in association with lupus erythematosus-type lesions. A 35-year-old woman, mother of a child with X-linked CGD presented an 8-year history of erythematous plaques with an arciform pattern on the upper trunk, back and arms. The nitroblue tetrazolium test revealed the carrier status of the patient. Haematological, biochemical and immunological tests (including ANA, DNA, SSA-Ro, SSB-La, RNP, SM and Jo1 antibodies) were normal or negative except for a polyclonal hypergammaglobulinaemia with high serum IgA. Histological examination showed a papillary and perifollicular lymphohistiocytic infiltrate. Direct immunofluorescence was negative. We report a female carrier of X-linked CGD who developed clinical subacute lupus erythematosus-like lesions. We review the literature and discuss the pathogenetic mechanisms involved in the condition.

Adult↗
PubMed2000

Expansions of CD8+CD28- and CD8+TcRVbeta5.2+ T cells in peripheral blood of heavy alcohol drinkers.

BACKGROUND: Despite heavy alcohol consumption, only a low percentage of heavy drinkers develop liver disease. Imbalances in T-cell subsets and iron metabolism parameters are common findings in heavy drinkers, yet the possible role played by discrete T-lymphocyte subsets under heavy alcohol consumption remains unclear. METHODS: To gain new insights into the possible role played by T lymphocytes during alcohol consumption, characterization of CD28 expression and TcR repertoire in peripheral blood CD4+ and CD8+ T cells by two and three-color flow cytometry was performed. A group of heavy alcohol drinkers (AHD, n = 71) and a group of age-matched controls (n = 81), both HLA-phenotyped and HFE-genotyped, constituted the groups under study. RESULTS: Marked expansions of CD28- T cells within the CD8+ but not the CD4+ T-cell pool were observed in AHD compared with controls. These CD8+CD28- expansions were paralleled by expansions of CD8+ T cells bearing specific TcR Valpha/beta chains, namely VP5.2. Moreover, AHD, but not controls, carrying the H63D mutation in the HFE gene showed significantly higher percentages of CD28- T cells within the CD8+ T-cell pool than AHD carrying the normal HFE gene. Finally, high numbers of CD8+CD28- T cells in AHD were associated with lower levels of the liver-related enzymes ALT and GGT. CONCLUSIONS: This study showed that under active ethanol consumption, expansions of discrete CD8+ T-cell subsets occur within the CD8+ T-cell pool, that molecules of the MHC-class I locus seem to influence the extent of the expansions, and that high numbers of CD8+CD28- T cells are associated with low levels of liver enzymes in AHD.

Adult↗
PubMed1999

Induction of hyperacute graft-vs-host disease after donor leukocyte infusions.

BACKGROUND: Infusions of leukocytes obtained from the original bone marrow donor is a new approach for treating patients who have a relapse of leukemia after allogeneic bone marrow transplantation. Up to 90% of patients who achieved remission developed graft-vs-host disease (GVHD). However, any description of the clinical and histologic features in these cases is lacking. OBSERVATIONS: We describe 2 patients in whom a severe, peculiar, hyperacute, fatal GVHD developed after treatment with donor leukocyte infusions and interferon alfa. The patients had not received any additional chemotherapy or GVHD prophylaxis. In both patients, the eruption started with the appearance of erythematous plaques at the interferon alfa injection sites, and a generalized maculopapular eruption subsequently developed. The clinical lesions evolved from acute to lichenoid within several days. The histologic examination also demonstrated unusual findings and showed features of both acute and chronic lichenoid GVHD. CONCLUSIONS: Donor leukocyte infusions without GVHD prophylaxis may provoke a severe fatal hyperacute GVHD. In the cases presented herein, we discuss the significance of the rapid clinical evolution from acute to lichenoid and the combination of histologic features of both acute and chronic GVHD in the biopsy specimens.

Acute Disease↗