Prognostic Significance of Serum Copper in Patients With Cutaneous T-cell Lymphoma.

BACKGROUND: Serum copper has been reported to be increased in various cancers, including lymphoma. The purpose of the present study was to investigate the clinical and prognostic importance of serum copper levels in patients with cutaneous T-cell lymphoma (CTCL). PATIENTS AND METHODS: Serum copper was measured in 60 men and 38 women with mycosis fungoides (MF) and 14 men and 3 women with erythrodermic CTCL (6 with Sézary syndrome) consecutively evaluated from July 1980 to June 1985. RESULTS: A greater than normal copper level was present in nearly 20% of patients and was associated with an increased risk of disease progression and shortened disease-specific survival for patients with patch or plaque phase MF, but not for those with tumor phase MF or erythrodermic CTCL. In contrast, the serum lactate dehydrogenase level and neutrophil/lymphocyte ratio were not significantly associated with prognosis in our patient cohort. CONCLUSION: The reason for the association between the high serum copper levels and adverse prognosis is unknown. We hypothesized that interleukin-6 is secreted primarily by non-neoplastic cells at MF skin sites, leading to release of copper by the liver. Local production of interleukin-6 at the lesion sites might conceivably also promote neoplastic cell progression by stimulation of the STAT3 pathway. Further studies on the relationship between activated tumor-associated macrophages, serum copper levels, interleukin-6, or C-reactive protein and prognosis might be informative.

Prognosis after Hospitalization for Erythroderma.

Erythrodermic psoriasis (EP) and erythroderma exfoliativa (EE) are acute and potentially life-threatening inflammatory reactions. We estimated hazard ratios (HRs) of 3-year mortality following hospitalization for EP or EE compared with general population controls, patients hospitalized for psoriasis vulgaris, and toxic epidermal necrolysis (TEN), respectively. We identified 26 and 48 patients with a first-time hospitalization (1997-2010) for EP and EE, respectively (10 matched population-controls for each patient), 1,998 patients with psoriasis vulgaris, and 60 patients with TEN. During follow-up, 8 (30.8%) patients with EP, 19 (39.6%) patients with EE, and 34 (56.7%) patients with TEN died. Compared with population-controls, adjusted HRs were 4.40 (95% CI 1.66-11.70) for EP and 2.16 (1.21-3.82) for EE. Compared with psoriasis vulgaris, adjusted HRs were 1.83 (0.90-3.73) for EP, and 1.28 (1.01-1.63) for EE. The risk was significantly lower in EP (0.38 (0.16-0.91)) and in EE (0.50 (0.36-0.71)), compared with TEN. Mortality in EP and EE is high, and close follow-up is advised.

Exfoliative erythroderma at Kenyatta National Hospital, Nairobi.

BACKGROUND: Exfoliative erythroderma (EE), (Synonyms: Exfoliative dermatitis, Red man syndrome) is a clinical syndrome characterised by generalised erythema and scale. It is an important cause of functional skin failure and associated high morbidity and variable mortality rates. OBJECTIVES: To study demographics, aetiology, complications and clinical outcomes of exfoliative erythroderma (EE) on patients attending Kenyatta National Hospital (KNH). DESIGN: Cross- sectional descriptive study. SETTING: Kenyatta National Hospital, Dermatology Unit. SUBJECTS: All available medical records on inpatients seen by qualified dermatologists at KNH with generalised erythema and scale from 1996 to 2006. MAIN OUTCOME MEASURES: Discharge or death. RESULTS: Incidence exfoliative erythroderma was documented in 146 out of all 123 admissions (13%) into the dermatology unit from 1996-2006. Demographic mean age was 47 years, M: F ratio was 3:2, 67% had no income and 53% and 30% were residents of Nairobi and adjacent districts respectively. Sixty three percent were due to skin diseases, 23% due to systemic diseases of which 20% were due to HIV/AIDS and 14% due to adverse cutaneous drug reactions. Ninety percent of patients were treated and discharged and 10% died; 50% of whom had dermatoses and 29% due to HIV associated antituberculous drugs. CONCLUSIONS: Exfoliative erythroderma is an important cause of morbidity, admission and mortality in patients attending KNH. Dermatoses and HIV / AIDS were the most frequent causes. The mortality rate was relatively low and attributable to controllable diseases.

Life-threatening erythroderma: diagnosing and treating the "red man".

Exfoliative erythroderma, or diffuse erythema and scaling of the skin, may be the morphologic presentation of a variety of cutaneous and systemic diseases. Establishing the diagnosis of the underlying disease is often difficult and, not uncommonly, erythroderma is classified as idiopathic. Several cases are presented to demonstrate the diversity of presentation of this disease. Laboratory findings are typically unhelpful in establishing the etiology of erythroderma. Clinical data combined with multiple skin biopsies over time are necessary. Systemic complications of erythroderma include infection, fluid and electrolyte imbalances, thermoregulatory disturbance, high output cardiac failure, and acute respiratory distress syndrome. The initial approach to the management of erythroderma of any etiology includes attention to nutrition, fluid and electrolyte replacement, and the institution of gentle local skin care measures. Oatmeal baths and wet dressings to weeping or crusted sites should be followed by application of bland emollients and low-potency topical corticosteroids. Systemic dermatologic therapy may be required to maintain improvement achieved with local measures or to control erythroderma refractory to local measures. The prognosis of erythroderma is dependent on the underlying etiology.

Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy.

BACKGROUND: This original cohort of patients with erythrodermic cutaneous T-cell lymphoma (CTCL) was reported to have clinical improvement with photopheresis during the 12 months of the original study. No long-term follow-up data have been available to examine the impact of this therapy on the disease. OBJECTIVE: Our purpose was to provide long-term follow-up on the original 29 erythrodermic CTCL patients treated with photopheresis and to compare these results with historical controls. METHODS: Files of patients from the original photopheresis study centers were reviewed and their current status was documented. RESULTS: The median survival of the treated patients was 60.33 months from the date of diagnosis and 47.9 months from the date of the start of photopheresis therapy. A complete remission has been maintained in four of the six patients who achieved complete responses in the original study. The best responses were seen in patients with a lower CD4/CD8 ratio in the peripheral blood at the start of therapy. CONCLUSION: Photopheresis can influence the natural history of erythrodermic CTCL by inducing remissions and prolonging survival with minimal toxicity.

Fatal erythroderma (suspected graft-versus-host disease) after cholecystectomy. Retrospective analysis.

Fatal postoperative erythroderma (POE) developed in a 52-year-old woman with gallstones who underwent elective cholecystectomy. During surgery, she was transfused with 3 units of unirradiated packed red cells stored in the liquid state for at least 4 days after collection. The POE is believed to have been the result of transfusion-associated graft-versus-host disease (TA-GVHD). The diagnosis of GVHD was based upon the characteristic clinical picture and retrospective HLA typing. The implicated donor was homozygous for an HLA haplotype that appeared to be shared with the recipient: A24-CBL-Bw52-DR2-DRw52-DQw1, the most common haplotype in the Japanese population. This case raises the possibility that a transfusion of relatively fresh blood from a donor who has no HLA antigens incompatible with the recipient may result in GVHD in patients with no apparent immunoincompetence who are undergoing relatively minor surgery with no chemotherapy or radiation therapy.

Clinical, histological, and immunohistological studies of postoperative erythroderma.

We report 7 cases of acute fatal illness characterized by fever, diffuse erythematous rash, and progressive leukopenia occurring 10 days after surgical operation. The outcome was uniformly fatal. The biopsy findings consisted of eosinophilic individual necrosis of epidermal cells, satellite cell necrosis, basal liquefaction degeneration, and scanty cell infiltration into the dermis. T lymphocytes were found in the epidermis but Langerhans cells disappeared. These findings are compatible with acute graft-vs-host disease following blood transfusion. Explanations based upon drug allergy, infection, toxic shock syndrome, or toxic epidermal necrolysis seem less reasonable.