Generalised erythematous skin eruptions induced by sorafenib: cutaneous toxicity and treatment outcome.

A woman diagnosed of a renal cell carcinoma in 1989 had a metastatic kidney cancer localised in subcutaneous nodules, gut and lung in 2007. Sorafenib treatment was initiated a 400 mg orally twice a day. The patient developed generalised erythematous skin eruptions and two weeks later a widespread erythematous maculopapular eruption located exclusively on the legs and arms, along with an objective response. The most likely cause of the generalised erythematous skin eruptions was considered to be sorafenib because of the close temporal relationship between exposure to the drug and onset of symptoms. Furthermore, a relationship between sorafenib skin toxicity and treatment efficacy was observed. This therapeutic efficacy of EGFR inhibitors and cutaneous side effects should be better assessed in large cohorts or trials to determine whether the skin toxicity of patients can be linked to an objective antitumour response.

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.

Generalized mucinosis in a patient with erythroderma.

We describe an 81-year-old Japanese patient with erythroderma overlapping with widespread and symmetrical deposits of mucin in the upper dermis. Clinically, the mucinous lesions on the nape and upper trunk were localized papular mucinosis. Histologically, there was a perivascular infiltrate of lymphohistiocytic cells mingled with plasma cells in the upper dermis but no sclerosis. Immunohistochemical staining revealed that more than 90% of these infiltrating plasma cells produced immunoglobulin lambda-chain. Both the erythroderma and generalized mucinosis responded to topical steroid and PUVA therapy. To the best of our knowledge, this is the first case of erythroderma accompanied by generalized mucinosis.

Mycosis fungoides presenting as Ofuji's papuloerythroderma.

We report three patients presented with clinical features of Ofuji's papuloerythroderma (pruritic erythematous papules and extensive erythema sparing all skin folds), however, showing histopathological findings of mycosis fungoides (Pautrier's microabscess, haloed lymphocytes, disproportionate epidermotropism, and wiry collagen bundles). One case was associated with plaque stage of mycosis fungoides and follicular mucinosis. T-cell receptor (TCR) gene rearrangement analysis in the lesional skin tissue demonstrated rearrangement of the gamma chain in all cases. HTLV-1 serology was negative for two patients who conducted HTLV-1 test. We think that Ofuji's papuloerythroderma might be a variant of early mycosis fungoides rather than secondary skin manifestations to certain cutaneous inflammatory diseases.

Metabolic epidermal necrosis-hepatocutaneous syndrome.

It is clear that cutaneous lesions of metabolic epidermal necrosis in the dog can occur either with a demonstrable glucagon-secreting tumor or with hepatic disease without any detectable glucagonoma. Additional clinical case reports of the disease in cats are needed to better characterize the disease in this species. The lesions of NME-MEN may not represent a specific physiological mechanism of cutaneous disease but instead a pathophysiological process that can be triggered by several systemic metabolic abnormalities. The fact that NME is observed in association with a variety of conditions supports the theory that an overall metabolic derangement results in the rash. The prognosis for canine MEN is poor; however, some affected dogs have been maintained for many months with dietary management. High-quality protein diets such as Hill's Prescription Diet a/d (Hill's Pet Products) or other "recovery" diets may be helpful. Zinc and essential fatty acid supplementation may help some patients. Dietary supplementation with cooked egg yolks may be helpful. It is prudent to avoid corticosteroids in these cases, as development of diabetes mellitus worsens the prognosis. Histopathological examination of the pancreas coupled with determination of plasma glucagon may help define the characteristics of GS versus HS in dogs. It is possible that some dogs diagnosed with MEN-HS may have an undetected pancreatic tumor. Although the hepatic ultrasound findings in dogs with MEN-HS are becoming well characterized, it is possible for dogs with pancreatic neuroendocrine tumors to also have abnormal hepatic ultrasonography. As the presence of MEN and hepatic disease does not necessarily rule out the presence of a pancreatic tumor, prospective studies correlating plasma glucagon levels with pancreatic histopathology in cases of MEN-GS versus MEN-HS seem warranted.

[Ofuji papuloerythroderma: PUVA bath treatment]. / Papuloerythroderma Ofuji: Bade-PUVA-Behandlung.

Papuloerythroderma of Ofuji is a rare skin disorder described primarily in Japanese patients. It occurs primarily in elderly men. The initial lesions are diffuse red papules, sparing the face, palms and soles. Later the papules coalesce into an erythroderma, with typical sparing of the skin folds and creases (the deck chair sign). Pruritus is usually intense. Lymphadenopathy, peripheral blood eosinophilia and elevated IgE levels all are common. Both systemic corticosteroids and systemic PUVA therapy have been recommended. We describe a German male who fulfilled the diagnostic criteria for papuloerythroderma of Ofuji and responded well to PUVA bath therapy with both improvement in skin findings and reduction in pruritus.

Eritrodermia psoriásica y psoriasis pustulosa generalizada: tratamiento con etretinato / Psoriatic erythroderma and generalized pustular psoriasis: treatment with etretinate

Se presentan dos formas graves de psoriasis infantil, el primero para remarcar los factores precipitantes tales como el uso de corticoides orales y PUVA en la eritrodermia psoriásica y el segundo por las escasas comunicaciones de psoriasis pustulosa generalizada en niños menores de un año de edad; se comenta el tratamiento exitoso con el etretinato en ambos casos. (AU)

Eritrodermia psoriásica y psoriasis pustulosa generalizada: tratamiento con etretinato / Psoriatic erythroderma and generalized pustular psoriasis: treatment with etretinate

Se presentan dos formas graves de psoriasis infantil, el primero para remarcar los factores precipitantes tales como el uso de corticoides orales y PUVA en la eritrodermia psoriásica y el segundo por las escasas comunicaciones de psoriasis pustulosa generalizada en niños menores de un año de edad; se comenta el tratamiento exitoso con el etretinato en ambos casos.

Cutaneous T-cell lymphoma presenting as papuloerythroderma--a case and review of the literature.

Papuloerythroderma has been described as a distinct clinical entity. It is characterized by pruritus, red flat-topped papules with skin crease sparing, blood eosinophilia and lymphopenia. It has been described almost exclusively in elderly men. We report a case of cutaneous T-cell lymphoma which, at presentation, was indistinguishable from papuloerythroderma, in a 79-year-old male. An excellent clinical response to low dose oral corticosteroids and photochemotherapy was observed. There have only been two previous reports linking papuloerythroderma with T-cell lymphoma. This patient is in keeping with the two previously reported cases and would support the view that papuloerythroderma may predispose to, or in fact represent, an early form of cutaneous T-cell lymphoma.

Pre-Sézary syndrome.

Eighteen patients with erythroderma, recurrent cycles of circulating Sézary cells of less than 1,000 cells/mm3, and a chronic course were followed for a mean time of nearly 5 years and were diagnosed as having pre-Sézary syndrome. Only one patient died, and none developed lymphoproliferative disease. All ten patients who underwent patch testing showed positive results. The elevation of IgE was striking when this group was compared with a group with Sézary syndrome. Most patients achieved partial or complete remission on low-dose chlorambucil and prednisone therapy. Some patients had lymphocytic or lymphomatoid bands on skin biopsy specimens and were like previously reported patients with pre-Sézary syndrome whose condition progressed to Sézary syndrome. A nontoxic chemotherapy or an anti-T cell treatment program can control this chronic erythroderma state.

Exfoliative dermatitis from Chinese herbs (decoction)--an example of purely epidermal contact-type hypersensitivity to ingested medicines.

A patient, aged 78, with exfoliative dermatitis due to the ingestion of Chinese herbs (decoction) was presented. Provocative medication showed that he was sensitive to 4 composite materials (Radix angelicae acutilobae, Rhizoma zingiberis, Radix paeoniae and Radix glycyrrhizae). Patch tests were positive to each of these materials while the intradermal test was negative. These results indicate that systemic contact-type hypersensitivity to the ingested medicines is involved in evoking the exfoliative dermatitis lesion and that, in addition, purely epidermal contact sensitivity as defined by S. Epstein and Baer plays an important role.