Papuloerythroderma of Ofuji

Papuloerythroderma of Ofuji (PEO) is a rare skin condition first described in 1984 and characterized by diffuse erythroderma composed of papules coalescing into plaques with sparing of skin folds, known as the deck-chair sign. The disease is almost exclusively seen in the elderly and affects men more frequently than women. Common laboratory findings include peripheral and tissue eosinophilia, elevated levels of immunoglobulin E, and lymphopenia. The diagnosis entails exclusion of potentially causative pathologies, including drug intake, atopy, malignancy, and infection. These factors have frequently been found in association with PEO, but their role in the etiopathogenesis of the disease is poorly understood. A dysregulated immune system, with particular involvement of T-helper (Th)2 and Th22 cells, seems to be important in the development of PEO. Controversy exists as to whether PEO exists as an independent entity or as a clinical pattern of a variety of distinct conditions. Treatment necessitates first addressing any coexisting circumstances that may have a causal relationship with PEO. In idiopathic cases, topical and oral corticosteroids, ultraviolet light therapies, and immunosuppressive/immunomodulating therapies have been used with variable results. Future studies are needed to further understand the disease process and to establish guidelines for diagnostic workup and treatment.     

太藤丘疹红皮病一例

患者男,82岁,因躯干、四肢红斑伴瘙痒两年余于2013年3月来我科就诊。入院前一年曾于外院诊断湿疹应用多种抗组胺药口服及糖皮质激素软膏外用治疗,效果不佳,自觉瘙痒剧烈……     

太藤丘疹性红皮病一例

太藤丘疹红皮病是一种较为罕见的皮肤疾病,由Ofuji等于1984年首次报道,本病诊断要点为融合成片的红褐色扁平丘疹、"帆布椅"征和剧烈瘙痒.实验室检查通常可见嗜酸粒细胞增多和血清IgE水平的升高.本文报道1例太藤丘疹性红皮病.     

Papuloerythroderma of Ofuji responding to treatment with cyclosporin

We describe a patient with papuloerythroderma of Ofuji (PE) of unknown cause, in whom no underlying malignancy was found. Topical steroids and systemic antihistamines did not resolve the rash. Systemic steroids induced a remission of the PE, which was not maintained on reduction of the dose. Therefore, cyclosporin was added which did lead to rapid clearing of the skin. Remission was maintained after discontinuation of treatment.     

Papuloerythroderma of Ofuji associated with acute myeloid leukaemia

Papuloerythroderma of Ofuji (PEO) is an uncommon skin condition most commonly described in Japan. The aetiology of PEO is unknown and treatment may be difficult. There are several reports of an association between PEO and malignancy, most particularly with T-and B-cell lymphomas. We describe PEO complicated by acute myeloid leukaemia occurring in a 62-year-old man. This association has not been reported previously.     

Primary Cutaneous Histoplasmosis in Papuloerythroderma (Ofuji)

We report the first Japanese patient with primary cutaneous histoplasmosis who was infected inside Japan. He was not an immunocompromised host but his cutaneous lesions developed on preexisting papuloerythroderma (Ofuji). The long-term topical corticosteroid treatment for papuloerythroderma, which could have suppressed the local immunological defence system of the skin, might have been responsible for the histoplasma infection in the present case.     

Ofuji Papuloerythroderma Associated with Follicular Mucinosis in Mycosis Fungoides

Ofuji papuloerythroderma is a distinctive clinical entity of unknown etiology, which may occasionally be associated with B cell and T cell lymphoma or visceral malignancy. We report a case of papuloerythroderma associated with follicular mucinosis in mycosis fungoides (MF) that raises the possibility of papuloerythroderma as a form of prelymphomatous skin eruption. This specific papuloerythroderma responded well to the Re-PUVA treatment, which is a combination of etretinate and PUVA photochemotherapy.     

Papuloerythroderma of Ofuji: a report of three cases and review of the literature

Papuloerythroderma of Ofuji is a rare, distinctive entity featuring widespread erythematous, flattopped papules, with a striking sparing of body folds (the so-called‘deck-chair’sign), and peripheral eosinophilia. We report three elderly men who showed the typical features of this disease, and who responded to corticosteroid therapy. Immunohistochemical studies with anti-S-100 and UCHL-1 (CD45 RO) antibodies indicated the presence of abundant dendritic cells and mature T cells in the dermis. In addition, one patient had concomitant tinea corporis. Treatment with griseofulvin alone resulted in clearing of the fungal infection and improvement of his papuloerythroderma, and worsening of his papuloerythroderma occurred when the tinea relapsed.     

Papuloerythroderma

报告1例丘疹性红皮病.患者女,58岁.因躯干、双上肢伸侧及臀部红褐色丘疹伴瘙痒2年就诊.皮肤科检查:躯干、双上肢伸侧及臀部可见较多红褐色的扁平丘疹,表面少许鳞屑,有融合趋向,呈红皮病样损害.实验室检查:外周血淋巴细胞减少,血清IgE增高.皮损组织病理学检查:角化过度,棘层肥厚,表皮突细长下延,真皮浅层血管周围少量淋巴细胞浸润.皮损免疫组化:CD3、CD45RO、CD79a及KP-1阳性,CD20弱阳性,granzyme B及CD56阴性.诊断:丘疹性红皮病.     

Rapid and successful effects of combining dupilumab with ultraviolet B radiation therapy in the treatment of Papuloerythroderma of Ofuji

Papuloerythroderma of Ofuji (PEO) is an uncommon disease characterised by widespread erythroderma composed of intensely pruritic solid papules coalescing into plaques sparing the skin folds (deck-chair sign). The pathogenesis of PEO remains unclear, although T helper (Th) 2 and Th22 cells may play an important role. Dupilumab is an interleukin (IL)-4 receptor α-antagonist that effectively reduces Th2 responses, which has drawn increasing attention in the treatment of PEO patients. Here, we reported a successful case of dupilumab treatment in combination with ultraviolet B (UVB) radiation therapy, which is well known and effective for chronic itch. The patient had a significant decrease in visual analogue scale (VAS) score and eosinophil after only 1 week of treatment, which may be due to the combination effect.     

Papuloerythroderma

The main macroscopic features of 4 cases presented here are erythroderma-like lesions and solid papules. The former covers extensive areas over entire trunk and extremities, especially extensor surfaces. The latter is observed mainly on flexor surfaces of extremities and around the former. The axillae, inguinal regions, cubital and popliteal fossae, and big furrows on abdomen are spared from these lesions. Rashes appear first as papules and form erythroderma-like lesions through their assembly and confluence. Histologically, both show almost the same findings. Patients are older males with no general symptoms except itching. Although the course is chronic, lesions improve slowly and heal only after years.     

Ofuji papuloerythroderma

Ofuji papuloerythrodema is a rare type of dermatosis marked by an erythrodermic manifestation which is intensely pruritic and results from the coalescing of brownish papules which in the main do not involve the great folds. The authors present the case history of a 72-year-old female, whose dermatosis appeared 8 years after the diagnosis of malignant lymphocytic lymphoma. The Authors describe the clinical and histopathological picture, illustrating the laboratory results and stressing the importance of the association of the two pathologies in a possible nosological overview.     

Papuloerythroderma of ofuji

A 62-year old female had intensely pruritic eruption consisting of widespread coalescing sheets of uniform erythematous papules with characterstic sparing of body folds (deck-chair sign). Papuloerhthroderma of Ofuji is a rare disease of unknown aetiology.