Dermatosis eosinofílicas (I)

The eosinophil is an effector cell of the immune response with important affinity for the tissues. Skin diseases characterized by a dermal infiltration of eosinophils constitute an apparently heterogeneous group. We review the characteristic features of such well-defined entities, known as eosinophilic dermatoses.In the first part of this work we included eosinophilic pustular folliculitis, eosinophilic cellulitis, angiolymphoid hyperplasia with eosinophilia, granuloma faciale, eosinophilic ulcer of the tongue, papuloerythroderma and the pachydermatous eosinophilic dermatitis.In analogy to the accepted unifying concept of neutrophilic dermatosis, we will discuss the possible pathogenic unity of this group of diseases.     

Hypereosinophilic dermatitis (Nir-Westfried). A variant in the spectrum of the hypereosinophilia syndrome

Hypereosinophilic dermatitis represents a clinically distinct disorder in the spectrum of eosinophilic dermatoses. Its major clinical symptoms include pruriginous papular skin eruptions associated with blood eosinophilia. Histological examination reveals a diffuse, dense infiltration with eosinophils. We discuss the clinical picture, differential diagnosis, and therapy in the light of two cases.     

Churg‐Strauss syndrome with coexistence of eosinophilic vasculitis,granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid‐like blisters

The main histopathological features in the cutaneous lesions of Churg‐Strauss syndrome (CSS) are dermal leukocytoclastic vasculitis with a variable eosinophilic infiltrate and non‐vasculitic tissue eosinophilia with granuloma formation. This wide histopathological spectrum may account for the various skin manifestations of CSS. However, the unique histopathological combination of dermal eosinophilic vasculitis and subcutaneous granulomatous phlebitis accompanied by bulla formation has not been previously described. We report an unusual CSS case showing dermal necrotizing eosinophilic vasculitis and granulomatous phlebitis in purpuric lesions coupled with subepidermal blistering. The blisters showed dermal granulomatous dermatitis and eosinophilia without evidence of vasculitis. Dermal necrotizing eosinophilic vasculitis was characterized by fibrinoid alteration of the vessel wall, a prominent perivascular eosinophilic infiltrate, a few infiltrating histiocytes along the affected vessel wall, and the absence of neutrophilic infiltration. The underlying subcutaneous granulomatous phlebitis was characterized by an angiocentric histiocytic infiltrate surrounded by marked eosinophilic infiltrate. Deposition of cytotoxic proteins and radicals derived from eosinophils in the vessel walls and papillary dermis followed by a secondary granulomatous response may account for the unique clinical and histopathological features in this case. Ishibashi M, Kudo S, Yamamoto K, Shimai N, Chen K‐R. Churg‐Strauss syndrome with coexistence of eosinophilic vasculitis, granulomatous phlebitis and granulomatous dermatitis in bullous pemphigoid‐like blisters.     

Pustular skin disorders: diagnosis and treatment

The differential diagnosis for pustular skin disorders is extensive. The distribution of the lesions and the age of the patient are characteristics that may provide strong clues to the etiology of cutaneous pustular eruptions. In adults, generalized pustular dermatoses include pustular psoriasis, Reiter's disease and subcorneal pustular dermatosis. Medications can cause generalized pustular eruptions, such as in the case of acute generalized exanthematous pustulosis; or more localized reactions, such as acneiform drug eruptions, which usually involve the face, chest and back. Localized pustular eruptions are seen on the hands and feet in adults with pustulosis palmaris et plantaris and acrodermatitis continua (both of which may be variants of psoriasis); on the face in patients with acne vulgaris, rosacea, and perioral dermatitis; and on the trunk and/or extremities in patients with folliculitis. A separate condition known as eosinophilic folliculitis occurs in individuals with advanced human immunodeficiency disease. Severely pruritic, sterile, eosinophilic pustules are found on the chest, proximal extremities, head and neck. Elevated serum immunoglobulin E and eosinophilia are often concurrently found. In neonates, it is especially important to make the correct diagnosis with respect to pustular skin disorders, since pustules can be a manifestation of sepsis or other serious infectious diseases. Generalized pustular eruptions in neonates include erythema toxicum neonatorum and transient neonatal pustular melanosis, both of which are non-infectious. Pustules are seen in infants with congenital cutaneous candidiasis, which may or may not involve disseminated disease. Ofuji's syndrome is an uncommon generalized pustular dermatosis of infancy with associated eosinophilia. As in adults, neonates and infants may develop acne or scabies infestations. In this article, we review the most common pustular dermatoses and offer a systematic approach to making a diagnosis. We also report the most up-to-date information on the treatment of these various cutaneous pustular conditions.     

Wells' Syndrome Associated with Churg-Strauss Syndrome

Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells'' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features.     

嗜酸粒细胞增多综合征的治疗进展

嗜酸粒细胞增多综合征是病因不明,以骨髓、外周血和组织中嗜酸粒细胞增多为特点,并累及多器官的一组疾病,仅累及皮肤者称为嗜酸粒细胞增多性皮炎.传统药物如糖皮质激素、羟基脲、环孢素等均为非特异性作用药物,长期服用不良反应明显.近年发现,该病与嗜酸系融合基因或异常T淋巴细胞克隆有关,酪氨酸激酶抑制剂、干扰素α、白介素-5单克隆抗体等对该病有特异性疗效.     

Hyperimmunoglobulin E syndrome

A 24-year old woman had the major features of the hyperimmunoglobulin E syndrome: recurrent staphylococcal skin infections (from the age of 6 months), an extremely elevated serum immunoglobulin E level (25,000 units/ml), and defective neutrophil chemotaxis. This patient also had peripheral blood eosinophilia and cutaneous candidiasis. There was a family history of asthma, but the patient herself did not have a history of asthma or hay fever, and, on examination, had no evidence of atopic dermatitis. The patient has not had any systemic infections. Results of the skin biopsy showed dermal edema and a perivascular infiltrate with eosinophils and an increased number of mast cells. The clinical spectrum of the hyperimmunoglobulin E syndrome may include atopic dermatitis, mucocutaneous candidiasis, systemic infections, and/or the features of Job's syndrome.     

Localization of Eosinophil Granule Major Basic Protein in Incontinentia Pigmenti

We report a case of incontinentia pigmenti and demonstrate the deposition and localization of eosinophil major basic protein (MBP) in the vesicular stage of this neurocutaneous syndrome. The initial stage of incontinentia pigmenti is histologically characterized by intraepidermal vesicles associated with eosinophilic spongiotic dermatitis. Pathologic examination of a lesional tissue specimen from our patient demonstrated epidermal necrosis, spongiosis, and vesicle formation. Indirect immunofluorescence with affinity-chromatography purified antibody to human eosinophil granule MBP demonstrated many intact eosinophils within vesicles and scattered throughout the epidermis and dermis, and extracellular deposition of granular MBP in the tissue. The characteristic finding of extensive tissue eosinophilia in incontinentia pigmenti, as well as the new finding of extracellular deposition of an eosinophil granule protein in lesional tissue, suggests the involvement of eosinophils in the pathogenesis of the disease.     

Wells' syndrome associated with idiopathic hypereosinophilic syndrome

Wells’ syndrome, or eosinophilic cellulitis, is characterized by recurrent cutaneous swellings which resemble acute bacterial cellulitis, and by distinctive histopathological changes. Skin lesions show dermal eosinophilic infiltration and the characteristic‘flame figures, which are composed of eosinophil major protein deposited on collagen bundles. The idiopathic hypereosinophilic syndrome is a multisystem disease with a high mortality rate. It is characterized by peripheral blood eosinophilia and eosinophilic infiltration of many organs, including the skin. The most common skin lesions are pruritic maculopapules and nodules over the trunk and limbs, with urticaria and angio-oedema. In contrast to Wells’ syndrome, the pathology of these skin lesions is non-specific with variable eosinophil infiltration. We report overlapping clinical and histopathological findings characteristic of both syndromes in one patient. Our data favour the hypothesis that both syndromes represent an abnormal eosinophilic response to a variety of underlying diseases or causative agents and thus are different expressions of one disease entity linked to theimmunobiology of eosinophils.     

Eosinophilic Cellulitis (Wells' Syndrome)

Abstract: Two patients with eosinophilic cellulitis are reported and 22 additional cases from the literature are reviewed. Cutaneous lesions are variable in appearance and may be confused with cellulitis, urticaria, insect bites, or contact dermatitis. Microscopically, there is a dense dermal infiltrate of eosinophils. Subsequently, granulomatous features with characteristic "flame figures" become apparent. Peripheral eosinophilia is common. The etiology of eosinophilic cellutitis is unknown, although a hypersensitivity mechanism is suspected. Treatment with systemic corticosteroids is frequently effective, but the disease Is often characterized by relapses that can occur for several years.     

Frequency, levels, and significance of blood eosinophilia in systemic sclerosis, localized scleroderma, and eosinophilic fasciitis

Blood eosinophilia is a common feature of eosinophilic fasciitis and is variably reported in systemic sclerosis and localized scleroderma. Since these diseases share cutaneous fibrosis as the final outcome and have other clinical and pathologic features that are difficult to differentiate, the presence of blood eosinophilia may be a further source of confusion. In this study, we examined the frequency and level of blood eosinophilia in 715 patients with systemic sclerosis, 72 patients with localized scleroderma, and 22 patients with clinically active eosinophilic fasciitis. When defined as greater than 400 cells/mm3, eosinophilia was present in 7% of patients with systemic sclerosis, 31% of patients with localized scleroderma, and 83% of patients with eosinophilic fasciitis. Greater than 1000 eosinophils/mm3 were present less frequently in systemic sclerosis (1%) and localized scleroderma (8%) than in eosinophilic fasciitis (61%). No difference in the frequency of eosinophilia was present in patients with the limited cutaneous CREST syndrome or the diffuse cutaneous variety of systemic sclerosis, and in these patients the presence of eosinophilia did not correlate with the extent of cutaneous or internal organ involvement or with other laboratory abnormalities. Among patients with localized scleroderma, eosinophilia was more common in those with linear scleroderma and generalized morphea than in those with morphea, and both the frequency and level of eosinophilia were greater in individuals with clinically active disease (p less than 0.02). Eosinophilia was a persistent feature in untreated patients with active eosinophilic fasciitis, even up to 30 months of disease duration.     

Wells' syndrome. Recurrent granulomatous dermatitis with eosinophilia.

Two cases of granulomatous dermatitis with eosinophilla (Wells' syndrome) are reported. With Wells' original four cases, these two cases define a distinctive dermatosis with onset as cellulitis and formation of solid edema and either final spontaneous resolution or resolution with steroid therapy. Microscopic study showed diffuse tissue eosinophilia and fibrinoid flame figures, evolution of associated focal necrobiosis, and formation of focal microgranulomas associated with eosinophils. Biopsy of muscle and fascia showed comparable fasciitis and eosinophilic myositis. Immunofluorescence in one case disclosed fibrin in the dermis and lgM, lgA, and C3 in the blood vessels of the muscle. Recurrences of the lesions often appeared to be related to drug administration or surgery.     

Eosinophilic Cellulitis (Wells'' Syndrome)

Two patients with eosinophilic cellulitis are reported and 22 additional cases from the literature are reviewed. Cutaneous lesions are variable in appearance and may be confused with cellulitis, urticaria, insect bites, or contact dermatitis. Microscopically, there is a dense dermal infiltrate of eosinophils. Subsequently, granulomatous features with characteristic "flame figures" become apparent. Peripheral eosinophilia is common. The etiology of eosinophilic cellulitis is unknown, although a hypersensitivity mechanism is suspected. Treatment with systemic corticosteroids is frequently effective, but the disease is often characterized by relapses that can occur for several years.     

The histopathogenesis of the flame figure in Wells' syndrome based on five cases

Characteristics of Wells' syndrome are recurrent episodes of edema and erythema of sudden onset, often covering large areas of the skin. Microscopy shows marked eosinophilia and the presence of so-called flame figures. The flame figures have been considered to be either secondary to aggregates of expelled eosinophilic granules and disintergrating eosinophils, or foci of necrobiotic collagen. Our study indicates that the flame figure is secondary to disintegration of eosinophils and consists of aggregates of eosinophilic granules and nuclear fragments and not of necrobiotic collagen. We consider Wells' syndrome to be a distinctive clinical and histological reaction, which can be triggered by many different, mostly unknown factors.     

Role of slit skin smear examination in cutaneous T-cell lymphomas and other chronic dermatoses

The purpose of this study was to evaluate the utility of slit-skin smear examination in the diagnosis of various chronic dermatoses. The study included 24 patients with chronic dermatoses who presented to the skin outpatient department. Ten patients had idiopathic erythroderma, seven were diagnosed with airborne contact dermatitis, four had cutaneous T-cell lymphoma, two had chronic actinic dermatoses, and a single patient had panniculitis. Slit skin smears were obtained from all patients, stained with Leishman stain, and examined under microscope. Out of 24 patients, all four cases of cutaneous T-cell lymphoma showed abnormal cells suggestive of lymphomatous infiltration, two patients with airborne contact dermatitis showed reactive lymphocytes, and two idiopathic erythroderma cases showed numerous eosinophils in the smear. Slit skin smear examination is a simple rapid, decisive test in the diagnosis of cutaneous T-cell lymphoma. It is a useful screening test, especially in Sezary syndrome and diseases with specific skin infiltrate.     

Wells' syndrome: a clinical and histopathologic review of seven cases

Wells' syndrome, or eosinophilic cellulitis, is characterized clinically by an acute dermatitis resembling cellulitis, which evolves into violaceous plaques that resolve spontaneously without scarring. The histopathologic features are dynamic, starting with dermal edema and infiltration of eosinophils, the development of "flame figures," and finishing with the appearance of phagocytic histiocytes. We present the clinical and histopathologic features of seven cases of eosinophilic cellulitis.     

胸腺基质淋巴细胞生成素在炎症性皮肤病中的作用及相关机制研究进展

 

胸腺基质淋巴细胞生成素（TSLP）是一种调节皮肤粘膜炎症反应、维持免疫稳态的IL 7样细胞因子。近年来研究显示,TSLP不但在特应性皮炎、接触性皮炎等变态反应性皮肤病中发挥重要作用,也与玫瑰痤疮、银屑病、嗜酸性皮病和白塞病等多种炎症性皮肤病相关。现就TSLP的一般生物学特性、在常见炎症性皮肤病中的相关作用机制及TSLP单克隆抗体生物制剂的临床疗效作一综述,为临床诊断、治疗提供新思路。

     

Eosinophilic cellulitis and dermographism

A 26-year-old man presented with a history of intermittent erythematous plaques on his hands and legs. A peripheral blood eosinophilia was noted. Histopathologic examination showed numerous eosinophils and characteristic flame figures. The clinical presentation and histopathologic alterations are consistent with the diagnosis of Wells' syndrome, which is also known as eosinophilic cellulitis. Wells' syndrome is a rare condition of unclear etiology. We discuss its diagnosis and possible association with other conditions that manifest peripheral eosinophilia.     

Neutrophilic dermatoses: part II

This article addresses neutrophilic dermatoses, thus complementing the previous article (part I). The following dermatoses are introduced and discussed: subcorneal pustular dermatosis (Sneddon-Wilkinson disease), dermatitis cruris pustulosa et atrophicans, acute generalized exanthematous pustulosis, continuous Hallopeau acrodermatitis, palmoplantar pustulosis, infantile acropustulosis, Andrews' pustular bacteride and eosinophilic pustular folliculitis. A brief review of neutrophilic dermatoses in pediatric patients is also conducted.