头皮糜烂性脓疱性皮病一例

患者,女,50岁。头皮红斑、脓疱伴疼痛6个月。皮肤科查体：头皮弥漫性分布紫红色浸润性斑片,其上见脓疱,局部见斑片状脱发区。实验室检查：取脓疱疱液多次行细菌及真菌培养均阴性。组织病理：表皮角化过度伴角化不全;真皮浅中层弥漫性淋巴细胞、浆细胞、中性粒细胞浸润。抗生素治疗效果不佳,口服糖皮质激素及外用他克莫司治疗后有效。诊断：头皮糜烂性脓疱性皮病。     

Erosive pustular dermatosis of the leg: long-term control with topical tacrolimus

Erosive pustular dermatosis of the leg is an unusual form of sterile pustulosis that typically affects the lower limbs of elderly patients. We report the cases of two women who developed erythematous skin plaques with pustules that coalesced and evolved into erosions and crusted areas. Histology showed epidermal spongiosis with subcorneal pustules and a dermal infiltrate with eosinophils and neutrophils. Lesions were treated with topical clobetasol propionate 0.05% for 10 days followed by topical tacrolimus daily until complete resolution, and then twice weekly for 1 year, without relapse. The response to topical corticosteroids and tacrolimus further support the close relationship with erosive pustular dermatosis of the scalp. Topical therapy with tacrolimus may offer good long-term disease control.     

Erosive pustular dermatosis of the leg: report of three cases

Erosive pustular dermatosis of the leg is a distinct form of spongiform amicrobial pustulosis. The disorder typically affects the lower limbs of elderly patients presenting with chronic venous insufficiency and stasis dermatitis, and has a chronic course. Three elderly patients with chronic venous ulcers are described, who developed pustules and moist eroded lesions on the leg. The clinical and histological features were typical for erosive pustular dermatosis. The lesions rapidly responded to topical treatment with either tacrolimus or corticosteroids. Of note, this condition was associated with a diverticular disease in two patients, while in another patient an epidermoid carcinoma of the tongue was present. Erosive pustular dermatosis of the leg is an uncommon but distinct skin disorder typically associated with trophic changes of the lower limbs. Our observations raise the question of the relation of erosive pustular dermatosis of the leg with the group of neutrophilic dermatoses. Topical immunotherapy with tacrolimus may constitute a novel therapeutic option for this frequently recalcitrant condition.     

Erosive pustular dermatosis of the scalp: a successful treatment with photodynamic therapy

Erosive pustular dermatosis of the scalp (EPDS) is a rare inflammatory disease of unknown aetiology that usually occurs in the elderly. It is characterized by sterile pustules, chronic crusted erosions, cicatricial alopecia, and skin atrophy. The histopathology is nonspecific, and its pathophysiology remains undetermined, with various types of local trauma possibly acting as the triggering factor. We describe a case of EPDS in a 75-year-old female in whom there was a marked response to photodynamic therapy with methyl 5-aminolaevulinic acid.     

米诺环素治疗角层下脓疱病一例并文献复习

报道米诺环素治疗角层下脓疱病1例并对相关文献进行复习。患者，女,14岁，臀部、腹股沟、双下肢屈侧反复出现红斑、脓疱6年，加重半月。组织病理学表现为角质层下脓疱，疱内有中性粒细胞浸润，免疫荧光示表皮细胞间及基底膜IgG、IgA、IgM、C3阴性。结合临床表现及病理学检查，确诊为角层下脓疱病。给予口服米诺环素，外用地奈德乳膏治疗，1个月后皮损消退,维持治疗2个月，随访6个月，未复发。     

Pustular Drug Eruption Due to Bacampicilin Hydrochloride in a Patient with Psoriasis

We report a case of pustular drug eruption due to bacampicilin hydrochloride which developed in a patient with pustular psoriasis. The patient was a 45-year-old Japanese woman with psoriasis which started as pustular psoriasis twenty years previously. In 1994, she developed generalized erythema with pustules accompanied by high fever and liver injury. Clinical and histological findings of this pustular eruption were different from her previous episodes of pustular psoriasis. Erythemas and pustules disappeared and her abnormal transaminase returned to normal rapidly when she discontinued bacampicilin hydrochloride. Her positive reaction to a patch test and a lymphocyte stimulation test also suggested that our case had a pustular drug eruption rather than pustular psoriasis induced by a drug.     

Erosive pustular dermatosis of the leg associated with compression bandaging and fungal infection

BACKGROUND: Erosive pustular dermatosis of the leg (EPDL) has been described in association with venous insufficiency and atrophy of the skin of the lower leg. Like erosive pustular dermatosis of the scalp, this disease has also been reported to be a non-infective condition. OBJECTIVES: To investigate the clinicopathological features and, where possible, the aetiology of clinical EPDL. METHODS: We identified a group of patients undergoing continuous compression bandaging for venous dermatitis of the legs and/or predominantly venous leg ulceration with clinical features described in patients with EPDL. They were investigated by skin biopsy, patch testing and microbiological tests for the presence of bacteria and fungi. RESULTS: Twenty-four of 400 (6%) patients were noted to have pustules on the leg(s). There was laboratory evidence of fungal infection in 13 of 24 patients (54%), with complete and sustained resolution of pustules after antifungal treatment. Pustulation in the other 11 patients (46%) was unresponsive to antibiotics for confirmed bacterial infection; some improvement was seen with potent topical corticosteroids but full clearance was achieved only after a switch from continuous four-layer compression bandaging to the use of intermittent long stretch compression. CONCLUSIONS: EPDL is a fairly common clinical picture seen in patients undergoing continuous compression bandaging. It may be produced by opportunistic, particularly fungal, infection. In almost half an infective aetiology cannot be demonstrated and a pyoderma gangrenosum-like process may be implicated.     

Pustular dermatosis of the scalp associated with autoimmune diseases

A 36-year-old woman visited our hospital with a five month history of persistent pustulation, crusting, and alopecia on the vertex of the scalp. No pathological organisms were isolated from the lesions. Histological examination revealed non-specific changes of chronic inflammation with destroyed follicles. Antibiotic therapy produced no response, but steroid therapy was effective. From these observations, a diagnosis of erosive pustular dermatosis of the scalp (EPDS), as described by Pye et al., was made. The patient also had Hashimoto's thyroiditis, autoimmune hepatitis, and Takayasu's aortitis. The laboratory studies revealed an increased erythrocyte sedimentation rate, C-reactive protein 3+, hypergammaglobulinemia, and various auto-antibodies, suggesting the possibility of a pathogenesis common to both this dermatosis and the autoimmune diseases.     

角层下脓疱性皮病合并结缔组织病二例

报道2例角层下脓疱性皮病，皮损均表现为红斑基础上的水疱、脓疱，组织病理学特征为角层下脓疱，直接免疫荧光阴性；脓疱疱液细菌培养阴性。患者1，女，53岁，合并类风湿关节炎。口服阿维A，糖皮质激素和羟氯喹治疗。患者2，女，69岁，合并干燥综合症及自身免疫性肝炎，予糖皮质激素及米诺环素治疗。2例患者均好转。     

Post-traumatic erosive dermatosis of the scalp: A hypergranulated variant

Erosive pustular dermatosis of the scalp is a slowly progressive chronic inflammatory disease that predominantly affects elderly male patients with marked actinic damage. The clinical evolution consists firstly of keratotic and erosive plaques surmounted by yellow-brown crusts and non-follicular pustules; later, the active crusting lesions regress in number and the scarring process causes diffuse cutaneous thinning and loss of hair follicles. However, manifestations may be atypical, leading to frequent misdiagnosis. We present a case series of post-traumatic erosive pustular dermatosis on the scalp of 4 elderly patients. The characterising feature was the presence of erosion consisting of abundant hypergranulation tissue, with an almost total lack of crusts and pustules. Dermoscopy showed a unique pattern of stretched and dilated linear, telangiectatic and polymorphous on-focus vessels, milky-red areas and white scarring areas. This clinical entity is rarely reported in the literature. The majority of reported cases were located on the legs.     

Eosinophil-infiltrating folliculitis in childhood--report of a case

A 5-year-old Japanese girl developed follicular pustules with erythematous plaques on the lower extremities. The histopathology was compatible with eosinophilic pustular folliculitis (EPF). However, the clinical findings were not typical of EPF; the pustules did not appear in crops, the pustules became ulcers, and the skin lesions appeared exclusively on non-seborrheic areas.     

Subcorneal pustular dermatosis type of IgA pemphigus: demonstration of autoantibodies to desmocollin-1 and clinical review

We describe a 40-year-old Japanese man with a 3-year history of vesiculopustular lesions resembling subcorneal pustular dermatosis. Histopathology showed subcorneal pustules containing a few acantholytic cells, and direct immunofluorescence disclosed IgA deposition in the intercellular space of the upper epidermis. Circulating IgA autoantibodies of very low titre were also demonstrated by indirect immunofluorescence. A novel cDNA transfection technique clearly detected IgA autoantibodies reactive with human desmocollin-1. Combined therapy with dapsone and etretinate improved the skin lesions. We review the clinical features of 49 patients in the literature who presented with vesiculopustular lesions and intraepidermal IgA deposition.     

Erosive Pustular Dermatosis of the Scalp Following Herpes Zoster: Successful Treatment with Topical Tacrolimus

Erosive pustular dermatosis of the scalp (EPDS) is a rare disorder of the elderly characterized by multiple pustular lesions with erosions and crusting that result in scarring alopecia. EPDS typically develops in aged or sun-damaged skin with a history of trauma. Histopathologically, EPDS is nonspecific, showing atrophic epidermis and chronic inflammation. Bacteriological and mycological investigations of EPDS are generally negative. Although herpes zoster is a common disorder in elderly people, previously reported cases of EPDS were rarely associated with herpes zoster. We present a rare case of EPDS following herpes zoster treated successfully with topical tacrolimus.     

Eosinophilic pustular folliculitis in infancy: an unusual case

INTRODUCTION: Eosinophilic pustular folliculitis in children is a follicular inflammatory dermatosis, usually occurring early in life. The disease progresses in flares of prurigenous plaques studded with papules and sterile pustules of the scalp and other areas of the skin. OBSERVATION: A 7 year-old boy presented with itching papular vesicular and pustular plaques on the scalp and the face. Pigmented plaques with pustular border, located on the trunk, were associated with pustular and erosive lesions of the side of the lower lip and in the nostrils. A specimen taken from the pustules did not show bacterial or fungal infection. Histologic examination of a biopsy specimen showed subcorneal pustules with eosinophilic and neutrophilic infiltrates of follicles. Clinical improvement was obtained only by the combination of steroids and dapsone, but recurrence followed withdrawal of treatment. DISCUSSION: Eosinophilic pustular folliculitis in children is rare. Our case report combines features of the infancy form (lesions located on the scalp and face) and the adult form (location on the trunk and limbs with annular distribution), expressing the conceptual confusion that remains between both forms. The mucosal involvement seen in our patient has never been reported in the literature neither in the infancy nor in the adult form.     

Case of acrodermatitis continua of Hallopeau following psoriasis with atypical clinical presentation

We present a 63‐year‐old Japanese woman who had clinically unique symmetrical skin rashes on her lower face, inframammary area, back and extremities, with some pustules on the cheeks. Skin biopsy specimens showed typical findings of psoriasis, and Psoriasis Area and Severity Index score was 5.9. After the skin lesions were treated successfully with vitamin D3 ointment, pustules developed on the tips of the fingers and toes, with paronychial and subungual involvement. The pathology of the nail matrix was consistent with pustular psoriasis, and the patient was diagnosed with acrodermatitis continua of Hallopeau (ACH) following psoriasis with an unusual clinical presentation. ACH was well controlled with a low dose of cyclosporin. Our patient is a rare case chronologically affected by two diseases in the same category. We confirmed that ACH is a variant of pustular psoriasis, and believe that the patient could provide another clue to determining the entity of ACH.     

头皮糜烂性脓疱性皮病1例

  报告1例头皮糜烂性脓疱性皮病。患者女,82岁,因右侧头皮颞部反复出现红斑、脓疱、糜烂、结痂3个月就诊。皮肤科检查：右侧头皮颞部见大片红斑、糜烂、结痂,中间部分毛发脱落,皮肤轻度萎缩,浅表疤痕形成。皮损组织病理示：浅表糜烂结痂,表皮不规则增生,部分表皮轻度萎缩,真皮浅层可见以淋巴、组织细胞、浆细胞及少量中性粒细胞为主的混合炎症细胞浸润,毛囊萎缩,数量减少,局部仅见立毛肌,毛囊结构消失,部分区域胶原纤维增生硬化,PAS染色与抗酸染色均阴性。诊断：头皮糜烂性脓疱性皮病。治疗：予以口服异维A酸,外涂他克莫司与卡泊三醇软膏,皮疹较前稍好转。现随访观察中。     

Eosinophilic pustular folliculitis of infancy suppressed with cetirizine

Eosinophilic pustular folliculitis of infancy is a rare inflammatory disease characterized by recurrent, itchy sterile pustules mainly located on the scalp. Topical agents are mostly effective to suppress the symptoms in the majority of cases. However, systemic agents that are safe for long‐term use are required for patients with widespread or resistant lesions. We present a case with eosinophilic pustular folliculitis of infancy whose disease was suppressed with cetirizine but recurred after ceasing the drug.     

Pentoxyfilline as a treatment for subcorneal pustular dermatosis

Subcorneal pustular dermatosis (SPD) is a rare pustular neutrophilic dermatosis in which groups of sterile pustules appear in the superficial (subcorneal) skin. This chronic condition can be associated with significant morbidity and decreased quality of life. Dapsone is the first‐line therapy for SPD, but some patients fail to respond or cannot tolerate it. In these instances, patients may be treated with second‐line therapies such as phototherapy, topical corticosteroids, or systemic agents including glucocorticoids, acitretin, immunosuppressive, or biologic medications. These therapies may not always be efficacious and can be associated with intolerable adverse effects. Here, we report a case of a patient who sustained long‐term remission and no side effects with the novel use of pentoxifylline, a tumor necrosis factor‐alpha inhibitor, as monotherapy. Pentoxifylline should be considered as a possible therapy in patients with SPD intolerant to dapsone.     

增殖性脓性皮炎-脓性口炎一例

【摘要】 患者女,58岁,唇、头皮、皱褶部位反复发生红斑、脓疱、增殖性斑块3个月。患者有溃疡性结肠炎病史2年,皮疹发生前肠炎加重。皮肤科检查：双唇弥漫性增厚,上有密集米粒至绿豆大脓疱,部分脓疱融合,唇缘黄色结痂;左颊黏膜有一1.5 cm × 2 cm的增殖性斑块,边缘脓疱呈环状排列;头皮、颈、左腋窝、脐周、左腹股沟可见多发性大小不等的增殖性斑块、糜烂、黄色结痂,中央和边缘有环状排列的水疱和脓疱,皮疹消退后留色素沉着斑;右腋4个新发脓疱。下唇缘皮损组织病理学检查：表皮轻度增厚伴中性粒细胞微脓肿,真皮密集混合性炎症细胞浸润,可见中性粒细胞和嗜酸性粒细胞微脓肿;下唇皮疹边缘正常皮肤直接免疫荧光检查阴性。血常规检查示嗜酸性粒细胞比例增高。脓疱细菌、真菌培养阴性。诊断：增殖性脓性皮炎-脓性口炎。 【关键词】 增殖性脓性皮炎-脓性口炎     

Folliculitis decalvans-like pustular plaques on the limbs sparing the scalp

Folliculitis decalvans is a neutrophilic cicatricial alopecia characterised by progressive pustular folliculitis. Folliculitis decalvans is seen as a condition usually limited exclusively to the scalp and rarely affects the limbs. We present a case of a 63-year-old man with a 3-year history of progressive pustular folliculitis with inflammatory patches and central scarring alopecia on both forearms and a circumscribed patch on his right lower leg. His presentation, clinical course and isolation of Staphylococcus aureus together with the histopathological findings all supported a folliculitis decalvans-like pustular folliculitis limited to the limbs. Biopsies revealed follicular pustules, gross interfollicular fibrosis with plasma cells and concentric perifollicular fibrosis with lymphocytes, all features seen with folliculitis decalvans. The positive response to antibiotics combined with topical corticosteroids mirrored the response seen with scalp folliculitis decalvans. In contrast to the previously reported cases, the patient had no evidence of folliculitis decalvans on the scalp.