Neutrophilic eccrine hidradenitis secondary to infection with Serratia marcescens

Neutrophilic eccrine hidradenitis (NEH) is a rare dermatosis which usually develops after administration of chemotherapeutic treatments. An infective origin is exceptional. We report a patient, previously operated on for ependymoma, who presented with an eruption typical of NEH even though he had not received chemotherapy. Culture of a skin biopsy revealed Serratia marcescens. The dermatosis improved after antibiotic therapy but recurred twice and culture again isolated S. marcescens; electron microscopy revealed cytoplasmic inclusions within neutrophils, suggestive of bacteria. The disease improved every time with appropriate antibiotic therapy. An infective aetiology for NEH is rare: three such cases have been reported, of which one was due to S. marcescens. The originality of our case is the recurrence of the disease on three occasions with the same bacterium isolated on each occasion, with disease remission after antibiotic therapy. This case confirms that infections may be a possible cause of NEH and underlines the necessity to search for infective agents, especially in patients immunocompromised by haematopoietic malignancies and/or chemotherapeutic treatments.     

Eccrine hidradenitis sine neutrophils: a toxic response to chemotherapy

We present a case of hidradenitis occurring in a patient after chemotherapy for acute myeloid leukemia (AML) in the setting of profound neutropenia. Neutrophilic eccrine hidradenitis (NEH) presents as tender erythematous papules and plaques and is often associated with chemotherapy for AML. NEH is postulated to be due to toxic injury to the sweat glands followed by neutrophilic inflammation. Alternatively, some hypothesize that NEH represents a primary neutrophilic process. Our patient's clinical presentation was similar to previously reported cases of NEH; however, degenerative changes of the sweat ducts were noted on microscopy without neutrophilic inflammation. She had fewer than 0.01 thousand neutrophils per microliter for 4 days preceding the biopsy. At the same time, a separate area of superficial skin infection developed because of Staphylococcus epidermidis and also lacked neutrophilic inflammation. The similar clinical course and shared histopathologic features between our case and NEH argue that neutrophils are a secondary response to a toxic effect rather than the primary effector in NEH. Neutrophil-poor variants of hidradenitis, both infectious and due to drug toxicity, should be considered diagnostically in neutropenic patients.     

嗜中性小汗腺炎

报告1例伴有子宫内膜癌的嗜中性小汗腺炎.患者女,63岁.因下腹部、会阴部和双大腿内侧反复出现水肿性红斑2年就诊.皮损每次发作3 d左右可自行消退.皮损组织病理检查示真皮深层小汗腺分泌部和导管周围有中性粒细胞浸润,并且有局灶性腺上皮坏死和空泡变性.抗酸染色和PAS染色均阴性.     

Idiopathic plantar hidradenitis

We describe four cases of idiopathic plantar hidradenitis (IPH), a form of neutrophilic eccrine hidradenitis (NEH) localized on the feet of otherwise healthy young persons. Our patients were all males, 8–17 years old, in apparently good health, but presenting erythematous painful papules, plaques and nodules on the soles. They were afebrile and there were no constitutional symptoms. The differential diagnoses included erythema nodosum, vasculitis and traumatic plantar urticaria. The histological features of IPH are similar to those of NEH, except for the absence of syringosquamous metaplasia. The patients were treated with topical steroids and oral NSAID, with total resolution of the lesions in 1 or 2 weeks.     

Neutrophilic eccrine hidradenitis heralding the onset of chronic myelogenous leukaemia

Neutrophilic eccrine hidradenitis was initially described in acute myelogenous leukaemic patients undergoing chemotherapy, suggesting a drug-induced mechanism. It has been reported in some with various neoplastic and non-neoplastic conditions as well as in healthy individuals. However, most the reported cases have been described in acute myelogenous leukaemic cases receiving chemotherapy. We describe a neutrophilic eccrine hidradenitis case unassociated with chemotherapy in a woman with chronic myelogenous leukaemia.     

Chronic pruritic neutrophilic eccrine hidradenitis in a patient with Behçet's disease

Summary Neutrophilic eccrine hidradenitis (NEH) is a rare distinct entity that usually presents as asymptomatic erythematous papules that disappear spontaneously in 1–3 weeks. However, its appearance may be polymorphic, pruritic, recurrent or even chronic as is described in this case. The histological combination of neutrophilic infiltration in and necrosis of the eccrine secretory gland epithelium is highly characteristic for NEH. It typically occurs in patients receiving chemotherapeutic drugs for malignancies, but other associations have also been reported. To our knowledge, we report the first case of NEH in a patient with Behçet's disease (BD). Cutaneous manifestations of BD, an inflammatory systemic disorder of unknown origin, include neutrophilic dermatoses such as Sweet's syndrome and pyoderma gangrenosum, although these are unusual in BD. NEH could be another neutrophilic dermatosis related to BD. This observation suggests that NEH is not strictly related to chemotherapeutic drugs and malignancies. It appears to be a reactive dermatosis associated with other factors as well, including BD. Treatment was successful with dapsone 100 mg daily.     

A Case of Multiple Eccrine Porocarcinoma with Stepping-Stone Distribution on the Foot

An 89-year-old woman had three tumors of several years' duration in a stepping-stone distribution on the dorsum of her right foot. The two larger ones were diagnosed histologically as eccrine porocarcinomas, and the smallest of the three shared many clinical similarities with them. Eccrine porocarcinoma is a rare malignant tumor. We describe this peculiar case in which three tumors developed in the same region and discuss how they might have occurred. It is most likely that the three tumors of this case originated from nevoid tumors, and that the individual lesions exhibited malignant changes.     

Papillary Eccrine Adenoma with a Pomegranate-like Appearance

Papillary eccrine adenoma (PEA) is a rare cutaneous tumor which histopathologically presents numerous intradermal tubular structures with inward papillary projections. Only a few cases of PEA have been reported recently. We report a case of PEA of a 58-year-old Japanese man. The marked hyperkeratosis and pits gave the tumor the clinical appearance of a burst-open pomegranate. Compact hyperkeratosis within proliferated epidermis contained spiral ducts mimicking intraepidermal eccrine sweat ducts histopathologically. These keratinous structures were thought to correspond to the pores. Several tubular structures running up to the overlying thickened epidermis were observed in the upper dermis. With these findings and with immunohistochemical studies, we proposed that this tumor originated from eccrine sweat ducts.     

A case of infectious eccrine hidradenitis

We report a case of "infectious" neutrophilic eccrine hidradenitis who developed papules on the upper arm and trunk. Histological findings revealed vacuolar degeneration and necrosis of epithelial cells in the eccrine sweat ducts and neutrophils that had migrated through ductal epithelium to the lumen. A microabscess was also seen in the eccrine sweat gland coil. Our patient had received no chemotherapy such as cytarabine. Gram-positive cocci were present in the lesional eccrine duct indicating an infective origin of the disease. Human beta defensin-2, one of epithelial antimicrobial peptides, was present in the lesional epidermis and eccrine duct.     

Multiple vascular eccrine spiradenomas: a case report and published work review of multiple eccrine spiradenomas

Eccrine spiradenoma (ES) usually occurs as a solitary small nodule. It presents rarely as multifocal or multiple localized tumors arranged in a linear, zosteriform or nevoid distribution. We present a rare case of a 55-year-old woman who had a 48-year history of multiple vascular eccrine spiradenomas (VES) localized on the left side of the submandibular region and neck. All five tumors were skin-colored or pinkish-purple, and ranged in size 1.5-2.5 cm. Histologically, each tumor was composed of two characteristic cell types and many dilated vascular spaces were noted in the stroma. Contrast-enhanced computed tomography showed irregularly shaped, enhanced areas at the center of the tumors. A published work search revealed 35 cases of multiple ES, but multiple VES was extremely rare. We summarized the features of previously reported multiple ES and discuss the clinical and histological classification of ES.     

A case report of temple squamoid eccrine ductal carcinoma: A diagnostic challenge beneath the tip of the iceberg

Eccrine carcinomas are a rare tumor entity which originates from eccrine glands and comprise <0.01% of all cutaneous tumors. Eccrine carcinoma subtypes are classified based on their distinct histopathological pattern. Squamoid eccrine ductal carcinoma (SEDC) is a rare subtype, characterized by both squamous and adnexal ductal differentiation, and resembles squamous cell carcinoma on clinical examination. Given the rarity of these tumors, there remains a lack of clear diagnostic criteria or treatment guidelines for the management of SEDC. We report a rare case of SEDC with perineural invasion in an elderly gentleman who was treated with wide local excision.     

Twenty‐four‐week interim analysis from a phase 3 open‐label trial of adalimumab in Japanese patients with moderate to severe hidradenitis suppurativa

Hidradenitis suppurativa (HS) is a chronic skin disease characterized by recurrent painful inflamed nodules/abscesses and draining fistulas that negatively impact quality of life. Adalimumab, a monoclonal antibody against tumor necrosis factor‐α, has been approved in the EU, USA and Japan for the treatment of moderate to severe HS. This is an interim analysis of an ongoing phase 3, multicenter, open‐label, single‐arm study of the safety and efficacy of adalimumab weekly dosing in Japanese patients with moderate to severe HS. Fifteen patients received adalimumab 160 mg at week 0, 80 mg at week 2 and 40 mg every week thereafter starting at week 4. The fulfillment of Hidradenitis Suppurativa Clinical Response was assessed under adalimumab treatment; clinical response was assessed by skin pain, total abscess and inflammatory nodule count and modified Sartorius score; and quality of life and safety were assessed. At week 12, 86.7% of patients achieved clinical response, with improvements at week 12 across the primary and secondary end points generally sustained through week 24. Adalimumab weekly dosing was generally safe and well tolerated with no new safety findings through week 24. These results suggest that adalimumab is effective and well tolerated in Japanese patients with moderate to severe HS.     

Bowen's disease with invasive adnexal carcinoma: the pluripotential nature of Bowen's disease cells

Bowen's disease rarely exhibits multiple combinations of premalignant and/or malignant skin lesions. Bowen's disease with invasive adnexal carcinoma was originally described by Kao, but is not well recognized by clinicians due to its rarity and lack of specific clinical features of this condition. Herein, we describe three unusual cases of Bowen's disease with invasive adnexal carcinoma. The two distinct neoplastic areas exhibited continuity both clinically and histologically. The plaque lesions possessed clinical features typical of Bowen's disease. In cases 1 and 3, we confirmed the adnexal tumor within tumors of Bowen's disease, the diagnosis of which is eccrine porocarcinoma. The tumor in case 2 was characteristic to trichilemmal carcinoma. Immunohistochemically, the tumor cells of Bowen's disease and the adnexal carcinoma differed in antigenicities. The present cases support a notion that Bowen's disease maintains a pluripotential nature.     

Neutrophilic superficial eccrine ductitis: Proposal of a new disease concept

A 3‐year‐old Japanese boy presented with a 3‐month history of itchy erythematous papules on his trunk and forearms, the histologic findings of which were characterized by predominantly neutrophilic inflammation within and around the eccrine sweat ducts and obliteration and disruption of the superficial eccrine sweat ducts. Although the skin disorder had some clinical and histopathologic similarity to miliaria and neutrophilic eccrine hidradenitis, it was ultimately conformed to be neither disorder. Based on this and a case report in the Japanese literature of a 1‐year‐old boy with erythematous eruptions whose clinical and histopathologic findings were similar to those in our case, we propose the skin disorder in our case, referred to as “neutrophilic superficial eccrine ductitis,” as a unique entity.     

Eccrine angiokeratomatous hamartoma combined with solitary angiokeratoma or verrucous venous malformation: Report of two cases and comprehensive review of the literature

First reported in 2006, eccrine angiokeratomatous hamartoma is a very rare vascular malformation of the skin, with only few described cases. It has a peculiar histopathology with features deriving from the combination of two different vascular malformations of the skin: solitary angiokeratoma and eccrine angiomatous hamartoma. In the past, other authors described similar hamartomatous lesions with features deriving from verrucous venous malformation and eccrine angiomatous hamartoma. We believe that these lesions are clearly overlapping from clinical, histopathological, and immunohistochemical points of view and the term “eccrine angiokeratomatous hamartoma” should be used to indicate the whole spectrum of these lesions as suggested by Kanitakis et al. Herein we present two cases of this rare vascular hamartoma, with clinical, histopathological and immunohistochemical characterization. In addition, for the first time we report a complete and detailed review of the literature to clarify the clinical, epidemiological, and histopathological features of this unique entity.     

头皮小汗腺痣合并乳头状汗管囊腺瘤1例

报告1例罕见头皮小汗腺痣合并乳头状汗管囊腺瘤.患者男,78岁,出生时即发现头皮黄豆大小肿物.皮肤科检查:头皮顶部可见一肤色及玫瑰红色斑块.皮损组织病理检查示:部分表皮呈疣状增生,真皮深层可见大量结构正常的小汗腺腺体及导管;另一部分表皮向真皮凹陷形成囊状腔,囊状腔下部可见乳头状突起,乳头状突起由两排细胞组成,乳头状突起基...     

Two cases of multiple eccrine spiradenoma with linear or localized formation

We report two rare cases of recurrent, multiple eccrine spiradenoma. Both cases presented with extensive lesions comprised of multiple red papules of various sizes and a soft blue-red nodule. The first case was a 30-year-old woman. Her lesions followed a linear arrangement on her chin, and extended down the right side of the neck with spontaneous pain. The second case was a 57-year-old woman with tumors in a localized group on the left occipital region without pain. A search of the literature revealed only 15 reported cases of linear/zosteriform/nevoid multiple eccrine spiradenoma. Both cases were treated by surgical excision. Most of the red papules displayed typical histological features including two cell types: large clear cells with low-density cytoplasm; and small dark cells with high-density cytoplasm. The large soft tumors exhibited a variable histological appearance. In the first case, the cystic tumors displayed an homogeneous structure comprised of eosinophilic material. In the second case, the cystic tumors included abundant interstitial tissue.     

Two diseases one remedy? Systemic amyloidosis secondary to hidradenitis suppurativa: Treatment with infliximab

Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Herein, we present such a patient that had developed AA amyloidosis during the course of hidradenitis suppurativa. Both AA amyloidosis and hidradenitis suppurativa have responded to infliximab therapy which was shown by clinical recovery and by the improvement in renal functions.