Neutrophilic dermatoses: Pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.

Neutrophilic dermatoses are a heterogeneous group of inflammatory skin disorders that present with unique clinical features but are unified by the presence of a sterile, predominantly neutrophilic infiltrate on histopathology. The morphology of cutaneous lesions associated with these disorders is heterogeneous, which renders diagnosis challenging. Moreover, a thorough evaluation is required to exclude diseases that mimic these disorders and to diagnose potential associated infectious, inflammatory, and neoplastic processes. While some neutrophilic dermatoses may resolve spontaneously, most require treatment to achieve remission. Delays in diagnosis and treatment can lead to significant patient morbidity and even mortality. Therapeutic modalities range from systemic corticosteroids to novel biologic agents, and the treatment literature is rapidly expanding. The first article in this continuing medical education series explores the pathogenesis of neutrophilic dermatoses and reviews the epidemiology, clinical and histopathologic features, diagnosis, and management of Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.

Clinicopathologic analysis of atypical hand, foot, and mouth disease in adult patients.

BACKGROUND: Hand, foot, and mouth disease is a contagious viral infection usually affecting children. A resurgence of cases in adults, mainly caused by coxsackievirus A6 and with an atypical and more severe presentation, has taken place. OBJECTIVE: The goal was to examine the clinical, histologic, and immunohistochemical features of this disease in adults. METHODS: This is a retrospective study on documented cases of adult hand, foot, and mouth disease from France's Dermatology Department of Strasbourg University Hospital and Bel-Air Hospital in Thionville. RESULTS: Six patients with severe and atypical presentation were included, 4 caused by coxsackievirus A6. The histologic features were: spongiosis, neutrophilic exocytosis, massive keratinocyte necrosis, shadow cells in the upper epidermis, vacuolization of basal cells, necrotic cells in follicles and sweat glands, dense superficial dermal infiltrate of CD3+ lymphocytes, and strong granulysin expression. LIMITATIONS: This is a retrospective case series. CONCLUSION: In adult patients presenting with atypical hand, foot, and mouth disease caused by coxsackievirus A6, biopsy specimens show distinctive changes in the epidermis but also in adnexal structures. The inflammatory infiltrate is made of T cells with a cytotoxic profile, with numerous granulysin-positive cells, as observed in severe drug-induced eruption with necrosis of keratinocytes.

Palmar Eccrine Hidradenitis Secondary to Trauma from Computer Gaming in an Adolescent After Bone Marrow Transplantation.

A 14-year-old boy who had undergone a matched sibling bone marrow transplant for acute lymphoblastic leukemia presented with painful nodules on his palms after prolonged gaming on his computer and mobile phone. Histology showed a neutrophilic inflammatory infiltrate surrounding the acrosyringium and eccrine sweat coils in the deep dermis. The lesions resolved spontaneously with conservative management.

[Hidradenitis suppurativa/acne inversa: An update]. / Hidradenitis suppurativa/Acne inversa : Ein Update.

Hidradenitis suppurativa/acne inversa (HS/AI) is a chronic inflammatory disease characterized by painful nodules, abscesses, fistulas, sinus tracts and scarring, which may lead to severe functional and psychological impairment. Patients often suffer for many years before the right diagnosis is finally made. HS/AI is still a therapeutic challenge. Conservative therapies play a role in mild stages of the disease; however they do not result in healing. Therapy of choice associated with the lowest recurrence rate is a radical wide excision of involved skin.

Infectious eccrine hidradenitis: sweat glands as the portal of entry for cellulitis.

Neutrophilic eccrine hidradenitis (NEH) is a rare neutrophilic dermatosis involving the eccrine glands. It is commonly associated with haematological malignancy and administration of chemotherapy. An infective aetiology for NEH is termed infectious eccrine hidradenitis (IEH). Pathogens that have been associated with IEH include Nocardia, Serratia, Enterobacter sp., Staphylococcus aureus and Mycobacterium chelonae We describe a case of IEH in a patient with prolonged use of a compression sleeve for their upper limb lymphoedema. The histopathological findings of NEH and IEH are almost identical. Skin tissue culture and rapid clinical improvement with antibiotic therapy are keys in delineating the two subtypes.

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.

Occlusive eccrine hidradenitis presented as a reticulated eruption on the buttocks.

A 13-month-old girl presented a reticulated eruption of 2 months duration located on her buttocks. Histologically, a mononuclear cell inflammatory infiltration was found within and around the eccrine sweat glands, with epithelial cell damage. The lesions were related to several traumatic mechanisms involving the buttocks, such as crawling and repeated trauma with a bathmat carpet.

Neutrophilic dermatoses: Kids are not just little people.

Neutrophilic dermatoses are a group of inflammatory skin disorders characterized by an overactive innate immune system with dysregulation of neutrophils without underlying infectious etiology. The major representative conditions discussed are Sweet syndrome; pyoderma gangrenosum; neutrophilic eccrine hidradenitis; palmoplantar eccrine hidradenitis; subcorneal pustular dermatoses; bowel-associated dermatosis arthritis syndrome; and synovitis, acne, pustulosis, hyperostosis, and osteitis. We will also discuss other neutrophilic conditions present almost exclusively in the pediatric population, including congenital erosive and vesicular dermatosis with reticulated supple scarring and the recently described group of autoinflammatory diseases. The clinical characteristics, diagnostic approach, and treatment management in the pediatric and adult population are discussed.

Modern Electronic Devices: An Increasingly Common Cause of Skin Disorders in Consumers.

: The modern conveniences and enjoyment brought about by electronic devices bring with them some health concerns. In particular, personal electronic devices are responsible for rising cases of several skin disorders, including pressure, friction, contact dermatitis, and other physical dermatitis. The universal use of such devices, either for work or recreational purposes, will probably increase the occurrence of polymorphous skin manifestations over time. It is important for clinicians to consider electronics as potential sources of dermatological ailments, for proper patient management. We performed a literature review on skin disorders associated with the personal use of modern technology, including personal computers and laptops, personal computer accessories, mobile phones, tablets, video games, and consoles.

[Neutrophilic eccrine hidradenitis in a healthy patient]. / Hidradénite eccrine neutrophilique chez un sujet sain.

BACKGROUND: Neutrophilic eccrine hidradenitis occurs mostly in patients receiving chemotherapy for acute myeloblastic leukemia, rarely in healthy patients. Histopathologic pattern is characteristic and includes selective necrosis of the eccrine glands with a local neutrophilic infiltrate. CASE REPORT: We report the case of a 41 year-old woman with no medical past-history who developed during 2 years successive papulous skin eruptions. The latter always resolved spontaneously. Skin biopsy led to the diagnosis showing typical features of neutrophilic eccrine hidradenitis. DISCUSSION: Our case is original because the neutrophilic eccrine hidradenitis had occurred in a healthy woman. Neither malignant hematologic disease nor visceral cancer was disclosed in this patient in the following 31 months.

Neutrophilic dermatoses during granulocytopenia.

BACKGROUND AND DESIGN: Noninfectious cutaneous neutrophilic lesions can occur during granulocytopenia, but their mechanism remains unknown. We undertook a retrospective study of the neutrophilic dermatoses that developed during granulocytopenia induced by chemotherapy for acute myelogenous leukemia. RESULTS: Seven men and one woman were included (2.6% of treated cases of acute myelogenous leukemia); half had acute myelogenous leukemia subtypes 4 and 5. The male-to-female ratio was 7:1. Neutrophilic eccrine hidradenitis was diagnosed in five cases, Sweet's syndrome in two cases, and difficult-to-classify neutrophilic dermatoses in one case. Cutaneous lesions appeared 12.5 days after the start of chemotherapy, and the mean leukocyte count was 0.426 x 10(9)/L. Three patients needed corticosteroids systemically. CONCLUSION: Neutrophilic dermatoses during chemotherapy-induced granulocytopenia seem to occur more frequently in men with acute myelogenous leukemia subtypes 4 and 5.

Neutrophilic eccrine hidradenitis in actinic reticuloid syndrome.

Neutrophilic eccrine hidradenitis (NEH) is a rare, transient complication, which usually occurs in patients with leukemia receiving various chemotherapeutic regimens. However, similar eruptions have been observed in other conditions, including HIV-positive patients, and are often preceded by the onset of malignancies. We report the first case of NEH arising in a patient with actinic reticuloid syndrome who had been treated with methotrexate.

Neutrophil eccrine hidradenitis in a patient with AIDS.

Neutrophilic eccrine hidradenitis (NEH), first described as a rare, transient, and benign complication of various chemotherapy regimens for acute leukemia, has also been observed in other conditions, including three HIV-positive patients and even in otherwise healthy individuals (1-3). A similar histological pattern was described after intradermal bleomycin injections into normal human skin (4). We report the first case of NEH in a hemophilic HIV infected patient treated with stavudine, a new reverse transcriptase inhibitor.

Mastitis in the lactating mink female (Mustela vison S.) and the development of "greasy kits".

"Greasy kits" is the result of a multifactorial disease complex with few known definitive aetiological factors. Mastitis has been hypothesized as a triggering factor although classical clinical signs of mastitis (rubor, tumor, dolor, calor) are rarely seen in lactating Danish mink females. In this study we sacrificed 2 groups of lactating mink females with a total of 78 mammary glands at day 19-30 after giving birth. The first group had raised normal mink kits while the other group had suffered severe attacks of greasy kits. We found no clinical or histopathological evidence of mastitis but isolated streptococci and staphylococci from 2 mammary glands in females raising greasy kits. These glands showed no clinical or histological signs of inflammation attributable to bacteria and we conclude that mastitis is not necessary for the generation of greasy kits.

[Pseudomonas eccrine hidradenitis in a child revealing acute lymphoblastic leukemia]. / Hidradénite eccrine à Pseudomonas de l'enfant révélatrice d'une leucémie aiguë lymphoblastique.

INTRODUCTION: We report the case of a Pseudomonas (P.) aeruginosa eccrine hidradenitis in a child, or a "Pseudomonas Hot Foot Syndrome", revealing an acute lymphoblastic leukemia. OBSERVATION: A 10 year-old girl consulted for the sudden onset of painful and necrotic palmoplantar nodules in a context of fever and shivering. Histology of a cutaneous biopsy found necrosis of the eccrine glands and, on culture, P. aeruginosa. The blood count revealed pancytopenia and the myelogram acute lymphoblastic leukemia. All the hemocultures and other microbiological samples were negative. The cutaneous signs had appeared 48 hours after bathing in an aquatic amusement park. Diagnosis of Pseudomonas eccrine hidradenitis, or "Pseudomonas Hot Foot Syndrome" was retained, although the local sanitary authorities were not able to demonstrate P. aeruginosa contamination of the water in the park. COMMENTS: Lesions evoking juvenile Pseudomonas aeruginosa eccrine hidradenitis without obvious traumatic factor must lead to the search for P. aeruginosa contamination from water and the subsequent sanitary and epidemiological consequences. Furthermore, severe P. aeruginosa cutaneous infections in children must also lead to the search for an underlying immunosuppression and notably an acute leukemic process.

Infectious eccrine hidradenitis associated with intense sun exposure.

Infectious eccrine hidradenitis (IEH), which usually manifests as singular or multiple erythematous papules or plaques, is a rare dermatosis involving an infectious agent and histologic findings identical to that of neutrophilic eccrine hidradenitis (NEH). We report a case of IEH in a 24-year-old woman who developed a pruritic, erythematous, papular rash after a sunburn. A culture of a pustule revealed methicillin-sensitive Staphylococcus aureus. Our patient had complete resolution of her rash within 2 weeks of starting amoxicillin and clavulanate. This case of IEH and NEH related to both intense sun exposure and infection supports the hypothesis that NEH is a response to nonspecific stimuli and may occur in many different clinical settings.