Sweet's syndrome and pyoderma gangrenosum associated with ulcerative colitis

A young woman with ulcerative colitis developed pyoderma gangrenosum during the active phase of the disease and Sweet's syndrome (acute febrile neutrophilic dermatosis) three months after panproctocolectomy.     

Neutrophilic Dermatoses

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses – conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions – including systemic drugs, topical agents, and other treatment modalities – for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-α antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis – either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen andUVAradiation. Topical agents can have an adjuvant role in themanagement of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.     

Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia

Pyoderma gangrenosum is a neutrophilic dermatosis of unknown aetiology. Visceral involvement by pyoderma gangrenosum is rare, the lung being the most frequent site of extracutaneous disease. We describe a 73-year-old man with pyoderma gangrenosum and chronic myelomonocytic leukaemia in whom aseptic hepatosplenic abscesses and bony lesions were associated.     

From acute febrile neutrophilic dermatosis to neutrophilic disease: forty years of clinical research

In 1964, Sweet described an acute febrile neutrophilic dermatosis. It is now widely accepted that Sweet's syndrome belongs to a group of associated neutrophilic dermatoses. Although clinically dissimilar, Sweet's syndrome, pyoderma gangrenosum, subcorneal pustular dermatosis, erythema elevatum diutinum, and a few other conditions can be considered a part of this same pathologic spectrum of inflammatory disorders because of (1) the existence of transitional and overlap forms; (2) the similar histopathologic feature of an infiltrate by normal polymorphonuclear leukocytes; (3) the possible occurrence of extracutaneous neutrophilic infiltrates, defining the neutrophilic disease; and (4) the frequent association with systemic diseases. According to the localization of the neutrophilic infiltrate, we describe neutrophilic dermatoses en plaques (dermal), superficial (epidermal), and deep (dermal and hypodermal). Almost every organ of the body may be involved by a neutrophilic aseptic inflammation. The main systemic diseases associated with neutrophlic dermatoses are hematologic, gastrointestinal, and rheumatologic diseases. Although the pathophysiology of these conditions is still poorly understood, treatment with systemic anti-inflammatory agents is usually efficacious.     

Cutaneous manifestations of neutrophilic disease. A study of seven cases.

Seven patients with a complex form of neutrophilic dermatosis are reported. Clinically, they had variable associations of four types of lesions: blisters/pustules, plaques, nodules and ulcerations. Histologically, a neutrophilic infiltrate was observed at variable levels in the epidermis, dermis and subcutis. Systemic manifestations were present in all cases (general symptoms, joint, renal, ocular and lung involvements). Three patients had an associated disease (myelodysplasia, metastatic carcinoma, IgG gammopathy). Steroids were the most efficient treatment. These observations, as well as a review of the literature, support the opinion that the neutrophilic dermatosis represents a continuous spectrum encompassing four well-defined entities: subcorneal pustular dermatosis, Sweet's syndrome, erythema elevatum diutinum and pyoderma gangrenosum. We propose that the different patterns of the neutrophilic dermatosis are the most obvious manifestations of a potentially multisystemic neutrophilic disease and allow its recognition.     

坏疽性脓皮病发病机制的研究进展

 坏疽性脓皮病(PG)是一种病因不明的嗜中性皮肤病。近年来研究显示，MEFV、PSTPIP-1、NOD2、Ptpn6等易感基因可能与PG相关，中性粒细胞功能障碍、补体系统异常、多种细胞因子（如TNF-α、IL-1α、IL-1β、IL-8、IL-17和IL-25等）分泌的异常亦在PG的发病中起着关键作用。本文就近年来PG发病机制中可能存在的遗传易感因素、免疫异常方面的研究进展作一综述。     

Case of multiple myeloma associated with extramedullary cutaneous plasmacytoma and pyoderma gangrenosum

A variety of cutaneous manifestations has been described in multiple myeloma including extramedullary cutaneous plasmacytomas, cutaneous amyloidosis, pyoderma gangrenosum, leukocytoclastic vasculitis, necrobiotic xanthogranuloma, scleromyxedema, Sweet's syndrome, subcorneal pustular dermatosis, scleredema, and plane xanthomas etc. An 89-year-old Korean man, who had been suffering from multiple myeloma 1 year previous, presented for evaluation of two nodules on the right side of the forehead, left side of the chest (7th rib area), and multiple ulcers with papulopustules on both the thigh and the left side of the chest (2nd rib area) during 15 days, which developed at the same time. A biopsy of a lesion which manifested as a cutaneous nodule on the right side of the forehead revealed dermal infiltration by well-differentiated plasma cells, similar to those found on a bone marrow biopsy, and a biopsy of the lesion manifested as a painful ulceration on the right thigh area showing dermal neutrophilic infiltration. Histologic findings were consistent with plasmacytoma and pyoderma gangrenosum, respectively. We present a case of multiple myeloma which developed extramedullary cutaneous plasmacytoma and pyoderma gangrenosum simultaneously, which is very rare. The patient was treated with a systemic steroid and conservative therapy.     

Neutrophilic dermatosis of the dorsal hands in a farmer

Neutrophilic dermatosis of the dorsal hands is a recently described disorder, which is similar to Sweet's syndrome. It is characterized by erythematous plaques, pustules and haemorrhagic bullae located solely on the dorsal surface of the hands. We describe a 57-year-old man with neutrophilic dermatosis of the dorsal hands that occurred following exposure to a chemical fertilizer. There are few cases reported in the literature regarding neutrophilic dermatosis and the aetiology remains unclear. For the present case, we propose that neutrophilic dermatosis of the dorsal hands might have been induced by the chemical fertilizer.     

Management of neutrophilic dermatoses

Neutrophilic dermatoses, including Sweet's syndrome, pyoderma gangrenosum, and rheumatoid neutrophilic dermatitis, are inflammatory conditions of the skin often associated with underlying systemic disease. These are characterized by the accumulation of neutrophils in the skin. The associated conditions, potential for systemic neutrophilic infiltration, and therapeutic management of these disorders can be similar. Sweet's syndrome can often be effectively treated with a brief course of systemic corticosteroids. Pyoderma gangrenosum, however, can be recurrent, and early initiation of a steroid-sparing agent is prudent. Second-line treatment for both of these conditions includes medications affecting neutrophil function, in addition to immunosuppressant medications.     

Pediatric pyoderma gangrenosum associated with leukocyte adhesion deficiency type 1: A case report and review of the literature

Pyoderma gangrenosum is a rare neutrophilic dermatosis characterized by painful skin ulcers with necrotic, undermined margins. In severe cases, particularly in pediatric patients, work-up for an associated autoimmune, inflammatory, malignant, or genetic disorder should be considered based on the clinical presentation. We report a unique case of pediatric pyoderma gangrenosum with a leukemoid reaction, secondary to an autosomal recessive leukocyte adhesion deficiency type 1.     

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.     

Drug-induced Sweet's syndrome in acne caused by different tetracyclines: case report and review of the literature

Sweet's syndrome was first described in 1964. It is characterized by an acute onset of non-pruritic, painful reddish nodules on the head and neck, chest and/or the upper limbs, mostly accompanied by fever, general malaise and leucocytosis. Histopathological examination shows a diffuse dermal neutrophilic infiltrate. The pathogenesis is still not fully understood, and different diseases have been shown to be associated with this syndrome. However, although still very rare, there is an increase of reports on Sweet's syndrome induced by drugs. We describe a 30-year-old man who experienced acute neutrophilic dermatosis after systemic treatment with minocycline. Additionally, there is a strong possibility that the same patient developed a drug-induced Sweet's syndrome after oral administration of tetracycline and doxycycline.     

Neutrophilic dermatosis of the hands after influenza vaccination

An otherwise healthy 72-year-old man presented with a painful eruption composed of grouped hemorrhagic purulent blisters on erythematous plaques, on both palms of his hands, which appeared 12 h after he had been vaccinated against influenza.
Based on the patient's history, physical examination, histopathological and laboratory findings, the diagnosis of neutrophilic dermatosis of the hands (NDH) was made.
NDH is currently considered a distributional variant of Sweet's syndrome (SS). It is identical to atypical pyoderma gangrenosum (PG) and pustular vasculitis of the hands.
There are only seven reported cases of SS after vaccination, none of them with lesions confined solely to the palms or soles. Our current case is the third one of SS following influenza vaccination.     

Sweet''s syndrome progressing to pyoderma gangrenosum—a spectrum of neutrophilic skin disease in association with cryptogenic cirrhosis

A 78-year-old Caucasian woman developed Sweet's syndrome which progressed over 3 weeks to pyoderma gangrenosum and subcorneal pustule formation. In spite of treatment the patient died and post-mortem examination revealed cryptogenic cirrhosis which could have explained the spectrum of neutrophilic skin disease observed in this patient.     

Neutrophilic dermatosis of the dorsal hands related to cocaine abuse

Neutrophilic dermatoses encompass a wide spectrum of diseases characterized by a dense infiltration mainly composed of neutrophils. Neutrophilic dermatosis of the dorsal hands is currently considered a localized variant of Sweet syndrome. Cocaine abuse has been related to a wide range of mucocutaneous manifestations, including neutrophilic dermatoses such as pyoderma gangrenosum. The authors of this study present a patient with neutrophilic dermatosis of the dorsal hands, in which cocaine abuse was identified as a probable trigger.     

Sweet's syndrome: a spectrum of unusual clinical presentations and associations

BACKGROUND: Sweet's syndrome (SS) is the prototypic neutrophilic dermatosis. First described in 1964, the characterization of new clinical associations, unique histopathological findings and clinical variants have stimulated much interest and discussion recently. However, the prevalence of these unusual variants and clinical associations within a single cohort of patients, has not been described. OBJECTIVES: To describe and evaluate the prevalence of unusual clinical and histopathological features, as well as the clinical associations of SS seen in patients from the National Skin Centre, Singapore. METHODS: This is a retrospective study of all consecutive cases of SS seen at our centre over a 5.5-year period (June 1999-December 2004). Data on associated systemic diseases was obtained from the medical records and matched with information from the National Cancer Registry, Singapore. Patients not actively followed up for more than 3 months were contacted for their updated health status. RESULTS: Thirty-seven patients were identified. Ten (27%) had non-idiopathic SS. These were associated with haematological disorders, connective tissue disorders, infections or a drug. Twenty-nine patients (78%) had at least one atypical clinical or histopathological feature. Atypical clinical features included bullous lesions, SS with hand involvement or neutrophilic dermatoses of the hands and the concomitant existence of subcutaneous SS with pyoderma gangrenosum. SS was the presenting feature in three patients with infections caused by atypical organisms, including Mycobacterium chelonae, Penicillium species and Salmonella type D. Unique histopathological variants included subcutaneous SS and lesions containing an admixture of mature and immature neutrophils. Subcutaneous neutrophilic inflammation seemed to be more common in patients with an underlying haematological disorder. This group of patients also had a lower mean haemoglobin level. CONCLUSIONS: Unusual clinical and histopathological variants of SS described in the literature are similarly encountered in our cohort of patients, with some features being more common than others. We highlight and discuss some unique clinical and histopathological observations seen in our patients with SS.     

Multiple neutrophilic dermatoses in myelodysplastic syndrome

A 72-year-old woman developed three consecutive processes that showed characteristics of different neutrophilic dermatoses. First, she developed a picture resembling granuloma faciale, followed by a Sweet's syndrome-like eruption, and then by a superficial pyoderma gangrenosum. She was later diagnosed with myelodysplastic syndrome. This case demonstrates that neutrophilic dermatoses form a spectrum of entities that do not necessarily occur in isolation.     

The relationship between neutrophilic dermatosis of the dorsal hands and sweet syndrome: report of 9 cases and comparison to atypical pyoderma gangrenosum

BACKGROUND: Neutrophilic dermatoses are a collection of diseases with varying presentation unified by clinical and histologic features. Neutrophilic dermatosis of the dorsal hands is a recently described clinical entity and an evolving disease concept. Its relationship to acute febrile neutrophilic dermatosis (Sweet syndrome), pyoderma gangrenosum, and a primary vasculitis has been debated. OBSERVATIONS: We present 9 cases (8 women and 1 man) of neutrophilic dermatosis of the dorsal hands, all with consistent histologic features. Two cases had histologic evidence of vasculitis, and 3 had clinical extension of lesions onto the forearms. Most showed fever, leukocytosis, and/or elevated erythrocyte sedimentation rate. Individual cases were associated with leukemia, lung carcinoma, and inflammatory bowel disease. All 9 patients responded to systemic corticosteroid therapy, with additional response to dapsone, methotrexate, and potassium iodide therapies in several cases. Of the 9 patients, 5 showed complete resolution of their skin disease, whereas 4 required ongoing therapy. We assessed the 43 cases previously reported in the literature. CONCLUSION: The clinical presentation, laboratory data, histologic features, and response to corticosteroid therapy offer strong evidence that neutrophilic dermatosis of the dorsal hands is a localized variant of Sweet syndrome and is also identical to atypical pyoderma gangrenosum when that condition presents on the hands.