Sweet's syndrome (acute febrile neutrophilic dermatosis)

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a condition characterized by the sudden onset of fever, leukocytosis, and tender,erythematous, well-demarcated papules and plaques which show dense neutrophilic infiltrates on histologic examination. Although it ma occur in the absence of other known disease, Sweet's syndrome is often associated with hematologic disease (including leukemia), and immunologic disease (rheumatoid arthritis, inflammatory bowel disease). Treatment with systemic corticosteroids is usually successful. skin, Sweet syndrome, neutrophilic dermatosis, corticosteroids,     

以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例

 报告以急性发热性嗜中性皮病为首发症状的急性髓系白血病1例。患者女,50岁,反复全身红色斑块伴疼痛4个月,加重2 d。皮肤科检查：颈部及双上肢分布大小不等红色斑块,皮损边缘略隆起于皮面,呈环状,未见水疱及破溃,触痛阳性。上唇少许糜烂面。组织病理符合急性发热性嗜中性皮病改变。入院后查血液系统异常,诊断急性髓系白血病,以急性发热性嗜中性皮病为首发症状。予EA方案预化疗。三个月后患者因严重脑出血死亡     

Pyoderma gangrenosum associated with biphenotypic acute leukemia

Pyoderma gangrenosum is a neutrophilic dermatosis that may be associated with myeloid malignancies. Less information is available about the association of pyoderma gangrenosum with lymphoid malignancies. We present, to our knowledge, the first case of pyoderma gangrenosum associated with biphenotypic acute leukemia wherein the malignant cells show a phenotype specific for myelogenic and lymphocytic leukemia. Histopathologic examination revealed rather nonspecific features without involvement of leukemic cells in the skin lesions. Treatment with systemic steroids was followed by characteristically rapid healing of the skin lesion.     

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.     

Acute febrile neutrophilic dermatosis (Sweet's syndrome) in a patient with hairy-cell leukemia

A case of hairy-cell leukemia that started with cutaneous lesions similar to those of acute febrile neutrophilic dermatosis (Sweet's syndrome) is reported. The patient had leukopenia and a recurrent eruption for a year prior to the diagnosis of the hematologic disorder. Bone marrow examination eventually demonstrated characteristic "hairy" cells with tartrate-resistant, acid-phosphatase activity. Biopsy of a cutaneous lesion suggested an abscess showing a dense neutrophilic dermal infiltrate with perivascular predilection. The findings of IgA, IgM, C3, and fibrinogen in vessel walls by immunofluorescence and vascular disruption by electron microscopy contribute additional evidence for an immunologic determinant in this instance of Sweet's syndrome. It is known that neutrophilic infiltrates of Sweet's syndrome may have a variety of clinical presentations. This is the second case of hairy-cell leukemia with which Sweet's syndrome was associated.     

手部嗜中性皮病

报告3例手部嗜中性皮病。3例患者均为女性,有相似的临床和组织病理学特点,无发热、关节痛等全身症状。2例患者组织病理上有明显的血管炎改变。3例患者均未并发骨髓增生不良性疾病、髓细胞性白血病和炎症性肠病;对121服泼尼松、雷公藤多苷和5％碘化钾均有明显疗效。3例患者的临床表现、组织病理学改变以及对糖皮质激素等治疗的反应均支持手部嗜中性皮病是一种局限型的Sweet综合征。     

Neutrophilic eccrine hidradenitis. A distinctive type of neutrophilic dermatosis associated with myelogenous leukemia and chemotherapy

On two occasions, erythematous edematous plaques developed on the left side of the neck and the left shoulder of a man undergoing induction chemotherapy for acute myelogenous leukemia. The lesions resolved after several days in both instances. Histologically, numerous neutrophils surrounded and focally infiltrated the eccrine secretory coils, in which epithelial necrosis was observed. The "fixed" nature of the plaques and temporal relationship to chemotherapy suggest that the lesions represent an unusual reaction to chemotherapeutic agents. It is possible that this unique clinicopathologic picture represents a neutrophilic dermatosis associated with leukemia.     

Atypical pyoderma gangrenosum as a manifestation of childhood acute lymphoblastic leukemia

Pyoderma gangrenosum is a neutrophilic dermatosis that may occur idiopathically or in association with various systemic diseases and malignancy. Although the association of this entity with myeloid malignancies is well known, its association with lymphoid malignancy is extremely rare. We describe atypical pyoderma gangrenosum in association with acute lymphoblastic leukemia in a 2-year-old child, an occurrence not reported before.     

Neutrophilic dermatosis of myeloproliferative disorders: Atypical forms of pyoderma gangrenosum and Sweet's syndrome associated with myeloproliferative disorders

Atypical forms of pyoderma gangrenosum (PG) and Sweet's syndrome (SS) (acute febrile neutrophilic dermatosis) have been separately reported in association with various forms of leukemia over the past decade. A case in which both atypical bullous PG and atypical SS occurred in a patient with myeloid metaplasia is presented, and the literature is reviewed concerning the association of these dermatoses with neoplasias of myeloid origin. The current case and review support the thesis that these dermatoses, when associated with myeloproliferative disorders, represent points on a continuum of noninfectious, nonmetastatic, inflammatory neutrophilic dermatoses that may occur in patients with derangements in myeloid cell proliferation.     

ACUTE FEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME) WITH MYELOID PROLIFERATION

A case of acute febrile neutrophilic dermatosis in a 21-year-old man is presented. Onset of the dermatosis followed sore throat, gastro-enteritis treated with sulphonamides and trimethoprim, and signs of meningism. Accompanying the pronounced neutrophilic leucocytosis and dermal polymorph infiltrate, there was marked myeloblastic proliferation in the bone marrow. While distinction from acute myeloid leukaemia was difficult, there was a rapid response to steroid therapy. The findings emphasize that myeloid proliferation may be a central feature in at least some cases of acute neutrophilic dermatosis.     

Sweet's syndrome associated with trans-retinoic acid treatment in acute promyelocytic leukemia

A case of acute promyelocytic leukemia is reported in which Sweet's syndrome developed at the time of an improvement in white blood cell count due to transretinoic acid. Acute febrile neutrophilic dermatosis (Sweet's syndrome) is a disorder characterized by acute onset of inflammatory skin nodules associated with systemic features which include malaise, fever and neutrophilia. Many triggers and associated disorders have been identified since the syndrome was described, the most important being the association with haematological diseases including leukemia' and other myeloproliferative disorders.² We describe a case apparently provoked by drug therapy.     

Neutrophilic eccrine hidradenitis: a case report of an unusual annular presentation

Neutrophilic eccrine hidradenitis (NEH) is an acute, self-limited, inflammatory neutrophilic dermatosis. It is most commonly described in patients with acute myelogenous leukemia (AML) following chemotherapy. NEH generally presents as erythematous papules and plaques on the head, trunk, and extremities. Histologically, NEH is characterized by a neutrophilic infiltrate surrounding eccrine ducts and coils with occasional necrosis. We describe a case of NEH with an unusual presentation of annular plaques. A search of the literature revealed only one other case report of NEH presenting as an annular eruption.     

Panniculitis in a 3‐year‐old child with Fanconi anemia‐associated bone marrow hypoplasia heralds transformation to acute myeloid leukemia

Fanconi anemia is a rare, autosomal recessive genomic instability disorder characterized by congenital limb anomalies, panmyelopathy and a high risk of malignancy, principally acute myeloid leukemia. Hematologic malignancy presenting with acute febrile neutrophilic dermatosis (Sweet syndrome), both deep and superficial forms, is well described in Fanconi anemia patients but is a rare phenomenon in otherwise healthy children. We present a case of panniculitis (presumptive subcutaneous Sweet syndrome) heralding transformation to acute myeloid leukemia in a 3‐year‐old boy with a severe Fanconi anemia phenotype.     

Letter: Neutrophilic eccrine hidroadenitis with atypical findings

Neutrophilic eccrine hidradenitis (NEH) was originally described as a distinctive dermatosis occurring in patients undergoing chemotherapy, especially associated with the use of cytarabine in the treatment of acute myelogenous leukemia (AML). This disorder is characterized by a neutrophilic infiltrate around the eccrine glands and coils and is associated with necrosis. However, atypical findings in the histology have been reported. To the best of our knowledge, leukocytoclastic vasculitis has not been described in association with NEH. We hypothesize that a toxic effect of cytarabine may damage the vessel wall to some extent, causing a vasculitic phenomenon.     

An Unusual Association between Sweet's Syndrome and Metastatic Papillary Follicular Thyroid Carcinoma

Sweet''s syndrome (SS), or acute febrile neutrophilic dermatosis, is marked by fever, leukocytosis, and painful erythematous papules/plaques resulting from neutrophil migration and accumulation in the dermis. This condition has been associated with underlying hematologic as well as solid malignancies. We describe a unique case of SS in a patient with metastatic papillary follicular thyroid carcinoma and group A streptococcal pharyngitis. The distribution of the patient''s SS rash was similar to the rash of neutrophilic dermatosis (pustular vasculitis) of the dorsal hands.     

Acute febrile neutrophilic dermatosis (Sweet's syndrome) following BCG vaccination

Several factors may trigger or be associated with acute febrile neutrophilic dermatosis (AFND). We report a case of AFND following BCG vaccination as an interesting association although no direct interrelation can be certified.     

Sweet's syndrome masquerading as facial cellulitis

Sweet's syndrome, or acute febrile neutrophilic dermatosis, is a cutaneous condition that typically occurs as tender red plaques or nodules. However, atypical presentations may occur and, in our case, Sweet's syndrome masqueraded as facial cellulitis and soft tissue infections of the extremities in a sporotrichoid pattern. Despite treatment with broad-spectrum antibiotics, the cutaneous lesions progressed. Results of skin biopsy specimens of the facial plaque and a nodule on the right upper extremity were diagnostic of Sweet's syndrome. Simultaneous to diagnosis, the patient also was found to have acute myelogenous leukemia (AML).     

A Case of Neutrophilic Dermatosis of the Dorsal Hands with Concomitant Involvement of the Lips

Neutrophilic dermatosis of the dorsal hands (NDDH) is a localized, pustular variant of acute febrile neutrophilic dermatosis (Sweet syndrome). The lesions of NDDH clinically resemble those of Sweet syndrome (SS), but they differ from classic SS according to their locations (NDDH is predominantly restricted to the dorsal hands) and the smaller number of accompanying systemic symptoms. The histology of the NDDH lesion shows a dense dermal neutrophilic infiltration. The lesions rapidly resolve after systemic corticosteroid and/or dapsone therapy. We herein report on a case of neutrophilic dermatosis of the dorsal hands in a 34-year-old woman. The patient also had skin changes with erythematous plaque on the right lips.     

Association of Sweet's syndrome and acute sarcoidosis: report of a case and review of the literature

Sweet's syndrome (acute febrile neutrophilic dermatosis, SS) may be considered a reactional dermatosis with numerous associated diseases. We describe a rare association between SS and acute sarcoidosis in a 55-year-old woman. The atypical feature of positive tuberculin reaction is discussed. In this association, diagnosis may be challenging with coexistent skin lesions of both diseases. Acute sarcoidosis should be considered when SS is accompanied with mediastinal lymphadenopathy, uveitis or erythema nodosum. Deep-seated nodular lesions in the context of SS are not always 'deep' SS lesions.