ACUTE FEBRILE NEUTROPHILIC DERMATOSIS (SWEET'S SYNDROME)

Background. Sweet's syndrome is well recognized and not infrequently diagnosed in Spain; however, the range of clinical and pathologic expression may not have been fully realized. Methods. We reviewed 30 consecutive Spanish cases of Sweet's syndrome diagnosed in our department from 1979 to 1990, with special attention to clinical and histopathologic findings. Results. Distinctive clinical features in our series included oral mucosa lesions in four patients (13%), development of pathergy phenomenon in one case, concurrent nodular lesions resembling erythema nodosum on the limbs in nine cases (30%), and lung involvement in two patients. Infectious disease and drug treatment were recorded as possible triggering factors of Sweet's syndrome in eight and seven patients respectively. Associated underlying systemic disorders were present in 15 (50%) of our patients. The most frequent associations were hematologic neoplasia in four patients, solid neoplasia in two, and chronic idiopathic inflammatory bowel disease in three patients. Dressler's syndrome and sicca syndrome were found in one patient each. Histopathologic studies of skin biopsy specimens obtained at presentation disclosed typical features of Sweet's syndrome in all cases. Epidermal involvement, with variable degrees of spongiosis, exocytosis of polymorphonuclear leukocytes and keratinocyte necrosis, was a prominent feature in 83% of biopsy specimens. Conclusions. Further characterization of the clinicopathologic spectrum of Sweet's syndrome is necessary as the recognition of the full spectrum of this syndrome will improve our diagnostic abilities and provide a solid clinical basis for prospective studies that allow dissection of the intricate pathomechanisms involved in this fascinating disorder.     

Unusual cutaneous manifestations of myelodysplastic syndrome

We report a 58-year-old patient with acute myeloid leukaemia who developed an oedematous erythema resembling Sweet's syndrome, accompanied by atypical erythema nodosum and bullous pyoderma gangrenosum. Examination of skin biopsies showed dense infiltration with mature neutrophils. although there was peripheral blood leucocytopenia. The oedematous erythema worsened after he was treated with granulocyte-colony-stimulating factor (G-CSF), which was given For his leucocytopenia. We suggest that when a neutrophilic dermatosis complicates leukaemia, alternatives to G-CSF should be considered for the treatment of leucocytopenia. Corticosteroids were very effective in controlling the skin lesions in our patient.     

Sweet's Syndrome with Small Cell Carcinoma of the Lung

We report a 59-year-old male with Sweet's syndrome who developed small cell carcinoma of the lung one year after the initial diagnosis. To the best of our knowledge, this is the first report of Sweet's syndrome in conjunction with lung cancer. In cases of Sweet's syndrome, a search for not only hematologic disorders but also for solid tumors should be made.     

Acute febrile neutrophilic dermatosis (Sweet''s syndrome) and adenocarcinoma of the rectum

Sweet's syndrome is a distinctive dermatosis that usually occurs as an idiopathic disorder. About 10% of cases have occurred in patients with myeloproliferative disease. Seven cases have been described with Sweet's syndrome in conjunction with a solid malignancy. We report a patient with Sweet's syndrome in association with an adenocarcinoma of the rectum. We believe that this syndrome may reflect a clinical manifestation of underlying myeloproliferative or solid malignancy. Sweet's syndrome is a characteristic dermatosis of acute onset, consisting of pseudovesicular, tender nodules and plaques, fever, arthritis and a neutrophilic leukocytosis. The associaton of Sweet's syndrome with myeloproliferative diseases has been reported several times. Acute myeloid leukaemia may occur in up to 10% of patients with Sweet's syndrome.^1,2 Reports of associations of the syndrome with non-myeloproliferative malignancies are uncommon. We report herein a patient with Sweet's syndrome and an adenocarcinoma of the rectum.     

Concurrence of Sweet's Syndrome,Pathergy Phenomenon and Erythema Nodosum-like Lesions

We report the case of a 54-year-old woman with concurrent Sweet''s syndrome, pathergy phenomenon and eythema nodosum-like lesions associated with suppurative tonsillitis. Tender, violaceous and highly edematous papules and plaques were detected on the forearms and legs, some of which were pseudovesicular. Similar edematous papules were detected on the dorsum of the hands at the sites of intravenous injection. Biopsies of a plaque on the forearm and a papule at the site of intravenous injection confirmed the presence of Sweet''s syndrome. Multiple, tender, violet-red, subcutaneous erythematous nodules were revealed on the legs. A biopsy taken from a nodule on the leg revealed septal panniculitis. To our knowledge, no patient with concurrent Sweet''s syndrome, pathergy phenomenon, and erythema nodosum-lesions had been reported yet in previous literature.     

Tumour necrosis factor-alpha promoter polymorphism in erythema nodosum.

Erythema nodosum is a common skin disease characterized by erythematous, tender subcutaneous nodules, mostly located on the lower extremities. Little is known about its pathogenesis, although a wide variety of aetiological factors (e.g. bacterial and viral infections, neoplastic diseases and drugs) have been described. Sarcoidosis, a typical granulomatous disease, often occurs in association with erythema nodosum (Loefgren syndrome). Since granulomatous diseases have been closely linked to a deregulated tumour necrosis factor (TNF)-alpha production, it was tempting to speculate whether TNF-alpha might play a role in the pathogenesis of erythema nodosum, at least in cases associated with sarcoidosis. A previously described nucleotide exchange, (G-A) at position -308 in the human TNF-alpha gene promoter, has been shown to be a major cause for enhanced TNF-alpha production. In the present report, we investigated the genomic TNF-alpha promoter region in patients suffering from EN with and without underlying sarcoidosis. Our results showed a strong correlation between the uncommon TNF A II allele and sarcoidosis-associated erythema nodosum. Patients with erythema nodosum without underlying sarcoidosis displayed a similar allele frequency compared with controls. Taken together, we provide evidence that erythema nodosum in association with sarcoidosis might be pathogenically linked to altered TNF-alpha production due to a genetic promoter polymorphism.     

Drug-induced Sweet's syndrome (acute febrile neutrophilic dermatosis) associated with hydralazine

We report a case of Sweet's syndrome associated with hydralazine. The association of Sweet's syndrome with hydralazine, and with the oral contraceptive, minocycline, and trimethoprim/ sulphamethoxazole, has been reported previously. We suggest that a drug aetiology should be sought in cases of Sweet's syndrome.     

TREATMENT OF ERYTHEMATOUS DERMATOSES WITH POTASSIUM IODIDE

Forty-seven patients with various erythematous dermatoses were treated with systemic administration of potassium iodide. There were fifteen patients with erythema nodosum, ten with nodular vasculitis, fourteen with erythema multiforme, and eight with Sweet's syndrome. Potassium iodide was dramatically effective in a substantial number of cases. In responsive patients, improvement of the cutaneous changes was evident within a few days. The effect of the drug was impressive in patients with lesional tenderness, joint pains, and/or high fever. Relief of these subjective symptoms occurred within twenty-four hours. The possible mode of the action of potassium iodide and pathogenesis of erythematous dermatoses were also discussed.     

Skin gangrene as an extraintestinal manifestation of inflammatory bowel disease

Inflammatory bowel diseases can commonly present many cutaneous lesions which can contribute to the diagnosis of the disease or its activity. The most frequent cutaneous or mucocutaneous manifestations suggesting ulcerative rectocolitis activity are erythema nodosum (3-10%), pyoderma gangrenosum (5-12%) and aphthous stomatitis (4%). Other reactive skin manifestations related to immunological mechanisms associated with the inflammatory bowel disease are: Sweet''s syndrome, arthritis-dermatitis syndrome associated with inflammatory bowel disease and leukocytoclastic vasculitis. We describe the case of a young man with diagnosis of ulcerative rectocolitis, which presented an extensive cutaneous gangrene secondary to microvascular thrombosis. The case represents a dermatologic rarity and should be recognized as a cutaneous manifestation related to the hypercoagulability state observed in the disease''s activity.     

Subcutaneous histiocytoid Sweet's syndrome in a patient with myelodysplastic syndrome and acute myeloblastic leukemia

Subcutaneous histiocytoid Sweet's syndrome is a rare variant of histiocytoid Sweet's syndrome (SS). We present a 68‐year‐old woman with subcutaneous histiocytoid SS in association with refractory myelodysplastic syndrome transformed to acute myeloblastic leukemia (AML), status post induction chemotherapy and with persistent blasts (50%) in the bone marrow and blood, accompanied with neutropenia. The patient presented to the emergency room with fever and altered mental status. Clinical examination revealed approximately 20 scattered 0.5–2 cm, pink to pink‐purple non‐tender firm nodules on the legs and left arm. The differential diagnosis included Sweet's syndrome (deep), leukemia cutis, infection, polyarteritis nodosa and erythema nodosum. Histopathologic examination of a biopsy from the left arm revealed a nodular infiltrate of neutrophils and histiocytoid mononuclear cells solely in the lobular compartment of the subcutaneous fat with focal areas of necrosis. Most cells in the infiltrate labeled with myeloperoxidase (MPO) including the histiocytoid cells. The cells were negative for CD34 and CD117. All special stains for microorganisms were negative. A diagnosis of subcutaneous histiocytoid SS was made. A subcutaneous histiocytoid SS should be suspected when a neutrophilic/histiocytoid panniculitis, occurring in the setting of myeloid disorders, is encountered and after exclusion of an infectious process and leukemia cutis.     

Cutaneous sarcoidosis

We report on three clinical cases of cutaneous sarcoidosis. One of them was associated with erythema nodosum, another with erythema annulare centrifugum. The third case showed an annular lesion having persisted for 10 years; X-ray of the lungs revealed stage 3 disease.     

Inflammatory Nodules of the Lower Legs: A Clinical and Histological Analysis of 134 Cases in Korea

We analysed 134 Korean cases with inflammatory nodules of the lower legs on the basis of clinicopathological findings, responsiveness to various therapeutic agents, and clinical course. There were 53 cases of erythema induratum (EI), 18 of erythema nodosum (EN), 40 of EN-like lesions of Behçet's disease, 15 of other entities, including superficial migratory thrombophlebitis, cutaneous periarteritis nodosa, sarcoidosis, malignant lymphoma, Churg-Strauss syndrome, and parasitosis, and eight unclassified cases. The unclassified group was composed of a spectrum of diseases with clinicopathologic features ranging between those typical of EN and EI. The present study revealed that the profiles of diseases featuring inflammatory nodules of the lower legs in Korea differed from those found in other areas. These geographic and demographic differences should be kept in mind when managing a patient with inflammatory nodules of the lower legs.     

Sweet's syndrome in association with non-Hodgkin's lymphoma

Sweet's syndrome is an acute neutrophilic dermatosis frequently found in association with other conditions, particularly inflammatory and neoplastic disease. We report here a patient who developed the condition 2 years after the diagnosis of non-Hodgkin's lymphoma (NHL), in this case affecting: a tonsil, the thirteenth report of such an association. We discuss the diagnosis, investigation and management of Sweet's syndrome in the context of the current case.     

Löfgren's syndrome presenting with erythema nodosum-like eruption

L?fgren's syndrome is an acute form of sarcoidosis that mostly presents with erythema nodosum, periarticular ankle inflammation, arthritis, conjunctivitis, and unilateral or bilateral hilar or right paratracheal lymphadenopathy. Erythema nodosum is the most common cutaneous lesion in acute sarcoidosis, but it is histologically nonspecific, as it does not reveal sarcoidal granulomas upon microscopic examination. We present a case of a 57-year-old woman with acute onset of tender erythematous nodules on the calves, which were clinically erythema nodosum-like lesions. Histologic analysis revealed sarcoidal granulomas located in the dermis and subcutaneous tissue. Upon further work-up, the patient was found to have both pulmonary and ocular sarcoidosis. Based on this case, we recommend that all patients with erythema nodosum-like lesions undergo biopsy and further work-up for sarcoidosis is recommended in case of biopsy proven sarcoidal granulomas.     

Behçet''s disease with Sweet''s syndrome-like presentation—a report of six cases

We describe six cases of Behcet's disease, which showed Sweet's syndrome-like features. The clinical findings in our patients closely resemble those of the cases described in previous reports on Sweet's syndrome. However, oral and genital mucosal involvement, non-tenderness of the lesions and a pustular eruption elsewhere made us consider Behcet's disease. Besides the clinical findings, vasculitis was clearly demonstrated in the skin biopsy specimens.     

Sweet's syndrome—therapy with cyclosporin

We report the case of a 39-year-old female patient suffering from Sweet's syndrome after an upper respiratory tract infection. Cyclosporin A at a dose of 10 mg/kg per day was given as initial treatment. Skin lesions and general malaise resolved within 9 days. The cyclosporin dose was decreased within 21 days, without recurrence of the eruption. Cyclosporin is a potent inhibitor of T lymphocytes, but affects granulocyte and monocyte functions as well. Success of treatment in our case shows that cyclosporin represents an alternative to steroid treatment in patients with Sweet's syndrome.     

SWEET'S SYNDROME: CLINICOPATHOLOGIC STUDY OF ELEVEN CASES

Background. The clinicopathologic manifestations of Sweet's syndrome associated with malignancy have been rarely studied in a large series. Methods. We describe the clinical features of Sweet's syndrome in 11 patients seen during the past 12 years. Histopathologic findings of these patients are discussed. The clinicopathologic manifestations of 249 cases of Sweet's syndrome reported in the literature were reviewed. Results. The clinicopathologic manifestations of Sweet's syndrome associated with hematologic disorders (HSS) were more severe and characterized by bullous pyoderma with more pronounced epidermal changes and ulceration, anemia, giant platelets and abnormal platelet counts. Approximately 40% of the Sweet's syndrome reported in the literature were associated with hematologic malignancy, and the other 7% of patients had solid tumors. Conclusions. Sweet's syndrome can be classified into three types: (1) association with hematologic disorders (HSS); (2) association with solid tumors (SSS); and (3) idiopathic or associated with various other disorders (ISS).     

Sweet's syndrome associated with Behçet's disease

A case of acute febrile neutrophilic dermatosis (Sweet's syndrome) associated with Behçet's disease occurring in a 48 year old woman is reported. She fulfilled the diagnostic criteria for both Sweet's syndrome and Behçet's disease. There have been several reports of this association. Herpes simplex virus was isolated from a genital ulcer in our patient. The possible role of herpes simplex virus in this interesting association of Behçet's disease and Sweet's syndrome is discussed and a review of the literature is made.     

Acute Febrile Neutrophilic Dermatosis (Sweet's Syndrome) Associated with Extreme Infiltration of Eosinophils

A remarkable infiltration of eosinophils was observed in skin biopsy specimens in two cases of Sweet's syndrome. One patient was a 29-year-old woman, whose clinical and histological features included associated asthma, a prior respiratory tract infection, red plaques, blood eosinophilia, and a dense dermal infiltrate of neutrophils and eosinophils, without evidence of vasculitis. The other patient was a 61-year-old man characterized by a fever, red plaques, erythema nodosum-like lesions, hepatic dysfunction, a dermal infiltrate of neutrophils accompanied by eosinophils, and a subcutaneous prominent infiltrate of eosinophils. Clinical and histological evidence supported the diagnosis of Sweet's syndrome in both of these patients. While the literature describes eosinophil infiltration in this disease, extreme eosinophil infiltration could be misleading in the diagnosis.     

Azathioprine‐induced Sweet's syndrome: A case series and review of the literature

We present three patients with azathioprine‐induced Sweet's syndrome (AISS) who attended our tertiary institution within a 12‐month period. Established associations exist between Sweet's syndrome and some medications; however, to date links to azathioprine are tentative. While there are case reports of AISS, most have occurred in patients with inflammatory bowel disease (IBD), an underlying predisposition for Sweet's syndrome. Our case series adds to the evidence that the entity of AISS truly exists independent of confounding factors such as concurrent IBD.