Bronchial manifestation of acute febrile neutrophilic dermatosis (Sweet''s syndrome)

Eighteen isolates of Blastomyces dermatitidis were evaluated for their in vitro susceptibilities to ketoconazole, itraconazole, and fluconazole. The MIC ranges were 0.1 to 0.4 microg/ml for ketoconazole, < or =0.018 to 0.07 microg/ml for itraconazole, and 2.5 to 4.0 microg/ml for fluconazole. The ranges for the minimal lethal concentrations were 0.2 to 0.8 microg/ml for ketoconazole, < or =0.018 to 0.07 microg/ml for itraconazole, and 10 to 40 microg/ml for fluconazole. Itraconazole was the most active agent against B. dermatitidis in vitro, while fluconazole was the least active. These results correlate with the clinical efficacies noted to date with doses of these agents used to treat blastomycosis.     

Chronic bullous dermatosis in childhood (linear IgA dermatosis)

A 5-year-old boy presented with disseminated, partly grouped blisters indicative of chronic bullous dermatosis of childhood (CBDC) following a gastrointestinal infection 2 weeks earlier. CBDC has long been differentiated from adult linear IgA disease. Clinical and laboratory studies revealed substantial clinical and immunological overlap between the two blistering disorders, whereas recent investigations suggest heterogeneity of the target antigen involved. Pathohistological and immunofluorescence-microscopical characteristics of a subepidermal blister and linear IgA and granular C3 deposition at the basement membrane together with the typical history and clinical signs were decisive in the differential diagnosis. The disease promptly cleared up after daily administration of 16 mg methylprednisolone-21-acetate tapering and 25 mg dapsone. Immunohistological detection of collagen IV at the base of a blister made it possible to localize the split above the lamina densa. The demonstration of collagen IV stresses the importance of immunodermatopathology in the differential diagnosis of subepidermal blistering diseases.     

Pregnancy-associated Sweet's syndrome: world literature review

Sweet's syndrome (acute febrile neutrophilic dermatosis) is a steroid-responsive dermatosis characterized by pyrexia, neutrophilia, and painful erythematous plaques that histologically show a dense dermal infiltrate of neutrophils. Pregnancy-associated Sweet's syndrome is defined as the initial appearance or recurrent episode of Sweet's syndrome in a pregnant woman. Sweet's syndrome occurring during pregnancy has only been described in four women. The clinical characteristics of the women with pregnancy-associated Sweet's syndrome are reviewed and the differential diagnosis of pregnancy-associated dermatoses that clinically mimic Sweet's syndrome are discussed.     

Acute interstitial pneumonia (Hamman Rich syndrome)

We report the case of a 65-year-old woman with no history of respiratory disease who suffered onset of dyspnea after an episode of pseudoinfluenza. Dyspnea progressed such that within 15 days it was triggered by minimal effort. The patient died 15 hours after admission to our hospital, with a clinical picture of adult respiratory distress. Autopsy allowed us to rule out several diseases and arrive at a diagnosis of acute interstitial pneumonia, consistent with clinical course, anatomical and pathological findings as described in the literature.     

Nonsecretory IgA1 autoantibodies targeting desmosomal component desmoglein 3 in intraepidermal neutrophilic IgA dermatosis

Intraepidermal neutrophilic IgA dermatosis, a rare skin disease entity manifested with blisters and pustules clinically and lower epidermal blister, acantholysis, and neutrophilic infiltration pathologically, was first reported in 1985. Although the disease is characterized by IgA autoantibodies targeting the epithelial cell surface component, the target antigen has not been determined. We investigated a patient with this disease by histopathology, direct and indirect immunofluorescence, immunoblotting, and immunoadsorption studies. The pustular lesion was characterized by blister at the lower epidermis, acantholysis, and neutrophilic infiltration. Nonsecretory IgA1 subclass autoantibodies targeting the lower epithelial cell surfaces were detected in the patient's skin and serum. The patient's IgA autoantibodies labeled a recombinant desmosomal protein desmoglein 3 on immunoblotting and the immunolabeling of epithelial cell surfaces was eliminated by preadsorption with desmoglein 3. Thus, desmoglein 3 is identified as a target antigen in intraepidermal neutrophilic IgA dermatosis. The ability of IgA1 autoantibodies to bind neutrophils may be responsible for the prominent neutrophilic infiltration observed histopathologically and for the pustular lesions observed clinically.     

Neutrophilic dermatoses: pyoderma gangrenosum and Sweet's syndrome

Pyoderma gangrenosum and Sweet's syndrome are classified as neutrophilic dermatoses as they exhibit intense dermal inflammatory infiltrates composed of neutrophils with little evidence of a primary vasculitis. They share several characteristics and respond to immunosuppressives. Aetiology is felt to represent a manifestation of altered immunologic reactivity. Patients with both conditions concurrently have been described. Diagnosis is based on clinical and histopathological findings. However, clinically the typical forms of the two conditions are quite distinct: pyoderma showing cutaneous ulceration with a purple undermined border and Sweet's syndrome having tender, erythematous, nonulcerated plaques and nodules. Approximately 50% of cases of pyoderma are associated with a specific systemic disorder. These include inflammatory bowel disease, rheumatoid arthritis, non-Hodgkin's lymphoma and myeloproliferative disorders. Many associations with Sweet's syndrome have been described, including acute myeloid leukaemia, myeloma and adenocarcinomas, and haematological malignancy. There is overlap between the two conditions with lesions categorised as Sweet's syndrome being clinically more characteristic of atypical pyoderma and vice versa. We believe that pyoderma and Sweet's syndrome represent a continuum of spectrum of disease. The reason for the clinical differences between the conditions is unclear and merits further investigation but may be explained by varying levels of intensity and extent of the inflammatory process. This review will describe the pathogenesis, clinical features, diagnosis, associations and treatment of the two conditions.     

Sweet's syndrome with arthritis in an 8-month-old boy

Sweet's syndrome was diagnosed in a 4-month-old boy. He was successfully treated with systemic corticosteroids. At the age of 8 months, he developed acute arthritis in his right knee. The synovial fluid was analyzed and revealed a very high neutrophil count and neutrophil activation with a detectable level of intraarticular granulocyte-monocyte colony stimulating factor (GM-CSF). Prednisone injection into the knee led to dramatic improvement. No recurrence occurred. Although arthritis and/or arthralgia are common features in adult patients with Sweet's syndrome, this is the first reported case of Sweet's arthritis in a child.     

Arthritis with an inflammatory dermatosis resembling Sweet's syndrome. Report of a unique case and review of the literature on arthritis associated with the inflammatory dermatoses

A patient with a unique case of chronic episodic arthritis coincident with flares of acneform, pustular, nodular and ulcerating skin lesions was observed over a five-year period. This patient and a review of the literature on arthritis associated with the inflammatory dermatoses provide evidence which may interrelate several of these nosologically confusing skin conditions, e.g., the family of leukocytoclastic angiitides with the newly posited acute febrile neutrophilic dermatosis of Sweet. Systemic manifestations and a variety of acneform, pustular, nodular and ulcerating cutaneous lesions in the inflammatory dermatoses are best explained by small vessel involvement, with individual syndromes being determined by the type and degree of vascular change. Perivascular neutrophilic infiltration is the unifying histologic feature of these small vessel diseases. Neutrophil infiltration differentiates these entities, and our patient, from the histologically nonspecific inflammations of the skin, e.g., Behcet's disease and pyoderma gangrenosum, which, although capable of causing identically appearing skin lesions, consist predominantly of lymphocytic dermal infiltrates even in the earlier stages. It appears important to recognized these morphologically varied acute inflammatory dermatoses with perivascular neutrophilic infiltration in view of their systemic features and the dramatic efficacy of corticosteroid therapy.     

Relationship between autoantibody and dermatosis in myelodysplastic syndrome

We analyzed the relationship between autoantibody and dermatosis in 22 patients with myelodysplastic syndrome (MDS). These MDS patients consisted of five cases with refractory anemia (RA), three RA with ringed sideroblasts (RARS), eight RA with excess of blasts (RAEB), four RAEB in transformation (RAEB-t), and two chronic myelomonocytic leukemia (CMMoL) according to the FAB classification of MDS. The autoantibody was detected in seven patients, of whom four had rheumatoid factor (RF) and three had antinuclear antibody (ANA). Neither RF-positive nor ANA-positive MDS patients had other autoantibodies. Dermatosis was observed in nine cases of these 22 MDS patients. Five of 7 MDS patients (71%) with autoantibody developed dermatosis in their clinical course, as did four of 15 MDS patients (27%) without autoantibody. All four MDS patients with RF had dermatosis such as anaphylactoid purpura, xerotic dermatitis, thrombophlebitis, ephelides, and genital herpes. One of three MDS patients with ANA had pruritus senilis. The four MDS patients without autoantibody had dermatosis such as erythema nodosum, ichthyosis vulgaris, Sweet syndrome, and thrombophlebitis. Three of four MDS patients with RF had normal liver function tests, while three MDS patients with ANA showed liver dysfunction. Our studies presented here suggested that the dermatosis could develop frequently in MDS patients with autoantibody and that RF was closely related to development of dermatosis in MDS patients, although the dermatosis is not specially fixed.     

Acute respiratory distress syndrome (ARDS) in neonates and children

ARDS remains a syndrome which despite all efforts poses problems in exact definition (cause, course and severity). Most of the existing information comes from clinical observations and uncontrolled studies and is therefore of limited value. Despite the advent of new treatment modalities mortality from ARDS has remained high and is influenced or caused by several factors like underlying disease, previous health status, presence of MOSF, complications of therapy or ultimate failure of gas exchange. Therapy is directed at elimination of the cause of ARDS if possible, but then mainly supportive, considering all organs and systems. With the introduction of gentler respiratory support techniques (small tidal volumes and pressure limitation, permissive hypercapnia and HFO) and appropriate measures to reduce oxygen toxicity (titration of PEEP, possibly NO), iatrogenic lung injury, indistinguishable from ARDS, can be reduced, and this might improve survival rates. For the future, modulation of the host's inflammatory response may hold great promises for prevention and treatment of ARDS, but such strategies need to be explored with well controlled clinical trials, respecting the complexity of the issue.     

Sweet's syndrome associated with spinal surgical intervention. A case report

STUDY DESIGN: A patient with a medical history of Sweet's Syndrome, an acute neutrophilic dermatosis, was seen at the authors' institution for cervical pain. After undergoing a thorough history-taking and physical examination and after experiencing no relief with conservative therapy, the patient underwent cervical spine surgery. After the surgical procedure, the patient developed multiple cutaneous lesions that were consistent with the findings associated with an acute recurrence of Sweet's Syndrome. OBJECTIVES: To characterize the authors' experience with this unusual histologically documented dermatologic disorder. SUMMARY OF BACKGROUND DATA: Sweet's Syndrome is a rare form of neutrophilic dermatosis characterized by recurrent eruptions of painful, edematous, red, tender plaques that are found predominantly on the torso in middle-aged women. After an extensive literature search, it was noted that this rare and unusual disorder has not been reported previously in association with surgical intervention of any type, including spinal operations. METHODS: The patient's postoperative course was documented, and all medical records were reviewed retrospectively. RESULTS: The patient's rash resolved spontaneously. Solid fusion of C5-C6 occurred. The patient remained neurovascularly intact, and her axial cervical pain decreased significantly from its preoperative levels. CONCLUSIONS: Sweet's Syndrome remains a rare dermatologic disorder, which may complicate a routine postoperative course. Patients with Sweet's Syndrome have an exceedingly high rate of other serious medical illness. The effect of Sweet's Syndrome on physiologic bone healing is unknown. In this patient, there was nonunion of the cervical spine, with eventual solid bony union. Perioperatively, patients with this disorder are treated with oral prednisone and oral antibiotics to prevent secondary complications at the surgical wound.     

Sweet's syndrome. Presentation of six cases and review of the literature

The acute febrile neutrophilic dermatosis or Sweet syndrome, initially described in 1964 by Robert Sweet (1). It is characterized fever, neutrophilic leucocytosis, abrupt appearance of erythematous, painful, cutaneous plaquets and dense dermal infiltrate consisting of mature neutrophils without vasculitis sings. Malignancy has been described in the 10-15% of the reported cases. We report our series of 6 patients diagnosticated of this illness in our department. One of this patients has Sweet syndrome associated with a malignancy disorder. All of them had diagnostic criteria of the described disease and had good response to corticotherapy. We also report a bibliographic review of this infrequent syndrome.     

Acute pseudo-obstruction of the colon (Ogilvie's syndrome): advances in management

The management of the patients with acute pseudo-obstruction of the colon (APOC) still represents a matter of debate. To better evaluate and compare the effectiveness of various therapeutic approaches in the management of APOC 29 patients were considered. These were included according to three consecutive periods in: group A (1977-1982) concerning patients who underwent medical treatment alone (n = 8) or endoscopic (n = 4) and surgical (n = 1) decompression; group B (1983-1990) in which the management was based on simple endoscopic decompression (n = 10); group C (1991-1995) including patients in whom placement, under fluoroscopic control, of a tube in the cecum following endoscopic decompression was provided (n = 6). Mean time required for resolution of colonic distension was 2.3 (+/- 0.50 SD) days in patients who underwent endoscopic decompression and tube placement, as compared to 4.5 (+/- 2.47 SD) days in the group of patients treated either with conservative measures or simple endoscopic decompression (p = 0.04). No recurrence occurred after colonoscopic decompression and tube placement while colonic distension recurred in 4 of 14 patients managed by simple endoscopic decompression (0% vs. 28.6%, n.s.). Our experience showed that endoscopic decompression is an effective method, moreover if associated with the placement of an indwelling tube into the right colon. This method, for its easiness and safeness, besides its effectiveness in preventing the recurrence of colonic distension, may be surely considered an advance in the management of acute pseudo-obstruction of the colon.     

CHILD syndrome. Case report of a rare genetic dermatosis

We report on a 2-month-old girl who developed unilateral ichthyosiform erythrodermia on the right side of her trunk and the right leg 1 week after birth. In addition, there was hexadactyly of her right hand. Apart from that, close physical examinations did not reveal any other defects. We diagnosed this condition as CHILD syndrome (congenital hemidysplasia with ichthyosiform nevus and limb defects).