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 patho-mechanisms involved in this fascinating disorder.     

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.     

Therapy of acute polyradiculoneuritis (Guillain-Barré syndrome)

The Guillain-Barre syndrome (GBS) is the most common cause of acute paralysis. It requires early institution of therapy to avoid life-threatening complications, to speed up recovery and minimize residual handicap. Advances in supportive care (treatment of infections, anticoagulation) and the introduction of intensive care (assisted ventilation, treatment of autonomic disturbances) have greatly improved the management of this disease and consequently its prognosis. A better understanding of the underlying immune mechanisms prompted pathogenetically oriented therapy. Plasma exchange and high dose intravenous immunoglobulin have been demonstrated in large controlled trials to hasten recovery in GBS. Problems of these studies are discussed and the presumed mode of action of these therapies is reviewed. Corticosteroids have shown no benefit. Ongoing trials are listed and future strategies for immunointervention are outlined.     

The acute phase reaction syndrome: the acute phase reactants (a review)

The acute phase reaction of the organism, which determines the response that follows the injury of tissues and organs and helps the survival of the individual is reviewed. The main participants, the induction of the reaction and the regulatory mechanisms are shortly discussed, with special respect to the prominent role of interleukin-6. The relationship between the production and synthesis of the acute phase reactants under physiological and pathophysiological conditions is analyzed. The particular functions of orosomucoid and alpha 2HS-glycoprotein are summarized. Finally, the common biological actions of IL-6 and the acute phase reactants are mentioned.     

Rescue therapy for the acute respiratory distress syndrome (ARDS)

The potential risk of feto-maternal haemorrhage following coelocentesis was examined in 17 singleton pregnancies at 6-11 weeks of gestation by measuring maternal serum concentration of alpha fetoprotein (AFP) before and 1 and 10 min after the procedure. There was no significant difference between the maternal serum AFP concentration before coelocentesis (median 7.5, range 4.5-21.5 IU) compared to the values at 1 min (median 8.6, range 3.9-17.8; Z = -0.504, P = 0.614), and 10 min (median 7.5, range 5.7-20.6; Z = -0.432, P = 0.666) after the procedure. These findings demonstrate that coelocentesis is not associated with significant feto-maternal haemorrhage.     

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).     

Ocular manifestation of subacute sclerosing panencephalitis (SSPE) (author's transl)

A case history of a patient with subacute sclerosing panencephalitis (SSPE) is presented with emphasis on the initial ocular manifestation of the disease. Acute necrotising retinitis was found clinically and proved histologically. Clinical and histological findings are compared with reports in the literature.     

Zinc-responsive dermatosis in northern-breed dogs: 17 cases (1990-1996)

In situ hybridization is performed on oligodendrocytes using oligo- nucleotide probes to determine the subcellular distribution of myelin basic protein (MBP) encoding messages which are associated with the cytoskeleton, relative to sub-cellular distribution of all MBP-encoding messages in the cells.     

Rhabdomyolysis and acute renal failure caused by haloperidol-decanoate (neuroleptic malignant syndrome)

A case of rhabomyolysis with attendant severe acute renal failure, arisen in a 59-year-old male treated with haloperidol-decanoate, is presented. The patient has been affected by paranoia schizophrenia since childhood, and he was treated with electroshock and successively with neuroleptics p.o. Four years before our observation, a therapy with haloperidol decanoate (50 mg i.m. monthly) was started. After some time, catatonic like episodes appeared, which got more and more frequent, until they appeared weekly. In occasion of the last of them, he was admitted to our hospital. At the objective examination he presented psychomotory arrest, perspiration, mytacism, severe muscle rigidity, moderate oedems to lower limbs. Laboratory findings showed a pattern consistent with rabdomyolysis and severe renal failure. After that haloperidol decanoate was stopped and rehydration and intensive diuretic therapy was started, the clinical and laboratory pattern went normal, persisting however a light creatinine increase. Probably the rhabdomyolysis was induced by the haloperidol decanoate, and renal failure by secondary severe hyvolemia. This case comes into the so-called neuroleptic malignant syndrome which can rarely arise in patients treated with antipsycotic agents and which causes high mortality, particularly when there are rhabdomyolysis and acute renal failure.     

Intracerebral hematoma as an acute manifestation of intracranial tumors

Sixteen patients with spontaneous intracerebral haematoma due to intracranial tumours are discussed. The total number of patients with intracranial tumour treated in the period concerned was 594, so the incidence of tumour haemorrhage was 2.7%. In most of the patients we found metastatic neoplasm (n = 6) and glioblastoma (n = 3). All patients were acute admissions. Seven were comatose, seven were somnolent, and two patients were alert. In ten patients the haemorrhage represented the first reliable clinical sign. In six patients a tumour disease was known. Fourteen patients were operated on. Six patients died. Extensive neuro-radiological examination is very important, particularly since 30% of these acute intracerebral haemorrhages occur in patients with benign intracranial tumours.