Pretibial myxoedema (PTM) in its most extreme form clinically resembles lymphoedema. Studies with quantitative lymphoscintigraphy and fluorescence microlymphography in two patients with extensive PTM confirmed the presence of both functional and structural changes in the lymphatic system. We suggest that mucin deposition in the dermis causes compression of the dermal ...

During the past 15 years, the clinical spectrum associated with the anti-Ro(SS-A) antibody response has been defined. Various clinical presentations, including subacute cutaneous lupus erythematosus, the neonatal lupus syndrome, the Sjögren's syndrome/lupus erythematous overlap syndrome, and primary Sjögren's syndrome, have been detected in association with the anti-Ro(SS-A) response. The anti-Ro(SS-A) ...

A 27 year old woman with systemic lupus erythematosus (SLE) developed vertical diplopia with an apparent bilateral inferior oblique muscle palsy, resulting in a limitation of elevation of the globe in adduction. It resolved with systemic steroid treatment. A transient tenosynovitis affecting the superior oblique tendons was the probable underlying ...

We analyzed the cutaneous reactions to systemic analgesic-antipyretics and non-steroidal anti-inflammatory drugs reported to the spontaneous reporting system of the Gruppo Italiano Studi Epidemiologici in Dermatologia (GISED). The system has been active since 1988, with periodic intensive surveillance exercises, and 202 dermatologists have collaborated. Up to December 1991, 2,137 reactions ...

A 65-year-old woman with facial erythema and hypergammaglobulinemia developed excessive fatigability. A diagnosis of Lambert-Eaton myasthenic syndrome (LEMS) was made from electrophysiological studies. She had symptoms and laboratory data compatible with probable Sjögren's syndrome. Skin biopsy revealed the histological findings of discoid lupus erythematosus. Treatment with 3,4-diaminopyridine resulted in the ...

The pseudoexfoliation syndrome has recently been suggested to represent the local manifestation of a more widespread disorder. In this study, a case of classic bilateral pseudoexfoliation syndrome with systemic distribution of pseudoexfoliation material involving a variety of organ systems is described. Using transmission electron microscopy, typical pseudoexfoliation fibers were identified ...

We examined three patients from two families of Jewish-Iraqi origin who had progressive reduction of visual acuity and childhood onset of bilateral optic nerve atrophy without additional retinal abnormalities. They had neurologic symptoms compatible with Behr's syndrome. Neurologic signs included increased tendon reflexes, a positive Babinski sign, progressive spastic paraplegia, ...

OBJECTIVE: To report a case of cephalexin-induced Stevens-Johnson syndrome (SJS), a devastating adverse drug reaction that involves the entire skin surface and mucosal areas of the body. DATA SOURCES: MEDLINE search (key terms cephalosporins, Stevens-Johnson syndrome, erythema multiforme, and systemic lupus erythematosus) and references identified from bibliographies of pertinent articles. ...

We report on an infant with the Marden-Walker syndrome. In addition to the consistent neurological abnormalities described previously in this syndrome, the infant had a striking neurological constellation, absence of primitive reflexes, jerky eye movements, failure to habituate to repeated stimuli, inadequate behavior development, and absence of orientation responses to ...

Multiorgan thrombotic disorders have been described in a variety of conditions including systemic lupus erythematosus. Among these are the 'catastrophic' antiphospholipid syndrome, disseminated intravascular coagulation and the thrombotic thrombocytopenic purpura-haemolytic uraemic syndrome. In this review we briefly analyse some specific clinical and haematological characteristics of these conditions and attempt to ...

The case is reported of a 41 year old white woman who developed systemic lupus erythematosus (SLE) seven years after primary Sjögren's syndrome and four years after the association of Sjögren's syndrome with Jaccoud's arthritis. The SLE was detected by a benign intracranial hypertension, which is a rare neuro-ophthalmic presentation ...

We previously found that the Clq precipitin in sera from patients with hypocomplementemic urticarial vasculitis syndrome is an IgG autoantibody to Clq. We report here a prevalence study of this autoantibody in 162 patients with musculoskeletal or rheumatic diseases including hypocomplementemic urticarial vasculitis syndrome, systemic lupus erythematosus (SLE), and rheumatoid ...

POEMS syndrome [plasma cell dyscrasia with polyneuropathy, organomegaly, endocrinopathy, monoclonal (M) protein, skin changes] although mimicking the connective tissue diseases in several ways has not been reported in a patient who also meets criteria for the diagnosis of systemic lupus erythematosus (SLE). We describe a patient with POEMS syndrome and ...

PURPOSE: Patients with systemic capillary leak syndrome have a characteristic triad of hypotension, hemoconcentration, and monoclonal gammopathy. They have frequent and severe attacks of hemoconcentration and hypotension accompanied by marked plasma shifts. The exact role of this monoclonal protein is unknown, but it probably leads, in some way, to an ...

Anti-Ro autoantibodies are frequently found in the sera of patients with Sjögren's syndrome, systemic lupus erythematosus, and subacute cutaneous lupus erythematosus as well as in the sera of mothers of infants with the neonatal lupus syndrome. Close associations have been found between anti-Ro and a number of clinical manifestations, particularly ...

Takayasu's arteritis (TA) rarely coexists with systemic lupus erythematosus (SLE). Eighteen cases of TA associated with SLE found by worldwide literature search are reviewed, and an additional unique case is reported in association with the presence of anti-cardiolipin antibody. Patients with TA and/or SLE have similar age of onset and ...

A 47-year-old man began to suffer from progressive truncal ataxia and mental alterations typical of Wernicke-Korsakoff syndrome. He showed confusional state, hallucinations, delirium of jealousy and a serious impairment of recent memory. The symptomatology lasted 13 months, but only in the last weeks was it complicated by myoclonias. Triphasic pseudoperiodic ...

The term fasciitis-panniculitis syndrome (FPS) is proposed as a novel compilation encompassing several disorders, common to which is subcutaneous induration caused by cicatrizing fasciitis as well as septal and lobular panniculitis and perimysial fibrosis. Included herein are Shulman's eosinophilic fasciitis, morphea profunda, lupus profundus, venous lipodermatosclerosis, toxic oil syndrome, altered ...

We are reporting a young lady with protracted deep vein thrombosis of her left leg which turned out to be antiphospholipid (anticardiolipin) antibody syndrome of ANA positive systemic lupus erythematosus. Lupus anticoagulant was demonstrated by prolongation of activated partial thromboplastin time and Russell's viper venom time. She had no anti-thrombin ...

A 66-year-old man presented with massive bilateral scleral thickening and calcification associated with a unilateral exudative retinal detachment which did not respond to systemic anti-inflammatory agents including steroids and cyclophosphamide but improved with scleral resection. This patient shows the features of both posterior scleritis and the uveal effusion syndrome, providing ...

A 57-yr-old woman developed Crow-Fukase syndrome 18 yr after resection of plasmacytoma of the rib. Irradiation applied to the relapsed plasmacytoma and systemic chemotherapy alleviated symptoms and signs, but the tumor relapsed in the unirradiated cervical lymph node and she died of Pseudomonas pneumonia during chemotherapy 3 yr after diagnosis. ...

Patients addicted to alcohol and other psychoactive substances are frequently seen by family physicians. When admitted to the hospital, these patients may develop abstinence syndromes that require pharmacologic intervention. The pharmacologic management of acute abstinence syndrome from discontinuation of central nervous system depressants involves substitution of another sedative-hypnotic agent and ...

We have correlated the clinical and histopathologic features of the eyes and central nervous system in a patient with Hallervorden-Spatz syndrome who died at age 11 years. The main ocular findings included degeneration of photoreceptors, marked thinning of the outer nuclear and outer plexiform layers, retinal gliosis, narrowing and obliteration ...

We report here a new case of oculomasticatory syndrome related to systemic Whipple's disease. The patient presented typical ophthalmoparesis associated with ocular myorhythmia consisting of 1 Hz convergent oscillations of both eyes. The masticatory involvement was remarkable and consisted of a permanent bruxism leading to severe tooth abrasions. Possible pathophysiology ...

While the etiology of connective tissue diseases remains unknown, the classification of individual cases will continue to depend on identifying certain patterns of clinical and laboratory features. As many as 25% of connective tissue disease patients present with an overlap syndrome with features of systemic lupus erythematosus, systemic sclerosis, polymyositis, ...

Eclampsia is a syndrome that may be characterized by functional derangement of multiple organ systems including the central nervous system. Cerebral manifestations are rare in eclampsia but may include cerebral hemorrhage, blindness, and coma. Management of eclampsia involves the control of convulsions and hypertension as well as delivery. Cerebral manifestations ...

Paraneoplastic syndromes affect the nervous system in a very small percentage of patients with cancer. In those patients, portions of the nervous system are damaged or destroyed, usually in association with a small and often occult neoplasm elsewhere in the body. Current evidence suggests that these syndromes occur when the ...

Here we report a case of neonatal lupus erythematosus syndrome presenting with multisystem organ involvement, including anemia, thrombocytopenia, purpura, bloody diarrhea, enzymatic liver abnormalities, splenomegaly and pneumonitis. These findings preceded the cutaneous rash that was the clue for the diagnosis. The patient's mother had an undiagnosed subacute cutaneous lupus erythematosus. ...

Three cases of neonatal lupus erythematosus (NLE) syndrome are described. The key diagnostic sign is the erythematous and annular clinical appearance of lesions. The histopathologic findings may be subtle in NLE syndrome compared with discoid or subacute cutaneous lupus erythematosus. Direct immunofluorescence testing may be useful in supplementing the histopathologic ...

With recent advances in understanding of the organization, complexity, and function of the major histocompatibility complex and other genes involved in the immune response, a rational approach to interpreting associations of systemic lupus erythematosus and Sjögren's syndrome with these genes is now possible. For example, both systemic lupus erythematosus and ...

Central motor pathways conduction abnormalities after magnetic stimulation of the brain are reported in two siblings with Cockayne's syndrome. Reduced amplitude of the responses with large central conduction time were the main changes. These results are consistent with signs of diffuse white matter hypomyelination found on MRI. Conversely, only mild ...

Annular erythema developed in 22 patients with Sjögren syndrome. Clinically, the annular erythema was subdivided into three forms: Sweet disease-like annular erythema with an elevated border (14 cases); subacute cutaneous lupus erythematosus (SCLE)-like marginally scaled erythema (5 cases); and papular erythema (3 cases). Histopathologically, features commonly seen in annular erythema ...

We present a Burmese patient with widespread annular erythema associated with Sjögren's syndrome. Unlike previously described cases, the disease occurred in the setting of systemic lupus erythematosus. Photoprovocation testing revealed light sensitivity in the UVA range with elicitation of subacute cutaneous lupus erythematosus-like lesions. The presence of an erythema annulare ...

An iliac crest bone marrow aspiration in a 24-year-old man was followed by severe haemorrhage into the iliopsoas muscle. A lupus anticoagulant and severe hypoprothrombinaemia, as well as clinical and laboratory pointers to suggest the presence of a systemic lupus erythematosus-like syndrome, were demonstrated. Therapy with prednisone was commenced following ...

Many neurologic syndromes are named for literary characters. For example, the "Alice in Wonderland" syndrome of altered body perceptions, usually caused by migrainous ischemia, is so called because of the resemblance of its symptoms to the fluctuations in size and shape that plague the main character in Lewis Carroll's 1865 ...

The syndrome of inappropriate secretion of antidiuretic hormone (SIADH) has been reported rarely in systemic lupus erythematosus (SLE) with central nervous system disease. Previous case reports linking SIADH with lupus have either not documented clearly that SLE was the sole cause of the problem, or have not demonstrated a correlation ...

OBJECTIVE: To characterize an unusual mode of presentation of systemic lupus erythematosus: acute and severe pancytopenia related to reactive hemophagocytosis. DESIGN: Retrospective case series. SETTING: Two general community hospitals in Hong Kong. PATIENTS: Six patients presenting with a reactive hemophagocytic syndrome, identified over a 3.5 year period, diagnosed with systemic ...

Two young Akitas were examined because of manifestation of a juvenile-onset form of polyarthritis. A search of medical records at the New York State College of Veterinary Medicine found 6 more similarly affected Akitas. The clinical manifestations were marked by cyclic febrile illness and signs of profound joint-related pain. Two ...

A patient with Sjögren's syndrome and seronegative polyarthritis is reported. After piroxicam intake and sun exposure she developed subacute cutaneous lupus erythematosus lesions with Ro antibodies. Despite drug withdrawal, typical cutaneous lesions and serological markers of systemic lupus erythematosus (SLE) progressively appeared. The use of piroxicam and other nonsteroidal anti-inflammatory ...

Three patients with the chief complaints of dizziness while standing up or blackouts were diagnosed correctly as having the Shy-Drager syndrome at the Department of Otolaryngology. Both the electrocardiogram R-peak to R-peak interval and the Schellong test revealed disorders of the autonomic nervous system in each of these cases. Electronystagmography ...

Parotidectomy may be associated with a significant depression in the retromandibular region and a significant incidence of gustatory sweating (Frey's syndrome). Superiorly and inferiorly based sternocleidomastoid flaps and posterior plication of the superficial musculoaponeurotic system were evaluated for their ability to ameliorate both consequences. Sixteen patients with sternocleidomastoid flaps and ...

A 47-year-old woman with overlap scleroderma-polymyositis syndrome and positive circulating lupus anticoagulant developed scleroderma nephropathy, characterized by rapidly progressive renal failure caused by thrombotic microangiopathy with widespread thrombi in small arteries and glomeruli. The possible relationship between lupus anticoagulant and the development of thrombosis at the small renal vessels level ...

Recurrent chorea is described in a 61-year-old woman who had had chorea gravidarum when she was younger. The recurrent chorea appeared to be induced by a topical vaginal cream that contained conjugated estrogen. This case is consistent with the existence of a recurrent syndrome of hormone-induced chorea. The effect of ...

A 35-year-old female with pyoderma gangrenosum developed paraparesis with a sensory level at L1. Three months later she complained of diplopia and was found to have bilateral internuclear ophthalmoplegia with exotropia and no ocular convergence. The term Webino syndrome has been coined to design this set of neuro-ophthalmologic findings. Although ...

Muscle involvement was identified in 14 patients with scleroderma or a connective tissue disease overlap syndrome with predominant features of scleroderma. Patients presented with symmetrical proximal weakness indistinguishable from other inflammatory myopathies. Creatine kinase and electromyography were useful to demonstrate muscle involvement. Muscle histopathology demonstrated primarily the vasculopathy of scleroderma ...

The authors report a patient with Miller-Fisher syndrome in whom MRI of the brain stem showed increased signal density on T2 sequence anterior to the fourth ventricle, on the right and the left. The authors discuss the relation between these MRI abnormalities and some clinical features of the syndrome. The ...

Vogt-Koyanagi-Harada (VKH) syndrome represents a spectrum of bilateral panuveitis with associated central nervous system and dermatologic manifestations. The diagnosis is based on clinical and angiographic findings, but some of the characteristic features may be either absent or difficult to visualize in the presence of opaque media. With the use of ...

Three patients presented with septal fasciitis and panniculitis, associated with clinical and laboratory features which precluded straight-forward classification into eosinophilic fasciitis, localised scleroderma, or lupus erythematosus profundus. Treatment with cimetidine caused the remission of cutaneous manifestations and the extracutaneous abnormalities, such as nailfold capillary disturbances and the presence of antithyroid ...

The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. Patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute ...

As a result of follow-up studies published in 1937 and 1939, Langfeldt divided schizophrenia into two groups; 'typical schizophrenia' which had a poor outcome, and the 'schizophreniform psychoses' which had a less typical clinical picture of schizophrenia and a good outcome. Langfeldt's cases of schizophreniform psychoses were reclassified according to ...