A rare case of systemic lupus erythematosus (SLE), with massive bilateral pleural effusions as the first manifestation, is described. The patient was a previously healthy 20-year-old soldier. Initial investigations were unrevealing, but after 3 months the patient developed the full-blown syndrome. He responded well to corticosteroids and cyclophosphamide with resolution ...

A patient with systemic lupus erythematosus who presented with subcutaneous nodules over the flexor aspect of the fingers in association with arthritis and Raynaud's phenomenon is described. Histopathological examination of the nodules showed appearances consistent with rheumatoid nodules. Further investigations revealed leucopenia and circulating anti-nuclear antibody but negative rheumatoid factor. ...

The Syndrome of Inappropriate Secretion of Antidiuretic Hormone (ADH) or SIADH has been reported in a great variety of diseases and disorders of the central nervous system. However, to our knowledge only three cases [Kaplan et al., Decaux et al. 1981, Agus et al. 1983] of SIADH associated with systemic ...

A case of myelofibrosis in association with systemic lupus erythematosus (SLE) is reported. Acute thrombocytopenia and a bleeding tendency developed in a 24-year-old woman with SLE. Bone marrow aspiration was unsuccessful due to myelofibrosis. Pulse therapy with methylprednisolone reversed both thrombocytopenia and myelofibrosis. A review of the literature revealed that ...

We present the case of a 26-year-old Caucasian woman with systemic lupus erythematosus (SLE) of 9 years duration, mainly involving the kidney. Her clinical course was complicated in the last year by several infectious and noninfectious events, and after pancytopenia, fever, and extremely high levels of lactate dehydrogenase developed, the ...

Sarcoidosis resolves spontaneously in many cases on a long-term basis. However, there are many cases whose resolution is delayed. We have experienced a frequent association of chronic tonsillitis (CTL), and dental caries in those patients of persistent course. We hypothesized that the presence of chronic or recurrent inflammatory foci in ...

Nodular regenerative hyperplasia (NRH) of the liver is an uncommon pathologic finding associated, in most cases, with rheumatic and hematologic diseases. Although its pathogenesis remains unclear, NRH probably results from liver regeneration to maintain its functional capacity after ischemia-induced injury. An intrahepatic microvascular occlusive mechanism has been considered most likely ...

Multicentric reticulohistiocytosis is a rare multisystem disorder that reflects a reactive inflammatory response to an undetermined stimulus. While the disease is characterized as a dermatoarthritis, multiple organ systems including cardiac and skeletal muscle, the pleura and gastrointestinal tract have been involved in reported cases. The synovitis can be quite destructive ...

The facial nerve is commonly involved in neural sarcoidosis, but there is only one reported case in which bilateral facial paralysis was the sole manifestation of sarcoidosis. We report a case of sarcoidosis presenting with bilateral facial paralysis as the only sign. The pathology, clinical presentation, pathophysiology and diagnosis of ...

We report the case of a female patient with necrobiosis lipoidica of the lower legs and coexistent systemic and cutaneous sarcoidosis. We review the six previously reported patients with coexisting necrobiosis lipoidica and sarcoidosis. The associations between the granulomatous disorders of the skin, especially necrobiosis lipoidica, sarcoidosis and granuloma annulare, ...

Acute symmetric demyelinating polyneuropathy of the Guillain-Barré type is known in systemic lupus erythematosus (SLE). Chronic idiopathic demyelinating polyneuropathy (CIDP) has been reported rarely with SLE. A case is reported of CIDP accompanying SLE with autoantibodies against GM1- and GM3-gangliosides. There was no historical evidence to suggest SLE, and CIDP ...

Pyoderma gangrenosum in a 35-year-old woman with long-standing systemic lupus erythematosus was treated with pulse steroid therapy. Significant improvement in the ulceration and control of the lupus erythematosus occurred. The unusual association of these disorders is reviewed, and the efficacy of pulse therapy in the treatment of refractory cases of ...

The Budd-Chiari syndrome (BCS) was diagnosed in a 30-year-old male hospitalized with hepatomegaly, abdominal collateral vessels and hepatic veins and inferior vena cava thrombosis (IVC) in 1988. The presence of circulating lupus anticoagulant (LAC) was suspected and demonstrated on this occasion in view of an earlier diagnosis of systemic lupus ...

Lymphocytic hypophysitis is one of the causes of hypopituitarism in the postpartum period. Some of these patients have been reported to have other organ-specific autoimmune diseases, and the disease is considered to be autoimmune in nature. The authors describe a unique case of lymphocytic hypophysitis in a young woman. She ...

Pemphigus vulgaris is extremely rare in childhood. Only 35 cases of juvenile pemphigus vulgaris have been reported in the literature up until to now. During the pre-steroid era, the outcome of the disease was usually regarded as fatal. Today, the prognosis has improved because of the possibility of early diagnosis ...

The marrow findings of 23 lupus patients with pancytopenia were reported. The most common findings were dyserythropoiesis and hypoplasia, both occurring in 9/23 (39%) of the cases. Neither feature was definitely related to cytotoxic drug therapy since most cases were treated by steroids only. Three of the hypoplastic marrows also ...

A 33-yr-old black female with systemic sarcoidosis developed chylous ascites. A CT scan showed extensive mesenteric lymphadenopathy. At laparotomy, the mesenteric and para-aortic lymph nodes were enlarged and diffusely matted. Histologically, normal lymph node morphology was virtually replaced with noncaseating granulomas. Corticosteroids and dietary manipulations were unsuccessful in managing the ...

A 13 year old girl presented with miliary tuberculosis and active systemic lupus erythematosus (S.L.E.). She responded to a combination of antituberculous drugs and systemic steroids. This case illustrates the fact that S.L.E. presenting in childhood may be rare but not unknown and exemplifies the need for vigilance in detecting ...

We report a 49-year-old woman who developed lobular glomerulonephritis with prevalent deposition of material positive for IgG, C1q and lambda light chain, but which was not stained by Congo red. Glomeruli revealed massive electron-dense deposits with a microlamellar structure in the mesangial matrix and peripheral capillary loops. Clinically, the patient ...

A patient with inactive systemic lupus erythematosus was successfully treated for pneumococcal sepsis complicated by disseminated intravascular coagulation, shock, renal failure, and functional asplenia. Functional asplenia was diagnosed from the total absence of uptake of intravenously administered 99mtechnetium-labeled sulfur colloid. Ten similar cases of functional asplenia occurring in patients with ...

Sarcoidosis is a relatively uncommon disease, seldom seen by ear, nose and throat departments. There are interesting head and neck and, more specifically, nasal manifestations. A patient with sarcoidosis presented at our department with nasal obstruction and deformity. Although these were the predominant features of the disease, the diagnosis was ...

The occurrence of sarcoidosis in more than one member in the same family is a fact that has been noted by many authors. It is now recognized as Familial Sarcoidosis, and nearly 450 cases have been reported in the world literature. Included in this figure are consaguineal cases as well ...

A case of segmental neurofibromatosis (SNF) in an octogenarian is presented. The patient's lesions were first noted when he was 74 years of age. This variant of neurofibromatosis (Riccardi's classification, NF-V) is most typically seen in childhood or young adulthood. To the best of our knowledge, our patient's SNF is ...

We report a case of endogenous Nocardia endophthalmitis in a patient with systemic lupus erythematosus (SLE). He developed a parafoveal lesion in the right fundus while on systemic corticosteroid and antibiotic treatment. Initially we suspected a fungal origin and treated him with antifungal drugs. The intraocular disease progressed without improvement ...

This is the second case report of hydralazine induced Sweet's syndrome and lupus erythematosus. Our patient had the characteristic rash of Sweet's syndrome, confirmed histopathologically, as well as laboratory features consistent with drug induced lupus, including positive serum antinuclear and antihistone antibody tests. The association between these 2 clinical entities ...

The authors report a case of eosinophilic fasciitis with an histopathological pattern of inflammatory neutrophilic vasculitis associated with the typical inflammatory infiltrate. The presence of this type of vasculitis which may be observed in the initial forms of scleroderma supports the hypothesis of a considerable overlap between these two entities.

Fifty-eight cases of septal granulomatous panniculitis were reviewed; 14 cases were diagnosed as erythema nodosum migrans (migratory panniculitis) and 36 as chronic erythema nodosum on the basis of clinical and histopathologic features. Erythema nodosum migrans was characterized by markedly thickened and fibrotic septae, marked capillary proliferation (like granulation tissue), and ...

Primary anetoderma (PA) has occasionally been described in association with lupus erythematosus (LE). The present study was performed to elucidate a possible causal link between PA and LE by the use of direct and indirect immunofluorescence (IF) methods. Two patients with PA were studied. Biopsy specimens were obtained from early ...

Patients with systemic lupus erythematosus (SLE) appear to be at increased risk for development of neoplastic disease. We describe the case of a male teenager with SLE and Burkitt's lymphoma. His presentation was similar to that of an exacerbation of his underlying SLE. We believe this to be the first ...

Two patients with esophageal involvement of pemphigus vulgaris are presented, who were diagnosed histopathologically and by direct immunofluorescence. In most dermatological textbooks the possibility of esophageal involvement is not mentioned. In the English medical literature 11 cases have been reported. It seems that if upper gastrointestinal endoscopy had been done ...

Hemodynamically significant lupus valvulitis, requiring valve replacement, is rare: 21 cases have been reported so far in the literature, and only 2 of these have involved double valve replacement. We describe an additional case of double valve replacement in a patient with systemic lupus erythematosus. The histopathologic and clinical features ...

A case of pemphigus vulgaris with esophageal involvement is discussed and 10 other reported cases are reviewed. Esophageal involvement is a rare but serious occurrence. It is most readily diagnosed by endoscopy when dysphagia or odynophagia in a patient with pemphigus vulgaris or with a history of pemphigus vulgaris does ...

We report the case of a 16-year-old female who developed pulmonary infection due to Pneumocystis carinii within 3 months of the clinical diagnosis of systemic lupus erythematosus (SLE). Steroid therapy doses never exceeded Prednisone 40 mg/day, and no cytotoxic drugs were used. The case presents several unique aspects of importance ...

A 15-year-old girl with systemic lupus erythematosus suddenly developed fever, meningismus, and herpes zoster. Within 48 hours, transverse myelitis developed at the level of the nerve root involvement of the herpes zoster. Since both systemic lupus erythematosus and varicella-zoster have been reported to cause myelitis, therapy was initiated for both. ...

High-grade atrioventricular heart block (HGAVB) occurring as a manifestation of systemic lupus erythematosus is an exceedingly rare event. We describe a patient with HGAVB who had myositis and antibodies to nuclear RNP (previously associated with myocarditis). A review of the literature on HGAVB associated with systemic lupus erythematosus is also ...

A case of naproxen-induced bullous photodermatitis ("pseudoporphyria") is presented. A 37-year-old woman with systemic lupus erythematosus developed tense bullae on her hands and lips, and was found to have a clinical and histological condition that was similar to porphyria cutanea tarda without the associated haematological abnormalities. The lesions remitted upon ...

Alveolar macrophages (AMs) recovered from the bronchoalveolar lavage (BAL) of 44 patients with sarcoidosis were evaluated for their ability to release type IV collagenolytic metalloproteinase (IV-Case). This enzyme, which is produced by peripheral blood monocytes (PBMs) but not by tissue macrophages, degrades type IV collagen, the major structural component of ...

We reviewed 62 cases (26 men and 36 women, median age 55 years) of previously diagnosed lichenoid dermatitis and correlated the histologic and immunodermatologic findings with the clinical diagnosis and course. The clinical diagnostic groups were contact dermatitis (6 cases), drug eruption (14), lupus erythematosus (6), lichen planus (3), cutaneous ...

Intermittent claudication and limb-threatening ischemia caused by occlusion of large and medium-sized arteries are rare manifestations of systemic lupus erythematosus. So far only eight documented cases have been reported, predominantly in young women. In this report two more patients are described. There is no common opinion concerning pathophysiologic mechanism, but ...

Two patients are described in whom myasthenia gravis was associated with systemic lupus erythematosus. The review of the literature shows that such association is not frequent and that it affects mostly women (6 women:1 man), and that myasthenia gravis usually precedes lupus erythematosus. Thymectomy was a precipitating factor in 25% ...

A man with arthritis, gynecomastia and a rash histologically showing leukocytoclastic vasculitis, dermatitis herpetiformis and immunoglobulin G deposits at the dermoepidermal junction is reported. In contrast to cases of systemic lupus erythematosus (SLE) associated with vesiculobullous eruptions and similar histological features, our patient had neither bullae nor serological evidence of ...

We report the case of a 13-yr-old patient with retarded growth and a 2-yr background of asthenia, anorexia, and fever, whose laboratory data revealed anemia, thrombocytosis, an elevated erythrocyte sedimentation rate, ferropenia, hyperglobulinemia, hyperfibrinogenemia, and presence of a lupus-like circulating anticoagulant. Clinical studies revealed a tumor-like overgrowth in the gastric ...

Two cases of propylthiouracil-associated acute hepatitis, one case of fatal methimazole-associated hepatocellular necrosis and one case of propylthiouracil-associated lupus-like syndrome are described. The literature related to antithyroid drug side effects and the mechanisms for their occurrence are reviewed and the efficacy and complications of thyroidectomy and radioiodine compared to those ...

Two patients (male, 60 and 66 years of age) who developed systemic lupus erythematosus (SLE) in the 6th decade are described. Both patients presented with a polymyalgia rheumatica (PMR) syndrome. In both cases there was an underlying muscle involvement (nonspecific in the first case and true myositis in the second ...

A case of lupus lymphadenitis with frozen section immunohistologic studies is presented. Clinically, the patient had well-documented systemic lupus erythematosus (SLE) when rapid development of generalized lymphadenopathy raised the possibility of a diagnosis of malignant lymphoma. Histologically, the findings of paracortical foci of necrosis and hematoxylin bodies were diagnostic of ...

A case is reported in which the treatment of the ranula consisted of dissection of the cystic wall along with excision of the sublingual gland of origin. The histological picture of the gland was consistent with sarcoidosis. Thereafter, all other examinations were negative for sarcoidosis. However, there remains the possibility ...

A 42-year-old man presented with systemic lupus erythematosus, universal alopecia and non-pruritic hyperpigmented papular mucinosis. The latter was most evident on acral areas. In hyperpigmented areas of the face the immunofluorescence showed deposits as in LE and with alcian blue and colloidal iron an abundance of mucin was demonstrated in ...

A patient with antinuclear antibody (ANA) negative systemic lupus erythematosus (SLE) developed pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum. The literature of pneumatosis cystoides intestinalis and spontaneous pneumoperitoneum is reviewed, the benignancy of these conditions is emphasized, and particular attention directed to their association with and implications in the collagen vascular ...

We report on the cases of two patients in whom linear perilesional hypopigmentation and atrophy developed after intralesional injection of corticosteroids for treatment of keloids. Evaluation of our patients and the previously reported cases showed that perilesional linear atrophy or hypopigmentation (or both) is a distinct complication after intralesional or ...

Phenothiazine-induced lupus with circulating anticoagulant (CAC) has been reported infrequently, and is rarely associated with significant symptoms. We describe a patient who we suspect had perphenazine-induced lupus erythematosus with CAC. She had recurrent episodes of venous thrombosis and ultimately developed superior vena cava syndrome. The relationships among phenothiazine-induced lupus, CAC, ...