OBJECTIVE: Posterior reversible encephalopathy syndrome (PRES) is a rare, recently described neurologic condition identifiable by clinical presentation and magnetic resonance image (MRI) appearance. It is associated with renal insufficiency, hypertension, and rheumatologic diseases. Patients present with headache, seizures, loss of vision and altered mental function, and a pattern on imaging ...

Sarcoidosis is a multisystemic disease that usually involves the lungs and lymph nodes, but almost any organ can be involved. Genitourinary involvement with sarcoidosis is extremely rare. We report the case of a 30-year-old African-American male who presented with a right-sided intrascrotal mass and diffuse lymphadenopathy. On further workup, he ...

Sarcoidosis of the vulva is a very rare condition. Until now, only four cases have been reported in English literature. The patient, a 48-year-old, gravid 3, para 2 postmenopausal Greek woman presented with a 6 months history of perineal pain. The patient underwent a wide local excision of the painful ...

Hypertrichosis lanuginosa acquisita (HLA) is an unusual condition which is characterized by subtle and progressive development of multiple, long, thin, unmedullated hairs ("lanugo hairs") distributed preferentially on the face. Most cases are associated with malignant tumors or non-malignant condition such as porphyria cutanea tarda, AIDS, anorexia nervosa, thyrotoxicosis, or secondary ...

Pemphigus foliaceus (PF) is a rare autoimmune blistering disease presenting in endemic and sporadic forms. The typical presentation is recurrent shallow erosions in a seborrheic distribution. We present a case of a 58-year-old woman with PF who was successfully treated with a combination of oral corticosteroids and dapsone. We also ...

Hot tub lung is a form of hypersensitivity pneumonitis (HP) reported to be associated with exposure to large aerosolized inocula of Mycobacterium avium complex (MAC). Although the pathogenesis of the disease is still poorly understood, the pathology of MAC-related HP can be similar to that of sarcoidosis, with well-formed granulomas. ...

Various autoimmune diseases have been reported to occur in patients with sarcoidosis. However, coexistence of sarcoidosis and antiphospholipid syndrome (APS) is extremely rare. We describe a 59-year-old female patient with pulmonary sarcoidosis who had preceding APS. Her previous medical history consisted of a miscarriage and ischemic colitis. She was diagnosed ...

Membranous lipodystrophy represents a peculiar type of fat necrosis that is present in patients with various types of skin disease. It is characterized by the presence of microcysts and macrocysts and is lined by amorphous eosinophilic material with a crenelated arabesque appearance. These findings have been associated with lupus erythematosus, ...

Celiac disease (CD) is an inflammatory condition of the gut with a known autoimmune pathogenesis. Many similarities exist between the pathogenesis of CD and systemic lupus erythematosus (SLE); it is still unknown whether there is an association. There are 13 case reports in the literature of both diseases occurring simultaneously. ...

Sarcoidosis involving the pancreas is a rare occurrence. Isolated cases of localized or diffuse involvement of the pancreas have been reported in the literature. The preoperative diagnosis of this entity is a clinical challenge, and surgical intervention is usually needed to make a definitive diagnosis. We report a patient that ...

We present a 47-year-old man with recurrent intramedullary sarcoidosis. This condition, without any other manifestations of the disease, is rare, with less than 20 reports in the literature. Further, to our knowledge our presentation is the only reported case of intramedullary sarcoidosis recurring in a separate, distant region of the ...

Rituximab is a chimeric monoclonal antibody specific for human CD20 that causes selective transient depletion of the CD20+ B-cell subpopulation. We report the first case of systemic lupus erythematosus (SLE) pneumonitis resistant to conventional treatments that responded well to rituximab and review current reports on the use of rituximab in ...

The antiphospholipid syndrome (APS) is characterized by obstetric and thrombotic complications in the presence of antiphospholipid antibodies. It can happen in an isolated way or in association with diffuse connective tissue diseases, mainly systemic lupus erythematosus. The association of APS with Takayasu arteritis (TA) is rarely described in the literature. ...

Systemic lupus erythematosus (SLE) is a systemic, autoimmune disease. SLE patients are prone to infections, and their hospital admissions and mortality are most commonly associated with infections. Necrotizing fasciitis (NF) is a rare, life-threatening infection of the subcutaneous tissue. In this report, NF associated with Streptococcus pneumoniae (SPN) that developed ...

The most common cardiovascular manifestation of Systemic Lupus Erythematosus is pericardial disease. Tamponade in SLE is rarely described. The patient discussed in this case report presented with symptoms of heart failure. Physical exam, laboratory testing, echocardiography, and right heart catheterization revealed multiple morbid conditions including tamponade. The diagnoses satisfied four ...

Localized salmonella soft tissue infections have been rarely described in humans. We report a case of necrotizing fasciitis caused by Salmonella serogroup C in a patient with systemic lupus erythematosus who was successfully treated with surgical debridement and cefoperazone-sulbactam. In addition, we provide a literature review on risk factors and ...

Interferons (IFNs) are widely used for the treatment of various medical diseases. They have marked immunomodulatory effects, and many reports have been published associating IFN therapy with the induction of autoimmune phenomena and other disorders of immune regulation such as sarcoidosis. The clinical presentation of IFN-induced sarcoidosis (IIS) is insidious ...

Although neurological involvement occurs in about 10% of patients with mixed connective tissue disease (MCTD), acute transverse myelitis (TM) has only been described in seven cases of MCTD. We hereby report a case of 70-year-old white female with transverse myelitis complicating her underlying MCTD. Our patient presented with lower extremity ...

We describe a 24-year old male patient with systemic lupus erythematosus (SLE) with the gastrointestinal manifestations of protein-losing enteropathy (PLE) and primary sclerosing cholangitis (PSC). He presented with periorbital, scrotal and lower limb oedema. PLE was diagnosed because of hypoalbuminaemia together with an elevation of alpha-1-antitrypsin stool clearance and absence ...

Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a benign and self-limited lymphadenitis commonly found in young women. It often shares clinical features with systemic lupus erythematosus (SLE), such as arthralgias, fever and leukopenia. The etiology of KFD remains unknown and controversial. Clinical course is favorable, with spontaneous remission in ...

Neurologic symptoms rarely occur as presenting feature of systemic lupus erythematosus (SLE). We describe a 37-year old woman who presented with several episodes of transverse myelitis and optic neuritis. Clinical, radiologic and laboratory findings were compatible with neuromyelitis optica (NMO). Seven years after disease onset clinical and laboratory findings were ...

Amicrobial pustulosis of the folds (APF) is characterized by relapsing, primary aseptic, pustular eruptions mainly affecting the cutaneous folds, scalp, and periorificial regions such as the mouth, external ear canal, and nostrils. APF is a rare condition that has previously been reported in 25 women exhibiting a wide spectrum of ...

BACKGROUND: Nephrogenic fibrosing dermopathy (NFD) is a newly proposed clinical entity resembling scleromyxedema. This is mainly found in patients with chronic renal failure who have undergone hemodialysis. Nearly all cases have been reported in the Western population. OBJECTIVE: We sought to assess whether NFD cases occurred in a non-Western population ...

There have been few reports of lupus erythematosus tumidus (LET) in the literature. Most of textbooks of dermatology or dermatopathology mention this entity only briefly, if at all. The authors describe an additional case of this underdiagnosed disorder that further supports its existence as a separate entity in the spectrum ...

We report the case of a 24-year-old Cuban-American man initially presenting with a 1-year history of bilateral testicular pain, subsequently diagnosed as sarcoidosis. The diagnosis was histologically confirmed intraoperatively with excisional biopsy of the right tunica. Genitourinary complaints are a rare initial presentation of this disease. Although therapeutic approaches have ...

Acute interstitial nephritis is commonly seen in kidney biopsies of patients with acute renal failure; however, granulomatous interstitial nephritis (GIN) is rare. We identified 46 cases of GIN in a 17-year period in this institution and we investigated their most probable etiologies. Complete clinical information was available in 38 patients. ...

Kikuchi-Fujimoto disease, also known as histiocytic necrotising lymphadenitis, is a self-limiting condition of uncertain aetiology characterised by lymphadenopathy, pyrexia, and neutropenia. Some reported cases have been associated with systemic lupus erythematosus and there have been suggestions that Kikuchi's disease could represent a mild form of lupus but without definite evidence. ...

Papulosquamous eruptions involving the palms and soles are thought to be particularly suggestive of secondary syphilis. Alternative diagnoses exist, however, and include psoriasis guttata, atypical pityriasis rosea, and pityriasis lichenoides chronica (PLC). We describe the case of a patient with an abrupt onset of psoriasis guttata and extensive palmoplantar involvement. ...

Toxic epidermal necrolysis (TEN) is an acute, rapidly evolving mucocutaneous reaction with a high mortality rate characterized by extensive painful cutaneous and mucosal exfoliation and systemic involvement that is frequently associated with medication use. The treatment of this condition is controversial. Systemic lupus erythematosus (SLE) is a generalized autoimmune disease ...

Chorea is a rare manifestation of paraneoplastic disease and is associated with CV2/CRMP-5 antibodies. Obsessive-compulsive disorder and large-scale white matter abnormalities on MRI have not been previously reported in association with these antibodies. We report on a case of CV2 paraneoplastic syndrome with obsessive-compulsive behavior preceding the motor manifestations of ...

Transverse myelitis is a rare manifestation of antiphospholipid syndrome, usually secondary to systemic lupus erythematosus (Rheum Dis Clin North Am 20:129-158, 1994). Only about 110 reports of this complication have been reported (Lupus 10:851-856, 2001). A connection has been demonstrated between positive serology for antiphospholipid and transverse myelitis (Lupus 8:109-115, ...

Hydranencephaly is characterized by absent cerebral hemispheres replaced by fluid-filled sacs in a normal skull. Herein we report the case of a premature neonate with hydranencephaly born to a mother with systemic lupus erythematosus. Although numerous etiologies have been proposed, an association between maternal systemic lupus erythematosus and neonatal hydranencephaly ...

A 5-year-old, female neutered, Australian Kelpie presented with a 2-month history of dramatic bilateral erythematous thickening of the third eyelids. Ophthalmic examination demonstrated raised, pink to red, irregular thickening of the entire palpebral surface of both third eyelids. There were no other ocular abnormalities. A surgical biopsy was taken from ...

PURPOSE: To describe a case of eyelid sarcoidosis without systemic manifestations with a three-year follow-up. METHODS: A 73-year-old woman presented complaining of a one-year history of swelling and hyperemia in the right upper eyelid. To confirm the diagnosis, we performed an incisional biopsy of the eyelid. RESULTS: Histopathological examination showed ...

A further new case of sarcoidosis associated with the use of interferon (IFN) in the classical treatment of chronic hepatitis C (CHC) is reported. During the last two years, more than 20 cases of interferon-induced sarcoidosis have been described in the literature and about half of these cases have involved ...

BACKGROUND: Lipedematous alopecia is a rare entity of uncertain origin characterized by a boggy scalp and varying alopecia. Of 11 cases in the literature, 7 occurred in African American women. Classic histopathologic findings include increased subcutaneous tissue, varying inflammation, and an absence of mucin. Some have sought to distinguish this ...

BACKGROUND CONTEXT: We present the case of a 20-year-old male with sarcoidosis with cryptococcal infection of the spine. PURPOSE: To present the case and add to the existing body of literature on clinical cryptococcal infection. STUDY DESIGN/SETTING: An academic tertiary care center. METHODS: Chart review and clinical examination.

We present a case of a pregnant patient with fulminant systemic lupus erythematosus complicated by alveolar hemorrhage, a rare and potentially fatal manifestation of lupus. It typically presents in the context of a pulmonary-renal syndrome. Active lupus nephritis with hypoalbuminemia is a major risk factor for alveolar hemorrhage. Treatment with ...

Sarcoidosis is a common systemic granulomatous disease affecting multiple organs. Oral involvement is relatively rare and, to our knowledge, there have been only 64 cases reported in the English literature. Most cases of oral sarcoidosis present with mobility of the teeth due to rapid alveolar bone loss. Other oral manifestations ...

Calcinosis cutis is common in several connective tissue diseases such as dermatomyositis, scleroderma or lupus erythematous. In dermatomyositis, it is more likely to concern children than adults but it is not exceptional in adults. Several treatments have been used empirically with inconsistent success. We report a case of adult cutis ...

Systemic lupus erythematosus is a disease of unknown cause that manifests with tissue and cellular alterations due to the deposition of antibodies and pathogenic immune complexes. The disease can be associated with anticardiolipin antibody syndrome, a disorder of recurrent vascular thrombosis and thrombocytopenia associated with a persistent anticardiolipin test positivity. ...

Cutaneous tuberculosis of the orofacial region is a rare condition and when it occurs, can cause confusion regarding the true nature of the lesion. This is compounded by the fact that neither mantoux test nor histopathology is confirmatory. In this report we discuss a case of lupus vulgaris with emphasis ...

We report a 40 years old woman with chronic urticaria and acute transverse myelitis associated with systemic lupus erythematosus. The urticaria appeared in her adolescence and after 26 years was followed by photosensitivity, peripheral polyarthritis and acute transverse myelitis, with positive antiphospholipid and antinuclear antibodies. Both chronic urticaria and acute ...

We report the case of a young woman with a single history of benign summer light eruption (BSLE) who developed delayed onset annular lupus-like lesions triggered by a polychromatic phototest, 6 weeks after the irradiation. BSLE of French authors is an idiopathic photodermatosis that corresponds to the minor form of ...

Neuropsychiatric lupus can be difficult to diagnose, and little prospective data exists to help direct management. In this case report we describe the acute onset of symptoms of depression, mania, and psychosis and their complete resolution 48 h following a 5-day treatment course of intravenous immunoglobulin (IVIG) in a 20-year-old ...

Histiocytic necrotizing lymphadenitis, called Kikuchi-Fujimoto's disease (KFD), is an idiopathic, self-limited condition rarely associated with systemic lupus erythematosus (SLE). The cause of concomitant KFD and SLE is still unknown. We describe a 19-year-old man simultaneously diagnosed with both KFD and SLE complicated with deep vein thrombosis (DVT). To the best ...

BACKGROUND: Systemic lupus erythematosus (SLE) is an autoimmune disorder with various systemic and cutaneous manifestations. Nail abnormalities, including onycholysis, red lunulae, pitting, and ridging, have been seen in patients with the disorder. To our knowledge, however, pincer nail deformity has yet to be reported coincident with the onset of SLE. ...

A 30-year-old black woman presented with a 1-year history of repeated episodes of worsening weight loss and fatigue. Physical examination showed no skin lesions and a chest roentgenogram was normal. Workup showed no obvious cause of recurrent renal stones. Finally, the detection of lymphadenopathy led to the diagnosis of extrapulmonary ...

We present the case of a man diagnosed with lupus and hypoparathyroidism. This combined diagnosis has very rarely been reported, which is surprising given the evidence that idiopathic hypoparathyroidism can be due to autoimmunity, the multiplicity of organ systems that lupus can affect and the evidence that other endocrinopathies such ...

Malar or discoid rash is the most frequent specific cutaneous lesion for systemic lupus erythematosus (SLE). Neutrophilic dermatosis as an initial presentation in SLE is unusual. We describe a 38-year old female patient who primarily suffered from erythematous tender plaques and fever. Examination of skin biopsy of the plaques showed ...