Spinal cord granulomatous vasculitis: an unusual clinical presentation of sarcoidosis.

A 52-year-old Caucasian man presented with isolated manifestations of myelopathy. Cervical magnetic resonance imaging showed a focus of increased signal intensity at the posterolateral left half of C5 and C6. Biopsy of spinal cord revealed the presence of active vasculitis associated with noncaseating granulomas. The patient responded to the combination of methotrexate (MTX) and corticosteroid treatment. Low-dose MTX was an effective, steroid-sparing regimen. This is a rare condition of isolated spinal cord involvement associated with sarcoid vasculitis.

Hypopigmented macules in acantholytic disorders.

BACKGROUND: Widespread hypopigmented macules are rarely seen in heavily pigmented patients with Darier's disease. Previous hypotheses concerning the cause of decreased pigmentation suggest it is a postinflammatory phenomenon or that the hypomelanosis is evidence of subclinical acantholysis. PATIENTS: This report presents 2 patients: a new case of disseminated guttate leukoderma in a black patient with Darier's disease and the first such case in a patient with transient acantholytic dermatosis (Grover's disease). Direct immunofluorescence and electron-microscopic studies were carried out on lesional biopsies. OBSERVATIONS: Numerous small hypopigmented macules were observed in two black patients followed for acantholytic disorders. Three biopsies of the hypopigmented macules revealed acantholysis, while one showed only decreased melanin. Direct immunofluorescence studies were negative. Electron-microscopic studies of the leukodermic macules showed sparse melanocytes and melanosomes that were mostly pigmented stage IV melanosomes. CONCLUSIONS: Disseminated guttate leukoderma can occur in transient acantholytic dermatosis, as well as in Darier's disease. It is readily apparent on darkly pigmented skin because of contrast. The etiology of this phenomenon is still unknown.

Jejunoileal bypass-induced liver dysfunction and bacterial translocation: effect of intraluminal glutamine-infusion.

We tested the effect of long-term intraluminal administration of glutamine on jejunoileal bypass (JIB) induced abnormalities in the plasma-liver profile in rats. Male Sprague Dawley rats (200-250 g) were subjected to an end to side JIB followed by daily intraluminal infusions of either 8 ml saline (n = 5), infused over a 4-hour period, or 8 ml saline containing 1g/Kg body weight glutamine (n = 7) for 3 weeks. Thirteen unoperated rats and four JIB rats without infusions served as controls. At the conclusion of the experiment, a cardiac blood sample was removed and analyzed for plasma cholesterol, albumin, total protein, gamma glutaril transferase, lactic dehydrogenase, glutamic-oxaloacetic transaminase, glutamic pyruvic transaminase, alkaline phosphatase, and bilirubin. Tissue samples from various segments of bowel, liver, mesenteric lymph nodes, and spleen underwent histopathologic examination. Bacteriological cultures were prepared from jejunum, ileum, mesenteric lymph nodes, liver, and spleen. Bacterial translocation occurred in both JIB-saline and JIB-glutamine infused rats. Glutamine-infused rats developed a significant decrease in the plasma cholesterol levels. However, glutamine did not prevent the JIB-induced alterations in the plasma-liver profile and bowel histopathology. It is suggested that experimental JIB procedure can be used as a model of bacterial translocation consequent to mucosal permeability and intestinal inflammatory diseases.

Eosinophilic hepatitis: a new feature of the clinical spectrum of the eosinophilia-myalgia syndrome.

The eosinophilia-myalgia syndrome associated with L-tryptophan-containing products is highlighted by eosinophilia, incapacitating myalgias, and diverse multisystemic manifestations. In addition to involvement of the skin, skeletal muscle, and peripheral nerves, visceral damage has been quite prominent, particularly affecting the lungs, the heart, and the liver. Hepatic involvement has been manifested by altered liver tests but is clinically silent. We report the unique case of a woman with this syndrome who developed abdominal pain, a clinical picture of hepatitis and chronically abnormal liver tests. Histologic examination of the liver disclosed eosinophilic hepatitis with piecemeal necrosis. The occurrence of clinically overt hepatic involvement has not been reported previously. Potential mechanisms of liver damage in eosinophilia-myalgia syndromes are discussed.

Fibroblast function in psoriatic arthritis. I. Alteration of cell kinetics and growth factor responses.

OBJECTIVE: To explore the potential role of fibroblasts in the pathogenesis of psoriasis and its related arthritis. Specifically, we analyzed the cell cycle of psoriatic fibroblasts obtained from skin and synovium by flow cytometry, and we also studied their response to several growth factors. METHODS: Fibroblast cultures were established from normal and psoriatic skin, uninvolved and involved, and synovium. NIH-3T3 cells were also used as indicator cells in some of the experiments. Fibroblasts DNA cell cycle analysis was performed by flow cytometry, and the data was analyzed by using the "Cytologic DNA applications software version 2." In addition, fibroblasts were stimulated with growth factors including epidermal growth factor, transforming growth factor-beta, and platelet derived growth factor. RESULTS: A significant increase of S and G2-M phase values in confluent cultures of psoriatic fibroblasts in both skin and synovium compared to normal fibroblasts was found. Psoriatic fibroblasts also exhibited a greater proliferative response to growth factors compared to normal fibroblasts. CONCLUSION: Data obtained clearly showed a significant intrinsic in vitro alteration in skin and synovium fibroblasts from patients with psoriasis.

Fibroblast function in psoriatic arthritis. II. Increased expression of beta platelet derived growth factor receptors and increased production of growth factor and cytokines.

OBJECTIVE: The main objective was to investigate the expression of platelet derived growth factor (PDGF) receptors, and production of growth factors and cytokines from psoriatic skin and synovium derived fibroblasts. METHODS: Fibroblast cultures were established from normal and psoriatic skin and synovium. Confluent cultures of fibroblasts were used for a receptor binding assay for PDGF, and then extracts were run on Western blot. The amount of immunoreactive A and B chain peptides present was determined with specific A or B chain antisera. Production of interleukin 1 beta and PDGF-beta was accomplished by neutralization with the use of commercially available antisera. A functional assay was used to measure transforming growth factor-beta (TGF-beta). RESULTS: There was an increased expression of the beta PDGF receptor in the psoriatic fibroblasts. Interleukin 1 beta and PDGF-beta production by psoriatic fibroblasts was also increased. However, TGF-beta production was similar in normal and psoriatic fibroblasts. CONCLUSION: Our data demonstrate an increased expression of beta PDGF receptor, and production of IL-1 beta and PDGF by psoriatic fibroblasts. The findings provide further support for an active role of this cell line in the pathogenesis of psoriasis and psoriatic arthritis.

Scurvy resembling cutaneous vasculitis.

Various conditions can imitate cutaneous vasculitis. Scurvy is a less appreciated cause of rash that can resemble vasculitis. Three patients were referred to our rheumatology service for "vasculitis," who subsequently were found to have scurvy. Findings included a purpuric skin rash, myalgias, and malaise. The patients had low vitamin C levels and findings on skin biopsy specimens indicative of scurvy. The three patients were on idiosyncratic diets deficient in vitamin C, and the two patients who allowed us to administer vitamin C therapy improved clinically. We emphasize the importance of a careful dietary history. Early recognition of scurvy is important because it can be treated specifically, and toxic treatment of vasculitis can be avoided.

Medulloblastoma metastatic to breast, diagnosed by fine-needle aspiration biopsy.

The extracranial spread of a primary intracranial tumor is an infrequent occurrence. The most common metastatic sites in order of frequency are bone, lymph nodes, and soft tissue. Analysis of the literature uncovered only two case reports of medulloblastoma that had metastasized to the breast. Both of these cases were diagnosed by open surgical biopsies. We present a case of medulloblastoma metastatic to the breast which was diagnosed by fine-needle aspiration biopsy. This diagnosis was subsequently confirmed by a tru-cut surgical biopsy and was further supported by immunohistochemical studies.

Nevus of Ota acquisita of late onset.

Nevus of Ota is a melanotic pigmentary disorder characterized by its distinctive cutaneous distribution involving skin innervated by the trigeminal nerve. Most cases are clinically manifest at birth or around puberty; however, acquired lesions in adults have been reported. We report a case of nevus of Ota acquisita that occurred in an eighty-one-year-old man.

Psoriatic arthritis: clinical response and side effects to methotrexate therapy.

In the last decade, methotrexate (MTX) has emerged as a useful second line agent for a variety of arthritides. However, there still exists some reluctance for its wider use mainly because of concerns about its liver side effects. We describe our clinical experience with this drug in psoriatic arthritis (PsA). The study group included 24 men and 16 women, with a mean age of 47 years (16-75), with oligoarticular (13) or polyarticular (27) involvement, with a mean disease duration of 12 years (1-36). Patients received a mean dose of 11.2 mg of MTX orally/week during a mean period of 34 months (6-132). Seven had been previously treated with other second line agents. Thirty-eight patients had an excellent or good response. In them, the erythrocyte sedimentation rate dropped in a mean of 38 mm/h. Only 2 patients had a rather poor response. Two patients discontinued the medication because of side effects: leukopenia in one and stomatitis in the other. Eleven patients presented with liver test abnormalities: 3 mild, 6 moderate and 2 severe. Seven patients had 11 liver biopsies. Except for one, none had evidence of cirrhosis or inflammation. Indeed, no changes were observed in the histopathology in those with repeated biopsies. The case reported as cirrhosis occurred very early in the course of MTX therapy. He continued taking MTX treatment without further deterioration of liver chemistry and/or histology. It is concluded that MTX is an effective and safe agent in PsA. Results also indicate that it is not necessary to perform liver biopsies on a routine basis.

There is an association between human immunodeficiency virus infection and spondyloarthropathies.

The presence of inflammatory musculoskeletal manifestations during the course of human immunodeficiency virus (HIV) infection is well established. A wide spectrum of rheumatic disorders have been reported since the first reports of Reiter's syndrome with HIV infection. Other reported associations include forms of arthropathies, psoriatic arthritis, Sjögren's syndrome, polymyositis-dermatomyositis, vasculitis, and septic arthritis.

Eruptive vellus hair cysts and neurologic abnormalities: two related conditions?

A fifteen-year-old white boy with a history of seizure disorder, borderline cognitive function, academic difficulty, and explosive temper outbursts was evaluated for asymptomatic flesh-colored papules appearing on his chest, abdomen, and axillae. Examination of a biopsy specimen showed eruptive vellus hair cysts; results of genetic, endocrinologic, and neuropsychiatric evaluation revealed abnormalities suggestive of a previously undescribed neurocutaneous syndrome.

Dowling-Degos disease, hidradenitis suppurativa, and multiple keratoacanthomas. A disorder that may be caused by a single underlying defect in pilosebaceous epithelial proliferation.

We report a case in which one patient had Dowling-Degos disease (reticulate pigmented anomaly of the flexures), hidradenitis suppurativa, and multiple keratoacanthomas. Abnormal epithelial proliferation involving mainly the pilosebaceous apparatus has been recognized in all three conditions. We speculate that a single underlying defect in follicular epithelial proliferation, characterized by variable expressivity, accounts for the coexistence of these clinically distinct disorders of follicular derivation.

Characteristics and pathogenesis of myositis in human immunodeficiency virus infection--distinction from azidothymidine-induced myopathy.

Inflammatory muscle involvement during the course of human immunodeficiency virus (HIV) infection is described and guidelines are suggested for its differentiation from the myopathy associated with azidothymidine (AZT) therapy. Six patients infected with HIV presented with proximal muscle weakness, biochemical and electromyographic abnormalities consistent with myositis. One patient had a skin rash characteristic of dermatomyositis. Muscle biopsy findings demonstrated the presence of an inflammatory cell infiltrate and HIV-p24 antigen. All patients developed their clinical picture prior to AZT therapy and responded to steroids with or without coadministration of AZT.

HIV associated arthropathy: HIV antigen demonstration in the synovial membrane.

Light and electron microscopic studies were performed on the synovial membranes of 5 patients with HIV associated arthropathy. An immunoperoxidase technique with the use of monoclonal antibodies against CD4, CD8, B and DR lymphocytes, and HIV p24 antigen was also used. Mild to moderate nonspecific proliferative changes and increased vascularity of the subsynovial space were seen. Electron dense deposits and viral-like particles were not observed. Immunohistochemical staining revealed HIV p24 positive staining in cells of the synovial lining layer and in the mononuclear cells of the subsynovial space. CD4, CD8, with predominance of CD8, and B and DR cells were also present. The presence of HIV p24 antigen may be indicative of a role, yet to be defined, in the pathogenesis of HIV associated rheumatic disorders.

Granulomatous myocarditis with unusual histologic features.

A 61-year-old white man was found to have granulomatous myocarditis associated with a carcinoid tumor in the ileum with local lymphatic metastasis, a mixed papillary and follicular carcinoma of the thyroid with regional lymph node metastasis, benign tubular cell adenomas of the kidneys, and chronic pancreatitis.

The hamster model of chronic Mycobacterium avium complex infection.

Male golden Syrian hamsters were evaluated as a model for the pathogenesis of human infection with Mycobacterium avium complex. Intratracheal inoculation produced a chronic, nonfatal, pulmonary and disseminated infection (overall rate, 86%). The frequency of infection in hamsters that received 5 x 10(8) versus 1 x 10(8) colony forming units (cfu) was not significantly different (87% and 92%, respectively), but 1 x 10(7) cfu produced infection in only 78% of inoculated animals (P = .034). The percentage of animals developing pulmonary infection with M. avium complex did not differ between inoculum groups (77%-80%). Disseminated infection occurred significantly less frequently in the 1 x 10(7) group (46%) compared with the 5 x 10(8) (79%) and 1 x 10(8) (68%) groups (P = .001 and .056, respectively). After seven weeks, partial clearance of M. avium complex from the lungs coincided with an increased number of animals with splenic involvement. The hamster may be a useful model for human infection with M. avium complex.

Human adjuvant disease following augmentation mammoplasty.

Two patients are described in whom a progressive systemic sclerosis-like illness developed several years after silicone augmentation mammoplasty. Both had removal of breast implants, followed by marked-to-complete recovery from clinical abnormalities. This entity is increasingly recognized and has become known as human adjuvant disease.

Dermatological manifestations of toxic agents.

A great variety of cutaneous disorders can be seen as reactions to toxic substances. To limit the extent of this review, cutaneous manifestations known to have an immunological mechanism are simply listed with pertinent references. The report has been focused to cover the cutaneous manifestations of toxic agents of non-immunological and unknown mechanisms. Entities discussed include acne, alopecia, dermatitis caused by spider bites, pityriasis rosea, and chemically-induced scleroderma.