Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe the histopathologic changes of skin, muscle, vessels, and fascia in 11 patients with eosinophilia myalgia syndrome, a newly described entity that has been linked to the ingestion of L-tryptophan. This syndrome is defined clinically by severe incapacitating myalgias and a peripheral eosinophilia. Arthralgias, edema of the extremities, morbilliform rashes, skin induration, weakness, fatigue, and respiratory weakness may be present as well. The earliest apparent histologic changes were observed at the septa between subcutaneous fat lobules and in the deep dermis or fascia. The septa and fascia were infiltrated with a sparse mixture of lymphocytes and histiocytes. In the deep fascia, in addition to inflammatory cells, there were distinctive, reactive mesenchymal cells that showed features of both histiocytes and fibrocytes. Minimal tissue eosinophilia was seen despite the extent of blood eosinophilia. Dermal thickening and homogenization of collagen bundles occurred with replacement of fat and adnexa (changes indistinguishable from scleroderma or morphea). Vessel walls in the dermis and fascia showed thickening and endothelial swelling, but no overt vasculitis was noted. Skeletal muscle biopsies showed a perimysial, epimysial, and/or fascial inflammatory infiltrate of lymphocytes and distinctive reactive mesenchymal cells with some eosinophils. Minimal myofiber atrophy, regeneration, or necrosis was seen despite the clinical history of severe myalgias in almost all patients. This syndrome should help gain insight into the mechanisms of fibrosis in environmental-induced, scleroderma-like syndromes and in idiopathic, scleroderma-like disorders as well.
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PMID:Pathologic manifestations of the eosinophilia myalgia syndrome: analysis of 11 cases. 156 45

We present a case of widespread subcutaneous symmetrical morphea that started as nodular vasculitis. The characteristics histologic features were abundant inflammatory infiltrates in the subcutis, fascia, and muscle, with numerous macrophages and extensive vascular changes.
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PMID:Subcutaneous symmetrical morphea with unusual onset as nodular vasculitis. 162 13

Biopsy specimens from 185 patients with 52 different skin disorders were investigated by indirect immunofluorescence staining for the presence of HLA-DR bearing keratinocytes and their association with an underlying inflammatory infiltrate and in particular with activated (HLA-DR-positive, Leu-4-positive) T lymphocytes. HLA-DR expression on keratinocytes was demonstrated in 38 dermatoses, including lymphocytic vasculitis, lupus erythematosus, morphea, vitiligo, lichen planus, cutaneous T-cell lymphoma, various infectious dermatoses, allergic contact dermatitis, granulomatous dermatoses, Sweet's syndrome, lichen sclerosus and erythema nodosum. In 27 of these this had not previously been reported. Occurrence of HLA-DR on keratinocytes was invariably linked to the presence of a lymphocytic infiltrate containing numerous activated T-cells (Leu-4 +, HLA-DR +) whereas such infiltrates were not accompanied by HLA-DR expression on keratinocytes in all the dermatoses investigated, as in pseudolymphoma and erythema anulare centrifugum. However, HLA-DR positive keratinocytes were consistently absent in skin disorders lacking any significant lymphocytic infiltration (e.g. leukocytoclastic vasculitis, bullous autoimmune dermatoses, genodermatoses and mastocytosis). Although it has been suggested that HLA-DR-positive keratinocytes are involved in various immune responses of the skin, their exact functional significance is, as yet, unknown.
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PMID:HLA-DR expression on keratinocytes is a common feature of diseased skin. 242 56

A deep sclerotic process developed on the shins of a 58-year-old man, and eosinophilic fasciitis or morphea profunda was suspected clinically. Bullae later arose on the plaques, and histologic examination of a skin biopsy specimen revealed sclerosis and inflammation of the deep dermis, panniculus, and fascia, with subepidermal edema causing formation of bullae. No lymphatic obstruction or vasculitis was seen. Two plaques of typical morphea on the penis were noticed 10 months later. The patient had no peripheral or tissue eosinophilia, hypergammaglobulinemia, hematologic abnormality, or history of exertion before the onset of the disease. The sclerotic process involved more than the fascia. In describing this deeper variant of morphea, the term "morphea profunda" appears to be more appropriate than "eosinophilic fasciitis."
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PMID:Bullous morphea profunda. 371 24

The POEMS syndrome is a synopsis of different symptoms such as polyneuropathy, organomegaly, endocrine disturbances, M-protein and skin changes. The leading symptoms are neuropathy and the skin symptoms. Additionally, a monoclonal light chain gammopathy is often found. The administration of immunosuppressive drugs yields a substantial improvement in some cases. We report here about a 72 year old lady who fell ill with a rapidly progressive neuropathy accompanied by hyperpigmentation and a morphea-like induration of the skin. A biopsy of the sural nerve showed a demyelinating axonal neuropathy and a focal vasculitis. Isoelectric focussing revealed oligoclonal bands in cerebrospinal fluid and serum. The cortisol serum level was very low and there were signs of a latent diabetes mellitus. These clinical features correspond to the POEMS syndrome. The prescription of initially 1 mg and later 0.5 mg prednisone improved the patient's condition dramatically.
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PMID:[POEMS syndrome: a contribution to differential diagnosis of polyneuropathy]. 850 14

Granuloma annulare (GA) and necrobiosis lipoidica (NL) are generally considered to be idiopathic cutaneous palisading granulomatous dermatitides. There are sporadic reports of such lesions occurring in patients with coexistent systemic diseases other than diabetes mellitus. Having encountered 49 patients whose skin biopsies showed GA or NL lesions in the setting of extracutaneous disease, the authors set out to assess their clinical and histopathological findings to determine if any parameters were predictive of underlying systemic disease. Fifty-two skin biopsies from 49 patients having either GA or NL in whom there was a clinical history of an associated systemic disease were analyzed by light microscopy. The main systemic disease associations were rheumatologic, endocrine, hematologic, infectious, and inflammatory bowel diseases, ANCA positive vasculitic syndromes, and sarcoidosis. The clinical and histomorphological features were compared with those of a control group of patients whose skin biopsies showed GA or NL and in whom there was no history of extracutaneous disease. For the systemic disease group, patients were selected either retrospectively or prospectively from 160,000 cases accessioned in a 24-month period in the dermatopathology databases of Pathology Services, Inc (Cambridge, MA) and Central Medical Laboratories (Winnipeg, Canada). All systemic disease cases from the former service were analyzed blindly by the second author and from the latter service were analyzed blindly by the first author. Patients in the control group were obtained retrospectively from the Pathology Services Inc. database by the authors. The location of the lesions was atypical in 30 of 34 biopsies from systemic disease patients with a GA tissue reaction versus 10 of 22 biopsies of GA in the control group (P = .001). Six of 18 biopsies from patients with NL tissue reactions in the systemic disease group showed an atypical location, versus only 1 of 9 biopsies of NL from the control group (P = .19). The clinical diagnostic considerations were much broader in the systemic disease group versus the control group and included vasculitis, panniculitis, and connective tissue diseases including morphea in the former. In 22 of 34 GA biopsies and 16 of 18 NL biopsies from the systemic disease group, an active vasculopathy of leukocytoclastic, granulomatous, or thrombogenic subtypes was demonstrable. None of the GA or NL biopsies from the control group showed a similar active vasculopathy. An active vasculopathy was predictive of systemic disease in patients having either a GA-like or an NL-like tissue reaction (P < .001). Fifteen of 34 GA and 7 of 18 NL biopsies in the systemic diseases group showed extravascular neutrophilia in contrast to 3 of 22 GA (P = .02) biopsies and 2 of 9 NL (P = .33) biopsies in the control group. The finding of an active vasculopathy in a skin biopsy specimen showing a GA- or NL-like tissue reaction, particularly in the setting of an atypical clinical presentation both with respect to the location and appearance of lesions, should prompt consideration of an underlying systemic disease, as should extravascular neutrophilia in a skin biopsy showing a GA-like tissue reaction.
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PMID:Granuloma annulare and necrobiosis lipoidica tissue reactions as a manifestation of systemic disease. 854 11

Clinicopathologic correlation of cutaneous biopsy specimens demonstrating typical lipomembranous fat necrosis was performed. Material from 732 biopsies of various subcutaneous inflammatory disorders seen at our institution in the past 5 years was screened for typical lipomembranous (membranocystic) changes in the panniculus, and 39 specimens from 38 patients with these changes were identified. The most common clinical context in which this condition was observed was in chronic sclerotic plaques of the lower legs associated with venous insufficiency (37% of the total cases). All patients were women, and the majority were obese. Typical lipomembranous fat necrosis was also observed in eight cases (21%) of erythema nodosum, three (8%) of morphea or subcutaneous morphea (or both), two (5%) of lupus panniculitis, two (5%) of necrobiosis lipoidica, and in single cases of polyarteritis nodosa, necrotizing vasculitis, and erysipelas. Six cases (16%) had no definite underlying disease. The mean age of all patients was 57 years (range 32-86 years), and 34 patients (89%) were women. Of the five major categories identified, lipomembranes lining macrocysts and microcysts were most prominent in the venous insufficiency- and morphea-related cases and were much less prominent in erythema nodosum, lupus panniculitis, and necrobiosis lipoidica, which generally showed histopathologic findings typical of these disorders. In addition to lining the macrocystic and microcystic cavities formed in the fat lobules, lipomembranes were prominent in areas of septal fibrosis in all cases associated with morphea and necrobiosis lipoidica and in 35% and 25% of venous insufficiency- and erythema nodosum-related cases, respectively. In lupus panniculitis, lipomembranes were most prominent in areas of hyaline necrosis. We conclude that lipomembranous fat necrosis is most likely a nonspecific form of ischemic fat degeneration that may be induced by various clinical entities. This change is most often seen in venous insufficiency-associated chronic sclerotic plaques typically observed in middle-aged obese women, and we propose the term stasis-associated lipomembranous panniculitis (SALP) to describe this most common form of lipomembranous fat necrosis.
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PMID:Lipomembranous (membranocystic) fat necrosis. Clinicopathologic correlation of 38 cases. 873 89

CD44 variant isoforms are frequently expressed on tissue-infiltrating lymphocytes. By the high incidence of autoimmune reactions of the skin and aiming at new strategies of therapeutic intervention, we became interested in evaluating the CD44 isoform expression profile in autoimmune reactions of the skin. Expression of CD44s, CD44v3, v5, v6, v7, v7-v8, and v10 was evaluated in 55 biopsies of lupus erythematosus, bullous pemphigoid, vasculitis, morphea, and pemphigus vulgaris. Biopsies did not contain CD44v5-, CD44v6-, CD44v7-, or CD44v7-v8-positive leukocytes. Staining with anti-CD44v10 was seen in vasculitis and occasionally in lupus erythematosus, morphea, and bullous pemphigoid. All biopsies contained CD44v3(+) leukocytes, the percentage of CD44v3(+) leukocytes being increased in autoimmune infiltrates with the exception of pemphigus vulgaris. CD44v3 was expressed by CD4(+) cells as well as by part of CD8(+) cells, Langerhans cells, and monocytes. Vascular endothelium also contained CD44v3(+) cells. Only monocytes expressed CD44v10. We assume that CD44v3 and CD44v10 may be targeting leukocytes toward the skin or allow for their retention and expansion via binding of cytokines and chemokines harbored by activated, skin-associated endothelium or provided by cells surrounding the infiltrate. The absence of CD44v6, frequently associated with lymphocyte activation, appears to be a peculiarity of skin-infiltrating leukocytes.
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PMID:CD44 variant isoform expression in a variety of skin-associated autoimmune diseases. 975 27

Generalized morphea is rarely associated with systemic overlap. We report an unusual case with generalized morphea involving cutaneous large vessel vasculitis, mononeuritis multiplex, and lupus anticoagulant without any evidence of the coexistent systemic lupus erythematosus.
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PMID:Necrotizing vasculitis in a patient with generalized morphea. 1171 63

The panniculitides represent a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. The specific diagnosis of these diseases requires histopathologic study because different panniculitides usually show the same clinical appearance, which consists of erythematous nodules on the lower extremities. However, the histopathologic study of panniculitis is difficult because of an inadequate clinicopathologic correlation and the changing evolutive nature of the lesions. In addition, large scalpel incisional biopsies are required. From histopathologic point of view, all panniculitides are somewhat mixed because the inflammatory infiltrate involves both the septa and lobules. However, nearly always the differential diagnosis between a mostly septal and a mostly lobular panniculitis is straightforward at scanning magnification on the basis of the structures more intensely involved by the inflammatory infiltrate. Mostly septal panniculitides with vasculitis are actually more vasculitis than panniculitis and include superficial thrombophlebitis and cutaneous polyarteritis nodosa. Mostly septal panniculitides with no vasculitis include erythema nodosum, necrobiosis lipoidica, deep morphea, subcutaneous granuloma annulare, rheumatoid nodule, and necrobiotic xanthogranuloma. Mostly lobular panniculitis with vasculitis is only represented by erythema induratum of Bazin. In contrast, mostly lobular panniculitides without vasculitis comprise a large series of disparate disorders, including sclerosing panniculitis, calciphylaxis, sclerema neonatorum, subcutaneous fat necrosis of the newborn, poststeroid panniculitis, lupus erythematosus profundus, pancreatic panniculitis, alpha(1)-antitrypsin deficiency panniculitis, subcutaneous Sweet syndrome, infective panniculitis, factitial panniculitis, lipodystrophy, traumatic panniculitis, subcutaneous sarcoidosis, and sclerosing postirradiation panniculitis. Finally, some cutaneous lymphomas may simulate panniculitis, both from clinical and histopathologic points of view and, for that reason, they will be included in this review, although they are not inflammatory processes, but authentic lymphocytic neoplasms involving subcutaneous tissue.
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PMID:Normal subcutaneous fat, necrosis of adipocytes and classification of the panniculitides. 1754 56


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