CURRENT CONCEPTS IN DERMATOLOGY. II. THE SKIN AND SYSTEMIC DISEASE.
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Many systemic diseases have cutaneous manifestations. In some diseases skin involvement is the predominant factor (Behcet's syndrome, urticaria pigmentosa, discoid lupus erythematosus and pseudoxanthoma elasticum); in others the skin manifestations, when present, are an important part of the condition (sarcoidosis, systemic lupus erythematosus, hypersensitivity angiitis, porphyria). This report includes descriptions of and comments on these cutaneous manifestations. Erythema nodosum and erythema multiforme are reaction patterns of the skin and mucous membrane which may have many causes. The relationship between discoid and systemic lupus erythematosus is discussed. There is little doubt that these are variations of the same basic disease process, even though the prognoses are very different. (+info)
HOMOLOGOUS DISEASE IN THE ADULT RAT, A MODEL FOR AUTOIMMUNE DISEASE. I. GENERAL FEATURES AND CUTANEOUS LESIONS.
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The cutaneous lesions of adult rats with homologous disease are described, and evidence is presented to indicate that they have an immunologic basis. The skin changes included erythema, purpura, edema, and a variety of inflammatory lesions. In the more active lesions, dermal infiltration, hydropic degeneration, acanthosis, and atrophy of the epidermis with hyperkeratosis and follicular plugging were present. In some cases, ulceration and sloughing were also observed. More chronic lesions were characterized by atrophy of the epidermis and collagenization of the dermis with disappearance of the skin appendages. Rejection of autografts was observed simultaneously with acceptance of homografts. The histologic appearance of autografts undergoing rejection was similar to that of the spontaneous skin lesions, suggesting that the latter, too, had an immunologic basis. In favor of this, also, was the specificity of the dermatitis for the skin of the host, with sparing of neighboring homograft tissue. There was a histologic similarity between the spontaneous skin lesions of homologous disease and those of lupus erythematosus on the one hand, and scleroderma on the other, thus supporting the possibility that the cutaneous lesions of these connective tissue diseases of man may also have an immunologic basis. It was concluded that the adult rat with homologous disease may furnish a model for human autoimmune disease. (+info)
HEMATOLOGICAL DISORDERS FOLLOWING EXPOSURE TO INSECTICIDES.
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The medical literature dealing with hematological disorders following exposure to insecticides (chiefly chlorinated hydrocarbons and organic phosphorus compounds) is briefly reviewed. The development of blood dyscrasias as a consequence of exposure to insecticides is considered unlikely. Reported cases are few in number and often involve persons with little contact with these materials. It is often impossible to prove (or to disprove) a cause-and-effect relation in the individual case. Pointers which may be of assistance in evaluating this relationship are described. Purpura as a result of allergic vascular changes after exposure to insecticides is also discussed. (+info)
Purpura annularis telangiectodes of Majocchi.
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A case is presented of a man with a 3-year history of ulcers in the setting of pigmented, annular and purpuric lesions of the lower extremities. A skin biopsy suggested a diagnosis of purpura annularis telangiectodes of Majocchi. First described in 1896 by Majocchi [1], purpura annularis telangiectodes is an uncommon pigmented purpuric eruption, which is characterized by symmetrical, purpuric, telangiectatic, and atrophic patches with a predilection for the lower extremities and buttocks. Histopathology and immunopathogenesis of this disease are similar to the other subtypes of pigmented purpuric dermatoses. (+info)
PTU-associated cutaneous vasculitis with ANCA anti-MPO and anti-PR3 antibodies.
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A 36-year-old woman presented at our clinic with symmetrical, tender, palpable purpuric lesions on her lower legs and buttocks after restarting PTU therapy for relapsing Graves' disease. PTU-induced vasculitis was diagnosed with remarkable ANCA anti-MPO and anti-PR3 antibody positivity. The purpuric skin lesions resolved immediately after discontinuation of the drug and the ANCA titres lowered. In the presence of activated neutrophils, PTU could induce a high cytotoxity and injure the vessel walls. Treatment of choice is discontinuation of the drug. Sometimes more aggressive therapy as cyclophosphamide or plasmapheresis is warranted. (+info)
The bleeding child; is it NAI?
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As a paediatric haematologist, the question of whether a child has been abused or whether they might have a bleeding diathesis is a question that I am regularly asked. When I first became a consultant, I would often find that not enough information was available; for example, incomplete histories had been taken or investigations were incomplete and difficult to interpret. This inevitably led to delays in confirming the cause of the bleeding and meant that if parents or carers contested a diagnosis of abuse, excluding a bleeding disorder was extremely difficult. I was also aware that carers of several of my patients with haemophilia or other bleeding disorders had initially been under suspicion of abuse, most usually at the time of the first few presentations. By highlighting important questions in history taking, having a specific haematological screen for children being investigated for bleeding in the context of non-accidental injury, and encouraging discussion of abnormal results with a haematologist, these difficulties can, for the most part, be avoided. (+info)
Infectious urticaria with purpura: a mild subtype of urticarial vasculitis?
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Urticaria is characterized by transient wheals. We report here five cases with long-lasting urticarial lesions persisting for more than 24 hours. Each lesion left purpura after fading. There was no systemic involvement. C-reactive protein and serum levels of complement were elevated or normal. Histologically, marked infiltration by eosinophils and neutrophils with karyorrhexis in the perivascular and intercollagenous spaces was observed, but there was no evidence of vasculitis (venulitis). Skin symptoms were resistant to systemic corticosteroids. In contrast, treatment of underlying bacterial infections resulted in marked improvement of skin lesions. E-selectin, VCAM-1 and ICAM-1 were expressed on endothelial cells. Marked deposition of C3a, C5a, neutrophil elastase and major basic protein in the dermis was observed. These urticarial lesions provoked by bacterial infections seem to lie on the continuum between urticaria and urticarial vasculitis. (+info)
Antibodies to lipooligosaccharide of a Brazilian purpuric fever isolate of Haemophilus influenzae biogroup aegyptius lack bactericidal and protective activity.
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The immunological basis for protection against Brazilian purpuric fever (BPF), a fulminant infection of young children associated with bacteremia with Haemophilus influenzae biogroup aegyptius, is unknown. Candidate antigens to which protective antibodies may be directed include cell surface proteins and lipooligosaccharide (LOS). We studied the activity of antisera to LOS purified from a BPF H. influenzae biogroup aegyptius isolate. Anti-LOS antisera contained anti-LOS antibody by enzyme immunoassay and immunoblot and no detectable anti-outer membrane protein antibodies by immunoblot. Anti-LOS antisera had minimal bactericidal activity and were not protective against the homologous strain in an infant rat model of bacteremia. Antiserum to whole bacterial cells had a titer of anti-LOS antibody similar to that of anti-LOS antisera and was bactericidal and protective. Removal of anti-LOS antibodies from anti-whole cell antiserum by affinity chromatography did not result in a loss of bactericidal activity. Serum from a normal adult contained anti-LOS antibodies and had bactericidal activity. However, anti-LOS antibodies purified from this serum did not have detectable bactericidal activity. These studies suggest that anti-LOS antibodies produced in rats are not bactericidal and do not contribute to protection against experimental bacteremia with BPF strains of H. influenzae biogroup aegyptius. (+info)