Biomedical subjects
Publications and source records attributed to W M Sams.
Photosensitivity is an abnormal cutaneous response to photosensitizing substances and sunlight or artificial light. Proper assessment of the photosensitive patient is important in defining photosensitive diseases and reactions.
Cutaneous silica granuloma is a poorly understood, uncommon condition that may mimic cutaneous sarcoidosis. We describe two cases of this entity and their characteristic latency period (between the time of silica exposure to the time of clinical onset of granuloma). We also review the histologic and energy dispersive x-ray analysis data, which prove the diagnosis. This condition should be recognized as an occupational dermatosis as well as the result of past incidental cuts or abrasions, which result in the development of granulomas, many in old wound scars. Differentiation from cutaneous sarcoidosis is possible with polarized light microscopy and energy-dispersive x-ray analysis.
A 64-year-old man had urticaria pigmentosa and myelodysplasia (refractory anemia with excess blast cells; partial trisomy 8 syndrome) without increased numbers of marrow mast cells. Clonal marrow assays in agar demonstrated normal to increased colony-forming units of granulocytes/macrophages. In long-term liquid cultures containing mast cell growth factor (interleukin 3), his marrow cells proliferated after 3 weeks to produce abnormal myeloid precursors similar to those in the corresponding marrow aspirate specimen. Cells with basophilic-staining granules were less abundant in comparison with normal marrow specimens cultured similarly. These results suggest that the mast cells in this patient are not of the same clone as the preleukemic marrow cells, although the possible marrow-cell origin of urticaria pigmentosa mast cells cannot be excluded. Previous reports suggest that urticaria pigmentosa without systemic mastocytosis occurs as a nonspecific abnormality in a variety of myeloid, lymphoid, and nonhematologic malignancies. Our data also support this hypothesis that urticaria pigmentosa is a reactive process rather than a manifestation of clonal proliferation of the primary malignancy.
Hypersensitivity angiitis is a disease in which patients present with palpable purpura dominant on the lower legs. As lesions evolve they become confluent, and sometimes hemorrhagic and ulcerate. Other organ systems may be involved, particularly the joints, gastrointestinal tract, and kidneys. Current evidence supports an immune complex pathogenesis in which a variety of antigens including bacteria, viruses, drugs, or chemicals are involved. Therapy consists of identifying the potential offending agent and administration of antiinflammatory drugs.
Human hypersensitivity angiitis is a disease involving deposition of immune complexes in which patients present with palpable purpuric lesions most often on the lower extremities and sometimes associated with multiple organ involvement. There are numerous potential antigens in the immune complex and the disease may be derived from an infectious organism such as Streptococcus or the hepatitis virus, from a drug, or from a variety of other chemicals which may be ingested or inhaled. In addition, the antigen may be part of another systemic disease. This complex of circulating antigen and antibody may become fixed within the vessel wall activating the complement cascade with elaboration of factors chemotactic for neutrophils. These cells then release lysosomal enzymes which result in destruction of the vessel wall and leakage of red blood cells into the tissue. This produces the typical purpura and palpability seen clinically. A central question in the pathogenesis of this disease is to explain why the immune complex is seemingly so selective in its site of deposition as it may be deposited in postcapillary venules in some patients and medium-sized muscular arteries in other patients. Part of the reason for this must be related to the lattice formation of a particular complex, while other reasons are likely related to alterations in vascular permeability, to the integrity of clearance mechanisms for the complex, or possibly even to a genetically determined defect of the phagocytic system.
Chronic sun exposure can result in numerous changes in human skin, particularly the skin of fair-skinned individuals. These changes include wrinkling, elastosis, actinic keratoses, irregular pigmentation, telangiectasia, and skin cancer. Most of these alterations had been considered to be caused by UVB wavelengths. However, a number of recent studies have demonstrated that UVA can also cause burning, elastosis, and skin cancer. Thus, individuals exposed to intense UVB from sunlight coupled with intense UVA from suntan parlors are likely at increased risk.
A 13-year-old child is described who presented with generalized pemphigus vulgaris associated with extraordinarily high titers of circulating autoantibodies against the pemphigus antigen. Because of the lack of response to treatment with reasonably high doses of oral corticosteroids, as well as the very high titer of circulating autoantibodies observed, this patient was treated with intravenous corticosteroid pulse therapy followed by plasmapheresis and then by combination immunosuppressive therapy (prednisone and azathioprine). A rapid clinical response was induced, correlating with reduction and subsequent elimination of the circulating pemphigus autoantibodies. Using such combination therapy, a remission of 12 months was achieved, and prednisone therapy was completely, albeit temporarily, tapered and then discontinued. Subsequent disease flare was then easily controlled with a short course of low-dose oral corticosteroid therapy.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Eight patients with livedo vasculitis of four to 30 years' duration that was unresponsive to a variety of medications were treated with pentoxifylline. Three patients experiences complete healing and remained free of active lesions while receiving the drug, four noted much improvement, and one had no change.
Peripheral blood mononuclear cells were obtained from 20 untreated condyloma acuminatum patients and from an equal number of sex- and age-matched controls and assayed for cell surface antigen expression, natural killer activity, and lymphokine production. Patient peripheral blood mononuclear cells had significantly lower helper-to-suppressor T-cell ratios (Leu3/Leu2) (P less than 0.05) and significantly higher percentages of Leu 2+ Tac+ cells (activated suppressor/cytotoxic cells) (P less than 0.05) and Leu 2+ OKM1+ cells (suppressor cells) (P less than 0.01). Natural killer activity of condyloma acuminatum patients was significantly lower (P less than 0.05) than that of controls. Production of interleukin-2 and interferon gamma, but not interferon alpha, was significantly (P less than 0.01) decreased in condyloma acuminatum patients. There was an inverse correlation between the in vitro production of interleukin-2 and interferon gamma and the percentage of Leu 2+ OKM1+ cells (suppressor) (P less than 0.01). Thus, patients with condyloma acuminatum differ from controls by demonstrating decreased natural killer-cell activity, decreased production of lymphokines which enhance natural killer-cell activity (i.e., interferon gamma and interleukin-2), and an increased proportion of T cells with a suppressor phenotype.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Bullous systemic lupus erythematosus is a newly recognized form of systemic lupus erythematosus characterized by a skin eruption clinically and histologically resembling dermatitis herpetiformis and responsive to dapsone. We report on a patient with bullous systemic lupus erythematosus who initially presented with lesions clinically resembling erythema multiforme, experienced exacerbation of her disease with dapsone, and had detectable circulating autoantibodies to the epidermolysis bullosa acquisita antigen.
Chronic sun exposure can result in numerous changes in human skin, particularly the skin of fair-skinned individuals. These changes include wrinkling, elastosis, actinic keratoses, irregular pigmentation, telangiectasia, and skin cancer. Most of these alterations had formerly been considered to be caused by UVB wavelengths, which are the wavelengths probably most responsible. However, a number of recent studies have demonstrated that UVA can also cause burning, elastosis, and skin cancer. Thus, individuals exposed to intense UVB from sunlight coupled with intense UVA from suntan parlors are likely at increased risk.
Human hypersensitivity angiitis is an immune complex disease in which patients present with palpable purpuric lesions of the skin and may often have multiple organ involvement. The antigen may be derived from an infectious organism such as the hepatitis virus, streptococcus, or a drug, and complexes with antibody. Under circumstances of vascular turbulence or vessel wall dilatation this complex may become fixed, activating the complement sequence with elaboration of chemotactic factors for neutrophils. These cells release lysosomal enzymes resulting in vessel wall destruction. Red blood cells leak into the tissue producing purpura and the inflammatory infiltrate accounts for the palpability. Although many patients have skin lesions only, others may have involvement of joints, gastrointestinal tract, kidneys, and even the lungs. The central question in the pathogenesis of this disease is why the immune complex is so selective in its site of deposition. Part of the reason must be related to the lattice formation of a particular complex, while other reasons are related to host factors of altered vascular permeability, integrity of clearance mechanisms or even a genetically determined defect of the phagocytic system.
Nail abnormalities secondary to systemic disease are important to the dermatologist because they are readily examined and may be the initial signal that systemic disease may be present. Some of the abnormal nail findings represent part of a symptom complex that may be useful in physical diagnosis. The knowledge of the correct onychopathologic etiology may give the patient the correct nail prognosis and may prevent institution of possible incorrect lengthy and costly treatment regimens. In this article, nail signs are grouped according to the characteristics of the change and according to systemic diseases producing signs.
Explore the source record for details and available documents.