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Biomedical subjects

B D Zelickson

Publications and source records attributed to B D Zelickson.

14 recordsLinked to original sources

Primary B-cell lymphoma with histologic features of a T-cell neoplasm.

A 58-year-old white man had dermatomyositis and primary cutaneous B-cell lymphoma. The cutaneous lymphoma was evidenced by a noduloulcerative disease of the lower extremities. Histologic results resembled a T-cell process with a diffuse, superficial infiltrate composed of small- and medium-sized lymphocytes with angioinvasion and epidermotropism. The infiltrate extended into the deep dermis and panniculus with scattered large lymphocytes and necrosis. With the help of gene-rearrangement analysis and immunophenotyping, the true B-cell lineage was discovered. The importance of gene-rearrangement analysis and immunophenotyping in the diagnosis of cutaneous lymphoma is emphasized.

Blotting, Southern

Epidermolysis bullosa acquisita induced by GM-CSF: a role for eosinophils in treatment-related toxicity.

A patient treated with granulocyte-macrophage colony-stimulating factor (GM-CSF) developed eosinophilia and epidermolysis bullosa acquisita. The bullae were subepidermal, and filled with an inflammatory infiltrate composed predominantly of eosinophils. Immunofluorescence studies disclosed linear deposition of IgG, IgA and C3 at the basement membrane zone and immunoelectron microscopy demonstrated antibody deposition in the lamina densa and sublamina densa region; however, the patient's serum did not contain circulating antibody to basement membrane zone antigens. Staining with monoclonal antibodies revealed dense deposits of both eosinophil peroxidase and eosinophil major basic protein at the dermal-epidermal junction. The eosinophilia and skin lesions resolved upon discontinuation of GM-CSF. This case provides evidence for two hypotheses: (1) GM-CSF induced proliferation and activation of eosinophils may contribute to some of the toxicities of GM-CSF treatment, and (2) activated granulocytes, including eosinophils, may mediate blister formation in epidermolysis bullosa acquisita.

Aged

Generalized pustular psoriasis in childhood. Report of thirteen cases.

Generalized pustular psoriasis is rare in children. Less than 100 cases have been reported. We describe 13 children with this type of psoriasis. Seven had acute onset of widespread sterile pustules coalescing into lakes of pus with subsequent exfoliation (the Zumbusch pattern). This usually occurred in infancy and was difficult to control; recurrences developed several times per year. Three had the subacute benign annular pattern. They tended to be older and often had resolution within several years. Three had a mixed pattern with Zumbusch flares preceded by an annular or acral pattern. Most patients had an eruption preceding the generalized pustular psoriasis and often had precipitating factors. Generally, generalized pustular psoriasis has little serious chronic morbidity. The condition in most patients was well controlled with topical therapy. Systemic steroids were not helpful.

Acute Disease

T-cell receptor gene rearrangement analysis: cutaneous T cell lymphoma, peripheral T cell lymphoma, and premalignant and benign cutaneous lymphoproliferative disorders.

T-cell receptor gene rearrangement analysis is a useful technique to detect clonality and determine lineage of lymphoid neoplasms. We examined 103 patients with mycosis fungoides, Sézary syndrome, peripheral T cell lymphoma, potentially malignant lymphoproliferative disorders including pre-Sézary syndrome, large plaque parapsoriasis, lymphomatoid papulosis and follicular mucinosis, and various benign inflammatory infiltrates. A clonal rearrangement was detected in skin samples in 20 of 24 patients with mycosis fungoides and in peripheral blood samples in 19 of 21 patients with Sézary syndrome. A clonal population was also detected in seven of eight cases classified as peripheral T cell lymphoma. The potentially malignant dermatoses tended to have clonal rearrangement, with the exception of large plaque parapsoriasis, and further follow-up is needed to correlate clonality with the disease course. These studies demonstrate the value of molecular genetics as an adjunct to morphology in the examination of patients with cutaneous lymphoproliferative disease.

Adolescent

Lipophagic panniculitis in re-excision specimens.

Lipophagic panniculitis consists of a macrophage infiltrate in the subcutaneous tissue. The macrophages transform into foam cells within the panniculus; they replace lipocytes and may form giant cells. Although those pathologic features have been described as diagnostic of Weber-Christian disease, we report the occurrence of lipophagic panniculitis in re-excision specimens. Among 252 re-excision specimens from previously biopsied skin tumors, 5 cases in which masses of lipophages were infiltrating and replacing the subcutaneous tissue were found. The infiltrate was localized to the deep dermis and superficial subcutaneous tissue below and beside the initial biopsy site. In 3 cases, suture or hair was detected within the tissue, and granulation tissue with foreign body giant cells was observed along the dermal suture line. In 4 cases there was evidence of phlebitis within or close to areas of infiltration. None of these patients developed symptomatic panniculitis. Lipophagia can be a normal response of wound healing in some patients.

Foam Cells

Generalized pustular psoriasis. A review of 63 cases.

BACKGROUND: Sixty-three patients with generalized pustular psoriasis were hospitalized during a 29-year period. They were classified into four subgroups on the basis of onset and morphologic pattern of disease: acute (von Zumbusch), subacute annular, chronic (acral), and mixed. This division provides a better understanding of the variability of the disease and helps in choosing treatment. OBSERVATIONS: The average age at onset was 50 years; male and female patients were affected about equally. In 11 patients, flares were precipitated by localized infections. Approximately one fourth of the patients had complications; most were superinfections. The average stay in the hospital was 30 days; factors correlating with a long hospitalization were hypocalcemia, female sex, and a previous history of psoriasis vulgaris or pustular psoriasis. CONCLUSIONS: Whereas topical therapy was helpful, systemic medications were often needed. Coal tar, ultraviolet light, and psoralen-ultraviolet A may be effective; however, they must be used with caution, because they may exacerbate the disease.

Acute Disease

Actinic cheilitis. Treatment with the carbon dioxide laser.

Actinic cheilitis is a premalignant condition that can be treated in several ways. A total of 43 patients with biopsy-proven actinic cheilitis were treated with the carbon dioxide (CO2) laser. After follow-up of at least 10 months, 26 patients thought that the lip was cosmetically improved, and 40 thought that the function of the lip was improved or had not changed. Complications were few and included only mild hypertrophic scarring, which resulted most often from the diagnostic biopsy and was corrected with topical or intralesional steroids or no therapy except simple massage. The CO2 laser is a simple, inexpensive, effective therapy for actinic cheilitis.

Adolescent

Patch testing in prurigo nodularis.

32 patients with prurigo nodularis evaluated at the Mayo Clinic from 1975 to 1987 have been patch tested for sensitivity to appropriate allergen series; 25 of these had relevant positive reactions and subsequent follow-up to 5 to 14 years was available for 11. 6 patients had persistent disease and 5 had resolution or marked improvement. 3 of these latter patients noted a strong positive correlation between improvement and avoidance of contact allergens. Screening for contact sensitivity may be helpful in the management of this refractory dermatosis, particularly if there is a coexistent dermatitis.

Adrenal Cortex Hormones

Intermittent leukapheresis: an adjunct to low-dose chemotherapy for Sézary syndrome.

Eleven patients with Sézary syndrome were treated with intermittent leukapheresis in addition to low-dose chlorambucil and prednisone. The results were as good as or better than those with chemotherapy alone. We believe the combined program with continuous leukapheresis to be optimal therapy but note that intermittent treatment offers some benefit for patients.

Chlorambucil

Transient acantholytic dermatosis associated with lymphomatous angioimmunoblastic lymphadenopathy.

Transient acantholytic dermatosis is a papulovesicular cutaneous eruption first described in 1970. There have been subsequent reports of similar disorders occurring in patients with malignancy. Angioimmunoblastic lymphadenopathy with dysproteinemia is a disorder characterized by an acute onset of generalized lymphadenopathy associated with fever, malaise, pruritus, night sweats, and hepatosplenomegaly. The patient described had a papular acantholytic dermatosis associated with the development of angioimmunoblastic lymphadenopathy with dysproteinemia-like T-cell lymphoma. The cutaneous manifestations of angioimmunoblastic lymphadenopathy with dysproteinemia are discussed.

Acantholysis

Polymorphonuclear leukocyte chemotaxis in generalized pustular psoriasis.

We studied polymorphonuclear leukocyte (PMNL) chemotaxis in 3 patients with generalized pustular psoriasis using 3 chemoattractants: Zymosan-activated serum from both the patients and normal individuals, and n-formyl-methionyl-leucyl-phenylalanine (FMLP). These attractants were assayed against both patient-derived and normal PMNLs. All patients had quiescent skin disease at the time of assay. We found that all patients had decreased PMNL chemotaxis to autologous Zymosan-activated serum, but not to FMLP. PMNLs from normal individuals responded normally to serum from the patients. We conclude that patients with generalized pustular psoriasis, when free of symptoms, may have an isolated PMNL chemotactic defect that is restricted to serum-derived attractants.

Adult

Oral drug reactions.

Oral drug reactions have many clinical manifestations and are produced by numerous medications. These reactions may be the result of an allergic reaction to systemically administered drugs or as an indirect effect of the action of the drug on other tissues. Other oral drug reactions may be the result of local or topical medications. These reactions are either a result of an allergic, delayed-type hypersensitivity, or a local primary irritation. The appearance may be nonspecific or it may resemble several distinct clinical entities. The diagnosis of these oral drug reactions is made with a good clinical history and examination, along with a high index of suspicion. Often there are multiple factors involved that complicate the clinical picture. The clinician who is familiar with the types of oral drug reactions caused by medications, the mechanisms by which these reactions occur, and which medications are most likely to cause the reaction will be prepared to make the correct diagnosis and treatment recommendations.

Anti-Bacterial Agents

Gene rearrangement analysis in lymphoid neoplasia.

Current uses for gene rearrangement analysis in clinical dermatology are listed in Table 3. This technique is useful for determining the existence of clonal populations within a background of polyclonal lymphoid cells; therefore, it is helpful in the diagnosis and staging of patients with CTCL and PTCL. Although dual genotypes do occur, this technique is usually capable of determining lineage in a clonal lymphoid infiltrate and elucidating and characterizing the etiopathogenesis of certain neoplasms. On the basis of this review of the literature and our own experience, we conclude that gene rearrangement analysis shows great promise for monitoring response to therapy and detecting progression or relapse in patients with CTCL and PTCL. With the recent technology of PCR, it is possible to amplify specific sequences of DNA, detect molecular alterations in individual malignant T cells, and even identify exogenous retroviral gene sequences in tissues of patients with CTCL. Although gene rearrangement analysis has supported or established the clonal nature of lymphomatoid papulosis, pre-Sézary syndrome, granulomatous slack skin syndrome, and follicular mucinosis, the clinical significance of these findings is not yet clear. In the case of primary cutaneous B-cell lymphoma and its benign counterpart, B-cell pseudolymphoma, further investigation will be needed to determine the clinical significance of clonal rearrangements.

Blotting, Southern