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Biomedical subjects

E Hodak

Publications and source records attributed to E Hodak.

At least 55 records · Page 3Linked to original sources
PubMed1993

Quantitative and qualitative assessment of plasma von Willebrand factor in classic Kaposi's sarcoma.

BACKGROUND: von Willebrand factor (vWF) is synthesized almost exclusively by endothelial cells and is stored there as ultra-high-molecular-weight multimers. The vWF multimers that are detected in the plasma are smaller than those stored within the endothelium. In two previous studies, comprising small series of cases with classic Kaposi's sarcoma (KS), an endothelium-derived tumor, increased levels of plasma von Willebrand factor antigen (VWF:Ag, the antigenic structure) were reported, suggesting that vWF:Ag may be a marker of endothelium proliferation. OBJECTIVE: Our purpose was to investigate the quantitative as well as qualitative alterations of plasma vWF in a large series of patients with classic KS at various stages of the disease. METHODS: Levels of plasma vWF:Ag were studied in 38 patients with classic KS confined to the skin at various stages of the disease and compared with a control group. Thirty-three patients had active KS (i.e., with skin lesions) and five were in remission. In five patients with active KS multimeric analysis of plasma vWF was also performed. RESULTS: The levels of vWF:Ag were significantly higher among KS patients than in the control group (n = 29, p < 0.01). Levels of vWF:Ag were also significantly higher in patients with active disease as compared with those in remission (p < 0.05). No correlation was found between vWF:Ag levels and the extent of KS. Analysis of the multimeric pattern of plasma vWF showed enhanced staining of all bands, particularly the intermediate and high molecular weight forms, which resemble the endothelial forms as opposed to normal circulating vWF multimers. CONCLUSION: Quantitative as well as qualitative alterations in plasma vWF were found in patients with KS, which may reflect the destruction or activation of endothelial cells within the lesions. vWF:Ag may serve as a marker of disease activity in classic KS; however, it is not a good marker for the extent of the disease.

Aged↗
PubMed1993

Kaposi's sarcoma with visceral involvement after intraarticular and epidural injections of corticosteroids.

Kaposi's sarcoma has been reported in patients receiving immunosuppressive therapy, most of whom are organ transplant recipients. The development of Kaposi's sarcoma after treatment with corticosteroids has been reported in only 38 patients who have not had acquired immunodeficiency syndrome or undergone organ transplantation. Cutaneous Kaposi's sarcoma developed 2 months after intraarticular steroid injections in a man with ulnar nerve entrapment. The lesions regressed spontaneously after 3 months but reappeared with visceral involvement 18 months later, shortly after initiation of a course of epidural steroid injections for treatment of low back pain. The cutaneous lesions and some visceral lesions rapidly regressed after cessation of treatment.

Administration, Topical↗
PubMed1993

A paraneoplastic mixed bullous skin disease associated with anti-skin antibodies and a B-cell lymphoma.

BACKGROUND: The full spectrum of bullous diseases associated with underlying cancers remains to be fully defined. OBSERVATION: We describe a patient with a mixed bullous disease exhibiting combined features of cicatricial pemphigoid and pemphigus and associated with a B-cell lymphoma producing an IgM paraprotein to intercellular antigens in human skin. The patient had the clinical features of cicatricial pemphigoid and the histologic and immunofluorescence abnormalities of both cicatricial pemphigoid and pemphigus. These included oral and cutaneous erosions; ocular scarring; subbasal and acantholytic intraepidermal bullae; and circulating and tissue-fixed basement membrane zone and intercellular antibodies. The antibodies were directed to a 140-kd antigen in dermal extracts of skin split with 1 mol/L of sodium chloride and to antigens with approximate molecular weights of 150, 180, 230, and 285 kd in the dermal extract. In contrast to paraneoplastic pemphigus, the intercellular antibodies did not react to mammalian bladder. The intercellular antibodies were of the IgM class and were associated with the paraprotein produced by the malignant B cells. CONCLUSIONS: We believe that this condition represents a novel bullous disease, which we refer to as paraneoplastic mixed bullous disease. This condition illustrates that distinct bullous diseases are associated with paraneoplastic syndromes and that at least one possible mechanism for such eruptions is the production of anti-skin antibodies by malignant B cells.

Autoantibodies↗
PubMed1992

Pachydermodactyly: first report in a woman.

Pachydermodactyly is a rare form of digital fibromatosis that is characterized by acquired, asymptomatic, protracted connective tissue swellings on the back and sides of a few proximal phalanges or proximal interphalangeal joints. This is the tenth reported case of pachydermodactyly. It has previously been reported only in men; to the best of our knowledge, this is the first woman described with this condition.

Adolescent↗
PubMed1992

Facial pyogenic granuloma-like lesions under isotretinoin therapy.

A male patient with severe cystic acne was treated with 13-cis-retinoic acid in a dosage of 1 mg/kg/day. Early flare-up of his acne and multiple pyogenic-like lesions on his face appeared during the second week of isotretinoin treatment. Histologic study of the lesions revealed granulation tissue. Possible etiologies for this phenomenon are discussed.

Acne Vulgaris↗
PubMed1992

Immunologic abnormalities associated with primary anetoderma.

BACKGROUND AND DESIGN: Primary anetoderma is a rare cutaneous elastolytic disorder, the etiopathogenesis of which has not yet been established. Six patients with primary anetoderma were studied in an attempt to assess the role of the immunologic system in the elastolytic process. The investigation included the medical history, physical examination, routine blood tests, specific tests for collagen diseases, prothrombin time, activated partial thromboplastin time, thyroxine, indirect immunofluorescence test, and skin biopsies for histopathologic study and direct immunofluorescence. RESULTS: Two of the patients presented with autoimmune disorders: the first had Graves' disease, lupus anticoagulant, and autoimmune hemolysis, and the second had systemic scleroderma. There were positive direct immunofluorescence findings in most of the patients. Furthermore, all of them were found to have serologic immunologic abnormalities, of which the most common was a positive antinuclear factor. CONCLUSIONS: These findings indicate that there is an immunologic involvement in primary anetoderma.

Adult↗
PubMed1991

Acne fulminans with hepatosplenomegaly and erythema nodosum migrans.

Acne fulminans is a rare form of acne characterized by ulcerative nodules and associated with systemic complications. We report the case of a 17-year-old boy with cystic acne who experienced sudden onset of acne fulminans associated with fever, weight loss, hepatosplenomegaly, and erythema nodosum migrans. To the best of our knowledge, this is the first reported case of acne fulminans manifested by hepatomegaly and erythema nodosum migrans.

Acne Vulgaris↗
PubMed1991

Primary anetoderma associated with a wide spectrum of autoimmune abnormalities.

Although the underlying pathologic mechanisms of primary anetoderma have not yet been identified, data suggest the participation of an immunologic mechanism in some cases. In a woman with clinical and histopathologic features of primary anetoderma (Jadassohn-Pellizzari type) of 30 years' duration, laboratory investigation disclosed positive antinuclear factor, hypocomplementemia, hypergammaglobulinemia, granular deposits of immunoreactants along the dermoepidermal junction, and fibrillar deposits in the papillary dermis. In addition, she was found to have autoimmune hemolysis and circulating lupus anticoagulant associated with recurrent deep-vein thrombosis and a history of Graves' disease (starting 5 years after onset of primary anetoderma). To our knowledge, none of the latter three autoimmune conditions has been previously associated with primary anetoderma.

Antibodies, Antinuclear↗
PubMed1990

Segmental neurofibromatosis.

Two patients had localized multiple cutaneous neurofibromas; one had bilateral involvement of the scalp and the other had true segmental neurofibromatosis. Other signs of neurofibromatosis were absent. Segmental neurofibromatosis may not be related to the generalized types of neurofibromatosis but may be a cutaneous hamartoma.

Aged↗
PubMed1990

Conjunctival involvement in pemphigus vulgaris: a clinical, histopathological and immunofluorescence study.

Eleven patients with pemphigus vulgaris and with eye complaints had histopathological and direct immunofluorescence (DIF) studies on biopsies of their conjunctivae. In eight of the patients the ocular symptoms preceded the other manifestations of pemphigus. None of the 11 patients had any detectable conjunctival blisters or erosions. The conjunctivitis in three of the patients proved on histopathology and DIF to be a manifestation of pemphigus vulgaris. In the remaining patients, conjunctival hyperaemia with or without a mucoid discharge was observed, but there were no specific histopathological features, although one of them had a positive DIF.

Adult↗
PubMed1990

Bullous pemphigoid--an adverse effect of ampicillin.

A young patient developed an acute self-limited bullous eruption shortly following ampicillin ingestion, with histopathological and immunofluorescent features fulfilling the criteria of bullous pemphigoid. A review of the relevant literature is made with special emphasis on the heterogenous and sometimes misleading faces of drug-induced bullous pemphigoid.

Adult↗
PubMed1990

The clinical and histopathological spectrum of IgA-pemphigus--report of two cases.

Two new cases of neutrophilic bullous disease exhibiting bound and circulating intercellular IgA in vivo in both direct and indirect immunofluorescence (IF), for which the term IgA-pemphigus was recently proposed, are presented. The first case showed a unique constellation of clinical and histopathological features not previously described for IgA-pemphigus, resembling both dermatitis herpetiformis and pemphigus, for which the designation IgA-herpetiform pemphigus seems most appropriate. The second case showed clinical and histopathological features mimicking subcorneal pustular dermatosis for which the previously used term IgA-pemphigus foliaceus seems most appropriate. The previously reported 11 cases showing similar direct IF findings, as well as our two patients, illustrate the main differences between IgA-pemphigus and classic forms of this condition: (a) different clinical manifestations with the absence of Nikolsky's sign; (b) scanty acantholysis; an abundance of neutrophils, with occasional neutrophilic spongiosis; (c) absence of C3 on direct IF; (d) the lower sensitivity of indirect IF and low levels of circulating antibodies; (e) the relatively benign course of the disease; (f) the apparent responsiveness to sulphones in many cases. It is suggested that IgA pemphigus should be considered as an entity separate from, but closely related to, classic pemphigus.

Diagnosis, Differential↗
PubMed1990

Semicircular lipoatrophy--a pressure-induced lipoatrophy?

A case of semicircular lipoatrophy, a rare form of localized lipoatrophy, is described in a 33-year-old woman. No precipitating factors such as trauma could be elicited by questioning. However, the patient subsequently realized that she had unwittingly been subjected to repeated daily trauma as she pressed the affected thigh against the edge of the wash bowl while applying make-up. This case thus supports a possible mechanical basis for this rare entity, and demonstrates the importance of taking a careful history in the search for the possible occurrence of minor episodes of trauma in such cases.

Adipose Tissue↗
PubMed1990

Effect of etretinate on peripheral T lymphocytes in psoriatic patients before, during and after 6 months of therapy.

Total peripheral T lymphocytes, OKT4 helper/inducer cells and OKT8 suppressor/cytotoxic cells, as well as T lymphocyte function determined by the local xenogeneic graft-versus-host reaction (GVHR), were investigated in 14 psoriatic patients prior to institution of treatment with etretinate, during the course of treatment and 6 months after its initiation. After approximately 2 months of treatment, there was a significant increase in the number of E-rosette-forming lymphocytes and OKT4 subpopulations with a return to normal levels after 6 months of treatment. The GVHR was positive in only 5/11 patients prior to therapy but in 9/11 patients after 2 and 6 months. Our results indicate that etretinate has a stimulatory effect on T lymphocytes and their subset counts.

Adult↗
PubMed1990

Fixed drug eruption following rifampin treatment.

A case of fixed drug eruption (FDE) associated with ingestion of rifampin in a young physician is reported. The drug was prophylactically administered because of a previous close contact with a meningococcemic patient. The eruption consisted of two solitary painless purplish lesions located over the extensor surface of the left forearm, characteristic of FDE, except for the absence of residual hyperpigmentation. It is suggested that slight lesions of FDE could be unnoticed or misdiagnosed, thus raising the possibility that such skin reactions to rifampin are more frequent than is reflected in the literature.

Adult↗