Biomedical subjects
Publications and source records attributed to M Sandbank.
We present a case of black grain mycetoma caused by Madurella mycetomatis. The diagnosis was made on the basis of conventional light microscopy and mycological studies. After 12 months of oral therapy with ketoconazole, the patient showed partial response. This is the first native Israeli case of black grain mycetoma.
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.
A 57-year-old woman who had undergone a lumpectomy for infiltrating duct cell carcinoma of the right breast was found to have morphea after receiving radiation therapy. The morphea occurred at the site of the irradiated field. It is suggested that the irradiation served as a provoking factor and that the morphea might be an isomorphic response to the trauma of the irradiation.
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Eleven newly diagnosed patients and one patient with pemphigus vulgaris who relapsed underwent endoscopy of the upper gastrointestinal tract. Three patients had blisters or erosions and two had longitudinal lines of erythema in the esophageal mucosa. In four patients histopathologic examination showed findings of pemphigus vulgaris but direct immunofluorescence was positive in all patients. This study demonstrates that the immunopathologic disturbance in pemphigus vulgaris involves the entire length of the esophagus, although only some patients have clinical or histologic involvement. To the best of our knowledge this is the first histopathologic and direct immunofluorescence study of esophageal involvement in patients with untreated pemphigus vulgaris.
Buschke-Ollendorff syndrome is an association of connective tissue nevi and osteopoikilosis that usually appears in the first decades of life. The nevi occur on the trunk and extremities. In a 48-year-old man with this rare syndrome the connective tissue nevus was located on the scalp and was pruritic. Histologically, involvement of the epidermis with cystic dilatation of the hair follicles and an abnormality of mucopolysaccharides were present. The nevi in this syndrome may involve an alteration in the three components of connective tissue to a variable degree.
The Papillon-Lefèvre syndrome is characterized by palmoplantar hyperkeratosis and juvenile periodontitis that results in the precocious loss of both the deciduous and permanent teeth. We report a patient with Papillon-Lefèvre syndrome associated with acroosteolysis, an association that has not been previously described.
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.
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.
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A retrospective review was made of 35 cases of Brown Recluse spider bites treated during a period of 21 years. The most common location of the bite was on the lower limbs, particularly the thigh. The main systemic manifestations were fever, malaise and maculopapular rash, but there were no cases of hemolysis, coagulopathy, or of renal or neurologic involvement. Signs indicating a poor prognosis appeared to be lymphangitis, generalized maculopapular rash and location of the bite on the thigh or abdomen. Most cases were treated effectively by rest, elevation of the affected part of the body, local cleansing, and prophylactic antibiotics. On the basis of this experience it was concluded that only when lesions show a necrotic area measuring 2 to 3 cm should patients be treated with systemic corticosteroids. In most such cases, administration of corticosteroids for 14-21 days proved sufficient to avoid the need for surgery. Only 5 cases required surgical intervention.
The diagnostic value of the macrophage migration inhibition factor (MIF) test was evaluated in 90 patients with the clinical diagnosis of drug eruption. There were 40 males and 50 females who ranged in age from 15-84 years (mean 61.0). Controls were 120 patients being treated with drugs without adverse reactions and 21 patients with dermatologic disorders unrelated to drugs. Positive MIF responses for 1 or more drugs were observed in 71.1% of the patients with drug eruptions as compared to 4.2% and 9.5% of the control groups, respectively (p less than 0.001 for both). The proportion of positive MIF responses in those in whom drugs were implicated (45.2%) was significantly greater (p less than 0.0001) than in those with eruptions in whom drugs were not implicated (18%). The MIF test may be of aid in the diagnosis of drug eruptions and in the identification of the offending drugs.
The purpose of this study was to assess the role of leukocyte adherence in the pathogenesis of the psoriatic lesion. Use was made of the fact that psoriasis and pemphigus differ considerably as to the presence of leukocytes in the respective lesions: abundance in psoriasis, and absence in pemphigus. The state of leukocyte adhesiveness/aggregation (LAA) was determined in the peripheral blood of 56 patients with psoriasis and 31 patients with pemphigus. Both classes of patients were subdivided into two categories according to the severity of the disease. It was found that in both diseases elevated values of LAA were obtained in the severe cases, whereas the mild cases did not differ significantly from normal controls. Thus, in psoriasis mean LAA values of 9.5% +/- 8% were recorded in the severe patients and 5.5% +/- 4.2% in the mild cases (p = 0.01), while in pemphigus the values were 15% +/- 9.6% and 6.6% +/- 3.7% respectively (p = 0.03). It is concluded that LAA per se does not play a primary role in causing the psoriatic lesion.
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.
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