Pigmented Paget's disease in a man previously treated with mammaplasty reduction for gynecomastia.
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Biomedical subjects
Publications and source records attributed to M A Barnadas.
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A 34-year-old female previously diagnosed of pseudoxanthoma elasticum developed an annular plaque with serpiginous borders of 42 by 30 mm in diameter on the inner left arm. A similar lesion later appeared on the inner right arm. Histopathological examination of a papule showed short, fragmented, granular, basophilic and calcified elastic fibers in the mid-reticular dermis. The epidermis showed hyperplasia surrounding degenerated and normal elastic fibers. Transepidermal elimination channels of these elastic fibers were also observed. These findings were consistent with the diagnosis of elastosis perforans serpiginosa. Abundant multinucleated giant cells were observed surrounding the area of epidermal hyperplasia and in the reticular dermis. The patient was treated with tazarotene, and the plaques disappeared in 9 months.
A 49-year-old male presented at our department with erythematous brownish plaques in the malar areas and left cheek of 9 years' evolution. Histopathological study revealed a dense, predominantly perivascular, inflammatory infiltrate in the reticular dermis. The infiltrate was composed of abundant neutrophils, lymphocytes, histiocytes, and eosinophils. Leukocytoclasia and fibrin in some vessel walls were also observed. The patient was diagnosed with granuloma faciale (GF). Direct immunofluorescence (DIF) study showed heavy immunoglobulin G (IgG) and less intense deposits of IgA, IgM, C(3) and C(1q) surrounding superficial and deep vessels of the reticular dermis. Perivascular and diffuse fibrinogen deposits were also present. No deposits were detected at the dermoepidermal junction. Our findings lend support to the hypothesis that classical pathway activation of complement may be involved in the development of vasculitis in GF.
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BACKGROUND: About 12% of patients with subepidermal autoimmune bullous disease and immunoglobulin G (IgG) at the dermal-epidermal junction present diseases other than bullous pemphigoid. MATERIALS AND METHODS: We report the clinical, histopathologic, and therapeutic aspects of eight cases of subepidermal bullous disorder with IgG on the floor of salt-split skin. RESULTS: A predominant neutrophilic infiltrate was detected in six of the eight patients. In one patient, the inflammatory infiltrate was neutrophilic and eosinophilic in the same proportion. A good response to dapsone alone or combined with prednisone was observed in six patients. CONCLUSIONS: The salt-split skin direct immunofluorescence test is useful for its diagnostic and therapeutic implications.
BACKGROUND: There have been reports suggesting the involvement of environmental factors in the disease process of pemphigus. Factors suggested include exposure to pesticides or certain drugs. OBJECTIVE: To analyze the association of pemphigus with environmental exposure to various agents, including smoking, recreational and occupational insults, drugs, and food. DESIGN AND SETTING: In-person interviews of pemphigus patients and control subjects were conducted by trained medical investigators using a structured questionnaire. Questions included occupational, behavioral, medical, and qualitative food frequency details. The multicenter study was conducted at outpatient services of teaching hospitals in Bulgaria, Brazil, India, Israel, Italy, Spain, and the USA. PARTICIPANTS: A total of 126 pemphigus patients (55 men, 71 women; age, 54 +/- 17 years) and 173 healthy controls (87 men, 86 women; age 50 +/- 19 years) were interviewed in the period between October 1, 1999 and March 31, 2000. The diagnosis of pemphigus was based on clinical, histologic, immunohistologic, and immunohistochemical criteria. The disease duration was 2-27 years (8.4 +/- 7.2 years). Individuals with skin diseases other than pemphigus were selected as control subjects. MAIN OUTCOME MEASURE: Information on drugs, foods, and occupational, environmental, constitutional, and other possible risk factors was analyzed by t-tests and chi-squared tests as applicable. A multivariate logistic regression model was applied to the data to study simultaneously the independent relationship between each risk factor and pemphigus vulgaris. RESULTS: The risk for pemphigus vulgaris was lower for ex-smokers and current smokers than for patients who had never smoked. Exposure to pesticides and occupational exposure to metal vapor were associated with an increased risk of pemphigus. Pemphigus patients had more pregnancies than controls. There were differences in environmental factors between countries, with exposure to gardening materials and pesticides being highest among patients from Bulgaria, followed by Israel. Disease characteristics also exhibited differences between countries. Bulgarian patients less frequently had oral mucous membrane lesions: 66% compared to 92% for Israeli patients and 83% for Italians. The distribution of the disease in skin and mucous membranes was similar among patients from all countries. Exclusive skin involvement was seen in 50% of patients, mucous membranes alone in 23% of patients, and both skin and mucous membranes in 27% of patients. CONCLUSIONS: The beneficial effect of smoking on pemphigus might be explained by its effect on the immune system. In addition, smoking has an antiestrogenic effect, while pesticides have an estrogenic effect. The lower numbers of smokers among patients, the higher exposure rates to pesticides, and the higher number of female patients who had been pregnant may point to the contribution of estrogens to the disease process. It remains to be determined whether measures, such as avoiding exposure to pesticides or metal vapor, may be beneficial in the clinical context. As the present study was a survey, more definitive studies should be conducted to validate the results.
We report a patient who presented with a papular pruritic eruption of a 3-month duration that histologically showed suprabasal acantholysis accompanied of an eosinophilic inflammatory infiltrate that was consistent with the diagnosis of Grover's disease. Later, erythematous plaques and vesicles appeared which showed a histopathological pattern of eosinophilic spongiosis. The direct immunofluorescence (DIF) study showed lineal IgG and C'3 at the dermal epidermal junction which was consistent with the diagnosis of bullous pemphigoid. No anti-intercellular deposits of immunoglobulin G (IgG) or C'3 were observed. We consider that suprabasal acantholysis may represent the early phase of bullous pemphigoid.
We report a patient with pernicious anaemia, primary autoimmune hypothyroidism and vitiligo, who presented with subcutaneous nodules. Histopathology of the nodules revealed noncaseating granulomas, consistent with a diagnosis of sarcoidosis. Mild pulmonary sarcoid was also detected. Although an association between sarcoidosis and other autoimmune diseases is well-recognized, the presence of the particular autoimmune diseases in our patient and the involvement of subcutaneous fat in the sarcoidal inflammation, appears to represent a most unusual clinicopathological combination.
We report the case of a patient with oral ulcerations that were likely traumatic in origin caused by biting of the tongue. Physical examination showed only deviation, fasciculations, and hemiatrophy of the right side of the tongue. Neurologic examination disclosed an isolated XII nerve palsy. A magnetic resonance image showed a lesion in the posterior fossa displacing the brain stem at a bulbar level. This was consistent with the diagnosis of a meningioma, later confirmed by the histopathologic study. It is very unusual that a meningioma produces a selective involvement of the XII nerve. Early diagnosis of a foramen magnum meningioma is important, both to improve prognosis and to avoid neurologic sequelae.
Angiomatous lesions appearing in chronic graft versus host disease (cGVHD) has been reported previously. We report a case of a patient in whom cutaneous exuberant granulation tissue developed in the course of a cGVHD after allo-peripheral blood progenitor cells transplantation.
Knowledge of autoimmune bullous diseases has greatly increased with the recognition of new entities, and the use of the direct immunofluorescence (DIF) using 1 molar per liter of sodium chloride (1 M NaCl) treated skin has been proposed. To estimate the frequency with which the different DIF patterns are present, we performed a systematic study of the skin or oral mucosa samples in which linear deposits of IgG at the basement membrane zone were detected by routine DIF in the last 6 years. The DIF tests were done on 56 samples before and after splitting the epidermis from the dermis with 1M NaCl. In 40 biopsies (72%) IgG was found on either the epidermal side or on both sides after 1M NaCl split. These cases corresponded to bullous pemphigoid (n=33), herpes gestationis (n=5) and cicatricial pemphigoid (n=2). In 6 cases (10.7%), IgG deposits were observed only on the floor, five corresponding to bullous pemphigoid and one to bullous pemphigoid-like eruption induced by amoxicillin. Repeat direct immunofluorescence using 1M NaCl split skin indicates that at least 12% of patients who were initially diagnosed as bullous pemphigoid, may in fact suffer a different entity, requiring other techniques to achieve the right diagnosis. This test can be a useful routine screening for autoimmune bullous diseases.
We report an 82-year-old man who presented with bullous pemphigoid and who later developed an acute glomerulonephritis with the histopathological and immunofluorescence pattern of a postinfectious glomerulonephritis. Antibodies directed against 230 and 180 kDa bullous pemphigoid antigens were detected in the patient's serum by means of the immunoprecipitation technique. Staphylococcus aureus and methicillin-resistant S. aureus were isolated from the patient's skin. We consider that in this particular patient the cutaneous infection played a part in the development of the kidney complication.
We report a 69-year-old female with erythroblastopenia and thymoma who developed lesions of Kaposi's sarcoma (KS) after thymectomy, 2 months after the initiation of therapy with methylprednisolone. Control of mucocutaneous KS lesions was obtained with radiotherapy, interferon alfa-2b and withdrawal of systemic immunosuppressive therapy. Erosive oral lichen planus appeared later, and after therapy with topical corticosteroids a new lesion of KS developed that regressed after withdrawal of topical corticosteroids. The detection of HHV-8 only in lesional skin supports the hypothesis that this virus can trigger the development of KS lesions.
We report three patients who developed a generalized rash with oral, genital or perianal ulcerations as a result of acute infection due to HIV. The primary infection was diagnosed by seroconversion (by means of EIA and Western blot techniques). Definitive diagnosis was established on days 52, 85 and 97 after the appearance of the rash. The p24 protein of the HIV was only detected in the early phase of the disorder in the two cases in which this study was carried out.
BACKGROUND: The prevalence of iron, folic acid and vitamin B12 deficiencies and their role in the development of recurrent oral ulcerations is not well known. The aim of this study was to determine the prevalence of these deficiencies in our patients. PATIENTS AND METHODS: Iron, folic acid and vitamin B12 levels were studied in 80 patients with recurrent oral ulcerations (ROU) and the results were compared with a control group of 29 patients with different oral diseases. RESULTS: In the recurrent oral ulcers patients, deficiencies were detected in 21/80 patients (26.2%). In 18 cases they were pure: iron (4), folic acid (10) and vitamin B12 (4). In 3 patients, combined deficiencies were detected, being secondary to pernicious anaemia in two patients. In the control group, deficiencies were observed in 4/29 cases (13.7%). In three cases they were isolated (one case suffered from ferropenic anaemia and two patients of pernicious anaemia). CONCLUSIONS: Patients with recurrent oral ulcerations have more frequently iron, folic acid and vitamin B12 deficiencies than those with other diseases of oral mucosa. However, there were not significant differences when the frequency of deficiency of each one of such elements were taken into account separately.
The histopathological changes observed in the cutaneous rash of three patients who suffered the acute phase of HIV infection are described. In all three patients a perivascular and interstitial inflammatory infiltrate was present in the upper and mid-reticular dermis. In one biopsy isolated areas of epidermal necrosis were observed and in the two other biopsies a perifollicular inflammatory infiltrate was detected with perforation in one case. Furthermore, a periductal infiltrate was observed in one of these biopsies.
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