Henoch-Schönlein purpura (HSP) during treatment with anastrozole.
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Biomedical subjects
Publications and source records attributed to C Mainetti.
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BACKGROUND: Localized vulval childhood pemphigoid is a rare variant within the pemphigoid group. Although its prognosis seems favorable, the best therapeutic strategy remains unclear. OBSERVATION: We here describe the case of an 8-year-old girl presenting with a 5-year history of relapsing vulval pain and lesions suggestive of lichen sclerosus. Clinical features, light microscopy and direct immunofluorescence microscopy were consistent with vulval cicatricial pemphigoid, although the autoantigen(s) involved could not be characterized. Her disease responded to treatment with topical tacrolimus ointment 0.1% within 3 months without any evidence for disease activity, except for slight residual scarring. After 12 months, her treatment was stopped without relapse. CONCLUSION: This observation suggests that in this rare immune-mediated blistering disease topical tacrolimus is an interesting therapeutic option without the adverse effects associated with topical steroids.
Classical Kaposi's sarcoma is a sporadic disease with a markedly higher incidence in the Mediterranean area. The two original forms of this disease, the Caucasian (classical) and the African, are now well-documented entities. The immunosuppression-related form is a new, recently described subset of the disease, arising from the human immune deficiency virus (HIV) infection and/or the widespread use of immunosuppressive treatments. Cutaneous lesions are the most common manifestation of classical Kaposi's sarcoma. The case described in the present report is characterised by a history of slowly evolving disease affecting the lower extremities without spread to thoracic or abdominal organs, a lengthy remission following initial radiation therapy, and local relapse with a further remission after a second course of radiation. Current treatments, in particular radiation therapy, achieve disease control or at least control of disease-related symptoms (mainly pain) in most cases, even when cure is not possible.
Primary cutaneous B-cell lymphomas have been associated with Borrelia burgdorferi, the spirochete responsible for Lyme disease. Recently, cutaneous marginal zone B-cell lymphoma has been proposed as a distinct clinical-pathological entity. We report a case of primary cutaneous marginal zone lymphoma, associated with B burgdorferi infection. Polymerase chain reaction (PCR) amplification of the third complementarity determining region (CDR3) of the immunoglobulin heavy chain gene showed the presence of a monoclonal lymphoproliferation, therefore strengthening the histological diagnosis of a malignant process. B burgdorfer-specific hbb gene sequences were detected by PCR in the lymphoma tissue at diagnosis but not after antibiotic treatment. A nearly complete clinical and histological regression was observed after B burgdorferi eradication, with immunohistochemistry studies showing disappearance of plasma cell differentiation and a marked decline in the number of CD3+ T cells and Ki-67+ cells. Our case confirms the link between B burgdorferi and some cutaneous lymphomas. The disappearance of the microorganism accompanied by the unequivocal decrease of most indicators of active T- and B-cell immune response strongly supported a pathogenetic role for B burgdorferi in sustaining an antigen-driven development and growth of this cutaneous marginal zone lymphoma. Antibiotic therapy (analogous to Helicobacter pylori infection in gastric MALT lymphoma) might be helpful with the aim of averting or at least deferring the indication for more aggressive treatment.
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Several growth factors, present in the plasma or delivered by blood cells, are involved in wound healing. We have treated 15 chronic atonic leg ulcers by topical application of autologous heparinized blood under a hydrocolloidal dressing. This 'topical haemotherapy' (THT) was applied every other day. After the first THT, 30% of the fibrinonecrotic material had already been removed. After a mean of 9 THT applications (range: 5-18), granulation tissue covered up to 75% of the surface of the ulcer (range: 30-100%) allowing autologous skin grafting. No local or systemic side-effects occurred during the treatment. THT is feasible and well tolerated; it is an easy and inexpensive treatment of chronic leg ulcers that rapidly induces granulation tissue. Growth factors and proteases produced by the blood cells could play a relevant role in this process.
We report a case of solid persistent facial oedema in a 18-year-old woman. A biopsy specimen from the nose demonstrating lympho-epithelioid granulomas and an electro-neuro-myographic examination revealing sub-clinical sequellae from an old right facial palsy, led to the definite diagnosis of Melkersson-Rosenthal syndrome (MRS). We focus on the differential diagnosis of persistent facial oedema and outline the difficulties to establish the diagnosis of MRS when all features of the classic triad "recurrent labial edema-facial nerve palsy-lingua plicata" are not present.
A 57-year-old woman with L-tryptophan-induced eosinophilia-myalgia syndrome, 23 months after the onset of symptoms, experienced development of a parasternal malignant fibrous histiocytoma on previously scleroderma-like skin areas and, almost concurrently, an extraabdominal desmoid tumor of the left arm muscle fascia. The malignant fibrous histiocytoma was treated by surgical excision without sign of recurrence or metastasis 19 months later. Radiation therapy was performed on the extraabdominal desmoid tumor. We suspect that these two connective tissue tumors in this patient were related to the exposure to contaminated L-tryptophan, which interfered with connective tissue metabolism.
In an attempt to stop the evolution of recent-onset severe alopecia areata (AA), we tested pulse corticotherapy on 9 patients. Acceptance into the study was based on the following criteria: recent-onset AA (< 1 year), AA in an active state, bald surface > 30% of the scalp, no contraindication to pulse corticotherapy. Each patient was given 250 mg i.v. of methylprednisolone twice a day on 3 successive days. In 8 patients the course of the ongoing episode of AA was stopped. At the 6-month follow-up, a regrowth on 80-100% of the bald surface was observed in 6 patients. One patient did not respond to treatment, and 2 had less than 50% of regrowth. This open study suggests that pulse corticotherapy: (1) can stop the course of severe AA in an active state, (2) is well tolerated without major side effects and (3) does not permit a stable control of AA of more than 1 year duration. This treatment seems to be indicated for severe AA of recent onset.
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Of 583 erysipelas/cellulitis seen in our Department between 1981 and 1991, six (1.04%) were localized to the buttock and hip. Five patients had had a dynamic hip screw implanted for coxarthritis on the side where the skin infection developed. This does not appear to be secondary to superinfection of the surgical wound as the erysipelas/cellulitis occurred weeks (7 to 520) after surgery. The possible surgical compromise of the venous/lymphatic circulation appears to be the causative factor. In all patients, the course was satisfactory upon systemic antibiotic therapy and no recurrence was seen after a mean follow up of 28 months.
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Abnormal metabolism of tryptophan is one of the possible aetiological factors in the L-tryptophan-induced eosinophilia-myalgia syndrome (EMS). We studied the plasma levels of tryptophan and serotonin and the urinary excretion of kynurenine and 5-hydroxyindoleacetic acid after oral intake of L-tryptophan in 1 subject with EMS and 2 healthy subjects. The test was repeated with concomitant administration of pyridoxine. In the patient there were elevated levels of plasma tryptophan during the loading and increased elimination of kynurenine in the urine both during and after the L-tryptophan test. During pyridoxine administration tryptophan levels and kynurenine elimination were much reduced, and kynurenine elimination was similar to that of controls. This study (i) confirms that an abnormal metabolism of L-tryptophan occurs in EMS patients and (ii) shows that this can be corrected by pyridoxine.
Two female patients who fulfilled the criteria for L-tryptophan-induced eosinophilia-myalgia syndrome (EMS) had, together with morphea-like and fasciitis-like sclerotic changes of the skin, lesions that clinically mimicked pseudoxanthoma elasticum (PXE). Histology was compatible with the diagnosis; electron microscopy did not reveal calcium deposits. PXE-like changes may represent an additional feature of the pleomorphic L-tryptophan-induced EMS.
Tuberculous lesions of the skin occur rarely nowadays. We therefore have mostly lost the knowledge to recognize this entity. Furthermore, the great variability of the clinical and particularly dermatologic manifestations of the disease can tax the most astute clinician. The challenge is even greater, when the patient has an intercurrent condition such as a malignancy or AIDS. In order to make understanding easier, we replace all descriptive terms, some carried over from the last century, by a useful pathogenetic and clinical, algorithmic classification. Finally the necessity of biopsy and specific cultures for proper diagnosis and treatment of any skin lesion consistent with skin tuberculosis is emphasized.