The Experts below are selected from a list of 213 Experts worldwide ranked by ideXlab platform

Franco Rongioletti - One of the best experts on this subject based on the ideXlab platform.

  • atypical discrete papular Lichen Myxedematosus associated with monoclonal gammopathy report of four cases and review of the literature
    Journal of The European Academy of Dermatology and Venereology, 2016
    Co-Authors: C Hermans, I Goldscheider, Thomas Ruzicka, Franco Rongioletti
    Abstract:

    BackgroundDiscrete papular Lichen Myxedematosus (DPLM) is a rare form of localized Lichen Myxedematosus that presents with skin involvement only and without systemic involvement. ObjectTo describe our experience with atypical cases of DPLM associated with monoclonal gammopathy. MethodsData were collected from patients with clinicopathological evidence of DPLM associated with monoclonal gammopathy who presented to the Department of Dermatology of two tertiary university-affiliated medical centres from 2000 to 2015 and were followed prospectively. ResultsThe sample included four patients (three males) with a mean age of 58years. No clinicopathological differences from typical cases of DPLM were observed, except for the presence of monoclonal gammopathy. The patients were followed up for a mean of 34months (6-72months) and no progression to scleromyxedema, multiple myeloma or systemic involvement was observed. No therapy was applied, except for topical tacrolimus or steroids, and the eruptions remained stable. ConclusionOur experience indicates an excellent prognosis of DPLM even for atypical cases in spite of the presence of monoclonal gammopathy.

  • scleromyxedema with an interstitial granulomatous like pattern a rare histologic variant mimicking granuloma annulare
    Journal of Cutaneous Pathology, 2010
    Co-Authors: Franco Rongioletti, Emanuele Cozzani, Aurora Parodi
    Abstract:

    Scleromyxedema is the generalized and sclerodermoid form of Lichen Myxedematosus. Its typical histological features include a diffuse deposition of mucin in the papillary and mid reticular dermis, an increased of collagen deposition, and a proliferation of irregularly arranged fibroblasts. We describe a 76-year-old man presenting with scleromyxedema associated with IgGλ monoclonal gammopathy whose biopsy showed histological features of an interstitial granulomatous-like process consistent with interstitial granuloma annulare. The significance of these unusual granulomatous findings in the setting of scleromyxedema are unknown and have been described only once in the literature. This observation expands the spectrum of scleromyxedema and highlights the difficulty in diagnosing this disabling condition. Rongioletti F, Cozzani E, Parodi A. Scleromyxedema with an interstitial granulomatous-like pattern: a rare histologic variant mimicking granuloma annulare.

  • Lichen Myxedematosus papular mucinosis new concepts and perspectives for an old disease
    Seminars in Cutaneous Medicine and Surgery, 2006
    Co-Authors: Franco Rongioletti
    Abstract:

    Lichen Myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM. In the literature, the terms LM, papular mucinosis, and scleromyxedema often have been used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. Actually, LM includes 2 clinicopathologic subsets: a generalized papular and sclerodermoid form (the only one which should be called scleromyxedema) with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. The localized form is subdivided into 4 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) papular mucinosis of infancy, a pediatric variant of the discrete form or the acral form of persistent papular mucinosis; and (4) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the subtypes, and (4) not well-specified cases.

  • updated classification of papular mucinosis Lichen Myxedematosus and scleromyxedema
    Journal of The American Academy of Dermatology, 2001
    Co-Authors: Franco Rongioletti, Alfredo Rebora
    Abstract:

    Abstract Lichen Myxedematosus (LM) is an idiopathic cutaneous mucinosis; its classification dates back to 1953, when Montgomery and Underwood distinguished 4 types of LM: a generalized Lichenoid eruption, later called scleromyxedema, a discrete papular form, a localized or generalized Lichenoid plaque form, and an urticarial plaque form. In the literature, the terms LM, papular mucinosis, and scleromyxedema have been often used indiscriminately as synonyms, but most reported cases of LM or papular mucinosis without indication of the subtype appear in fact to be cases of scleromyxedema. On the basis of personal experience, the anatomoclinical manifestations of published cases of LM, papular mucinosis, and scleromyxedema are reviewed to distinguish clearly between a generalized form with systemic, even lethal, manifestations and a localized form, which does not run a disabling course. LM includes two clinicopathologic subsets: a generalized papular and sclerodermoid form (also called scleromyxedema) and a localized papular form. Diagnosis of scleromyxedema should fulfill the following criteria: (1) generalized papular and sclerodermoid eruption; (2) mucin deposition, fibroblast proliferation, and fibrosis; (3) monoclonal gammopathy; and (4) the absence of thyroid disease. The criteria for localized LM are as follows: (1) papular or nodular/plaque eruption; (2) mucin deposition with variable fibroblast proliferation; and (3) the absence of both monoclonal gammopathy and thyroid disease. The localized form is subdivided into 5 subtypes: (1) a discrete papular form involving any site; (2) acral persistent papular mucinosis involving only the extensor surface of the hands and wrists; (3) self-healing papular mucinosis, of a juvenile and an adult type; (4) papular mucinosis of infancy, a pediatric variant of the discrete form or of acral persistent papular mucinosis; and (5) nodular form. A third group of atypical or intermediate forms, not meeting the criteria for either scleromyxedema or the localized form, includes cases of (1) scleromyxedema without monoclonal gammopathy, (2) localized forms with monoclonal gammopathy and/or systemic symptoms, (3) localized forms with mixed features of the 5 subtypes, and (4) not well-specified cases. (J Am Acad Dermatol 2001;44:273-81.)

  • worsening of Lichen Myxedematosus during interferon alfa 2a therapy for chronic active hepatitis c
    Journal of The American Academy of Dermatology, 1998
    Co-Authors: Franco Rongioletti, A Rebora
    Abstract:

    A 59-year-old woman with an asymptomatic papular eruption on both her upper limbs was diagnosed with Lichen Myxedematosus (LM). In the absence of any treatment, the disease remained stable for 18 years. One year after the onset of LM, she underwent three blood transfusions for major surgery. Chronic active HCV+ hepatitis was diagnosed in 1995. In 1996, examination revealed multiple 2 to 4 mm flesh-colored papules confined to her arms and forearms. No induration or thickening of the skin was seen. Laboratory studies including cryoglobulins, serum protein electrophoresis and immunoelectrophoresis, T3, T4, and TSH were normal. Antimicrosomes and antithyroglobulin antibodies were absent. Hepatitis C serology was positive. In a skin biopsy specimen, the Alcian blue stain at pH 2.5 disclosed an abundant mucin deposition. Again, no therapy was given for her skin lesions. Her transaminase blood level remained elevated for at least 6 months, so treatment with recombinant IFNα2a (Roferon A), 6 million IU 3 times a week, was begun. One month later, the skin lesions had increased in number and had spread to the shoulders, thighs, and trunk (Fig. 1). The IFN dosage was reduced to 3 million IU 3 times weekly, but new papules kept appearing, although to a lesser degree. When IFN was stopped the skin disease remained stable.

Madeleine Duvic - One of the best experts on this subject based on the ideXlab platform.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation
    Blood, 2006
    Co-Authors: Michele Donato, Adrienne M Feasel, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus–scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation commentary
    Blood, 2006
    Co-Authors: Morie A. Gertz, Adrienne M Feasel, Michele Donato, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m 2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus-scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

Michele Donato - One of the best experts on this subject based on the ideXlab platform.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation
    Blood, 2006
    Co-Authors: Michele Donato, Adrienne M Feasel, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus–scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation commentary
    Blood, 2006
    Co-Authors: Morie A. Gertz, Adrienne M Feasel, Michele Donato, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m 2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus-scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

Adrienne M Feasel - One of the best experts on this subject based on the ideXlab platform.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation
    Blood, 2006
    Co-Authors: Michele Donato, Adrienne M Feasel, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus–scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation commentary
    Blood, 2006
    Co-Authors: Morie A. Gertz, Adrienne M Feasel, Michele Donato, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m 2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus-scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

Donna M Weber - One of the best experts on this subject based on the ideXlab platform.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation
    Blood, 2006
    Co-Authors: Michele Donato, Adrienne M Feasel, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus–scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.

  • scleromyxedema role of high dose melphalan with autologous stem cell transplantation commentary
    Blood, 2006
    Co-Authors: Morie A. Gertz, Adrienne M Feasel, Michele Donato, Donna M Weber, Sergio Giralt, Richard E. Champlin, Victor G Prieto, Madeleine Duvic
    Abstract:

    Scleromyxedema, the most severe manifestation of the spectrum of Lichen Myxedematosus, is characterized by cutaneous mucinosis, extracutaneous manifestations, and a monoclonal gammopathy. Seven of 8 patients evaluated at our center were treated with high-dose melphalan (180 mg/m 2 intravenously) and autologous peripheral blood stem cell transplantation, with marked improvement of gastrointestinal, central nervous system, pulmonary manifestations, and Karnofsky performance status. Five patients obtained a cutaneous complete remission and 2 patients had partial remissions. Three patients with slight progression in the skin at 12, 8, and 4 months after treatment received a second cycle of high-dose melphalan and had further symptomatic improvement. The Lichen Myxedematosus-scleromyxedema spectrum appears to be a continuum that requires the presence of a serum paraprotein and differs in severity of skin lesions, extracutaneous manifestations, and performance status. High-dose melphalan followed by autologous transplantation appears effective for improving the symptoms and systemic manifestations of scleromyxedema.