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Stefano Sivolella - One of the best experts on this subject based on the ideXlab platform.

  • Melkersson–Rosenthal Syndrome: a case report of a rare disease with overlapping features
    2019
    Co-Authors: Mauro Cancian, Stefano Giovannini, Annalisa Angelini, Marny Fedrigo, Raffaele Bendo, Riccardo Senter, Stefano Sivolella
    Abstract:

    Abstract Background Melkersson–Rosenthal Syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. Case presentation A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphospholipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal Syndrome, which was confirmed by histologic findings of non caseating granulomas on lip biopsy. Conclusion Melkersson–Rosenthal Syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal biopsy to establish a correct diagnosis and prescribe targeted therapy

  • Melkersson Rosenthal Syndrome a case report of a rare disease with overlapping features
    2019
    Co-Authors: Mauro Cancian, Stefano Giovannini, Annalisa Angelini, Marny Fedrigo, Raffaele Bendo, Riccardo Senter, Stefano Sivolella
    Abstract:

    Melkersson–Rosenthal Syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphospholipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal Syndrome, which was confirmed by histologic findings of non caseating granulomas on lip biopsy. Melkersson–Rosenthal Syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal biopsy to establish a correct diagnosis and prescribe targeted therapy.

Andrea Peserico - One of the best experts on this subject based on the ideXlab platform.

  • successful treatment of Melkersson Rosenthal Syndrome with lymecycline
    2004
    Co-Authors: Barbara Pigozzi, Anna Belloni Fortina, Andrea Peserico
    Abstract:

    The cause of Melkersson-Rosenthal Syndrome, a granulomatous, inflammatory disease is still unknown. Many treatments have been tried with variable and often disappointing results. We report the case of a 31-year-old woman affected by Melkersson-Rosenthal Syndrome, who has been successfully treated with lymecycline, after variable results with steroids alone or combined with antihistamines, sulphasalazine and clofazimine.

A Carrizosa - One of the best experts on this subject based on the ideXlab platform.

Mauro Cancian - One of the best experts on this subject based on the ideXlab platform.

  • Melkersson–Rosenthal Syndrome: a case report of a rare disease with overlapping features
    2019
    Co-Authors: Mauro Cancian, Stefano Giovannini, Annalisa Angelini, Marny Fedrigo, Raffaele Bendo, Riccardo Senter, Stefano Sivolella
    Abstract:

    Abstract Background Melkersson–Rosenthal Syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. Case presentation A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphospholipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal Syndrome, which was confirmed by histologic findings of non caseating granulomas on lip biopsy. Conclusion Melkersson–Rosenthal Syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal biopsy to establish a correct diagnosis and prescribe targeted therapy

  • Melkersson Rosenthal Syndrome a case report of a rare disease with overlapping features
    2019
    Co-Authors: Mauro Cancian, Stefano Giovannini, Annalisa Angelini, Marny Fedrigo, Raffaele Bendo, Riccardo Senter, Stefano Sivolella
    Abstract:

    Melkersson–Rosenthal Syndrome (MRS) is a rare, neuro-mucocutaneous disease which presents as orofacial swelling, facial palsy and fissured tongue. These symptoms may occur simultaneously or, more frequently, with a oligosymptomatic or monosymptomatic pattern. Swelling, that is the most common initial finding, may mimic hereditary or acquired angioedema, a disorder caused by histamine or bradykinin-mediated plasma-leakage affecting subcutaneous and/or submucosal tissue. The differential diagnosis of MRS includes also chronic inflammatory and infective diseases characterized by granulomatous infiltration, as well as rosacea, contact dermatitis, allergic reactions and Bell’s palsy. A 71-year old, non-allergic female patient with no familial and personal history of angioedema presented, a few days after a possible herpes simplex or varicella-zoster virus infection, with monolateral facial paraesthesia and lower lip edema. After temporary remission of symptoms on oral steroids and antihistamines, she showed swelling recurrence refractory to valaciclovir therapy and a subsequent course of antihistamines. The clinical picture and a previous history of non-Hodgkin lymphoma prompted us to rule out an acquired form of paraneoplastic, C1-inhibitor (C1-INH) deficiency: C1q and both antigen and functional C1-INH tested normal, whilst we found low plasma levels of C3 and C4 possibly related to the parallel detection of antiphospholipid antibodies. Thus, we hypothesized a non-histaminergic, idiopathic form of angioedema and planned further therapy with tranexamic acid and the leukotriene receptor antagonist montelukast. Treatment failure with both drugs finally suggested a Melkersson–Rosenthal Syndrome, which was confirmed by histologic findings of non caseating granulomas on lip biopsy. Melkersson–Rosenthal Syndrome may occur with rather non-specific symptoms and overlap with alternative conditions, including recurrent angioedema. No specific biomarkers for MRS exist and clinical diagnosis is often of exclusion. The finding of complement or immune alterations, as in our patient, may be further confounding and justify the need for skin or mucosal biopsy to establish a correct diagnosis and prescribe targeted therapy.

Barbara Pigozzi - One of the best experts on this subject based on the ideXlab platform.

  • successful treatment of Melkersson Rosenthal Syndrome with lymecycline
    2004
    Co-Authors: Barbara Pigozzi, Anna Belloni Fortina, Andrea Peserico
    Abstract:

    The cause of Melkersson-Rosenthal Syndrome, a granulomatous, inflammatory disease is still unknown. Many treatments have been tried with variable and often disappointing results. We report the case of a 31-year-old woman affected by Melkersson-Rosenthal Syndrome, who has been successfully treated with lymecycline, after variable results with steroids alone or combined with antihistamines, sulphasalazine and clofazimine.