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

  • Cost of Intravenous Immunoglobulin Therapy Versus Conventional Immunosuppressive Therapy in Patients with Mucous Membrane Pemphigoid: A Preliminary Study
    2016
    Co-Authors: Yassine Daoud, Krishn Mohan, Ketan G Amin, Razzaque A Ahmed
    Abstract:

    Cicatricial pemphigoid, also known as mucous mem-brane pemphigoid (MMP), is a chronic systemic au-toimmune vesiculobullous disease characterized by the presence of autoantibodies to proteins in the basement membrane zone of mucosal tissues and the skin.1,2 It is a disease of the elderly, with a mean age of onset of 60 years.2 The mucosal membranes most frequently involved are oral, ocular, nasal, pharyngeal, laryngeal, tracheal, pe-nile, anal, and vaginal. The clinical diagnosis is confirmed by histologic, immunopathologic, and serologic studies. MMP may have serious complications, including blind-ness and death secondary to sudden asphyxiation. The sig-nificant and unusual aspect of MMP is that, as the blisters heal, they are followed by scarring. Consequently, fibrosis and stenosis of the nose, esophagus, vagina, penis, and anus create significant medical problems. Such sequelae have an unquestionable impact on quality of life and activ-ities of daily living. In most patients, the disease is chronic, can last for several years, and is debilitating

  • chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita
    Archives of Ophthalmology, 2006
    Co-Authors: Erik Letko, Razzaque A Ahmed, Kailash C. Bhol, Fahd Anzaar, Victor L Perez, Stephen C Foster
    Abstract:

    Objective To describe a nonconventional diagnostic technique used to diagnose a case of cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita. Methods Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, Intravenous Immunoglobulin Therapy was administered. Results Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of Intravenous Immunoglobulin Therapy and remained stable during follow-up. Conclusions Epidermolysis bullosa acquisita can manifest in the eye as chronic cicatrizing conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic cicatrizing conjunctivitis. Intravenous Immunoglobulin Therapy was effective against chronic cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita.

  • cost of Intravenous Immunoglobulin Therapy versus conventional immunosuppressive Therapy in patients with mucous membrane pemphigoid a preliminary study
    Annals of Pharmacotherapy, 2005
    Co-Authors: Yassine J Daoud, Ketan G Amin, Krishn Mohan, Razzaque A Ahmed
    Abstract:

    BACKGROUNDIntravenous Immunoglobulin (IVIG) is an expensive biologic agent used to treat patients with mucous membrane pemphigoid (MMP) nonresponsive to conventional immunosuppressive Therapy (CIST). The high cost of IVIG is of concern to healthcare providers and insurance companies.OBJECTIVETo compare the cost of IVIG with that of CIST in treating a cohort of 15 patients with severe and extensive MMP.METHODSFifteen patients with biopsy-proven MMP nonresponsive to CIST were subsequently treated with IVIG and demonstrated a positive clinical response. This was a comparative, retrospective study; the mean total duration of the observation period was 8.4 years. A comparison of the cost of IVIG with that of CIST during the study period and the annual cost was performed. The cost of CIST was defined as the actual cost of the drug plus the cost of management of the multiple adverse effects, including hospitalizations, produced by CIST. In the same patient cohort, no significant adverse effects to IVIG were obse...

  • consensus statement on the use of Intravenous Immunoglobulin Therapy in the treatment of autoimmune mucocutaneous blistering diseases
    Archives of Dermatology, 2003
    Co-Authors: Razzaque A Ahmed, Mark V Dahl
    Abstract:

    Objectives The purpose of the meeting of the Consensus Development Group was to critically evaluate the current published data on the use of Intravenous Immunoglobulin (IVIg) Therapy in the treatment of autoimmune mucocutaneous blistering diseases (AMBDs) and to discuss the industrial preparation and safety features of this biologic agent. Participants The participants were physicians who frequently treat patients with these diseases and included dermatologists, oral medicine specialists, ophthalmologists, and immunologists. The members of the group provided input and discussion in their areas of expertise. The participants were invited attendees. Evidence Data samples included only published information in the English-language literature. The expert opinions and experience of the members of the Consensus Development Group were vital to the discussion. Consensus Process A consensus was achieved by an open discussion and cumulative agreement on all issues relevant to the use of IVIg Therapy in the treatment of AMBDs. Special emphasis was placed on indications for its use, determination of outcome parameters, and development of a protocol for its therapeutic use. We also focused on its safety and on prevention of adverse effects. Conclusion This consensus statement outlines the scope of IVIg treatment; provides guidelines for its use, including indications, prescreening, premedications, dose, frequency, and monitoring; and defines the end point of Therapy.

  • Influence of Intravenous Immunoglobulin Therapy on autoantibody titers to desmoglein 3 and desmoglein 1 in pemphigus vulgaris
    European journal of dermatology : EJD, 2003
    Co-Authors: Naveed Sami, Kailash C. Bhol, Razzaque A Ahmed
    Abstract:

    Pemphigus vulgaris (PV) is an autoimmune mucocutaneous blistering disease. Recently, patients with mucosal involvement have been described to have autoantibodies to desmoglein 3 (dsg), while patients with mucocutaneous disease have autoantibodies to dsg 1 and dsg 3. The objective of this study was to prospectively analyze, over a 24-month period, the influence of Intravenous Immunoglobulin (i.v.Ig) Therapy on autoantibody titers to dsg 3 and dsg 1, in two groups of patients with severe PV. Group A consisted of 11 patients with mucocutaneous involvement and group B consisted of 10 patients with only mucosal involvement. Levels of autoantibodies to dsg 3 and 1 were measured by ELISA, at monthly intervals. Prior to Therapy initiation, group A patients' sera showed a high ELISA index value of both dsg 3 and 1 antibodies, while group B patients had a high index value to only dsg 3. During i.v.Ig Therapy, a progressive decline in the ELISA index values was observed in all patients. After the initiation of i.v.Ig Therapy, in group A, a statistically significant reduction (p < 0.05) in ELISA index value to dsg 3 and 1 was detected after four and five months, respectively. In Group B, a significant decline in the mean autoantibody titer values to dsg 3 (p < 0.05) was observed after six months of i.v.Ig Therapy. Group A patients had a negative ELISA index value to dsg 3 and 1 after a mean period of 21 and 20 months, respectively. Group B patients had a negative dsg 3 score after a mean period of 22 months. These results indicate that autoantibody titers to dsg 3 and 1, as measured by ELISA, can be used to monitor the serological response to treatment in PV patients. A sustained serological remission is observed in patients treated with i.v.Ig Therapy. .

Srini Kaveri - One of the best experts on this subject based on the ideXlab platform.

  • monoclonal antibody and Intravenous Immunoglobulin Therapy for rheumatic diseases rationale and mechanisms of action
    Nature Reviews Rheumatology, 2007
    Co-Authors: Jagadeesh Bayry, Michel D Kazatchkine, Sebastien Lacroixdesmazes, Srini Kaveri
    Abstract:

    Advances in our understanding of the pathogenesis of rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus have led to the emergence of Immunoglobulin-based Therapy as a major therapeutic force. Numerous monoclonal antibodies that target proinflammatory cytokines or their receptors (e.g. infliximab, adalimumab, tocilizumab, belimumab, HuMax-IL-15), and cell-surface or co-stimulatory molecules (e.g. rituximab) are either in clinical development or have been approved for clinical use. These antibodies are safe and effective in the long-term Therapy of many rheumatic diseases. In addition, polyclonal Immunoglobulins (Intravenous Immunoglobulin) obtained from pooled plasma from healthy blood donors are an effective therapeutic approach in certain rheumatic diseases. The mechanisms of action of monoclonal antibodies and Intravenous Immunoglobulin include cytolysis of target cells through complement or antibody-dependent cell-mediated cytotoxicity, induction of apoptosis of target cells, blockade of co-stimulatory molecules, and neutralization of pathogenic antibodies and soluble factors such as cytokines and their receptors, which ultimately lead to amelioration of the inflammatory process. The success of currently available therapeutic Immunoglobulins has led to considerable interest in the identification of novel molecular therapeutic targets in rheumatic diseases.

  • monoclonal antibody and Intravenous Immunoglobulin Therapy for rheumatic diseases rationale and mechanisms of action
    Nature Reviews Rheumatology, 2007
    Co-Authors: Jagadeesh Bayry, Michel D Kazatchkine, Sebastien Lacroixdesmazes, Srini Kaveri
    Abstract:

    Immunoglobulin-based therapies, including monoclonal antibodies and Intravenous Immunoglobulin, are effective therapeutic approaches for patients with rheumatic diseases who do not respond to conventional anti-inflammatory drugs. Several such therapies have been approved for clinical use; the mechanisms of action of these therapies are discussed in this Review. Advances in our understanding of the pathogenesis of rheumatic diseases such as rheumatoid arthritis and systemic lupus erythematosus have led to the emergence of Immunoglobulin-based Therapy as a major therapeutic force. Numerous monoclonal antibodies that target proinflammatory cytokines or their receptors (e.g. infliximab, adalimumab, tocilizumab, belimumab, HuMax-IL-15), and cell-surface or co-stimulatory molecules (e.g. rituximab) are either in clinical development or have been approved for clinical use. These antibodies are safe and effective in the long-term Therapy of many rheumatic diseases. In addition, polyclonal Immunoglobulins (Intravenous Immunoglobulin) obtained from pooled plasma from healthy blood donors are an effective therapeutic approach in certain rheumatic diseases. The mechanisms of action of monoclonal antibodies and Intravenous Immunoglobulin include cytolysis of target cells through complement or antibody-dependent cell-mediated cytotoxicity, induction of apoptosis of target cells, blockade of co-stimulatory molecules, and neutralization of pathogenic antibodies and soluble factors such as cytokines and their receptors, which ultimately lead to amelioration of the inflammatory process. The success of currently available therapeutic Immunoglobulins has led to considerable interest in the identification of novel molecular therapeutic targets in rheumatic diseases.

  • skin Immunoglobulin deposition following Intravenous Immunoglobulin Therapy in toxic epidermal necrolysis
    Experimental Dermatology, 2006
    Co-Authors: Philippe Paquet, Eric Jacob, Jean Pirson, Srini Kaveri, Pascale Quatresooz, Gérald Pierard
    Abstract:

    :  Human Intravenous Immunoglobulins (IVIg) which contain anti-CD95 antibodies have been proposed to treat toxic epidermal necrolysis (TEN). Presently, there is no evidence that IVIg reach the keratinocytes in TEN patients. The aim of this study was to assess the Ig distribution in the serum, blister fluid and skin of six consecutive TEN patients treated with IVIg (1 g/kg/day) for 3 days. They were compared with five TEN patients who only received supportive Therapy. In all patients, IgA, IgM and IgG concentrations were measured in the serum and blister fluid using an immuno-nephelometric method. Immunohistochemistry was performed on skin biopsies taken from both TEN clinically involved and uninvolved skin to search for IgG deposits. On admission, the IgG concentrations were significantly higher in both TEN serum and TEN blister fluid compared with their respective IgA and IgM contents. The IgG, IgA and IgM concentrations in blister fluid were significantly lower than their respective serum concentrations. The serum and blister fluid IgG concentrations, but not that of IgA and IgM, were markedly increased at the completion of the IVIg treatment. By contrast, they remained unchanged in the TEN patients that were untreated with IVIg. In the IVIg-treated patients, the IgG intraepidermal deposits raised markedly in both TEN-involved and uninvolved skin. This was not the case in patients who did not receive IVIg. These results suggest that IVIg perfusions brought a prominent increase in IgG concentration in the serum, blister fluid and epidermis of both TEN-involved and clinically uninvolved skin. The presence of potentially protective IgG in TEN epidermis following IVIg treatment could help limiting the disease progression.

Gen Sobue - One of the best experts on this subject based on the ideXlab platform.

  • acute superficial sensory neuropathy with generalized anhidrosis anosmia and ageusia
    Muscle & Nerve, 2011
    Co-Authors: Yu Miyazaki, Mizuki Ito, Naoki Atsuta, Haruki Koike, Masahisa Katsuno, Hirohisa Watanabe, Susumu Kusunoki, Gen Sobue
    Abstract:

    We report a 53-year-old woman with an unusual presentation characterized by acute onset of generalized sensory symptoms associated with anhidrosis, anosmia, ageusia, and elevated titers of anti–GalNAc-GD1a antibodies. After Intravenous Immunoglobulin Therapy, a remarkable improvement in the pain and temperature sensation was noted in her face, trunk, and extremities. The impaired pain and temperature sensation remained distributed along cranial and spinal dermatomes in a non–length-dependent manner, suggesting that the ganglionopathy affected small neurons. Muscle Nerve 43:287–289, 2011

  • acute superficial sensory neuropathy with generalized anhidrosis anosmia and ageusia
    Muscle & Nerve, 2011
    Co-Authors: Yu Miyazaki, Mizuki Ito, Naoki Atsuta, Haruki Koike, Masahisa Katsuno, Hirohisa Watanabe, Susumu Kusunoki, Gen Sobue
    Abstract:

    We report a 53-year-old woman with an unusual presentation characterized by acute onset of generalized sensory symptoms associated with anhidrosis, anosmia, ageusia, and elevated titers of anti-GalNAc-GD1a antibodies. After Intravenous Immunoglobulin Therapy, a remarkable improvement in the pain and temperature sensation was noted in her face, trunk, and extremities. The impaired pain and temperature sensation remained distributed along cranial and spinal dermatomes in a non-length-dependent manner, suggesting that the ganglionopathy affected small neurons.

Stephen C Foster - One of the best experts on this subject based on the ideXlab platform.

  • chronic cicatrizing conjunctivitis in a patient with epidermolysis bullosa acquisita
    Archives of Ophthalmology, 2006
    Co-Authors: Erik Letko, Razzaque A Ahmed, Kailash C. Bhol, Fahd Anzaar, Victor L Perez, Stephen C Foster
    Abstract:

    Objective To describe a nonconventional diagnostic technique used to diagnose a case of cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita. Methods Direct immunofluorescence of a biopsy specimen of the patient's conjunctiva was performed using fluorescein-conjugated rabbit antihuman antibodies against IgA, IgG, and IgM; complement C3; and fibrinogen. Immunoblot assay using healthy human skin as substrate was performed to investigate for the presence of antibodies in the patient's serum. After the diagnosis of systemic autoimmune disease was established, Intravenous Immunoglobulin Therapy was administered. Results Direct immunofluorescence of the conjunctiva revealed linear deposition of IgA and IgG, and C3 at the epithelial basement membrane zone. Immunoblot analysis demonstrated the presence of IgG antibodies in patient serum directed against a 290-kDa protein in human skin. A diagnosis of epidermolysis bullosa acquisita was established. All signs and symptoms improved dramatically 4 months after initiation of Intravenous Immunoglobulin Therapy and remained stable during follow-up. Conclusions Epidermolysis bullosa acquisita can manifest in the eye as chronic cicatrizing conjunctivitis indistinguishable from ocular cicatricial pemphigoid. A nonconventional diagnostic tool (immunoblot assay) might be helpful in establishing the diagnosis of an underlying systemic autoimmune disease in patients with chronic cicatrizing conjunctivitis. Intravenous Immunoglobulin Therapy was effective against chronic cicatrizing conjunctivitis associated with epidermolysis bullosa acquisita.

  • ocular cicatricial pemphigoid keratomycosis and Intravenous Immunoglobulin Therapy
    Cornea, 2004
    Co-Authors: Barbara Iaccheri, Stefanos Baltatzis, Thekla Papadaki, Manolette Roque, Tito Fiore, Benjamin Mathew, Barry Emara, A C Tokarewicz, Stephen C Foster
    Abstract:

    PURPOSE To report the case of a patient developing fungal keratitis in the context of uncontrolled ocular cicatricial pemphigoid (OCP), which, despite Intravenous Immunoglobulin (IVIg) and other immunomodulatory Therapy, progressed to end-stage pemphigoid, with corneal opacification, ankyloblepharon, and xerosis. Keratoprosthesis (KPro) restored functional vision for the patient. METHODS A 39-year-old man presented with uncontrolled CP and corneal ulcer in the left eye. Conjunctival biopsy diagnosed OCP; corneal scraping and biopsy diagnosed the cause of the corneal ulcer. OCP was treated with systemic steroids, immunosuppressive drugs, and IVIg. Visual rehabilitation was accomplished with Ahmed valve and a type II Dohlman KPro. RESULTS Immunohistology of the biopsied conjunctiva showed IgG at the epithelial basement membrane zone, confirming the clinical diagnosis of OCP. Microbiologic studies of the corneal biopsy specimen were negative for Acanthamoeba and herpes but positive for Aspergillus niger. The patient's keratomycosis resolved with topical antifungal Therapy. Treatment with Dapsone, Intravenous-pulse steroid, oral cyclophosphamide, and Intravenous Immunoglobulin (IVIg) failed to control the OCP, with resultant complete conjunctivization of the cornea. Keratoprosthesis improved the patient's visual acuity from hand movements to 20/20. CONCLUSIONS Patients with uncontrolled OCP are at increased risk of corneal infection. The difficulty in diagnosing keratomycosis and the relatively rare occurrence of OCP explain the uniqueness of our reported case. OCP may progress to "end-stage" disease despite Therapy. Keratoprosthesis can restore vision in selected otherwise seemingly hopeless cases.

  • linear iga bullous disease limited to the eye a diagnostic dilemma response to Intravenous Immunoglobulin Therapy
    Ophthalmology, 2000
    Co-Authors: Erik Letko, Kailash C. Bhol, Stephen C Foster, Razzaque A Ahmed
    Abstract:

    Abstract Purpose To report on a diagnostic dilemma and treatment challenge in a patient with chronic cicatrizing conjunctivitis without involvement of skin and other mucous membranes persisting for 6 years and not responding to topical and systemic steroids. Design Interventional case report. Methods We performed direct immunofluorescence of the conjunctiva with fluorescein-conjugated rabbit antihuman antibodies against Immunoglobulin A, G, and M, complement 3 component, and fibrinogen. To investigate the presence of circulating antibodies in patient's serum, indirect immunofluorescence using normal human conjunctiva, normal human skin, and monkey esophagus as substrate was done. In addition, we did immunoblot analysis using normal human epidermis as substrate to determine the molecular weight of an antigen. The patient was treated with Intravenous Immunoglobulin (IVIg). The correlation between the titer of circulating antibodies and the activity of conjunctival inflammation at various intervals during the course of IVIg Therapy was demonstrated by immunoblot assay with serial dilutions of the patient's serum. The highest dilution at which the binding was visible was considered the titer. Results Direct immunofluorescence of the conjunctiva and indirect immunofluorescence with both salt split skin and conjunctiva as substrate disclosed linear deposition of Immunoglobulin A (IgA) at the epithelial basement membrane. Immunoblot analysis demonstrated the presence of IgA circulating antibodies in patient's serum directed against a 97kDa protein in human epidermis. A continuous decrease in the titer of these antibodies correlating to improvement of clinical symptoms was observed during IVIg Therapy. Conclusions Use of a nonconventional diagnostic tool (immunoblot analysis), in addition to conventional immunohistologic studies, might be helpful in establishing the diagnosis of patients with chronic cicatrizing conjunctivitis. On the basis of results of these laboratory tests and clinical presentation, we believe that this patient has linear IgA bullous disease limited to the eye. IVIg Therapy decreased the titer of circulating antibodies and induced a remission in this patient.

Naveed Sami - One of the best experts on this subject based on the ideXlab platform.

  • Influence of Intravenous Immunoglobulin Therapy on autoantibody titers to desmoglein 3 and desmoglein 1 in pemphigus vulgaris
    European journal of dermatology : EJD, 2003
    Co-Authors: Naveed Sami, Kailash C. Bhol, Razzaque A Ahmed
    Abstract:

    Pemphigus vulgaris (PV) is an autoimmune mucocutaneous blistering disease. Recently, patients with mucosal involvement have been described to have autoantibodies to desmoglein 3 (dsg), while patients with mucocutaneous disease have autoantibodies to dsg 1 and dsg 3. The objective of this study was to prospectively analyze, over a 24-month period, the influence of Intravenous Immunoglobulin (i.v.Ig) Therapy on autoantibody titers to dsg 3 and dsg 1, in two groups of patients with severe PV. Group A consisted of 11 patients with mucocutaneous involvement and group B consisted of 10 patients with only mucosal involvement. Levels of autoantibodies to dsg 3 and 1 were measured by ELISA, at monthly intervals. Prior to Therapy initiation, group A patients' sera showed a high ELISA index value of both dsg 3 and 1 antibodies, while group B patients had a high index value to only dsg 3. During i.v.Ig Therapy, a progressive decline in the ELISA index values was observed in all patients. After the initiation of i.v.Ig Therapy, in group A, a statistically significant reduction (p < 0.05) in ELISA index value to dsg 3 and 1 was detected after four and five months, respectively. In Group B, a significant decline in the mean autoantibody titer values to dsg 3 (p < 0.05) was observed after six months of i.v.Ig Therapy. Group A patients had a negative ELISA index value to dsg 3 and 1 after a mean period of 21 and 20 months, respectively. Group B patients had a negative dsg 3 score after a mean period of 22 months. These results indicate that autoantibody titers to dsg 3 and 1, as measured by ELISA, can be used to monitor the serological response to treatment in PV patients. A sustained serological remission is observed in patients treated with i.v.Ig Therapy. .

  • Intravenous Immunoglobulin Therapy in patients with multiple mucosal involvement in mucous membrane pemphigoid
    Clinical Immunology, 2002
    Co-Authors: Naveed Sami, Kailash Hol, Razzaque A Ahmed
    Abstract:

    Mucous membrane pemphigoid (MMP), also known as cicatricial pemphigoid (CP), is an autoimmune mucocutaneous, blistering disease which can lead to blindness and/or death from sudden asphyxiation, secondary to a scarring process. Conventional Therapy for the treatment of MMP consists of high-dose systemic corticosteroids and/or immunosuppressive agents. Some patients do not respond to these treatments and develop multiple serious side effects, which can be potentially fatal. In such patients, alternative treatment modalities are needed. This study presents the use of Intravenous Immunoglobulin (IVIg) Therapy in 15 patients with severe MMP whose disease was nonresponsive to the prolonged use of high-dose systemic corticosteroids and immunosuppressive agents and who developed multiple side effects to them. All 15 patients received an IVIg dose of 1-2 g/kg/cycle. The following objective parameters were used to assess the clinical outcome pre- and post-IVIg Therapy: number of side effects, frequencies of recurrences and relapses, duration and total dosage of prednisone Therapy, and the quality of life. The differences in these variables between the pre- and post-IVIg data were statistically analyzed using the SAS UNIVARIATE software running the two-sided Wilcoxon signed-rank and sign tests. A statistically significant difference was observed between pre- and post-IVIg Therapy data when comparing the aforementioned variables. All 15 patients had an effective clinical response, were able to discontinue previous systemic therapies, and eventually achieved a prolonged clinical remission. IVIg improved the quality of life in all 15 patients and demonstrated a steroid-sparing effect. No serious side effects were observed. IVIg Therapy is a safe and effective alternative modality in the treatment of patients with nonresponsive and progressive MMP and can induce a sustained clinical remission.

  • Intravenous Immunoglobulin Therapy for patients with pemphigus foliaceus unresponsive to conventional Therapy
    Journal of The American Academy of Dermatology, 2002
    Co-Authors: Razzaque A Ahmed, Naveed Sami
    Abstract:

    Abstract Background: Pemphigus foliaceus (PF) is a chronic autoimmune blistering skin disease that is commonly treated with oral corticosteroids and immunosuppressive Therapy. In some patients, PF can be refractory to treatment and the resultant side effects of prolonged immune suppression can be potentially fatal. Alternative therapies are needed. Objective: The purpose of this study is to report treatment outcomes with IVIg Therapy in 11 patients with severe PF refractory to prednisone and other immunosuppressive Therapy. Methods: Selection criteria included documentation of a biopsy and immunopathology in 11 patients who were resistant to treatment or experienced side effects to conventional Therapy. IVIg was administered according to a defined protocol. The parameters used to assess clinical response to IVIg included time observed for effective control of disease, duration of IVIg maintenance Therapy, total duration of IVIg, number of IVIg cycles, systemic drug Therapy, and the frequency of recurrences and relapses. The pre-IVIg and post-IVIg data were statistically analyzed by means of the SAS UNIVARIATE and 2-sided Wilcoxon sign rank and sign tests. Results: All patients had an effective clinical response and remained in clinical remission for a mean period of 18.6 months after discontinuation of IVIg Therapy. Serious side effects from IVIg use were not observed. Conclusion: IVIg Therapy appears to have potential as a biologic alternative agent in inducing and maintaining clinical remissions in patients with PF who are resistant to more standard conventional treatment. IVIg is effective as monoTherapy and may be needed for a period of several months to achieve a long-term clinical remission. (J Am Acad Dermatol 2002;46:42-9.)