Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management

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Pathogenesis, Diagnosis, Management

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Autoimmune Diseases of the Skin

Quick Search Search Terms:. Set Session Filters. Search History. Selected Reading. Hertl, Michael. After the induction cycle, long-term infusion therapy every 4 or 12 weeks may be performed or according to clinical need. The most common adverse effects are associated with infusion reactions, often related to the rate of administration and hypersensitivity reactions fever, chills, bronchospasm, pruritus, and hypotension. Rituximab is contraindicated in pregnancy; patients of childbearing age should use effective contraception during its use and up to 12 months afterward.

In addition to conventional treatments, anti-TNF alpha agents have been reported to be effective in mucous membrane pemphigoid, perhaps merited by the high serum TNF-alpha levels in patients. They are generally indicated for those who are in need of aggressive systemic treatment, do not respond to conventional therapy, or have severe side effects or contraindications. Several reports in the literature have demonstrated the efficacy of etanercept and infliximab.

The clinical improvement after treatment with these agents supports the hypothesis that TNF-alpha plays an important role in the pathogenesis of mucous membrane pemphigoid. Mucous membrane pemphigoid is a chronic, often devastating, but rarely fatal disease.

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Its chronic inflammatory nature, tissue destruction, and scarring are responsible for the sequelae, which are often disabling. The therapeutic strategy will depend on the site, severity, and rate of progression.

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  • Patients with low risk oral mucosa with or without cutaneous involvement have a better prognosis and respond well to conservative approaches, whereas high-risk patients ocular, nasopharyngeal, laryngeal, esophageal, and anogenital mucosa have a worse prognosis, being poor responders and often experiencing scarring, despite treatment. In the latter, an early aggressive approach helps stop the inflammatory process and prevent scarring. Our experience indicates that the approach should be as aggressive as possible for high-risk patients. Some therapeutic options can be employed, depending on the clinical context and their availability.

    Bullous pemphigoid - causes, symptoms, diagnosis, treatment, pathology

    Pulse therapy with methylprednisolone, cyclophosphamide in combination with dexamethasone, and IVIG with rituximab have effected good responses in our service. We believe that long-term follow-up of these patients should be performed to detect relapses and initiate early therapeutic intervention, and previous pulses or cycles may be repeated. Therefore, the involvement of these structures results in the loss of dermoepidermal adhesion, with subsequent cutaneous fragility, vesicle and blister formation, and scarring.

    EBA has a wide spectrum of clinical presentations and can affect the skin and mucous membranes. EBA has 5 main phenotypes. The classical or mechanobullous form is observed in approximately one-third of EBA patients and is characterized by skin fragility in trauma-prone areas, with tense blisters, erosions, atrophic scars, milia formation, anonychia, and digital contracture, especially on the fingers. The bullous pemphigoid-like variant presents as vesicles and blisters with erythematous or urticarial lesions, usually without skin fragility or milia formation, mainly in the extremities, trunk, and skin folds.

    The cicatricial pemphigoid-like form presents with exclusive or predominant mucous involvement, affecting any stratified squamous cell epithelia, such as ocular, oral, nasal, laryngeal, esophageal, and genital mucosa.

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    • The Brunsting-Perry pemphigoid-like variant is defined as a vesiculobullous eruption on the head and neck, which may eventually have oral involvement. The linear IgA bullous dermatosis-like form is characterized by edematous plaques with tense blisters and vesicules that can present in annular or polycyclic arrangement. The differential diagnosis of EBA includes bullous systemic lupus erythematosus, porphyria cutanea tarda, and other subepidermal bullous dermatosis, as mentioned in the clinical variants.

      The diagnosis of EBA comprises histopathological analysis and direct DIF and indirect IIF immunofluorescence, which allow the diagnosis of subepidermal bullous dermatosis. However, they are not always conclusive for the diagnosis of EBA. The definite diagnosis of EBA requires additional techniques that are only available in research centers. The histopathological evaluation of injured skin in patients with EBA shows subepidermal cleavage, with varying degrees of inflammatory infiltrate.

      In the mechanobullous form, the inflammatory infiltrate is absent or minimal, whereas in the inflammatory forms of EBA, it is composed of neutrophils with variable numbers of eosinophils, monocytes, and lymphocytes. The salt-split skin technique, which produces BMZ cleavage in the lamina lucida, shows IgG deposition on the dermal side of the cleavage in EBA, whereas in bullous pemphigoid, this deposit is present on the epidermal or epidermal and dermal side of the cleavage. EBA is often refractory to various therapeutic modalities, which makes its long-term remission a challenge.

      Because it is a rare disease with several clinical presentations, there are no randomized clinical trials in the literature, compromising the selection of an ideal treatment. The inflammatory form of EBA apparently has a more favorable clinical response to conventional therapy with corticosteroids and corticosteroid-sparing agents than the mechanobullous form.

      Local care should be encouraged for better disease control. Similar to inherited epidermolysis bullosa, the general care includes the prevention of local trauma and infection and the use of non-adherent dressings. A Cochrane systematic review found 11 non-randomized studies of treatment for EBA, involving interventions in 20 adults and 11 children. The authors concluded that there is no recommendation for treating EBA, based on reliable evidence. EBA patients, especially those of the mechanobullous form, usually do not experience a good therapeutic response to systemic corticosteroids, such as in other autoimmune blistering diseases.

      Despite this limitation, systemic corticosteroids are still considered the first-line treatment for EBA, with usual dosages of 0. The main anti-inflammatory benefits of these medications are exerted by an antineutrophilic action. Dapsone use has been described in several cases of EBA. The efficacy of colchicine in EBA was reported for the first time in , with dosages ranging from 0.

      Minocycline is a broad-spectrum tetracycline that inhibits the recruitment of neutrophils and eosinophils as well as cytokine production. Immunosuppressants azathioprine, mycophenolate mofetil, cyclosporine, methotrexate. Azathioprine has varying results in the literature. Mycophenolate mofetil MMF is an immunosuppressive agent that alters the proliferation of B and T lymphocytes.

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      Methotrexate is a folic acid analogue and antimetabolite that inhibits the synthesis of DNA and RNA, impairing lymphocyte function. IVIG modulates the autoimmune response, reducing and neutralizing antibodies. No disease recurrence was observed at a mean follow-up of Immunoglobulin was also administered subcutaneously in a patient with severe and resistant disease, which can represent an alternative in cases with difficult venous puncture.

      ECP has proven to be efficient in inducing partial to complete remission in patients with recalcitrant EBA and is considered a well-tolerated therapy, with promising results. In the literature, a recent review revealed that 8 patients with severe and refractory EBA were treated with ECP, all of which progressed with clinical improvement. Rituximab is a chimeric anti-CD20 monoclonal antibody that causes B cell depletion through various mechanisms and is approved for the treatment of B cell lymphoma, rheumatoid arthritis, and granulomatosis with polyangiitis.

      Rituximab use in subepidermal bullous diseases, however, is more limited. In EBA, a few promising case reports have been described, usually in patients with recalcitrant disease who have undergone several treatments before rituximab such as systemic corticosteroid, dapsone, azathioprine, cyclosporine, mycophenolate, and cyclophosphamide , with an effective response.

      Autoimmune Diseases of the Skin - Pathogenesis, Diagnosis, Management | Michael Hertl | Springer

      Another proposed scheme is 1g, administered on Days 0 and In most cases, rituximab was administered following the rheumatoid arthritis protocol, at 1g IV on Day 0 and 1g IV on Day All patients needed a new drug cycle after 6 months. At present, 2 patients are in complete remission, 1 is in partial remission, and 2 experienced disease relapse after being in complete remission for 1 year.

      There is no standardized severity score for EBA; 2 recent classifications have been proposed in the literature:. In mild forms of EBA, use of oral steroids 0. Oral steroids Treatment suggested for severe forms of EBA involves the same as in moderate EBA, with the addition of one or a combination of steroid pulses, cyclosporine, plasmapheresis, IVIg or rituximab.

      In the non-severe localized form, topical corticosteroids are suggested, whereas in the non-severe, more diffuse form, the use of colchicine, dapsone, sulfasalazine, and topical corticosteroid is advised. Systemic corticosteroid therapy is reserved for urgent cases, such as laryngeal edema.

      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management

      In the non-severe form of EBA, the first treatment option suggested by the authors is colchicine 0. If a partial response occurs, prednisone should be added at 0. Figure 3. Immunosuppressive therapy MMF, cyclosporine, or azathioprine should be discontinued in case of no therapeutic response or side effects.

      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management

      In cases of partial response to such immunosuppressants, they may be maintained in combination with rituximab. However, one should consider the possible side effects of this association, especially infectious ones. The possibility of infectious prophylaxis should be considered.

      Afterward, a reduction of 10mg every 2 weeks should be made until the dose reaches approximately 0. When the dosage reaches 7. Attention should be given to stressful situations such as surgeries and infections , in which the dose of hydrocortisone should be doubled. In the case of pulse therapy, at the end of 3 days, corticosteroid therapy should be maintained through oral administration of prednisone at 0. EBA is a disease that can evolve with high morbidity due to its potential for scarring.

      The cutaneous involvement in EBA can progress with extensive erosions, atrophic scars, nail dystrophies, and fibrosis, with flexion contracture of fingers, limiting adequate mobility. Different mucosal sites with stratified squamous epithelium may be affected, such as the conjunctival, upper airway, oral, esophageal, genital, and anal areas. Ocular involvement in EBA is frequent, with various forms of presentation, including conjunctivitis, symblepharon, keratitis, subepithelial vesiculation, perforation, and corneal opacification.

      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management
      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management
      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management
      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management
      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management
      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management
      Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management Autoimmune Diseases of the Skin: Pathogenesis, Diagnosis, Management

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