The European Academy of Dermatology and Venereology (EADV) has provided updates to its S2K guideline on the management of pemphigus vulgaris and foliaceus, which were published in the Journal of the European Academy of Dermatology and Venereology.

The update was initiated by 62 members of the EADV’s autoimmune blistering diseases Task Force, all of whom voted upon current guideline recommendations according to degrees of consensus.

In the updated guideline, the EADV Task Force stated that the responsibility for creating a treatment plan for patients with pemphigus should fall on an experienced dermatologist in a hospital setting or specialized center. Before developing the treatment plan, the dermatologist and all other relevant clinicians should review the medical history and conduct or review findings from a physical examination. A pemphigus diagnosis should then be made based on clinical presentation, histopathology, direct immunofluorescence examination of skin or mucosal biopsy, and serological detection of autoantibodies.

For therapeutic management of pemphigus, the primary objective is to control and heal the bullous skin and mucous lesions without substantially increasing the burden of treatment-related side effects. Dapsone, topical corticosteroids, systemic corticosteroid therapy with prednisone, or rituximab could be considered as initial treatment of mild pemphigus foliaceus (PF), despite that few of these therapies have robust data to support their use in mild PF. In addition, the guideline suggests second-line management could include rituximab or systemic corticosteroid therapy with prednisone.


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The Task Force recommends tapering systemic corticosteroids during 4 to 6 months after the consolidation phase if treatment is associated with rituximab. The members of the Task Force were unable to reach a consensus on the best tapering regimen.

The updated guideline recommends clinicians inform their patients and their family members about the disease, including its clinical course and prognosis, relapse signs, and possible treatment-related adverse events. Clinicians should also recommend their patients seek out patient support groups for pemphigus.

Disclosure: Several study authors declared affiliations with the pharmaceutical industry. Please see the original reference for a full list of authors’ disclosures.

Reference

Joly P, Horwath B, Patsatsi Α, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. Published online August 24, 2020. doi:10.1111/jdv.16752