PEMPHIGUS HERPETIFORMIS

By Gary M. White, MD


Pemphigus Herpetiformis (PH) is an autoimmune blistering disease that combines clinical features of dermatitis herpetiformis and the immunologic characteristics of pemphigus.

Clinical

Bullous lesions show a herpetiform arrangement. Annular-shaped lesions are not uncommon. The trunk and proximal extremities are favored sites. Mucous membranes are spared in most cases [JAAD April 2014]. Some cases may appear eczematous with or without pompholyx-like lesions on the hands [JEADV 2016;30;540]. Histopathology may show spongiosis as well, confusing the clinical picture.

Immunoflourescence

Typically. there is deposition of IgG and C3 superficially in the intraepithelial intercellular zone. Less frequently, these immunoreactants can be seen in the lower layers of the epidermis mimicking the typical PV pattern, especially when circulating anti-Dsg3 autoantibodies are present.

Treatment

PH has a much better prognosis than classic pemphigus, although some cases may progress to classic PV. Dapsone is the treatment of choice and dosages from 100-300 mg/day usually control the disease well. Oral prednisone at a low dose is used as well or may be combined with dapsone. Other therapies used in various case reports include azathioprine, sulfapyridine, cyclophosphamide, mycophenolate mofetil, methotrexate, high-dose intravenous immunoglobulin, and plasmapheresis.

For children, prednisone alone or in combination with dapsone, methotrexate or azathioprine have been used [PD 2017;34;342].

References

Dermatology Online Journal, 17(6)

JAAD April 2014;Volume 70, Issue 4, Pages 780–787

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