(Last Updated On: October 30, 2018)

Authors: Ruby Ann B. Imson MD, Elizabeth P. Prieto, MD, FPDS

Abstract

Introduction: Pemphigus foliaceus is an autoimmune blistering disease of the skin which causes a characteristic inflammatory attack at the subcorneal layer of epidermis, which results in skin lesions that are scaly or crusted erosions with an erythematous base distributed along the seborrheic areas of the body. Mucosal involvement is absent even with widespread disease.

Case Summary: A 39-year- old male presents with a 7-month history of multiple skin colored papules and fragile vesicles on the scalp, face, chest and back leaving crusted erosions . This was accompanied by intractable pruritus and burning sensation. No systemic symptoms were noted. Histopathology result showed an intraepidermal blister with the plain of cleavage in the granular layer with neutrophils and acantholytic cells in the blister cavity consistent with a diagnosis of pemphigus foliaceus. The patient was treated with systemic corticosteroids (0.5mkd) along with antihistamines and emollients. The patient responded well with improvement of lesions.

Conclusion: There is no guideline that accurately describes the step by-step management of a patient with pemphigus foliaceus. Treatment goals include controlling the disease, avoiding complications due to prolonged use of steroids, induce remission and prevent relapses. As with other types of pemphigus, corticosteroids are still the mainstay of treatment of pemphigus foliaceus. However, in recalcitrant cases, other treatment methods such as use of immunosuppressive agents, intravenous immunoglobulin and plasmapheresis may be beneficial.

 

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Keywords

pemphigus foliaceus, steroids, antihistamine

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