Unilateral Dyshidrosiform Pemphigus Vulgaris

Abstract

AbstarctPemphigus vulgaris is an autoimmune disease characterized by fluid-filled blisters over body and mucosal surfaces. Localized pemphigus is a rare presentation of the disease. There are few reports of localized pemphigus in literature presenting as esophageal involvement, recurrent paronychia, conjunctival mass, foot ulcers, discoloration of toes with hyperkeratotic skin lesions, verrucous lesions, and dyshidrosiform pemphigus vulgaris. We present a case of dyshidrosiform pemphigus involving a foot following trauma. Histopathology and direct immunofluorescence confirmed our diagnosis. In our patient, the disease started as localized dyshidrosiform pemphigus and later became generalized. This unusual presentation led to a delay in diagnosis and proper treatment in this patient. Physicians should be aware of such rare presentations to aid in the timely diagnosis and management of such patients.

Bilateral dyshidrosiform pemphigus vulgaris: an unusual clinical presentation

“…Some rare presentations of pemphigus vulgaris have been reported, including isolated esophageal involvement, recurrent paronychia, conjunctival mass, foot ulcers, discoloration of toes with hyperkeratotic skin lesions and verrucous lesions. 1,3 Palmoplantar involvement in pemphigus vulgaris is a poor prognostic indicator. Dyshidrosiform pemphigus vulgaris is an unusual presentation which led to a delay in diagnosis.…”

“…Unlike dyshidrosiform pemphigus, dyshidrosiform pemphigoide is a well-known variant of localized pemphigoid presenting as vesiculobullous lesions on palms and soles. 1 Usually, an autoimmune bullous disease, is not initially considered in patients who present with blisters restricted to the palms and/or soles. Indeed, this dyshidrosiform bullous mimics several other conditions, such as allergic and irritant contact dermatitis, chronic bullous disease of childhood, cutaneous T-cell lymphoma, dermatophyte infection, dyshidrosis or pompholyx, epidermolysis bullosa acquisita, erythema multiforme, herpes gestationis, lichen planus, linear IgA disease, scabies, and systemic contact dermatitis.…”

“…However a hypothesis of dyshidrosiform pemphigoid was proposed incriminating local inflammatory process which can uncover the masked antigen and trigger the disease at local site. 1 This unusual presentation can lead to a delay in diagnosis and proper treatment. Hence, we present our case to highlight a rare morphologic variant of pemphigus.…”

“…Unlike dyshidrosiform pemphigoid, which is a well-known variant of localized pemphigoid, dyshidrosiform pemphigus remains very rare. 1 We report a case of a 59 year old man, with isolated bilateral large plantar erosion associated to pompholyx like lesions.…”

“…Pemphigus vulgaris is an autoimmune bullous disease clinically characterized by flaccid cutaneous blisters and erosions associated to mucosal involvement. 1 In addition to the cutaneous and mucosal surfaces, the pharynx, vocal folds, and anogenital mucosa may also be affected. 2 Unilateral or localized forms of pemphigus have been reported in literature.…”