Pemphigus foliaceus with circinated plaques and neutrophil pustules

Abstract

Pemphigus is a group of autoimmune intraepidermal bullous diseases; being pemphigus foliaceus (PF) and pemphigus vulgaris (PV) the most common subtypes. Pustular variants are scarcely reported for both PV and PF. The purpose of this manuscript was to describe the clinical, microscopic and immunologic findings of an atypical case of PF presenting with pustules, including a review of the literature. PF is described as blisters and because this entity is rare, it is not known for the general medical community that they are other clinical features that can be seen as this one we present here with pustules.

Pemphigus Foliaceous With Prominent Neutrophilic Pustules

“…PF with neutrophilic pustules is a rare presentation, with only a few cases been reported in literature. [ 1 2 3 4 ] All previously reported cases presented as erythroderma with extensive skin involvement similar to our patient. Clinically, the presence of predominant pustules in our patient was mimicking pustular psoriasis/IgA pemphigus/subcorneal pustular dermatosis (SCPD).…”

“…[ 1 ] and Méndez-Flores et al . [ 2 ] also reported frequent relapses and incomplete resolution on treatment (oral prednisolone and dapsone) in their cases who presented as erythrodermic PF with neutrophilic pustules. However, the addition of colchicine, along with steroids in our case, showed remarkable improvement and resolution of pustules in 2 weeks.…”

Assessment of Immune Cell Activation in Pemphigus

“…Due to minor differences in the pathomechanism of pemphigus variants, discrepancies in laboratory tests are also observed, as exemplified by the notable monocytosis and eosinophilia in pemphigus vegetans. However, it should be emphasized that activation of the immune system, manifested primarily by increased neutrophil [ 18 ] and lymphocyte [ 19 , 20 ], is significant in patients diagnosed with pemphigus. It is therefore essential that study is carried out to understand as precisely as possible the mechanism of immune cell activation in the course of this disease.…”

Manifestaciones atípicas del síndrome de vasculitis-angeítis- endotelitis (SVAE) cutánea

“…22,23 Esta "vasculitis leucocitoclasia" ocurre en varias entidades y el diagnóstico diferencial debe guiarse por la anamnesis, la presentación clínica, los exámenes paraclínicos y la histopatología (tabla 1). [23][24][25][26][27][28][29][30][31][32][33] A la luz de hoy, muchas de estas entidades neutrofílicas, están genética y epigenéticamente ligadas con disregulación de la actividad de linfocitos ayudadores y citotóxicos de diferenciación a citoquín productora de interleukina 17 (IL17), es decir Th17 y Tc17, y su red de interactoma con las citoquinas proinflamatorias de la familia de la interleukina 1 (IL1α, ILβ, IL36α/ IL1F6, IL36β/ IL1F8, e IL36γ/ IL1F9), IL12, IL17, IL23 y factor de necrosis tumoral alfa (TNFα), además de citoquinas antiinflamatorias de la familia de la IL1 (IL1RN/ IL1RA e IL36RN/IL1F5/IL36RA). Es también trascendental la deficiencia inmunofuncional en la actividad de los linfocitos T regulatorios (TREG) en la piel.…”