Localized Pemphigus Foliaceus with Unilateral Facial Involvement

Abstract

Pemphigus foliaceus is a superficial vesiculobullous disease that typically presents with widespread lesions. Localized presentations are less frequent, and they typically occur in middle-aged patients, following exposure to topical medications, and later on, become more disseminated. We present a case of a 19-year-old female with a localized presentation of pemphigus foliaceus unrelated to previous topical medications, that was a diagnostic and therapeutically challenging case. We also discuss the literature on localized cases, differences in presentations and responses to various treatment modalities. Pénfigo foliáceo; Localizado; Enfermedades ampollosasPénfigo foliáceo localizado con afectación facial unilateral Resumen El pénfigo foliáceo es una enfermedad vesículo-ampollosa superficial caracterizada por la aparición de lesiones generalizadas. Las presentaciones localizadas son menos frecuentes y suelen observarse en pacientes de mediana edad tras la exposición a medicamentos tópicos que posteriormente evolucionan a formas más diseminadas. Presentamos el caso de una mujer de 19 años de edad con pénfigo foliáceo localizado no asociado a medicamentos tópicos previos cuyo diagnóstico y tratamiento han supuesto un reto. También analizamos la literatura existente sobre los casos de pénfigo foliáceo localizado, las diferencias en las presentaciones clínicas y las respuestas a distintos tipos de tratamientos.

Actinic keratosis. Or maybe not?

“…This is exemplified in our case, in which the incidental superimposed AK distracted from the early features of localized PF. Similar to actinic damage, localized PF has a predilection for photoexposed facial skin . In our case, significant fluorescent uptake by the coexisting actinic damage during PDT (Fig.…”

Solitary erythematous crusted plaque on the neck

“…5 In the present case, indirect immunofluorescence was negative. 5 In the present case, indirect immunofluorescence was negative.…”

“…Immunopathologic investigations are usually negative in localized PF, although positive enzyme-linked immunosorbent assays (ELISAs) for anti-desmoglein-1 antibodies have been reported in a few cases. 5 In the present case, indirect immunofluorescence was negative. The absence or low rate of circulating autoantibodies may be explained by the localized nature of the lesions.…”

Acantholysis

“…Secondary acantholysis results from damage or alteration to keratinocytes with subsequent degeneration of desmosomes; examples include herpes virus infection, actinic keratosis, ASCC, elastolytic granuloma, acantholytic acanthoma, and many more. 5 We hope that this case brings attention to the prevention of further misdiagnosis and delay of appropriate treatment in patients presenting with atypical lesions of PV. In primary acantholytic disorders, keratinocytes tend to appear rounder and desmosomal spines appear destroyed rather than stretched as in a spongiotic inflammatory process.…”