An Unusual Case of Pemphigus Vulgaris

Abstract

Introduction

Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus (PF) and pemphigus vulgaris.

Case Presentation

We present the case of a young woman with scalp manifestations diagnosed as seborrhiasis who came to our office where a more careful history and clinical examination directed us toward another diagnostic suspicion. The histological examination confirmed our suspicion of pemphigus and therefore we believe it is important to report our experience to avoid misdiagnosis.

Discussion/Conclusion

Our case may be useful in the literature to identify cases of PF with atypical manifestations that may mimic other diseases.

Established Facts

• Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus and pemphigus vulgaris; to differentiate these 2 subtypes, a clinical and a histopathological exam are necessary, as well as the study of the different autoantibodies involved in the pathogenesis of the disease.

Novel Insights

• Nowadays, we believe it is necessary to frame pemphigus as differential diagnosis of many pathologies such as psoriasis, sebopsoriasis, and also seborrheic dermatitis.

Introduction/Literature Review

Pemphigus is a potential life-threatening skin disorder belonging to the group of the autoimmune bullous diseases affecting the skin and mucosa. The most common subtypes are pemphigus foliaceus (PF) and pemphigus vulgaris; to differentiate these 2 subtypes, a clinical and a histopathological exam are necessary, as well as the study of the different autoantibodies involved in the pathogenesis of the disease. PF is characterized by the development of superficial, subcorneal, acantholytic bullae that at the time of the clinical exam are usually seen as erosions or crusted plaques. In PF autoantibodies are targeted against the desmosomal cadherin desmoglein-1 antigen, which plays a pivotal role in the adhesion of nearby keratinocytes [1]. In the clinical practice, an unusual or atypical manifestation of this bullous disease can represent a diagnostic challenge, sometimes resulting in a delayed diagnosis, so that a variety of differential diagnoses depending on the clinical presentation should always be considered [2].

Case Report

We herein report a case of a 35-year-old female patient with PF attending our clinic. The patient during the anamnesis reported the presence of yellowish-white scales/crusts in the centre of the scalp, always interpreted as psoriasis or sebopsoriasis of the scalp, treated with topical corticosteroids. She also reported short periods of improvement with relapses. After months of alternating treatments, the patient noticed the appearance of alopecic areas upon removal of the scaly scabs and, frightened, interrupted every treatment. It seemed that the thing had stopped: she still had only small scales/crusts in the central areas of the scalp. The patient came to our observation for a strange eruption on the trunk (Fig. 1). On physical examination, erosions covered with serous crusts were noted in the upper chest anteriorly and posteriorly, the patient's clinical manifestations conflicted with the history she reported, but closer observation of the scalp revealed erosions surmounted by thin crusts very similar to those found on the trunk not attributable to psoriatic or sebopsoriatic lesions (Fig. 2). In the suspicion of autoimmune bullous disease all the necessary investigations were carried out including a skin biopsy with direct immunofluorescence. The histopathological exam with the direct immunofluorescence showed a superficial intra-epidermal bullous dermatosis, confirming the diagnosis of PF. At this point we decided to treat the patient with topical therapy (Vaseline 25%) to the scalp and oral corticosteroid therapy (50 mg/daily for 10 days, then tapered to 25 mg daily for 10 days). At follow-up visit, after 20 days of treatment, the patient reported a significant improvement of all her skin manifestations (Fig. 3). The patient is currently followed up in our structure with periodic outpatient visits. She is continuing systemic cortisosteroid therapy on a sliding scale (15 mg/day) with disappearance of clinical manifestations and only hyperchromic residuals.

Fig. 1.

Multiple crusted erosions localized to the anterior region of the trunk.

Fig. 2.

Serocrusts attached to the scalp overlying erosions.

Fig. 3.

Clinical picture after 20 days of topical and systemic therapy: complete remission of the lesions on the scalp and on the anterior region of the trunk with the presence only of hyperchromic lesions.

Discussion/Conclusion

Unusual or atypical manifestations of pemphigus could be easily misdiagnosed or leading to a delay in correct diagnosis [3]. In case of solitary lesions or uncommon localization of pemphigus, the number of potential differential diagnoses could increase as in the case of our patient. Unusual manifestations may mimic different skin disorders, often representing an important diagnostic challenge. A careful clinical examination and a histopathological examination are always required to avoid misdiagnosis and confirm the suspicious [4]. In conclusion, we feel it is necessary that sometimes we have to consider to pay attention approach “simple sign” which may suggest a diagnosis but actually hide a more important disease such as PF. Seborrheic dermatitis can simulate the pathology and for this reason this case can be important for young dermatologist.

Statements of Ethics

Written consent on photos and clinical data publication was given by the patient. Ethical approval was not required for this study in accordance with national guidelines.

Conflict of Interest Statement

The authors have no conflict of interest to disclose.

Funding Sources

None to declare.

Author Contributions

Dr. Laura Marano has given his contribution to manuscript conception and writing. Prof. Gabriella Fabbrocini has given her contribution reviewing the paper for important intellectual content. Prof. Giuseppe Monfrecola has given her contribution reviewing the paper for important intellectual content. Dr. Fabrizio Martora has given his contribution to manuscript conception.

Data Availability Statement

All data generated or analysed during this study are included in this article. Further enquiries can be directed to the corresponding author.