Pemphigus Vulgaris Localized to the Scalp with Complete Response to Topical Steroids.

Pemphigus vulgaris (PV) is an anti-cadherin autoimmune disease, characterized by the production of anti-desmoglein 3 (and to a lesser extent anti-desmoglein 1) antibodies, producing acanthosis, typically suprabasal, which clinically translates into flaccid blisters and erosions, generally starting on mucous membranes, with subsequent appearance of skin lesions. Only about 25 cases of PV of exclusively cutaneous location have been described in the literature. Treatment with potent topical steroids is usually sufficient to control the symptoms. We present the case of a 68-year-old female patient with localized PV in the scalp, presenting as alopecic and scabby plaque, with excellent response and hair regrowth with topical corticotherapy. Copyright:

INTRODUCTION

Pemphigus vulgaris (PV) is an anti-cadherin autoimmune bullous disease, characterized by the production of immunoglobulin G (IgG) autoantibodies against desmoglein 3 (Dsg-3) and desmoglein-1 (Dsg-1) antibodies (in up to 50% of patients).[1] Clinically, the first manifestation in 50%–60% of cases are mucosal erosions, intensely painful and refractory to treatment, typically in the oral mucosa, which may be accompanied, weeks or months later, by erosions or friable flaccid blisters on the skin (especially in the upper chest, face, scalp, and upper back).[2] Localized cutaneous PV is a very rare variant of PV with only other 25 cases described in the literature.

CASE REPORT

We present the case of a 68-year-old woman with no preexisting conditions, who comes to the dermatology clinic with a 6 months' history of intensely pruritic scabby, rounded, alopecic plaque in the vertex, without other skin or mucosal lesions [Figure 1]. In the anamnesis, the patient denies previous trauma, other symptoms, or drug intake.

Figure 1

Fissured scabby alopecic plaques with erythematous base

A skin biopsy sample showed a suprabasal acantholysis [Figure 2a], and in direct immunofluorescence, interkeratinocyte IgG and C3 deposits are observed [Figure 2b–d]. Antikeratinocyte antibody enzyme-linked immunosorbent assay is performed with positivity for anti-Dsg-3 and with negativity for anti-Dsg-1, anti-BP180, and anti-BP230.

Figure 2

(a) H-E (×20) showing suprabasal acantholysis; (b) direct immunofluorescence immunoglobulin G showing interkeratinocyte deposits; (c and d) direct immunofluorescence C3 showing interkeratinocyte deposits

Hair re-growth and resolution of the lesions were observed within 4 weeks after the initiation of treatment. No recurrence was observed in the 8-month follow-up with maintenance therapy with clobetasol three times per week.

DISCUSSION

PV is the most frequent form of autoimmune bullous disease representing 70% of cases. Antibodies against desmogleins inhibit its adhesive functions, leading to the loss of cell adhesion of keratinocytes causing intraepidermal acantholysis and to the clinical manifestation of blister formation. Although the scalp is commonly involved, and scalp erosions are a frequent finding, hair loss is rarely seen.[34]

Localized PV is a rare variant of PV. The few described cases of localized PV with exclusively cutaneous involvement[5] have been largely associated with local trauma (especially in the nose),[6] surgical scars,[7] and previously irradiated areas,[8] which could suggest a koebnerization phenomenon in PV.[78] Treatment with potent topical steroids is usually sufficient to control the symptoms, according to the isolated cases reported.[78]

As a conclusion, we present the case of localized PV, a rare clinical presentation of PV with complete resolution of the lesions and hair re-growth with treatment with clobetasol propionate 0.05% cream twice a day for 4 weeks with subsequent maintenance treatment of 3 nights per week for 6 months. Given the unusual of the picture and the possible differential diagnoses, high clinical suspicion and a correct histopathological study are essential for the correct filiation of this condition.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.