International Journal of Trichology International Journal of Trichology
 Print this page Email this page Small font sizeDefault font sizeIncrease font size
 
 
  Home | About IJT | Editorial board | Search | Ahead of print | Current Issue | Archives | Instructions | Online submission | Subscribe | Advertise | Contact us | Login   
 


 
 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 13  |  Issue : 6  |  Page : 36-38  

Pemphigus vulgaris localized to the scalp with complete response to topical steroids


Department of Dermatology, Principe De Asturias University Hospital, Alcalá De Henares, Spain

Date of Submission09-Jun-2020
Date of Decision29-Jul-2020
Date of Acceptance12-Jan-2021
Date of Web Publication22-Nov-2021

Correspondence Address:
David Vega-Diez
Department of Dermatology, Principe De Asturas University Hospital, Carretera De Alcalá-Meco S/N, CP 28805, Alcalá de Henares, Madrid, España
Spain
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijt.ijt_95_20

Rights and Permissions
   Abstract 


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.

Keywords: Autoimmune bullous disease, localized pemphigus vulgaris, pemphigus vulgaris, pemphigus


How to cite this article:
Vega-Diez D, Lario AR, Zubiaur AG, Medina S. Pemphigus vulgaris localized to the scalp with complete response to topical steroids. Int J Trichol 2021;13:36-8

How to cite this URL:
Vega-Diez D, Lario AR, Zubiaur AG, Medina S. Pemphigus vulgaris localized to the scalp with complete response to topical steroids. Int J Trichol [serial online] 2021 [cited 2021 Dec 5];13:36-8. Available from: https://www.ijtrichology.com/text.asp?2021/13/6/36/330780




   Introduction Top


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 Top


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

Click here to view


A skin biopsy sample showed a suprabasal acantholysis [Figure 2]a, and in direct immunofluorescence, interkeratinocyte IgG and C3 deposits are observed [Figure 2]b, [Figure 2]c, [Figure 2]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

Click here to view


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 Top


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.[3],[4]

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.[7],[8] Treatment with potent topical steroids is usually sufficient to control the symptoms, according to the isolated cases reported.[7],[8]

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.



 
   References Top

1.
Baum S, Sakka N, Artsi O, Trau H, Barzilai A. Diagnosis and classification of autoimmune blistering diseases. Autoimmun Rev 2014;13:482-9.  Back to cited text no. 1
    
2.
Kridin K. Pemphigus group: Overview, epidemiology, mortality, and comorbidities. Immunol Res 2018;66:255-70.  Back to cited text no. 2
    
3.
Veraitch O, Ohyama M, Yamagami J, Amagai M. Alopecia as a rare but distinct manifestation of pemphigus vulgaris. J Eur Acad Dermatol Venereol 2013;27:86-91.  Back to cited text no. 3
    
4.
Hadayer N, Ramot Y, Maly A, Zlotogorski A. Pemphigus vulgaris with loss of hair on the scalp. Int J Trichology 2013;5:157-8.  Back to cited text no. 4
    
5.
Zhang C, Goldscheider I, Ruzicka T, Sárdy M. Pemphigus vulgaris persistently localized to the nose with local and systemic response to topical steroids. Acta Derm Venereol 2017;97:1136-7.  Back to cited text no. 5
    
6.
Baykal C, Azizlerli G, Thoma-Uszynski S, Hertl M. Pemphigus vulgaris localized to the nose and cheeks. J Am Acad Dermatol 2002;47:875-80.  Back to cited text no. 6
    
7.
Hasson A, Requena L, Arias D, Martín L, de Castro A. Linear pemphigus vulgaris along a surgical scar. Dermatologica 1991;182:191-2.  Back to cited text no. 7
    
8.
Crocker C. Radiation-induced pemphigus in a patient with an invasive ductal carcinoma of the breast: A case report. Oxf Med Case Reports 2020;2020:omaa001.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2]



 

Top
 
 
  Search
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Access Statistics
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this article
    Abstract
   Introduction
   Case Report
   Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed170    
    Printed4    
    Emailed0    
    PDF Downloaded7    
    Comments [Add]    

Recommend this journal