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 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 33  |  Issue : 2  |  Page : 101-103

Pemphigus vegetans: A rare variant of pemphigus vulgaris


1 Department of Pathology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India
2 Department of Dermatology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pune, Maharashtra, India

Date of Submission06-Apr-2019
Date of Decision19-Apr-2019
Date of Acceptance10-Jun-2019
Date of Web Publication11-Feb-2020

Correspondence Address:
Banyameen Iqbal
Department of Pathology, Dr. D.Y. Patil Medical College, Hospital and Research Centre, Pimpri, Pune - 411 018, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jms.jms_20_19

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  Abstract 


Pemphigus vegetans (PV) is a rare clinical variant of pemphigus vulgaris and comprises up to 1%–2% of all pemphigus cases. It is characterized by vegetative plaques in the inguinal folds, flexural areas, oral mucosa, trunk, and thighs. It is accompanied by the presence of autoantibodies against desmoglein-3. We are presenting a case of a 36-year-old man with oral ulcers and multiple pustules in the flexural areas, inguinal region, and thighs. Systemic examination was uneventful. Skin biopsy was taken from the lesion over the thigh and subjected to light microscopy and immunofluorescence examinations. Based on clinical findings, histopathological characteristics, and direct immunofluorescence, a final diagnosis of PV was made.

Keywords: Autoimmune disease, pemphigus, pemphigus vegetans


How to cite this article:
Iqbal B, Raj A, Gupta S. Pemphigus vegetans: A rare variant of pemphigus vulgaris. J Med Soc 2019;33:101-3

How to cite this URL:
Iqbal B, Raj A, Gupta S. Pemphigus vegetans: A rare variant of pemphigus vulgaris. J Med Soc [serial online] 2019 [cited 2020 May 25];33:101-3. Available from: http://www.jmedsoc.org/text.asp?2019/33/2/101/278099




  Introduction Top


Pemphigus is a rare vesicobullous autoimmune disease that exhibits blistering of the skin and mucous membrane.[1] Autoantibodies directed against antigens on the surface of keratinocytes are responsible for the causation of pemphigus. The presence of circulating and skin-fixed autoantibodies is associated with all different forms of pemphigus. Pemphigus vegetans (PV) is a rare clinical variant of pemphigus vulgaris and comprises up to 1%–2% of all pemphigus cases. It is characterized by vegetative plaques in the inguinal folds, flexural areas, oral mucosa, trunk, and thighs. It is accompanied by the presence of autoantibodies against desmoglein-3.[2],[3] In the following case, we describe a rare presentation of PV in a 36-year-old man with complaints of itching and multiple pustules over the body along with scaly pruritic lesions in the groin area and thighs.


  Case Report Top


A 36-year-old man, shopkeeper by profession, came to the department of dermatology and venereology with a history of oral ulcers and multiple erythematous lesions and pustules in the flexural and inguinal regions for the last 8–10 months. He described the oral lesions as ulcers whereas the lesions on the trunk and flexural areas were described as scaly and itchy. His history revealed significant weight loss and intermittent diarrhea with more than 4 bowel movements per day. The patient had been on irregular treatment with antifungal ointments but reported no relief in symptoms. During examination, entire oral mucosa, oropharynx, tongue, and palate showed white, patchy, irregular, and rough lesions. Some of the oral lesions were ulcerated as well. Multiple blisters as well as raised, irregular, erythematous, and scaly lesions were noted predominantly in the inguinal region, groin, and on bilateral thighs [Figure 1]. Besides, gluteal cleft, axillary folds, penis, and perioral region were also affected with the same lesion albeit less severely. Systemic examination was uneventful.
Figure 1: Erythematous vegetative plaques on thighs

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Based on the history and clinical findings, a provisional diagnosis of pemphigus vulgaris was made. PV, pemphigus foliaceus, and pemphigus erythematosus were considered as differential diagnosis. Laboratory investigations revealed microcytic hypochromic anemia (hemoglobin - 9.9 mg/dl) and slightly elevated absolute eosinophil count (380/mm3). Skin biopsy was taken from the lesion (plaque) over the thigh and sent for histopathological and immunofluorescence examination. Light microscopy revealed acanthosis and papillomatosis in the epidermis [Figure 2] along with focal areas of intraepidermal microabscesses filled with eosinophils and polymorphs and diffuse infiltration of eosinophils and lymphocytes in the dermis [Figure 3] and [Figure 4]. Direct immunofluorescence showed bright intercellular deposition of immunoglobulin G (IgG) in the epidermis [Figure 5].
Figure 2: Exuberant proliferation and epitheliomatous hyperplasia of squamous epithelium (H and E, ×20)

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Figure 3: Intraepidermal microabscesses (H and E, ×40)

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Figure 4: Intraepidermal microabscesses filled with eosinophils (H and E, ×100)

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Figure 5: Direct immunofluorescence showing bright intercellular deposition of immunoglobulin G in the epidermis

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Based on clinical findings, histopathological characteristics, and direct immunofluorescence, a final diagnosis of PV was established. The patient was hospitalized for 14 days for inpatient corticosteroid therapy. Daily treatment with 40 mg of prednisone over a period of 2 weeks was given which effectively healed all skin and mucosal ulcerations. Once a clinical response was observed, and the oral ulcers and plaques in the inguinal and flexural regions subsided, the dose of systemic corticosteroids was tapered gradually to 10 mg daily and eventually discontinued over a period of 6 weeks. Anemia was treated with iron supplements and dietary measures. The patient continued using the same medications 6 months after being discharged from the hospital. At his 6th month follow-up, the patient was asymptomatic and without any recurrences.


  Discussion Top


PV is a rare clinical variant of pemphigus vulgaris, and it most commonly involves the oral mucosa. In certain patients, erosions have a tendency to develop into granulation tissue and crusting, known as vegetative lesions, often found on the groin, armpits, thighs, hands, eyelids, and in the perioral region.[4],[5] It is clinically classified as (1) Neumann type with oral lesions that resemble flaccid bullae and small ulcerated areas and (2) Hallopeau type with pustular lesions and a benign course with few relapses. As in pemphigus vulgaris, 50% of PV cases begin in the oral cavity months before skin lesions appears. Patients initially having cutaneous lesions will ultimately develop oral manifestations. These lesions are caused by intercellular autoantibodies against desmoglein 1 and 3 as adhesion molecules in the desmosomes of keratinocytes. Patients with the Hallopeau-type pemphigus are often considered to have a relatively benign disease. Hallopeau type of pemphigus usually requires a lower dose of systemic corticosteroids to control the mucocutaneous manifestations and usually have a prolonged remission. To get to a diagnosis, it is mandatory to identify both histopathology and direct immunofluorescence features of mucosal involvement. Our patient had a relatively benign course and belonged to Hallopeau type. Diagnostic findings for PV included eosinophilic spongiosis, epidermal hyperplasia, and intraepidermal abscesses filled with eosinophils as the lesions age, while direct immunofluorescence examination shows deposits of IgG and complement C3 on the keratinocytes. Indirect immunofluorescence is positive in up to 80%–90% of the patients and usually is correlated with the severity of the disease.[6] Treatment of PV is similar to that of pemphigus vulgaris. Systemic corticosteroids are the treatment of choice for these diseases. Some other modalities of treatment are dapsone, methotrexate, mycophenolate mofetil, and chlorambucil. Newer agents such as intravenous Ig therapy and rituximab (an anti-CD20 chimeric monoclonal antibody) are also being used. Prednisolone is usually administered in a dosage of 1–2 mg/kg body weight/day and is gradually tapered once clinical outcomes are achieved. Although the relapse rate in PV is high, the corticosteroids are usually supplemented with immunotherapy and immunomodulators to decrease the relapse rates. With this case report, we hope to add to the knowledge of the underlying mechanism of PV and the importance of a multidisciplinary approach to improve outcomes.


  Conclusion Top


PV is a rare variant of pemphigus vulgaris which causes chronic ulceration and blistering of skin and mucosa. The mouth maybe the only site of involvement in the initial stages, and this can lead to a delayed diagnosis and inappropriate treatment.[6] Newer diagnostic tests and better monitoring of the disease process can be achieved now with clear understanding of the role of anti-Dsg antibody–keratinocyte binding in blister formation. Treatment goals are suppression of circulating autoantibodies with the use of high-dose systemic corticosteroids. It proved to be an effective therapy in our patient and produced healing of all mucosal blisters/ulceration and inguinal and flexural plaques. The patient continued using the same medications 6 months after being discharged from the hospital without any recurrences. In future, antigen-specific immunotherapy may be an alternative to the current conventional treatment modalities. Recent data shed light on the long-term prognosis of the disease, suggesting that lasting remission maybe achievable more frequently in patients with PV than was thought.[7]

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.
Payne AS, Stanley JR. Pemphigus. Fitzpatrick's Dermatology in Medicine. 8th ed. New York: McGraw-Hill; 2012. p. 585-99.  Back to cited text no. 1
    
2.
Yuen KL, Yau KC. An old gentlemen with vegetative plaques and erosions: A case of pemphigus vegetans. Hong Kong J Dermatol Venereol 2012;20:179-82.  Back to cited text no. 2
    
3.
Son YM, Kang HK, Yun JH, Roh JY, Lee JR. The Neumann type of pemphigus vegetans treated with combination of dapsone and steroid. Ann Dermatol 2011;23:S310-3.  Back to cited text no. 3
    
4.
Bystryn JC, Rudolph JL. Pemphigus. Lancet 2005;366:61-73.  Back to cited text no. 4
    
5.
Becker BA, Gaspari AA. Pemphigus vulgaris and vegetans. Dermatol Clin 1993;11:429-52.  Back to cited text no. 5
    
6.
Ruiz-Roca JA, Berini-Aytés L, Gay-Escoda C. Pyostomatitis vegetans. Report of two cases and review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:447-54.  Back to cited text no. 6
    
7.
Yeh SW, Sami N, Ahmed RA. Treatment of pemphigus vulgaris: Current and emerging options. Am J Clin Dermatol 2005;6:327-42.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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