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CASE REPORT
Year : 2008  |  Volume : 20  |  Issue : 3  |  Page : 121-123 Table of Contents   

Pemphigus vulgaris


Department of Oral Medicine and Radiology, Krishnadevaraya College of Dental Science and Hospital, Hunasamaranhalli, via Yelankha, Bangaluru - 562 157, India

Date of Web Publication16-Jun-2009

Correspondence Address:
Vadi V Bharathan
Department of Oral Medicine and Radiology, Krishnadevaraya College of Dental Science and Hospital, Hunasamaranhalli, via Yelankha, Bangaluru - 562 157
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0972-1363.52781

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   Abstract 

Pemphigus vulgaris is the most common form of Pemphigus, is an autoimmune disease of the skin and mucous membrane characterized by impaired cell adhesion within the epidermis leading to the formation of intraepidermal bullae. The exact cause of the development of antibodies against the body's own tissues is unknown. Most patients are initially misdiagnosed and improperly treated.Diagnosis is mostly based on appearance and distribution of the skin lesions. Definitive diagnosis requires examination of a skin biopsy for histopathology and immunofluroscent study. Dental professionals must be sufficiently familiar with the clinical manifestations of pemphigus vulgaris to ensure early diagnosis and treatment, since this in turn determines the prognosis and course of the disease. Hence, we report a case of pemphigus vulgaris that was misdiagnosed in its early stage.

Keywords: Autoimmune disease, Intraepidermal bullae, Pemphigus, Pemphigus vulgaris, Tombstone appearance.


How to cite this article:
Vasudevan V, Bharathan VV, Radhika. Pemphigus vulgaris. J Indian Acad Oral Med Radiol 2008;20:121-3

How to cite this URL:
Vasudevan V, Bharathan VV, Radhika. Pemphigus vulgaris. J Indian Acad Oral Med Radiol [serial online] 2008 [cited 2020 Sep 26];20:121-3. Available from: http://www.jiaomr.in/text.asp?2008/20/3/121/52781


   Introduction Top


Pemphigus is an autoimmune disease of the skin and mucous membranes that manifests as vesicles (blisters) or bullae, which ultimately break down to form chronic painful ulcers. The name is derived from the Greek word pemphix, which means bubble or blister. It is an autoimmune disorder in which the immune system produces antibodies against specific proteins in the skin and mucous membrane. [1] These antibodies produce a reaction that leads to a separation of epidermal cells. The exact cause of the development of antibodies against the body's own tissues is unknown. [2]

The etiology of pemphigus is unknown. Pemphigus may be a side effect of certain blood pressure medications (ACE inhibitors) or chelating agents (medications, such as penicillamine, which remove certain materials from the blood). Occasionally, pemphigus may appear in reaction to medications, although this is rare.

Pemphigus is uncommon. It occurs almost exclusively in middle-aged or older people of all races and ethnic groups. About one-half of the cases of pemphigus vulgaris begin with blisters in the mouth, followed by skin blisters. The blisters (bullae) are relatively asymptomatic, but the lesions become widespread and complications develop rapidly and may be debilitating or fatal. [3] However, this condition has been differentiated from other chronic painful ulcerations, such as, aphthous ulcer, erythema multiforme, and so on, where there is no development of a vesicle or bullae. Pemphigus is differentiated from the aphthous ulceration by of the presence of an erythematous halo around the ulcer in the case of the aphthous ulcer and is absent in case of pemphigus. Erythema multiforme presents with crusting ulcers, with bleeding and ocular involvement.

Autoantibodies are formed against the cell protein desmoglein. Desmoglein forms the "glue" that attaches to the adjacent epidermal cells via attachment points called desmosomes. When autoantibodies attack desmogleins, the cells become separated from each other and the epidermis becomes unglued. This phenomenon is called acantholysis. This results in blisters that slough off and eventually turn into ulcers. Sometimes the whole body can be involved with a wide area of skin involvement. [2],[4],[5]

Types

There are three types of pemphigus that vary in severity: pemphigus vulgaris, pemphigus foliaceus, and paraneoplastic pemphigus. [1],[6],[7]

Diagnosis

Diagnosis is mostly based on the appearance and distribution of the skin lesions. Definitive diagnosis requires examination of a skin biopsy for histopathology that shows acantholysis and "tombstone appearancnone e".

Definitive diagnosis also requires the demonstration of anti-desmoglein autoantibodies, by direct immunoflorescence on the skin biopsy. Anti-desmoglein antibodies can also be detected in a blood sample using the ELISA technique. A high titer of cANCA (cellular Anti Neutrophil Cytoplasmic Antibody) is an important feature of the diseasenone . [5],[8]

Treatment

The most common treatment is the administration of oral steroids, such as, prednisone. To counteract the side effects of corticosteroids, steroid sparing or adjuvant drugs may be used along with the steriod. The immunosuppressant CellCept-like mycophenolic acidnone is among those being used in the drug. [8],[9]


   Case Report Top


A male patient, aged about 49 years, reported to the Department of Oral Medicine and Radiology at Krishnadevaraya College of Dental Sciences, Bangalore, with a chief complaint of multiple ulcers in the oral cavity as well as ulcers on the scalp, chest, and the back of his body.

The patient also gave a history of recurrent ulcers on the trunk and extremities. The blisters broke down spontaneously giving rise to painful ulcers. On clinical examination, the patient had multiple ulcers in the oral cavity and on the trunk and scalp. The ulcers were well demarcated and with a tendency to heal. However, there were no intact blisters or bullae on his body. Ulcers were present on the right and left buccal mucosa and floor of the mouth. Ulcers on the scalp were present in varying stages of the healing process [Figure 1],[Figure 2],[Figure 3],[Figure 4],[Figure 5].

A provisional diagnosis of pemphigus vulgaris was given and a further investigation, a Tzanck smear, was done [Figure 6].

Histopathology

A cytological smear showed normal squamous epithelial cells. However, a few noncohesive, circular-to-oval Tzank cells with prominent hyperchromatic nuclei were seen in the cytosmear.

Management

The patient was treated with steroids such as prednisolone 40-60 mg for a month, with tapering of the dose.

Patient evaluation and re-call

The patient was then re-called after a month for evaluation and the patient showed good response to the steroid treatment with regression of the lesions in his oral cavity as well as on the other surface of his body. The patient was then referred to a dermatologist for further management,

Special note

Pemphigus vulgaris is a chronic autoimmune mucocutaneous disease that initially manifests in the form of intraoral lesions, which spread to other mucous membranes and the skin. The etiology of pemphigus vulgaris is still unknown, although the disease has attracted considerable interest. The pemphigus group of diseases is characterized by the production of autoantibodies against intercellular substances and is thus classified as an autoimmune disease. Most patients are initially misdiagnosed and improperly treated for many months or even years. Dental professionals must be sufficiently familiar with the clinical manifestations of pemphigus vulgaris to ensure early diagnosis and treatment, since this in turn determines the prognosis and course of the disease. Here, we have reported a case of pemphigus vulgaris that was misdiagnosed in its earliest stage. [20]

 
   References Top

1.Khaled A, Fazaa B, Mrabet N, Zeglaoui F, Kamoun MR. Multiple autoimmune syndrome in a patient with pemphigus vulgaris: A new combination. Tunis Med 2008;86:595-7.  Back to cited text no. 1  [PUBMED]  
2.Cirillo N, Femiano F, Gombos F, Lanza A. High-dose pemphigus antibodies against linear epitopes of desmoglein 3 (Dsg3) can induce acantholysis and depletion of Dsg3 from keratinocytes. Immunol Lett 2009;122:208-13.   Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Farage MA, Miller KW, Berardesca E, Maibach HI. Clinical implications of aging skin: Cutaneous disorders in the elderly. Am J Clin Dermatol 2009;10:73-86.  Back to cited text no. 3    
4.Alecu M, Ursaciuc C, Surcel M, Coman G, Ciotaru D, Dobre M. CD28 T-cell costimulatory molecule expression in pemphigus vulgaris. J Eur Acad Dermatol Venereol 2008 in press.   Back to cited text no. 4    
5.Schmidt E, Waschke J. Apoptosis in pemphigus. Autoimmun Rev 2009 in press.   Back to cited text no. 5    
6.Kakurai M, Demitsu T, Iida E, Umemoto N, Yamada T, Yoneda K, et al . Coexistence of paraneoplastic pemphigus and bullous pemphigoid. J Eur Acad Dermatol Venereol 2009.   Back to cited text no. 6    
7.Sehgal VN, Srivastava G. Paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome. Int J Dermatol 2009;48:162-9.  Back to cited text no. 7  [PUBMED]  [FULLTEXT]
8.Heupel WM, Mueller TD, Efthymiadis A, Schmidt E, Drenckhahn D, Waschke J. Peptides targeting the desmoglein 3 adhesive interface prevent autoantibody-induced acantholysis in pemphigus. J Biol Chem 2009;284:8589-95.  Back to cited text no. 8    
9.Singal A, Pandhi D. Localized pemphigus vulgaris on cheeks responding to topical steroids. Indian J Dermatol Venereol Leprol 2009;75:80-1.   Back to cited text no. 9  [PUBMED]  Medknow Journal
10.Arnold DF, Burton J, Shine B, Wojnarowska F, Misbah SA. An 'n-of-1' placebo-controlled crossover trial of intravenous immunoglobulin as adjuvant therapy in refractory pemphigus vulgaris. Br J Dermatol 2009 in press.   Back to cited text no. 10    
11.Bangsgaard N, Rossen K, Skov L, Torpet LA. Picture of the month: Pemphigus vulgaris. Ugeskr Laeger 2009;171:533.   Back to cited text no. 11  [PUBMED]  
12.Parambil JG, Yi ES, Ryu JH. Obstructive bronchiolar disease identified by CT in the non-transplant population: Analysis of 29 consecutive cases. Respirology 2009;14:443-8.   Back to cited text no. 12  [PUBMED]  [FULLTEXT]
13.Kartono F, Shitabata PK, Magro CM, Rayhan D. Discohesive malignant melanoma simulating a bullous dermatoses. J Cutan Pathol 2009;36:274-9.  Back to cited text no. 13  [PUBMED]  [FULLTEXT]
14.Mignogna MD, Fortuna G, Leuci S, Adamo D, Ruoppo E. Delayed immunologic evidence in pemphigus vulgaris. J Eur Acad Dermatol Venereol 2009 in press.   Back to cited text no. 14    
15.Aguado L, Marquina M, Pretel M, Ruiz-Carrillo G, Espaρa A. Lesions of pemphigus vulgaris on irradiated skin. Clin Exp Dermatol 2009 in press.   Back to cited text no. 15    
16.Ramam M. Prolonged antimicrobial and oral cyclophosphamide therapy in pemphigus: Need for caution. Indian J Dermatol Venereol Leprol 2009;75:85.   Back to cited text no. 16  [PUBMED]  Medknow Journal
17.Singh S, Chaudhary R. Pulse therapy for pemphigus: The burden of proof. Indian J Dermatol Venereol Leprol 2009;75:82-4.   Back to cited text no. 17  [PUBMED]  Medknow Journal
18.El Fekih N, Kharfi M, Karaa A, Kamoun MR. Complete recovery from juvenile pemphigus vulgaris. Indian J Dermatol Venereol Leprol 2008;74:654-5.   Back to cited text no. 18    
19.Bertram F, Brφcker EB, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges 2009 in press.   Back to cited text no. 19    
20.Han JI, Mo IP, Na KJ. Pemphigus-like immune-mediated dermatosis in a Korean native black-bone fowl. Avian Dis 2008;52:714-7.  Back to cited text no. 20  [PUBMED]  


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]



 

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