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   Abstract
   Introduction
   Case Report
   Discussion
   References

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SHORT COMMUNICATIONS
Year : 2000  |  Volume : 66  |  Issue : 2  |  Page : 85-86

Transition of pemphigus vulgaris to pemphigus foliaceus




Correspondence Address:
Vibhu Mendiratta


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Source of Support: None, Conflict of Interest: None


PMID: 20877034

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  Abstract 

We report a case of pemphigus vulgaris in a 46-year-old woman who presented with scaly and crusted erosions over erythematous base associated with itching and burning mainly over the chest, submammary folds, face, abdomen and the back. Histopathology was consistent with pemphigus foliaceus. The patient responded to oral prednisolone (60 mg/day). The above findings suggest that pemphigus vulgaris showed a transition to the pemphigus foliaceus variant.


Keywords: Pemphigus vulgaris, Pemphigus foliaceus


How to cite this article:
Mendiratta V, Sarkar R, Sharma R C, Korann. Transition of pemphigus vulgaris to pemphigus foliaceus. Indian J Dermatol Venereol Leprol 2000;66:85-6

How to cite this URL:
Mendiratta V, Sarkar R, Sharma R C, Korann. Transition of pemphigus vulgaris to pemphigus foliaceus. Indian J Dermatol Venereol Leprol [serial online] 2000 [cited 2019 Aug 17];66:85-6. Available from: http://www.ijdvl.com/text.asp?2000/66/2/85/4876



  Introduction Top


Pemphigus is an autoimmune bullous disorder affecting the skin and the mucous membranes. Immunoblot techniques and immunoprecipitation have identified various antigens in pemphigus [1],[2] which subdivide pemphigus into pemphigus vulgaris (PV) and pemphigus foliaceus (PF). Desmoglein 3 [3] is a 130 KD protein belonging to the'cadherin' [4] family of molecules and is known as the PV antigen, whereas desmoglein 1 [1] is a 160 KD glycoprotein known as the PF antigen.

Biochemical similarity between the two desmogleins is responsible for the antibodies to either one of them recognizing epitopes on the other. Many patients with pemphigus have a mixture of antibodies which react with both PV (130 KD) and PF (160 KD) antigens [6],[7] hence, an admixture of clinical manifestations is possible in pemphigus due to the immunological cross-reactivity of antibodies.

We report a case of pemphigus vulgaris which showed a 'clinical transition' into pemphigus foliaceus in a woman.


  Case Report Top


A 46-year-old woman who was diagnosed as a case of pemphigus vulgaris in the past presented with erosive-crusted lesions over the face, chest and back of one-month duration. The patient had been taking her maintenance dose of steroid, tablet prednisolone 10 mg daily along with tablet cyclophosphamide 50 mg. There was no history of preceding drug intake or photosensitivity. Clinical examination revealed multiple superficial erosions with crusting over the submammary area, chest, back and face, associated with burning and itching. No intact vesiculobullous lesions were seen. Nikolsky's sign was postive. The laboratory investigations were normal except for increased blood sugar. On admission, the dose of steroid was increased to 60 mg of tablet prednisolone daily, along with systemic antibiotic and local therapy.

After admission, the lesions slowly increased in number to involve the entire chest, upper back and the submammary folds. Histopathological examination revealed acantholysis in the upper epidermis from the granular to the subcorneal region and focal eosinophilic spongiosis with a mixed inflammatory perivascular infiltrate in the dermis and was consistent with the diagnosis of pemphigus foliaceus. The oral corticosteroid dose was increased to 80 mg per day, to which the cutaneous as well as the oral lesions responded and completely disap­peared after two weeks leaving behind brown macular pigmentation.


  Discussion Top


Our patient was a diagnosed case of pemphigus vulgaris who subsequently developed superficial erosions with crusting over the seborrheic sites of the body without any intact vesiculobullous lesions, with a positive Nikolsky's sign, without mucous membrane involvement. Both the clinical features and the histopathology confirmed a transition to pemphigus foliaceus. Immunofluorescence / immunoblot technique could not be employed due to the nonavailability.

It is suggested that pemphigus foliaceus is more likely to show a transition to pemphigus vulgaris and patients with both. the antibodies will almost always exhibit clinical and histopathological features of PV, but the reverse is also true. [7] Hence the clinical expression of the disease may be determined by immunologic or genetic factors.







 
  References Top

1.Stanley JR, Year M, Hanoley - Nelson P, et al. Pemphigus antibodies identify a cell surface glycoprotein synthesized by human and mouse keratinocytes. J Clin Invest 1982; 70: 281 - 288.  Back to cited text no. 1    
2.Kirtsching G, Wojanarowska F. Autoimmune blistering disease: an update of diagnostic methods and investigations. Clin Exp Dermatol 1994; 19: 97-112.  Back to cited text no. 2    
3.Loomis CA, Korega J, Manabe M, et al. Characterization of a keratinocyte specific extracellular epitope of desmoglein. Implications for desmoglein heterogeneity and function. J Biol Chem 1992; 267: 1676-1684.  Back to cited text no. 3    
4.Buxton RS, Cowin P, Franke WW, et al. Nomenclature of the desmosomal cadherines. J Cell Bio 1993; 121 : 481-483.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Allen EM. Giudice GJ, Diaz LA. Subclass reactivity of pemphigus foliaceus autoantibodies with recombinant human desmoglein. J Invest Dermatol 1993; 100: 685-691.  Back to cited text no. 5    
6.Hashimoto T, Amagi M, Garrod DR, et al. Immunofluorescence and immunoblot studies on the reactivity of pemphigus foliaceus sera with desmoglein 3 and desmoglein 1. Epithelial Cell Biol 1995; 4 : 63-69.  Back to cited text no. 6    
7.Chang SN, Kum SC, Lee U, et al. Transition of pemphigus vulgaris to pemphigus foliaceus. Br J Dermatol.1997; 137 : 303 - 305.  Back to cited text no. 7    




 

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