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ORIGINAL CONTRIBUTIONS
Year : 1999  |  Volume : 65  |  Issue : 4  |  Page : 168-171

A clinico-pathological study of 70 cases of pemphigus




Correspondence Address:
S R Arya


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Source of Support: None, Conflict of Interest: None


PMID: 20921646

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  Abstract 

A clinicopalhological study of 70 cases of pemphigus observed over a span of four and a half years from January 1992 to June 1996 at the Sir J.J. Group of Hospitals and Grant Medical College, Mumbai is reported. Pemphigus vulgaris constituted the single largest group of 43 cases, followed by pemphigus foliaceus (25 cases) and pemphigus vegetans (2 cases). Majority of the cases were seen in the age group of 21-60 years, with a slight male predominance. The youngest patient was 14 years while the eldest was aged 75 years. Mucosal involvement was seen in 31 cases of pemphigus vulgaris, as opposed to only 5 cases of pemphigus foliaceus. Flaccid bullae were present in 100% cases. Pruritus was complained of in 14 cases, though it was more common in pemphigus vegetans and vulgaris. Salient histopathological features of pemphigus vulgaris observed were (I) intraepidermal suprabasal blisters (35 cases), (2) presence of acantholytic cells (40 cases), (3) "Row of tombstone appearance" (I8 cases) and (4) acantholysis involving follicular sheath (20 cases). Main histopathological features of pemphigus foliaceus were (1) subcorneal blister (15 case), (2) acantholysis (24 cases) and (3) bulla cavity containing inflammatory infiltrate (12 cases). Both cases of pemphigus vegetans showed hyperkeratosis, papillomatosis and irregular acanthosis with intra-epidermal eosinophilic abscesses besides suprabasal lacunae.


Keywords: Pemphigus vulgaris, Pemphigus foliaceus, Pemphigus vegetans


How to cite this article:
Arya S R, Valand A G, Krishna K. A clinico-pathological study of 70 cases of pemphigus. Indian J Dermatol Venereol Leprol 1999;65:168-71

How to cite this URL:
Arya S R, Valand A G, Krishna K. A clinico-pathological study of 70 cases of pemphigus. Indian J Dermatol Venereol Leprol [serial online] 1999 [cited 2018 Jan 20];65:168-71. Available from: http://www.ijdvl.com/text.asp?1999/65/4/168/4799



  Introduction Top


Pemphigus is a rare vesiculo-bullous disease having a grave prognosis. Acantholytic cells and clefts or bullae are formed in the epidermis as a result of interaction of antibodies with epidermal intercellular cement substance. In 1880, Auspitz recorded the histological findings of pemphigus blister and coined the term acantholysis.[1]

Lever has classified pemphigus into two categories based on the level of blisters. Pemphigus vegetans was considered a variant of pemphigus vulgaris with suprabasal clefts. Pemphigus erythematosus was considered a variant of pemphigus foliaceus due to a subcorneal bulla.

Though the disease is not uncommon in dermatological practice, only a few reports on this disease have been reported in Indian literature, especially from Northern India. The present study of 70 cases intends to highlight various clinicopathological aspects of the disease as it occurs in Mumbai region of Western Maharashtra.


  Materials and Methods Top


This study comprises of 70 cases of pemphigus seen at the Sir J.J. Group of Hospitals and Grant Medical College observed over four and a half years (January 1992 to June 1996).

A detailed history with particular reference to the mode of onset, characteristics and distribution of the lesions was taken. In addition to the routine hematological and urine investigations, elliptical skin biopsy of early lesion and surrounding skin tissue of all 70 cases were processed by paraffin section technique and stained by haematoxylin and eosin. Multiple serial sections of the biopsy were studied.


  Results Top


Out of the 70 cases of pemphigus studied, pemphigus vulgaris was the predominant type with 43 cases (61.4%) followed by 25 cases (35.7%) of pemphigus foliaceus and 2 cases (2.9%) of pemphigus vegetans.

Of the 43 cases of pemphigus vulgaris, 38 patients (88.4%) were between 21 and 60 years; and 2 cases (4.6%) were below 20 years of age. Only 3 cases (6.9%) were above 60 years. Out of the 25 cases of pemphigus foliaceus, 20 patients (80%) were in the age group of 21-60 years; while 2 patients (8%) were older than 60 years and 3 patients (12%) were below 20 years. Two cases of pemphigus vegetans were seen; both were between the age group 21-60 years.

There was a slight male preponderence in the ratio of 1.4:1 (25 males and 18 females)

Pemphigus vulgaris showed initial lesions involving mucous membranes in 37.2% cases and lesions including both skin and mucosae in 13.9% cases. Mucosal involvement at one time or the other was seen in 31 patients (72.1 %). On the other hand, in pemphigus foliaceus, only 4% cases showed initial mucosal involvement and 12% cases showed involvement of both skin and mucous membrane involvement.

The nature of the lesions observed during the course of pemphigus is shown in [Table - 1]. Flaccid bullae were found in all cases. Blisters seen arising on nonerythematous skin were seen in 42 cases (97.6%) of pemphigus vulgaris, which spontaneously ruptured to give rise to erosions in 27 cases (62.8%). Crusted lesions, erythematous plaques, vegetations and pustules were present less frequently. In pemphigus foliaceus, blisters arising on erythematous skin were seen in 17 cases (68%), crusted lesions in 13 cases (52%), erosions in 11 cases (44%) and pustules in 8% cases. Vegetations were seen in both cases of pemphigus vegetans.

Distribution of skin lesions showed common sites of involvement in pemphigus vulgaris were generalised involvement in 16 cases (37.2%), face in 9 Cases (20.9%), scalp in 16 cases (18.6%). On the other hand, in pemphigus foliaceus, there were lesions in generalised distribution in 8 cases (32%), face in 7 cases (28%), scalp in 4 cases (16%), trunk in 4 cases (56%) and involvement of extremities in 12 cases (48%).

Pruritus was seen in 14 cases (20%) of all pemphigus cases. Eleven cases (25.6%) of pemphigus vulgaris complained of pruritus. Both of our cases (100%) of pemphigus vegetans as compared to a solitary case (4%) of pemphigus foliceus had pruritus.

Of the 36 cases of pemphigus vulgaris and 19 cases of pemphigus foliaceus in which history of Nikolsky's sign was provided, it was positive in 35 cases (97.2%) and 18 cases (94.7%) respectively. Both cases of pemphigus vegetans showed positive Nikolsky's sign.

Of the 43 cases of pemphigus vulgaris, 35 cases (81.4%) showed intra-epidermal suprabasal vesicles and 8 cases (18.6%) showed mid-epidermal vesicles [Table - 2]. Midepidermal vesicles were seen in old bullae, due to regeneration of the cells from the floor of the bulla. Acantholysis was seen in 93% cases as groups of cells or single cells within the bulla cavity. Dyskeratosis, basal layer budding and pseudo-epitheliomatous proliferation was not seen in any of the cases. Acantholysis affecting follicular sheath was seen in 46.5% cases. An inflammatory infiltrate was present in the bulla cavity in 23 cases (53.5%). Neutrophils were predominant in 9 cases (20.9%) and eosinophils 11 cases (25.6%). Eosinophilic spongiosis was seen in 3 cases (6.9%). Two of them were known cases of pemphigus and had developed new lesions after discontinuation of steroids. In 8 cases (18.6%), the epidermis was lost during the process. Five of these cases (11.6%) had been reported negative previously and could be diagnosed on the basis of the above histological features.

Of the 25 cases of pemphigus foliaceus studied, 24 cases (96%) showed acantholysis [Table - 3]. Subcorneal bulla was seen in 15 cases (60%), subgranular cleavage from middle epidermis in 6 cases (24%). Dyskeratosis, basal layer budding and pseudoepitheliomatous proliferation were not seen in any of the cases. Acantholysis affecting follicular sheath was seen in 46.5% cases. An inflammatory infiltrate was present in the bulla cavity in 23 cases (53.5%). Neutrophils were predominant in 9 cases (20.9%) and eosinophils in 11 cases (25.6%). Eosinophilic spongiosis was seen in 3 cases (6.9%). Two of them were known cases of pemphigus and had developed new lesions after discontinuation of steriods. In 8 cases (18.6%), the epidermis was lost during the process. Five of these cases (11.6%) had been reported negative previously and could be diagnosed on the basis of the above histological features.

Of the 25 cases of pemphigus foliaceus studied, 24 cases (96%) showed acantholysis [Table - 3]. Subcorneal bulla was seen in 15 cases (60%), subgranular cleavage from middle epidermis in 6 cases (24%). Dyskeratosis was seen in only 2 cases (8%). Inflammatory cells were seen in the bulla cavity in 12 cases (48%). In 10 cases (83.3%) the infiltrate comprised of polymorphs. Spongiosis was seen in 10 cases (40%) and exocytosis in 8 cases (32%). Acanthosis, hyperkeratosis, parakeratosis, papillomatosis and increased pigment formation were seen in a few cases.

Both cases of pemphigus vegetans showed hyperkeratosis, papillomatosis and irregular acanthosis with intra-epidermal eosinophilic abscesses. Suprabasal lacunae with a few acantholytic cells were seen. Both the cases were of Neumann type. No cases of pemphigus erythematosus, drug-induced pemphigus and paraneoplastic pemphigus were seen in our study.


  Discussion Top


Our series is in accordance with other Indian series each of which has maximum cases of pemphigus vulgaris, followed by pemphigus erythematosus/ foliaceus and then by pemphigus vegetans.[2][3] Majority (85.7%) of our patients were between 21-60 years, akin to Indian literature.[3][4] Pemphigus vulgaris affects males and females equally,[5] although in the present study, there was a slight male predominance in the ratio 14.:1, which probably reflects the higher male attendance in the out-patient department. Male dominance has been reported in the same ratio.[6]

Nature and distribution of lesions as well as mucosal involvement in different types of pemphigus in our series has followed the pattern seen in earlier studies.[4][6][7][8][9] Flaccid bullae were seen in all the cases. Mucosal involvement was seen' in 31 patients (72.1 %) of pemphigus vulgaris, and only in 5 patients (20%) of pemphigus foliaceus. Pruritus was seen in 25.6% of our cases of pemphigus vulgaris, slightly lower than reported before. [3, 4] Nikolsky's sign was positive in 97.2% and 94.7% cases of pemphigus vulgaris and foliaceus respectively, in corroboration with reports in Indian literature.

The salient histological features of pemphigus vulgaris were (1) intraepidermal suprabasal blisters (2) presence of acantholytic cells (3) "Row of tombstone appearance" (4) Acantholysis involving follicular sheath.

Salient features of pemphigus foliaceus were (1) acantholysis (2) subcomeal cleavage (3) an inflammatory infiltrate.

Both of our cases of pemphigus vegetans were of Neumann type.

 
  References Top

1.Crosby DL, Diaz LA. Bullous disease: Introduction. Dermatol Clin 1993;11:373-378.  Back to cited text no. 1  [PUBMED]  [FULLTEXT]
2.Sehgal VN. Pemphigus in India. Indian J Dermatol Venereol 1972;18:5-7.  Back to cited text no. 2  [PUBMED]  [FULLTEXT]
3.Bedi BMS, Prasad TS. A study on pemphigus- Clinical and histological. Indian J Dermatol 1975;20:72-77.  Back to cited text no. 3    
4.Singh R, Pandhi RK, Pal D, et al. A clinico-pathological study of pemphigus. Indian J Dermatol Venereol 1973;39:126-132.  Back to cited text no. 4    
5.Krain LS. Pemphigus. Epidemiologic and survival characteristics of 59 patients from 1955-1973. Arch Dermatol 1974;110:862-865.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Handa F, Agarwal RR, Raj Kumar. A study of 85 cases of pemphigus. Indian J Dermatol Venerol 1975;39:106-111.  Back to cited text no. 6    
7.Fernandez JC, Dharani JB, Desai SC. A study of 100 cases of pemphigus-clinical features. Indian J Dermatol Venereol 1970;36:1-11.  Back to cited text no. 7    
8.Director W. Pemphigus vulgaris. A clinico-pathological study. Arch Dermatol 1952;65:155-169.  Back to cited text no. 8  [PUBMED]  [FULLTEXT]
9.Kandhari KC, Pasricha JS. Pemphigus in Northern India. Clinical studies in 34 patients. Indian J Dermatol Venereol 1965;31:62-71.  Back to cited text no. 9    


    Tables

[Table - 1], [Table - 2], [Table - 3]



 

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