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STUDIES
Year : 1994  |  Volume : 60  |  Issue : 3  |  Page : 140-143

Pemphigus : A clinical study of 109 cases from Tripoli, Libya




Correspondence Address:
M Shafi


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  Abstract 

From 1981 to 1992, 109 cases (90 females and 19 males) of pemphigus were seen in the Department of Dermatology, Central Hospital Tripoli, Libya. Age of onset of the disease varied from 17 to 85 years, while duration of the disease at the time of presentation varied from 3 days to 13 days. On the basis of clinical features and routine histological findings the cases were divided into various subtypes as : pemphigus foliaceus 65 cases, pemphigus vulgaris 34 patients, pemphigus erythematosus 5, herpetiform pemphigus 3 and vegetans type 2 cases. Three of our patients had diabetes mellitus preceding pemphigus, while 12 patients developed steroid induced diabetes. Significant secondary bacterial infection occurred in all cases at some stage of the disease while oral candidiasis occurred in 15 cases. Eczema herpeticum was seen in 4 patients, while 2 had extensive tinea corporis. One of 4 patients of pemphigus vulgaris had complete shedding of nail and 1 female patient had alternate phases of pemphigus foliaceus and generalized pustular psoriasis. The features in our cases of pemphigus foliaceus were somewhat similar to Brazilian pemphigus foliaceus and we had more cases of pemphigus foliaceus, almost exclusively affecting females.


Keywords: Pemphigus


How to cite this article:
Shafi M, Khatri M L, Mashina M, Ben-Ghazeil M. Pemphigus : A clinical study of 109 cases from Tripoli, Libya. Indian J Dermatol Venereol Leprol 1994;60:140-3

How to cite this URL:
Shafi M, Khatri M L, Mashina M, Ben-Ghazeil M. Pemphigus : A clinical study of 109 cases from Tripoli, Libya. Indian J Dermatol Venereol Leprol [serial online] 1994 [cited 2019 Sep 16];60:140-3. Available from: http://www.ijdvl.com/text.asp?1994/60/3/140/4020



  Introduction Top


Pemphigus is a group of blistering diseases, varying in clinical features and incidence in various population groups.

We have seen 109 cases of pemphigus since 1981 at the Central Hospital, Tripoli, Libya. An analysis of their clinical features is presented.


  Materials and Methods Top


All patients were admitted for initial assessment and treatment and many of them were re-admitted several times with exacerbation of the disease. Details of history, physical findings, laboratory data, treatment, and follow-up were recorded in a protocol. The diagnosis was based on clinical features and histopathology. Immunofluorescence studies could be arranged only in 7 patients.


  Results Top


Of the 109 cases only 2 men were Indian while others were Libyan nationals, 90 (75.6%) were female and 19 (24.4%) were male. The age at onset of the disease ranged between 17 years and 85 years in general, in cases of pemphigus vulgaris between 20 and 85 (median age 40 years), in cases of pemphigus vegetans between 23 and 40 years (median age 31 1/2 years), and in cases of pemphigus foliaceus between 17 and 85 years (median age 29 years). The most common age at onset was between 21 and 30 years [Table - 1].

The duration of the disease at the time of presentation to our department ranged between 3 days to 13 years. The onset of the disease process was gradual in 102 patients while it was acute in 7 patients.

The disease was asymptomatic in 81 (74.3%) but 28 (25.7%) patients complained of itching, mild in 15, moderate in 11, and severe in 2 patients.

The type of initial lesions and initial sites of involvement are given in [Table - 2],[Table - 3]

respectively. Out of 34 cases of pemphigus vulgaris 24 had lesions initially in oral mucosa and later developed skin lesions after an interval ranging from 15 days to 1'/z years; one patient had initial lesions on the conjunctivae while 6 patients developed mouth and body lesions simultaneoulsy and 3 developed skin lesions 1 week to 1 month earlier than the mouth lesions.

The nature of the various type of lesions observed during the course of the disease is given in [Table - 4]. One female patient with pemphigus vulgaris had onychomadesis of all the nails during the initial acute phase but later complete regrowth of the nails was observed after the control of the disease. The details of distribution of the lesions is given in [Table - 5].

On the basis of clinical features and histopathologic findings the cases were divided in five sub-types [Table - 6].

Other associated diseases present before and developing after steroid therapy are listed in [Table - 7]. Eczema herpeticum developed in 4 patients with pemphigus foliaceus while on high dose of steroid and 3 of them were on combination therapy of steroid and azathioprine. Variable degree of bacterial infection occurred in all cases at some stage of the disease. Pus culture of the infected lesions revealed growth of coagulase positive Staphylococci in 92, E Coli in 11, pseudorn_onas aeruginosa in 8, Streptococci in 5, and Froteus mirrabilis in 4 patients.


  Follow-up Top


One hundred and three cases could be followed for 6 months to 8 years, while 6 patients did not turn up after the first admission. Up to date follow-up was possible only in 34 cases. Six patients with pemphigus foliaceus went into remission ranging from 6 months to 2 years without treatment but later recurrence occured in 5 while one of them is still in complete remission. Longest duration of the disease recorded during follow-up was 16 years in cases with pemphigus vulgaris. Three patients with pemphigus vulgaris died in hospital due to complications directly related to the diseases and steroid therapy, 2 due to septicaemia and third due to cardiac arrest.


  Comments Top


Although we could not estimate the exact incidence of pemphigus in Libya, it seems to be quite high. High incidence in Mediterranean population particularly in the Jewish race has been documented.' In the previous studies [1],[3] sex ratio has been recorded to be equal in male and female but in the present study there was predominance of female (F:M=90:19), moreover pemphigus foliaceus patients were all female except 3 male. In our study median age at onset of pemphigus vulgaris was 40 years, pemphigus vegetans 31 1/2 years and pemphigus foliaceus 29 years, which is almost identical with previous reports.' However, in our cases of pemphigus foliaceus, the onset was mainly in young females. In 2 Indian patients with pemphigus vulgaris the age at onset was 26 years and 29 years. Early onset of pemphigus vulgaris is recorded in India . [2]

Itching was a significant complaint in 28 cases of pemphigus foliaceus in the present series while it has not been recorded as a prominant feature in the earlier studies. The course of pemphigus foliaceus has been recorded previously as comparatively benign but in the present study majority of the patients had a fulminant course although 6 patients showed temporary remission without steroid therapy. One female patient had alternate phase of pemphigus foliaceus and generalized pustular psoriasis. This patient developed attacks of generalised pustular psoriasis while on high dose of methylprednisolone, which could be controlled on administration of weekly methotrexate therapy.

Taking into consideration, the early age of onset, initial sites, type of lesions and course of disease in our cases of pemphigus foliaceus, the similarity to Brazilian pemphigus foliaceus is striking. However there are some dissimilarities. In Brazilian pemphigus, sex ratio is equal but in this series the pemphigus foliaceus cases were exclusively seen in female except 3 males. Moreover we did not record any evidence of the disease in other family menbers, which is a prominant feature of Brazilian pemphigus foliaceus. [3]

It is concluded that the incidence of pemphigus seems to be high in Libya, with high female predominance of the disease. For reasons, which need further study we have more cases of pemphigus foliaceus, almost exclusively affecting females.

 
  References Top

1.Pye R J. Bullous eruptions. In : Textbook of Dermatology (Champion R H, Burton J L, Ebling F J G, eds), 5th edn. Oxford Blackwell Scientific, 1992; 1638-47.  Back to cited text no. 1    
2.Desai S, Rao S. Hautarzt, 1960; 11 : 445.  Back to cited text no. 2  [PUBMED]  
3.Azulay R D. Brazilian pemphigus foliaceus. Int J Dermatol, 1982; 21 : 122-4.  Back to cited text no. 3    


    Tables

[Table - 1], [Table - 2], [Table - 3], [Table - 4], [Table - 5], [Table - 6], [Table - 7]

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