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ARTICLE
Year : 1968  |  Volume : 16  |  Issue : 3  |  Page : 152-153

Ectodermosis erosiva pluri-orificialis - Stevens Johnson syndrome


Burla Medical College, Sambalpur, Orissa, India

Date of Web Publication24-Dec-2007

Correspondence Address:
K Misra
Burla Medical College, Sambalpur, Orissa
India
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Source of Support: None, Conflict of Interest: None


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How to cite this article:
Misra K, Das C. Ectodermosis erosiva pluri-orificialis - Stevens Johnson syndrome . Indian J Ophthalmol 1968;16:152-3

How to cite this URL:
Misra K, Das C. Ectodermosis erosiva pluri-orificialis - Stevens Johnson syndrome . Indian J Ophthalmol [serial online] 1968 [cited 2020 Jul 5];16:152-3. Available from: http://www.ijo.in/text.asp?1968/16/3/152/37541

In the year 1922 STEVENS AND JOHNSON [7] reported two cases affect­ing children suffering from fever with multiple eruptions, stomatitis and ophthalmia. Since then the syndrome goes by their name. They emphasis­ed on micrococci (Staphylococci) as the septicEmia producing factor in this syndrome. KLAUDER [1] labelled this syndrome as "Ectodermosis Ero­siva Pluriorificalis," while ROBIN­SON AND McCRUMB [6] took it as a variant of Erythema multiforme. ROOK AND LEVER [5] considered this syndrome as a bullous type of Ery­thema multiforme with the features of pemphigus (butchers pemphigus). This syndrome resembles the human form of Foot and Mouth disease as reported by KLAUDER. [1] PRICE [5] takes it as a severe variant of erythe­ma multiforme.

Case note

M. S. 60 years was admitted on 22-6-66 to the eye ward of this Hos­pital with the following complaints.

i) Purulent discharge from both the eyes since three days.

ii) Inability to take food for five days.

iii) Fever and malaise since five days.

iv) Papules in the extremities since three days.

v) Muco-purulent discharge per urethra since three days.

On Examination

The patient was acutely ill with fever 103.4◦F, pulse 140/min, tongue coated and ulcerated. Ulcers in the lips compelled the patient to keep the mouth open. There was muco­purulent discharge from the eyes and urethra and multiple bullous swell­ings in the extremities, face and scalp, The skin was so easily giving way that it was a problem to give injec­tions. A little bit of digital pressure on scalp or face during examination would lead to erosion of that part of the skin.

Eye

Severe type of bulbar and tarsal congestion of conjuctiva with profuse mucopurulent discharge. Cornea was clear. Immature cataracts were pre­sent in both eyes.

Investigations:

Blood.
Wassermann reaction and V.D.R.L. were negative. Blood-sugar was 112 mg per cent Haemoglobin was 11.2 gm per cent. Total W.B.C, count 12,000/c.mm blood. Differential count: 72 per cent, neutrophils, 26 per cent, lymphocytes, 2 per cent eosinophils.

Urine: sugar and albumin nil. Lar­ge number of pus cells and R.B.C.'s.

Stools. Nothing abnormal. Conjunctival smear: Gram positive cocci cultivated.

Treatment

Predinisolon was given 40 mg daily in divided doses to start with and then tapered off. Achromycin 1 cap. 6 hourly - total 28 caps supplemented by Vitamin B complex and Vitamin C in heavy doses. Gentian violet was applied locally on the ulcers and erupted vesicles, Terramycin eye oint­ment was applied 3 times a day after proper washing of the eyes.

Course

The toxic features diminished re­markably within 36 hours and the patient was able to take food after 5 days. The patient was cured with­in 19 days stay in the Hospital. His affected skin shed off completely and got replaced by fresh skin. The pa­tient has been followed for more than a year and found to be free from any recurrence.


  Comments Top


Although STEVENS AND JOHN­SON [7] described the condition in 1922 in children with stomatitis and oph­thalmia, the condition has been des­cribed long before hat by RENDU [4] and later by FIESSINGER AND RENDU [1] who have emphasised the painless and non-recurring features, not necessarily confined to children. The condition need not also be con­fined to only two of the orifices as described by STEVENS AND JOHNSON, but all the orifices may he involved-mouth, nose, conjunc­tiva, urethra, vagina and anus. (Klauder [3] ). The veteran FUCHS[2] described the conjunctival involve­ment as early as in 1876 as an ocular manifestation in erythema multi­forme and called it "herpes iris con­junctiva". He described two varie­ties of the same, a mild form and a severe one with membrane forma­tion and involvement of the cornea, but evidently he saw no need of glorifying it as a clinical entity. In­deed, the skin lesions may develop without involvement of the mucous membrane at the orifices and vice­versa. It is possible at that rate for the conjunctiva or the other mucous membranes to be affected individu­ally or in combinations of two or more with or without skin involve­ment. It is therefore necessary to have a broader outlook on this con­dition, rather than consider it in the confines of a single syndrome.

Our patient proved to be a case of Stevens-Johnson syndrome for having multiple erosive cutaneous lesions, distressing mucosal involvements of the mouth, urethra and conjuctiva with general toxic features. But this patient is a little different from the other very few reported cases in that he was an older (60 years) patient. Also the eruptions were not limited to the extremities, but had spread to the face and scalp. PRICE [5] considers this syndrome to be due to hypersen­sitivity to a variety of known or sus­pected causes, in particular bacterial and virus infections and drug intoxi­cation. In this particular patient though a specific cause could not be traced, the presence of Gram - posi­tive cocci in the mucosal ulcers can be speculated as the etiological fac­tor.


  Summary Top


A case of Stevens Johsons Syn­drome is presented.

 
  References Top

1.
FIESSINGER N. AND RENDU R.: On a Syndrome characterized by si­multaneous Inflammation of all the ex­ternal Mucous Membranes coexistant with a vesicular Eruption of the four Members, painless and non-recurring (in French) Paris med. 23, 54, (1917) as quoted by KLAUDER in 2.  Back to cited text no. 1
    
2.
FUCHS, E.: Herpes Iris conjunctivae. Klin. Mbl. Augenh. 14, 333, (1876) as quoted by Klauder in 3.  Back to cited text no. 2
    
3.
KLAUDER J. V.: Ectodermosis Ero­siva Pluriorificialis - Resemblance to the Human Form of Foot and Mouth Disease and its relation to Erythema Exudativum Multiforme. Arch. Derm. and Syph. 36, 1067 (1937).  Back to cited text no. 3
    
4.
RENDU R.: On a syndrome charac­terised by simultaneous inflammation of all the external mucous membranes, co-existing with a varicelliform erup­tion, later purulent, of the four mem­branes (in French) Rev. Gen. de Cli­nic ib de Thurap 30, 351, (1916).  Back to cited text no. 4
    
5.
PRICE Text book of the Practice of Medicine, p. 946. Oxford Univers. Press. London 946 (1966). .  Back to cited text no. 5
    
6.
ROBINSON, H. M, and McCRUMB, F. R.: Comparative analysis of muco­cutaneous - ocular syndromes. Arch. Derm. Syph. 61, 539, (1950).  Back to cited text no. 6
    
7.
STEPHENS A. M. AND JOHNSON F. C.: A new eruptive Fever associat­ed with Stomatitis and Ophthalmia.­Report of two Cases n children. Amer. .J Dis. Child. 24, 526, (1922).  Back to cited text no. 7
    




 

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