Steven Jonhson Syndrome

Oral Manifestations in Stevens Johnson Syndrome

Authors: Bhavesh A. Mehta*, Alka Raka**, Vikas Sinha***
*Professor and Head Dermatology, **Resident Dermatology, ***Dean, Professor E.N.T.

Institution: Department of Dermatology and Department of Otolaryngology and Head and Neck Surgery
M.P.Shah Medical College, Jamnagar (Gujarat), India

Corresponding Author:

Dr. Bhavesh A. Mehta
Prof & Head Dermatology
M.P.Shah Medical College
Jamnagar -361 008 (Gujarat), India

E mail drbhavesh_skin@rediffmail.com

Abstract:

Stevens Johnson Syndrome is an acute life threatening mucocutaneous reaction characterized by extensive necrosis and detachment of epidermis with mucous membrane involvement. The aim of study is to find out commonest etiology, incidence of oral involvement, way of presentation and the survival rate of Stevens Johnson Syndrome. Total 20 cases were included in this study diagnosed in department of Dermatology in association with E.N.T. Department at M.P. Shah Medical College Jamnagar, India. The most common skin signs were erythematous dusky red macules with positive pseudo - Nikolsky Sign. The oral involvement was present in all the patients. Oral cavity had painful hemorrhagic ulcerative lesions over palate, tongue; lateral mucosa with crusting and white membrane over lips. It was associated with other mucous membrane involvement like ocular in 90% cases, genital in 60% cases, nasal mucosa involvement in 20% cases and anal mucosa involvement in 5% case. All patients were given symptomatic treatment. The 90% patients survived after successful treatment.

Introduction:

Stevens Johnson Syndrome is acute life threatening mucocutaneous reaction characterized by extensive necrosis and detachment of epidermis with mucous membrane involvement.¹ Stevens Johnson Syndrome can be differentiated from toxic epidermal necrolysis by the extent of area involvement. When 10% or less body area is involved it comes under Stevens Johnson Syndrome.² Overall incidence of Stevens Johnson Syndrome is 1 to 6 per million per year.³ Stevens and Johnson first reported two cases of disseminated cutaneous eruption associated with erosive stomatitis and severe ocular involvement.⁴ The incubation period is typically a few days to 3 weeks. Most cases of Stevens Johnson Syndrome are drug induced but the reaction is independent of the dose of drug and it is idiosyncratic.² The other rare causes of Stevens Johnson Syndrome are food additives, contact with chemicals and graft versus host reaction. Stevens Johnson Syndrome is a severe illness of usually sudden onset, associated with marked constitutional symptoms of high fever malaise, myalgia, arthralgia and extensive erythema multiforme of the trunk with occasional skin blisters and erosions. There is significant involvement of mucous membrane. Ashby &, Lazar⁵ in their review of 81 cases found involvement of oral mucosa 100%, eyes 91%, male genitalia 57% and anal mucous membrane 5%, while bronchitis & pneumonitis occurred in 6% and 23% of cases, respectively. Many times diagnosis at initial stage pays very crucial role in management. It increases the favorable outcome and also decreases complication.

Aim and Objective: The Aim and objective of this study to find out incidence of Stevens Johnson Syndrome, its oral involvement, presentation, complication and progbnosis.

Methods:

It is the case study of 20 cases of Stevens Johnson Syndrome carried out in department of Dermatology with help of department of ENT at M. P. Shah Medical College Jamnagar, India. The cases included in the study were 20 consequent cases of Stevens Johnson Syndrome from June 2010 to June 2011. Each patient was analyzed in detail with respect to history, its presentation and clinical examination. A special emphasis was given for history of any drug intake.

Observation:

The maximum number of cases was in between age group 20 – 30 years. The youngest patient in this study was 12 years female and the oldest was 80 year old male patient. The male female ratio was 1:2.3. Most of patients gave history of preceding drug intake within 6-8 weeks duration with non specific symptoms such as fever, headache, rhinitis, and myalgia. The 50% patients gave history of anticonvulsant like phenytoin, 30% patients gave history of NSAIDS like Paracetamol, Salicylates, 10% gave history of antibiotics like Cotrimoxazole and 10% patients gave no history of any drug intake.

Mucosal involvement preceded skin involvement in 60% patients (n=12). They complained of pain on swallowing and burning in oral cavity, stinging and redness in eye. Remaining 40% patients (n=12) give history of oral lesion accompanying skin lesions. Most of patients presented with mucosal symptoms which began with erythema followed by painful erosion of buccal cavity with lip swelling, painful hemorrhagic erosions coated by grayish white membrane and crusting over lips (Figures 1A, 2A, and 3A). There was also involvement of other mucous membranes like eye which presented with conjunctivitis, redness of eye, photophobia, and discharge with burning in eye.


Figure 1A: Before treatment, hemorrhagic erosion and crusting over lips.	Figure 2A: Before treatment. ulcerative lesions over tongue.	Figure 3A: Before treatment, hemorrhagic erosion and crusting over lips and ulcerative lesions over tongue.

Skin lesions were characterized by erythematous, dusky red purpuric macules. (Figures 1A, 2A,3 A). On examination lesions were bilaterally symmetrical with PseudoNikolsky Sign. The dislodgement of epidermis by lateral pressure was present on erythematous zones. The sign is elicited by applying lateral pressure with thumb or finger pad on skin over a bony prominence. This result in shearing force that dislodge epidermis hence this sign is also known as epidermal peeling sign. The underlying mechanism is the necrosis of epidermal cells and not acantholysis as in true Nikolskiy sign. The pseudo-Nikolskiy sign/epidermal peeling sign is positive in Stevens Johnson Syndrome, toxic epidermal necrolysis and in some cases of burns. It can be elicited only on involved or erythematous areas.

The oral cavity and tongue involvement was present in all of our patients which were associated with other mucous membrane involvement like eye in 90% cases, nasal mucosa involvement in 20% cases and anal mucosa involvement in 5% case. Oral cavity lesions ranged from lip edema, hemorrhagic crusting and erosions. Oral cavity showed painful hemorrhagic ulcerative lesions over palate, tongue (Figures 1A, 2A, 3A), lateral mucosa with perilesional pale halo.

Treatment:

The Treatment were given systemically by fluid replacement, aggressive nutritional support and short course of steroids. The intra venous fluid in the form of normal saline, dextrose normal saline, Ringer lactate with intra venous multivitamin supplement. Prednisolone in the dose of 2 mg/kg body weight which was tapered by 10 mg/weekly dose was given. The oral antibiotic (Azithromycin) was given to prevent secondary infection. Topically any antibiotic cream was applied for the skin lesion. During erosion of the skin the topical treatment consisted soframycin cream (framycetin 1%) or Fusidic acid (2%). If erythema was more, the mild steroid like betamethasone valerate 0.05% or momentasone furoate (0.1%) was added.
Oral cavity lesions were treated with systemic and topical antifungal and mild steroidal preparation.


Figure 1B: After treatment for hemorrhagic erosion and crusting over lips.	Figure 2B: After treatment for ulcerative lesions over tongue.	Figure 3B: After treatment for hemorrhagic erosion and crusting over lips and ulcerative lesions over tongue.

Discussion:

In our study of 20 patients of Stevens Johnson syndrome maximum number of patients in between age group 20-30 year age group.

In present study oral involvement was seen in 100% of patients, eye involvement in 90% of patients, 60% of patients had genital involvement, 20% of patients showed nasal mucosal involvement, and 5% of patients showed anal involvement which in accordance with other publish reports.⁵

Commonest cause of Stevens-Johnson syndrome is drug induced which similar to other study.⁶ Our study of 20 cases show most common drug causing Stevens Johnson Syndrome is anticonvulsant like phenytoin in 50% of patients. The 25% patients gives history of Nonsteroidal anti-inflammatory drug. 15% of patients gives history of antibiotic likecotrimazole. 10% of patients not giving any history of drug intake. So our study shows that the most common cause of Stevens Johnson Syndrome is anticonvulsant which in contrast to other study which show commonest cause is Nonsteroidal anti-inflammatory.⁷ Overall mortality is 10% in present study which matches with other study.⁸

Conclusion:

Stevens-Johnson syndrome is acute life threatening mucocutaneous reaction with most common etiology is drug .It is further concluded that, oral involvement present in majority of cases, which is significant cause of morbidity in patients. Early diagnosis and treatment helps in reducing morbidity and mortality and gives favorable outcome ( Figures 1B, 2B, 3B).

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