Study of genital manifestations of Stevens Johnson Syndrome/Toxic Epidermal Necrolysis

Abstract

Background: Stevens-Johnson syndrome/Toxic Epidermal Necrolysis (SJS/TEN) are severe mucocutaneous reactions, considered as variants of same pathologic process. It is characterized by epidermal blisters, necrosis and sloughing predominantly of drug induced etiology. Due to severe cutaneous reactions, the genital manifestations associated with SJS/TEN are less studied. The chronic sequelae of genital manifestations results in severe gynecological, urological and sexual related complications, and have a great impact on the quality of life of patients.

Aims and objectives: To study the genital manifestations associated with SJS/TEN.

Materials and methods: Patients of SJS/TEN attending the Out-patient and In-patient Department of Skin and VD in a tertiary care hospital, having genital manifestations were included in the study. Genital examination of all patients were observed and recorded with a follow up for 6 months.

Results: A total of 30 patients, twelve males (40%) and eighteen females (60%), were included. Genital manifestations were observed in 18 (60%) patients, with a male:female ratio of 1:2, i.e. males 6 (36.66%) and females 12 (63.33). All patients presented in the acute stage of the disease. Four female patients (33.3%) developed chronic manifestations in the form of labial synechiae in 2 (16.66%), vaginal synechiae in 1 (8.33%), vaginal strictures in 1 (8.33%) patient. None of the male patients developed chronic sequelae.

Conclusion: SJS/TEN are rare diseases, but the mucocutaneous involvement, especially of genitourinary system and their long-term sequelae have a major impact on the quality of life of affected patients. The genital manifestations are largely preventable by means of proper awareness and early intervention.

Keywords: Genital manifestations; Stevens–Johnson syndrome; scrotal bullae; scrotal erosions; toxic epidermal necrolysis; urethral erosions; vaginal discharge; vaginal erosions; vaginal synechiae.

Figure 1

This image shows (A) Labial erosions with sloughing of mucosa (B) Vaginal discharge in a patient with Stevens–Johnson syndrome

Figure 2

Acute manifestations in a female patient showing erosions and peeling of the skin over labia majora and minora; Foley's catheter in situ

Figure 3

Vaginal discharge in a female patient of Stevens–Johnson syndrome in the subacute stage

Figure 4

Development of chronic sequelae in a female patient, showing (A) Labial adhesions (B) Postinflammatory hypopigmentation over labia and thighs

Figure 5

Chronic sequelae of genital involvement in a female patient of toxic epidermal necrolysis, showing (A) Labial agglutination (B) Healing erosions in the perineal region

Figure 6

Chronic sequelae of genital involvement in a female Stevens–Johnson syndrome patient showing labial adhesions and healing of previously eroded areas with hypopigmentation

Figure 7

This image shows (A) Erosions with crusting over glans of the penis (B) Scrotal erosions in a patient with Stevens–Johnson syndrome

Figure 8

Acute findings in a male patient, with multiple erosions and peeling areas involving glans penis and scrotum