TEN Like Lupus: A Rare Initial Presentation of Lupus Erythematosus.

Sir,

Toxic epidermal necrolysis (TEN) is a severe life-threatening mucocutaneous adverse reaction, most commonly triggered by medications.[1] Lupus erythematosus (LE) is a multisystem, autoimmune disorder that commonly affects skin along with other organ systems.[2] However, cases of TEN in setting of LE are extremely uncommon; therefore, we report a case of TEN like Lupus, with TEN being the first disease manifestation.

19 years old healthy female presented with history of fever and rash over body along with oral ulceration of 03 days duration. There was history of erythema over the skin starting from the face, which gradually progressed to involve the trunk and extremities with development of blisters and erosions. There was no significant history of intake of medication in past prior to the onset of symptoms. On examination, she was found to have blistering with skin loss over 50% of body surface area (BSA) with Nikolsky sign positive, along with Erythema multiforme like target lesions and chill blain like lesions over palms and soles [Figures 1 and 2]. She also had extensive mucosal involvement with oral ulcers involving the buccal mucosa and lips, nasal mucosal erosions with hemorrhagic crusting, and conjunctival involvement. Cutaneous histopathology was consistent with TEN [Figure 3]. She was diagnosed as a case of TEN and started on cyclosporine (3 mg/kg) and prophylactic antibiotic therapy. The patient was monitored closely and managed as per IADVL guidelines for SJS/TEN in a burn center.[3]

Figure 1

Blistering with skin loss over 50% of body surface area (BSA) with Nikolsky sign positive

Figure 2

Erythema multiforme like target lesions along with chill blain like lesions over palms and soles

Figure 3

Necrosis of epidermis with separation of necrotic epithelium forming a sub-epidermal bulla and an extremely sparse lymphocytic infiltrate in papillary dermis

She was found to have moderate anemia (7.3 g/dL), leucopenia (2500/cm3), and decreased albumin level (2.0 g/dL) on evaluation. In the hospital, the patient developed bilateral conjunctival hemorrhage and bilateral corneal ulcers with gross diminution of vision (DVR-2/60, DVL-1/60). The hospital stay was further complicated by upper GI bleed along with hematuria which was managed by Inj tranexamic acid in consultation with a gastroenterologist. She was administered IVIg (3 gm/kg over 03 days), after which the skin lesions showed partial regression.

Due to persistent fever, prominent facial edema, chill blain-like lesions over extremities, and persistent leucopenia, possibility of TEN like LE was considered. On further evaluation, ANA was found to be positive 2+, coarse speckled pattern (titer 1:2560), DsDNA was also positive (titer 1:640) with low complement levels. She was diagnosed as a case of TEN like Lupus and managed with Inj methylprednisolone pulses in conjunction with the rheumatologist with good clinical improvement. She was discharged on oral prednisolone and hydroxychloroquine. She was later administered Inj Rituximab (RA protocol) after a month. Presently she is asymptomatic and is on regular follow-up [Figure 4].

Figure 4

Patient at discharge with complete epithelialization of skin

Cutaneous involvement in LE is the second most frequent presenting symptom, present in up to 75% cases and it can precede the onset of systemic symptoms in 25% patients.[4] TEN is classified under Acute cutaneous lupus erythematosus (ACLE) as per Gilliam and Sontheimer classification although it can be a feature of subacute cutaneous lupus erythematosus (SCLE) as well.[2] Lupus-associated TEN was first suggested by Mandelcorn and Shear in 2003 as a novel manifestation of LE, resulting from acute necrosis of the epidermis leading to widespread sloughing of skin and mucous membranes.[5]

Ting et al.[6] proposed the term 'Acute Syndrome of Apoptotic Pan-Epidermolysis (ASAP)' to refer to the clinical conditions characterized by acute cleavage of the epidermis due to basal cell apoptotic injury, which aimed to include drug-induced TEN along with TEN due to GVHD, psuedoporphyria, and ACLE. Although uncommon, cases of TEN have been reported secondary to viral infections (herpes infection, viral hepatitis, rubella), mycoplasma pneumoniae infections, after a bone marrow transplant, as acute graft versus host reaction and after vaccination in the pediatric population.[789]

Apart from TEN, other vesico-bullous lesions which can be seen in LE patients include Bullous SLE and associated autoimmune bullous disorders such as bullous pemphigoid, pemphigus vulgaris, dermatitis herpetiformis, epidermolysis bullosa acquisita, linear immunoglobulin A (IgA). LE patients are on multiple drugs which makes them prone to Fixed drug eruptions (FDE). TEN-like lesions have been mentioned secondary to methotrexate in LE patients.[10] These conditions were ruled out in our case based on history (onset and progression of lesions) along with examination and the histopathology findings.

High index of suspicion should be maintained as treating clinicians in cases of TEN when appropriate drug history is not available or in patients with unpredictable clinical course. As active skin damage (i.e. TEN like lupus) is an indicator of disease activity, a careful search for extracutaneous involvement should be mandatory to detect further complications.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.