Toxic epidermal necrolysis in a patient with lupus erythematosus

Stevens-Johnson syndrome and toxic epidermal necrolysis (TEN) are severe life-threatening conditions induced by pharmaceuticals. More than 100 pharmaceutical agents potentially implicated in the development of TEN have been reported. The medications most commonly causing TEN include allopurinol, carbamazepine, sulfonamides, antibiotics (aminopenicillins, cephalosporins, tetracyclines), antimycotics, antiepileptics, and non-steroidal anti-inflammatory drugs [1–3]. There have also been reported cases of the so-called TEN-like lupus erythematosus with a subacute course, extending over weeks to months, without a known drug trigger. TEN-like lupus erythematosus has been described in patients with systemic lupus erythematosus. Skin manifestations in patients with TEN-like lupus erythematosus occur initially in sites of previous skin eruptions typical of lupus and then gradually spread over the entire skin surface [4, 5]. The differentiation between toxic epidermal necrolysis and TEN-like lupus erythematosus remains a major diagnostic challenge.
In this paper, we present a case of subacute toxic epidermal necrolysis in a patient with lupus erythematosus. A 66-year-old woman with systemic lupus erythematosus, Sjögren syndrome and IgG k monoclonal gammopathy was admitted to the Department of Dermatology for the diagnosis and treatment of skin lesions persisting for approximately 6 months, in the absence of improvement after outpatient treatment (fig. 1). As reported by the patient, early skin lesions presenting as small, round focal erythematous and edematous lesions with a targetoid morphology were limited to the skin of the hands. However, they spread to the upper limbs and the trunk over the course of a few weeks. Two months prior to hospital admission, because of an exacerbation of skin lesions, the patient was consulted on an outpatient basis by a rheumatologist who prescribed treatment with hydroxychloroquine at a dose of 200 mg/day and sulfasalazine at a dose of 1000 mg/day. Despite the treatment, the patient’s skin lesions worsened, and multiple flaccid bullae and erosions of the vermilion border were observed. On admission to the Department of Dermatology, the patient was in general good condition. Physical examination revealed numerous confluent focal erythematous and edematous lesions. Within them, multiple flaccid blisters filled with serous fluid and erosions covered partially with yellow scabs were identified. Nikolsky’s sign was positive....


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