In October 2018, a 27-year-old woman presented with a 2-week history of a pruritic rash on her face and ears. Her past medical history was significant for SLE diagnosed 5 years ago with cutaneous, joint, and renal tropism. She was treated with different treatments; first, she received 6 Cyclophosphamide boluses with relay by Azathioprine for 3 years, stopped for hematological toxicity, then switched to Mycophenolate Mofetil which was stopped by the patient herself for 1 year due to lack of means. She also had a notion of septic arthritis of the knee having recurred despite well-adapted antibiotic treatment and her aunt was being treated for pulmonary tuberculosis. Therefore, she was hospitalized this time to carry out the SLE systematization assessment and to update her treatment. In the initial physical examination, we found a malar rash; erythematous plaques on her face, ears, and upper limbs; and a diffuse non-scarring alopecia. She also had arthralgia especially in her left knee, with swelling in that area.
A small left knee effusion was aspirated and the culture was positive for Mycobacterium tuberculosis; therefore, anti-tuberculous therapy (Rifampicin, Isoniazid, Pyrazinamide, and Ethambutol) was initiated. The cutaneous symptomatology worsened after 15 days from the start of tuberculosis treatment with extended rash at the trunk and neckline, erythematous and edematous plaques, and blisters in the photo-exposed areas. Nikolsky's sign was positive, the affected skin surface was estimated to be 30%, and the mucosal involvement was limited to a simple cheilitis (Figure 1, Figure 2). The antituberculosis treatment was initially discontinued because we had thought of the TEN.
Laboratory abnormalities showed proteinuria elevated to 2g/24h, bicytopenia (leucopenia at 3500 per cubic milliliter and anemia with hemoglobin at 10 grams per dl), an elevated anti-double stranded deoxyribonucleic acid (DNA) antibody with negative anti-histone antibodies and consumption of the complement C3 and C4. Moreover, skin biopsy showed diffuse epidermal necrosis, sub-epidermal detachment, interface dermatitis and presence of mucinosis. On direct immunofluorescence (DIF), a deposition of IgM and IgG in the basement membrane (lupus band) was present. Given the clinical history, lesion morphology, pathologic finding and laboratory studies, the diagnosis of TEN-like ACLE was established. After the administration of systemic corticosteroids, we have observed a healing of the skin lesions after two weeks. Anti-tuberculous treatment was reintroduced without any particular incident and Cyclophosphamide boluses were subsequently started. Joint swelling resolved within two months after the initiation of anti-tuberculous treatment and full joint range of motion was restored. There has been no recurrence of the bullous eruption to date.