Combination of vulgar pemphigus and toxic epidermal necrolysis: a case report

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A combination of vulgar pemphigus and toxic epidermal necrolysis is an extremely rare and clinically complex comorbidity characterized by divergent immune mechanisms of skin damage. We present a case report of a 33-year old patient with rapidly progressive bullous and erosive skin lesions. Immunofluorescence studies showed IgG and the C3 complement component deposits in the intercellular epidermal contacts; the diagnosis of vulgar pemphigus was confirmed. The Pemphigus Disease Area Index (PDAI) at the moment of maximal clinical symptoms was 78. Just before the hospitalization, the patient had been treated with antibiotics. Pathomorphological examination showed virtually total intra-epidermal detachment of the epidermis, in addition to IgG and C3 complement component deposits found by direct immunofluorescence. These findings, combined with clinical manifestations characteristic of toxic epidermal necrolysis, allowed to suspect two diseases. For 9 weeks, the patient was treated with high dose systemic glucocorticosteroids and plasmapheresis, which led to stabilization of the disease. At week 9 from the day of hospitalization, there was a dramatic deterioration in the patient’s condition. At week 10 of the hospitalization, rituximab was added to high dose systemic glucocorticosteroids resulting in clinical improvement at week 13. By week 24 after the treatment initiation, a significant clinical result was achieved, seen as complete epithelialization of the erosions and stable remission of the skin disease. The treatment strategy chosen has led to simultaneous exhaustion of B cell pool responsible for production of pathogenic auto-antibodies in pemphigus and to significant suppression of cell immunity, which plays a key role in toxic epidermal necrolysis. Toxic epidermal necrolysis should be excluded in patients with pemphigus, especially with atypical rapidly progressive course and medication-associated. Immunofluorescence methods play a decisive role in the diagnosis of these comorbid disorders. Combination therapy with rituximab and corticosteroids can be considered as a strategy targeting the pathogenic pathways of both diseases. This case report highlights the need for a thorough diagnostic analysis and the development of clear management algorithms for such complex cases.

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