Coexistence of pemphigus herpetiformis and systemic lupus erythematosus

Abstract

A female patient with coexistence of pemphigus herpetiformis and systemic lupus erythematosus is described. She presented to our Department with pruritic vesicles on her trunk and extremities, which were later accompanied with butterfly like erythema on her face and with central nervous system (CNS) manifestations. The diagnosis of pemphigus herpetiformis was based on the clinical picture and immunofluorescence finding, because the histopathologic finding is not always typical for the diagnosis. The diagnosis of systemic lupus erythematosus was based on positive ANA and anti-dsDNA, presence of butterfly-like erythema on her face, and CNS manifestations. The patient was treated by corticosteroids in combination with immunosuppressants, which should ensure good control of both diseases. The coexistence of pemphigus herpetiformis and systemic lupus erythematosus has not been reported in recent literature.

Concurrence of Systemic Lupus Erythematosus and Pemphigus: Coincidence or Correlation?

“…Pemphigus preceded SLE in 4 cases: in 2 of these 4 cases, SLE presented while pemphigus was still active [14, 15]. There was an interval of about 1 year between the development of the 2 diseases.…”

“…Seven patients had PV, and 1 [21] had PE. One had oral involvement only [14], 4 had cutaneous involvement only [15,19,20,21], and 2 had both oral and cutaneous involvement [17, 18]. Details of the clinical profile were not provided in the remaining 1 case [16].…”

“…In addition, 1 patient received dapsone for PV [14] and 2 hydroxychloroquine for SLE [16, 19]. One patient received azathioprine as treatment for both SLE and PV [15], while 1 received azathioprine for SLE [21] and 1 azathioprine for PV [18]. …”

“…In 2 patients both diseases were controlled [17, 19]. In 3 patients neither disease was controlled [14, 15, 20]; in 2 of these 3 patients, an exacerbation of PV occurred with the onset of SLE [14, 15]. In the 3 remaining patients only the pemphigus was controlled [16, 18, 21].…”

Dual diagnosis of pemphigus vulgaris and connective tissue disease

“…Systemic involvement such as pulmonary hypertension or renal involvement may develop over time. 6,7 Seven previous reports exist of a definitive dual diagnosis of SLE and PV, [8][9][10][11][12][13][14] but none of PV and MCTD. These reports lack detailed information on responses to treatment, relationship between the two diseases, and long-term follow-up.…”