Successful treatment of pemphigus vegetans by addition of etretinate to systemic steroids

Abstract

Pemphigus vegetans is a rare variant of pemphigus vulgaris and is characterized by flaccid bullae, which become eroded and form vegetations or papillomatous proliferations, especially in the intertriginous areas. Oral administration of corticosteroids alone does not always induce disease remission in pemphigus vegetans. We report a 44-year-old Japanese man with pemphigus vegetans. Although corticosteroid therapy resulted in healing of the oral ulcers and skin bullae, verrucous vegetations continued to develop. In contrast, by combining corticosteroid with etretinate, verrucous vegetations improved. Thus we propose that the combination therapy of steroid and etretinate might be an effective adjunct in the therapy of pemphigus vegetans.

Azathioprine in the Treatment of Autoimmune Blistering Diseases

“…22 For treatment of refractory patients, disease control has been reported under the corticosteroid/azathioprine regimen using additionally photopheresis or retinoids. 23,24 In paraneoplastic pemphigus, therapy for the underlying malignancy is essential. Concomitant treatment with azathioprine and other immunomodulatory drugs has been reported, but it is difficult to conclude whether any of them will reliably affect the prognosis of the disease because its mortality approaches 90%.…”

Pyoderma Vegetans

“…1,2,10 North American blastomycosis and pemphigus vegetans, an autoimmune disorder that is a rare variant of pemphigus vulgaris, closely resemble pyoderma vegetans clinically. 2,[12][13][14] Other deep fungal infections, such as coccidioidomycosis and phycomycosis, may rarely appear similar to pyoderma vegetans. 2 Botryomycosis is a rare bacterial infection that may mimic fungal disease and may also resemble pyoderma vegetans.…”

“…Laboratory examination revealed red blood cell count 3.51 M/ᮀ L [4.20-5.40], hemoglobin 10.0 g/dL [12][13][14][15][16] Abdominal ultrasound examination showed hepatomegaly with probable fatty infiltration and no evidence of hydronephrosis. A chest roentgenogram revealed no abnormalities.…”

Pemphigus Vegetans Associated With Verrucous Lesions - Expanding a Phenotype

“…However, oral administration of corticosteroids alone does not always induce disease remission. 12 In the literature reviewed, the association of systemic steroid therapy with azathioprine, dapsone, cyclophosphamide, and cyclosporine as effective treatment has been described. When it comes to persistent, verrucous vegetations, the combination of corticosteroid and etretinate resulted in healing of the lesions.…”

“…When it comes to persistent, verrucous vegetations, the combination of corticosteroid and etretinate resulted in healing of the lesions. 12 Moreover, the association of methotrexate, dicloxacillin, oral steroids, and periodic intralesional corticosteroid injections were effective in a relatively resistant scalp disease. 13 The vermillion and nail involvement, as well as the verrucous, hyperkeratotic acral plaques seen in this patient were also described in other cases of pemphigus vulgaris\vegetans, [6][7][8] and these should be considered, together with the classical vegetant lesions in the skin folds, characteristic of the verrucous variant of this condition.…”