Successful Treatment of Epidermolysis Bullosa Pruriginosa With Topical Tacrolimus

Dystrophic epidermolysis bullosa pruriginosa of elderly onset

“…The aims of treatment for DEB‐Pr are to ease the pruritus and to suppress the scratching activity that leads to the formation of blisters and/or prurigo‐like lesions; however, no universally successful treatment has been established. Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide 13–15 . McGrath et al.…”

“…Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide. [13][14][15] McGrath et al 1 reported that treatment with a systemic corticosteroid 10-30 mg ⁄ day up to 2 months did not appear to be effective, whereas in our case, oral PSL 30 mg ⁄ day was effective and improved the patient's symptoms. We therefore advocate use of topical tacrolimus and a higher dose PSL as potentially useful treatment options for DEB-Pr.…”

Dystrophic epidermolysis bullosa pruriginosa of elderly onset

“…The aims of treatment for DEB‐Pr are to ease the pruritus and to suppress the scratching activity that leads to the formation of blisters and/or prurigo‐like lesions; however, no universally successful treatment has been established. Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide 13–15 . McGrath et al.…”

“…Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide. [13][14][15] McGrath et al 1 reported that treatment with a systemic corticosteroid 10-30 mg ⁄ day up to 2 months did not appear to be effective, whereas in our case, oral PSL 30 mg ⁄ day was effective and improved the patient's symptoms. We therefore advocate use of topical tacrolimus and a higher dose PSL as potentially useful treatment options for DEB-Pr.…”

Dystrophic epidermolysis bullosa pruriginosa in Italy: clinical and molecular characterization

“…An amelioration of skin lesions and pruritus was observed in patient 5 during a-methylprednisolone treatment for asthma. The described clinical improvement of DEB-Pr patients with other immunomodulatory drugs, such as cyclosporine, thalidomide and tacrolimus, suggests the involvement of additional immunemediated factors in the pathogenesis of DEB-Pr (30)(31)(32). However, in our DEB-Pr patients, direct and indirect IF were negative for the presence of tissue-bound or circulating autoantibodies in the typical linear continuous pattern observed in autoimmune subepidermal blistering disorders.…”

Coinheritance of 2 New Potentially Damaging Heterozygous COL7A1 Variants in a Family With Autosomal Dominant Epidermolysis Bullosa Pruriginosa

“…The aims of treatment are to reduce pruritus, prevent scarring, and improve the patient’s overall quality of life. 8 Frequently recommended treatments include superpotent topical steroid ointments and topical tacrolimus ointment, 10 but these were unsuccessful in our patient. She experienced temporary relief from treatment with narrowband UVB phototherapy.…”

Dystrophic epidermolysis bullosa pruriginosa of elderly onset

“…The aims of treatment for DEB‐Pr are to ease the pruritus and to suppress the scratching activity that leads to the formation of blisters and/or prurigo‐like lesions; however, no universally successful treatment has been established. Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide 13–15 . McGrath et al.…”

“…Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide. [13][14][15] McGrath et al 1 reported that treatment with a systemic corticosteroid 10-30 mg ⁄ day up to 2 months did not appear to be effective, whereas in our case, oral PSL 30 mg ⁄ day was effective and improved the patient's symptoms. We therefore advocate use of topical tacrolimus and a higher dose PSL as potentially useful treatment options for DEB-Pr.…”

Dystrophic epidermolysis bullosa pruriginosa in Italy: clinical and molecular characterization

“…An amelioration of skin lesions and pruritus was observed in patient 5 during a-methylprednisolone treatment for asthma. The described clinical improvement of DEB-Pr patients with other immunomodulatory drugs, such as cyclosporine, thalidomide and tacrolimus, suggests the involvement of additional immunemediated factors in the pathogenesis of DEB-Pr (30)(31)(32). However, in our DEB-Pr patients, direct and indirect IF were negative for the presence of tissue-bound or circulating autoantibodies in the typical linear continuous pattern observed in autoimmune subepidermal blistering disorders.…”

Coinheritance of 2 New Potentially Damaging Heterozygous COL7A1 Variants in a Family With Autosomal Dominant Epidermolysis Bullosa Pruriginosa

“…The aims of treatment are to reduce pruritus, prevent scarring, and improve the patient’s overall quality of life. 8 Frequently recommended treatments include superpotent topical steroid ointments and topical tacrolimus ointment, 10 but these were unsuccessful in our patient. She experienced temporary relief from treatment with narrowband UVB phototherapy.…”

Dystrophic epidermolysis bullosa pruriginosa of elderly onset

“…The aims of treatment for DEB‐Pr are to ease the pruritus and to suppress the scratching activity that leads to the formation of blisters and/or prurigo‐like lesions; however, no universally successful treatment has been established. Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide 13–15 . McGrath et al.…”

“…Recent studies have described the efficacy of topical tacrolimus (as we also observed in our patient), systemic cyclosporine and thalidomide. [13][14][15] McGrath et al 1 reported that treatment with a systemic corticosteroid 10-30 mg ⁄ day up to 2 months did not appear to be effective, whereas in our case, oral PSL 30 mg ⁄ day was effective and improved the patient's symptoms. We therefore advocate use of topical tacrolimus and a higher dose PSL as potentially useful treatment options for DEB-Pr.…”

Dystrophic epidermolysis bullosa pruriginosa in Italy: clinical and molecular characterization

“…An amelioration of skin lesions and pruritus was observed in patient 5 during a-methylprednisolone treatment for asthma. The described clinical improvement of DEB-Pr patients with other immunomodulatory drugs, such as cyclosporine, thalidomide and tacrolimus, suggests the involvement of additional immunemediated factors in the pathogenesis of DEB-Pr (30)(31)(32). However, in our DEB-Pr patients, direct and indirect IF were negative for the presence of tissue-bound or circulating autoantibodies in the typical linear continuous pattern observed in autoimmune subepidermal blistering disorders.…”

Coinheritance of 2 New Potentially Damaging Heterozygous COL7A1 Variants in a Family With Autosomal Dominant Epidermolysis Bullosa Pruriginosa

“…The aims of treatment are to reduce pruritus, prevent scarring, and improve the patient’s overall quality of life. 8 Frequently recommended treatments include superpotent topical steroid ointments and topical tacrolimus ointment, 10 but these were unsuccessful in our patient. She experienced temporary relief from treatment with narrowband UVB phototherapy.…”