Successful Treatment of Epidermolysis Bullosa Pruriginosa With Topical Tacrolimus

Epidermolysis bullosa pruriginosa: A rare presentation with asymptomatic lesions

“…[2] The face and flexures are always spared, and nail dystrophy, albopapuloid lesions, blisters and milia are other common but variable features. [4] Age of onset of skin lesions in EBP is very variable; development of the first clinical sign in adulthood is not uncommon although the reason for the delayed presentation is not known. Lesions may develop at birth.…”

“…Clinical management of EBP is often difficult and treatment is usually unsatisfactory and includes general measures for pruritus like anti-histaminics and topical steroid with or without occlusion. [8] Trials of a few interventions which have been found to have a variable response in EBP are topical tacrolimus, [4] systemic ciclosporin, [9] Dapsone, [10] thalidomide, [7] and cryotherapy. [5] …”

Clinical and molecular dilemmas in the diagnosis of familial epidermolysis bullosa pruriginosa

“…Nevertheless, some reports of helpful interventions in EBP have been published. These include topical treatments (eg, tacrolimus), 14 systemic agents (eg, ciclosporin 17 or thalidomide 13 ), and cryotherapy. 12 In summary, we have investigated a family with EBP in whom we have identified a COL7A1 mutation in affected individuals as well as two clinically unaffected family members who may be at risk from developing EBP in the future.…”

“…Since the initial publication, a number of other cases of EBP have been reported, collectively highlighting the distinctive nature and clinical spectrum of this form of DEB. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] Inheritance of EBP may be dominant or recessive, but the cause of the pruritus is uncertain-it is not caused by a specific type of COL7A1 mutation. Other factors, such as atopy or iron deficiency, have been implicated, but no universal abnormality has been found to link all cases of EBP.…”

Treatment of pruritic diseases with topical calcineurin inhibitors

“…For example, a patient with epidermolysis bullosa pruriginosa was treated with tacrolimus 0.03% for otherwise intractable itching on the legs and experienced a relief within one week (Banky et al 2004). Patients with chronic irritative hand dermatitis experienced significant reduction of itch during topical treatment with pimecrolimus 1% (Cherill et al 2000).…”