title: Dialysis-associated pseudoporphyria successfully treated with vitamin D. Report of two cases.
abstract: Pseudoporphyria refers to a rare bullous dermatosis characterized by the clinical and histological features of porfiria cutanea tarda without abnormalities in porphyrin metabolism. The pathogenesis is heterogeneous and several exogenous factors may promote the bullous lesion formation, including medications, end stage renal disease, dialysis and tanning beds. Regarding treatment of this condition, in literature different therapy have been reported, such as glutathione and his precursor N-acetylcysteine, which presents anti-oxidant properties; however even more toxic drugs, such as chloroquine, are used. Moreover, in patients with drug-induced PP discontinuation of the offending agent, if possible, is a crucial aspect of the clinical management. We report two cases of dialysis patients presenting blisters on extremities, which healed with the avoidance of UV exposure and oral Vitamin D supplementation. Interestingly Vitamin D despite the lack of antioxidant properties led to a completely resolution of PP in both our patients within 30 days. A possible explanation of this finding is that Vitamin D, playing a key role in the regulation of serum Ca2+, can modulated cadherin-cadherin interactions and led to healing of pseudoporphyria bullous lesions. Finally we highlight the prominent role of UV-exposure in PP elicitation thus a good photoprotection is essential for all patients with pseudoporphyria.
authors.affiliation: Unit of Dermatology, NESMOS Department, Faculty of Medicine, Sant'Andrea" Hospital "Sapienza" University, Rome, Italy - cpranteda@hotmail.com.
authors.forename: G
authors.initials: G
authors.lastname: Pranteda
authors.forename: U
authors.initials: U
authors.lastname: Bottoni
authors.forename: M
authors.initials: M
authors.lastname: Tayefeh Jafari
authors.forename: G
authors.initials: G
authors.lastname: Pranteda
authors.forename: S
authors.initials: S
authors.lastname: De Micco
authors.forename: M
authors.initials: M
authors.lastname: Muscianese
authors.forename: P
authors.initials: P
authors.lastname: Menè
mesh_terms: D000368:Aged; D002118:Calcium / Q000502:physiology; D003357:Cosmetic Techniques / Q000009:adverse effects; D051181:Desmosomal Cadherins / Q000502:physiology; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D007365:Intercellular Junctions; D007676:Kidney Failure, Chronic / Q000150:complications / Q000628:therapy; D008875:Middle Aged; D009400:Nephrosclerosis / Q000150:complications; D010530:Peritoneal Dialysis / Q000009:adverse effects; D010787:Photosensitivity Disorders / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D017119:Porphyria Cutanea Tarda / Q000175:diagnosis; D011166:Porphyrins / Q000032:analysis; D006435:Renal Dialysis / Q000009:adverse effects*; D012872:Skin Diseases, Vesiculobullous / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D013472:Sunlight / Q000009:adverse effects*; D014466:Ultraviolet Rays / Q000009:adverse effects*; D014807:Vitamin D / Q000502:physiology / Q000627:therapeutic use*; D014808:Vitamin D Deficiency / Q000150:complications* / Q000188:drug therapy
chemical_list: D051181:Desmosomal Cadherins; D011166:Porphyrins; D014807:Vitamin D; D002118:Calcium