Pseudo porphyria or porphyria cutanea tarda?

• Autor: Modesto dos Santos, Vitorino
• Formato: artículo
• Fecha de Publicación: 2018
• Institución: Sociedad de Gastroenterología del Perú
• Repositorio: Revista de Gastroenterología del Perú
• Lenguaje: español
• OAI Identifier: oai:ojs.revistagastroperu.com:article/879
• Enlace del recurso: http://www.revistagastroperu.com/index.php/rgp/article/view/879
• Nivel de acceso: acceso abierto

Manuscripts about pseudoporphyria (PP) andporphyria cutanea tarda (PCT) are commented here,emphasizing some gastroenterological relationshipsand possible diagnostic challenges or misdiagnosis,in special for daily practice of primary health careworkers. Batrani et al. described PP associated withthe use of imatinib and manifested by bullae, erosions,scarring and milia on the dorsum of hands and feet,in absence of photosensitivity (1). Biopsy sampleshowed subepidermal blister, dermal festooning ofpapillae and thickened vessels; and the level of urinaryporphyrin was within the normal range (1). Imatinibhas been also associated with PCT; however, in thispatient the diagnosis was consistent with PP (1). Peláez-Castro et al. reported the first Peruvian case of PP ina hemodialytic woman with facial hyperpigmentation,bilateral bullae on the fingers, and some crusts onthe dorsum of hands (2). Biopsy study revealed subepidermal blisters, with dermal festooning and mildlymphocytic infiltrate; however, the normal profiles ofporphyrins allowed confirm the diagnosis of PP (2). Thiscondition is related to renal failure and hemodialysis,non-steroidal anti-inflammatory drugs, furosemide,retinoids, sulphonamides, tetracyclines, dapsone, andnalidixic acid (1,2). Hypertrichosis, hyperpigmentation,calcifications and sclerodermoid lesions areuncommon in PP; however, clinical, histopathologicaland immunofluorescence features can mimic PCT (1).Therefore, normal urinary and plasmatic porphyrins aremandatory for diagnosis of PP (1,2).