Maxillofacial Prosthetic Rehabilitation of Extensive Facial Defects in a Patient with Congenital Erythropoietic Porphyria Using a Multicomponent Silicone Prosthesis: A Case Report

Congenital Erythropoietic Porphyria (CEP), also known as Günther disease, is an exceptionally rare autosomal recessive disorder characterized by severe photosensitivity, recurrent blistering, ulceration, scarring, and progressive destruction of sun-exposed tissues. In advanced cases, extensive loss of facial structures can result in severe functional, esthetic, and psychosocial impairment. Maxillofacial prosthetic rehabilitation provides a conservative alternative when surgical reconstruction is limited by extensive tissue loss, compromised local tissues, or unpredictable healing. This case report describes the prosthetic rehabilitation of a 45-year-old male patient diagnosed with Congenital Erythropoietic Porphyria, presenting with extensive facial disfigurement involving the nasal, orbital, auricular, and adjacent facial regions. Following multidisciplinary medical evaluation and clearance, a comprehensive silicone-based maxillofacial rehabilitation was planned. Facial impressions were obtained using a reinforced irreversible hydrocolloid technique, followed by a definitive functional impression using addition silicone during facial movements. The definitive prosthesis was processed using medical-grade room-temperature vulcanizing silicone elastomer with intrinsic coloration and characterization. Retention was achieved using a water-soluble adhesive and spectacles. This case demonstrates the potential of a customized multicomponent silicone maxillofacial prosthesis in the rehabilitation of extensive facial defects associated with Congenital Erythropoietic Porphyria