We present a clinical case of a 56-year-old woman who developed bullous lesions on the dorsum of her hands in August 2022, prompting a biopsy revealing features consistent with bullous dermatitis such as porphyria cutanea tarda. Subsequently, she was referred to our unit for further evaluation.

Her medical history included hyperthyroidism, post-anesthesia recovery difficulties following two surgeries, recurrent episodes of diffuse abdominal pain radiating to the lumbar and inguinal regions accompanied by tachycardia, worsening asthenia, reduced diuresis, constipation, and insomnia. She also experienced episodes of lower extremity paresthesias and clonus.

Hospitalization in January 2023 included instrumental examinations to assess potential organic abnormalities related to reported symptoms, alongside genetic testing revealing a pathogenic variant c.917dup in the CPOX gene, confirming hereditary coproporphyria. Imaging studies identified two small aneurysms: one dysmorphic at the bifurcation of the right middle cerebral artery, and the other sac-like at the bifurcation of the left middle cerebral artery. Despite no apparent association with coproporphyria, these findings underscored the importance of preventing acute porphyria crises to mitigate aneurysmal rupture risk. Neurosurgical consultation recommended regular MRI monitoring.Initiation of Givosiran therapy was planned but delayed due to hospitalization in April 2023 for acute hepatitis secondary to initial HCV infection. After completing eradication therapy, she commenced Givosiran therapy in July 2023, resulting in prompt subjective symptom improvement and normalization of ALA and PBG levels. She remained asymptomatic, reporting enhanced quality of life post-treatment. This case highlights the co-occurrence of hereditary coproporphyria with multiple cerebral aneurysms, emphasizing the necessity for comprehensive diagnostic evaluations to exclude other underlying pathologies. Notably, it underscores the significant symptomatic improvement and enhanced quality of life achieved withGivosiran therapy, reflecting its efficacy in managing porphyria-associated symptoms.