Birt-Hogg-Dube syndrome coexisting with bullous pemphigoid and basal cell carcinoma

Introduction: Genodermatoses associated with malignant tumors constitute a diagnostically important group of dermatological diseases. One of them is the Birt-Hogg-Dube syndrome, characterized by numerous fibrofolliculomas, trichodiscomas, (benign nodules originating from hair follicles), lung cysts and an increased risk of kidney cancer. Case report: A 81-year-old man was admitted to our clinic with erythematous-edematous, bullous and erosive skin lesions located on his trunk and limbs. During hospitalization, bullous pemphigoid and two types of basal cell carcinoma were diagnosed. Additionally, attention was paid to the presence of numerous flesh-colored papules on the surface of the face, neck and ears, corresponding to fibrofolliculoma in the histopathological examination. An in-depth medical history and detailed imaging diagnostics allowed the patient to be diagnosed with the Birt-Hogg-Dube syndrome. Conclusions: Characteristic skin lesions and coexisting extra-cutaneous symptoms in Birt-Hogg-Dube syndrome allow early identification of patients at increased risk of kidney cancer. We describe the first case of Birt-Hogg-Dube syndrome associated with bullous pemphigoid and basal cell carcinoma.