Differential Diagnosis for Chronic Cutaneous Ulcers, Hepatosplenomegaly, Femoral Adenomegaly, and Necrotizing Granulomas with Vasculitis

Single Most Likely Diagnosis

• Wegener's Granulomatosis (Granulomatosis with Polyangiitis, GPA): This condition is characterized by the presence of necrotizing granulomas and vasculitis, which matches the biopsy findings. It often presents with systemic symptoms including skin ulcers, hepatosplenomegaly, and lymphadenopathy, making it a strong candidate for the single most likely diagnosis.

Other Likely Diagnoses

• Polyarteritis Nodosa (PAN): PAN is a systemic vasculitis that can cause cutaneous ulcers, hepatosplenomegaly, and lymphadenopathy. While it typically does not cause granulomas, the presence of vasculitis and other systemic symptoms makes it a plausible diagnosis.
• Churg-Strauss Syndrome (Eosinophilic Granulomatosis with Polyangiitis, EGPA): This condition is characterized by asthma, eosinophilia, and vasculitis, which can include necrotizing granulomas. It can also present with skin ulcers and systemic involvement, making it another likely diagnosis.
• Systemic Lupus Erythematosus (SLE): SLE is a systemic autoimmune disease that can cause a wide range of symptoms, including cutaneous ulcers, hepatosplenomegaly, lymphadenopathy, and vasculitis. While granulomas are less common, the broad spectrum of SLE manifestations makes it a consideration.

Do Not Miss Diagnoses

• Infectious Diseases (e.g., Tuberculosis, Fungal Infections): Certain infections can cause granulomatous inflammation and vasculitis. Missing an infectious cause could lead to inappropriate treatment and severe consequences, making it crucial not to overlook these possibilities.
• Malignancy (e.g., Lymphoma): Both Hodgkin and non-Hodgkin lymphoma can present with systemic symptoms including hepatosplenomegaly, lymphadenopathy, and cutaneous manifestations. The presence of necrotizing granulomas and vasculitis could be indicative of a lymphoproliferative disorder.

Rare Diagnoses

• Erdheim-Chester Disease: A rare form of histiocytosis that can cause systemic symptoms including skin lesions, lymphadenopathy, and hepatosplenomegaly. While less likely, its rarity and potential for severe outcomes warrant consideration in a differential diagnosis.
• Kawasaki Disease in Adults: Although primarily a disease of children, Kawasaki disease can rarely occur in adults and presents with vasculitis, lymphadenopathy, and sometimes hepatosplenomegaly. Its inclusion in the differential is based on the need to consider all potential causes of vasculitis and granulomatous inflammation.