Document Type
Conference Proceeding
Publication Date
5-3-2024
Abstract
Introduction: This case report highlights a 73-year-old male with biopsy-proven neutrophilic dermatosis with concern for Sweet Syndrome in the context of suspected hydralazine-induced systemic lupus erythematosus and autoimmune markers that initially pointed towards hydralazine-induced ANCA vasculitis. Case Presentation: The patient is a 73-year-old male with a past medical history significant for chronic kidney disease, urothelial cancer in 2021, renal cancer in 2013, and stage 1A left lung cancer. The patient additionally underwent workup for low white count and splenomegaly in months leading up to hospitalization, with bone marrow biopsy and workup negative for myelodysplastic syndrome or malignancy. Patient initially presented with acute COVID pneumonitis and received treatment with steroids and IV remdesivir. Admission was complicated by acute onset pancytopenia. Presentation also significant for a diffuse purpuric rash on his extremities, which was present for eight months since starting hydralazine and worsened with recent COVID infection. Laboratory evaluation revealed an elevated ANA titer, anti-histone antibodies, positive ANCA, myeloperoxidase antibodies, and low C3/C4. This raised concern for drug-induced lupus erythematosus and hydralazine-induced ANCA vasculitis. Hydralazine was discontinued and the patient was placed on darbepoetin, with improvement in pancytopenia. The patient was subsequently discharged. The patient was readmitted several days later with worsening pancytopenia, acute kidney failure, respiratory distress, and hemorrhagic bullae in addition to worsening purpuric maculopapular rash over extremities, face, and chest/back. Labs revealed elevated PT/aPTT, urinalysis with hematuria and proteinuria, elevated CRP, and normal ESR. No infection was found during the workup. Dermatology was consulted and numerous skin biopsies, which showed neutrophilic dermatosis characterized by marked dermal interstitial and perivascular neutrophilic infiltrate with karyorrhexis, basophilic debris, and edema with neutrophilic inflammation extending beyond perivascular areas. New CT imaging showing continued splenomegaly with new onset ascites found to be transudative in nature. Management and Outcome: The patient was treated with pulse dose steroids and transitioned to oral steroids as well as Cellcept with improvement in pancytopenia and rash/skin lesions without the formation of new lesions. Continued investigation for etiologies, including malignancy workup, is ongoing. Discussion: Neutrophilic dermatosis is commonly associated with Sweet Syndrome, a rare condition that features cutaneous eruption of erythematous & violaceous tender papules and plaques with occasional hemorrhagic bullous formation. The patient presented with similar findings, however, this case is unique as the patient did not exhibit the classic findings of fevers or leukocytosis before rash onset. This condition is associated with malignancies, infections, drugs, and autoimmune diseases. Several cases have documented hydralazine as a cause for Sweet Syndrome. Noncirrhotic portal HTN is a component of underlying hydralazine-induced DILE. Conclusion: The case demonstrates a diagnostic challenge and raises questions regarding the association of DILE and neutrophilic dermatosis in this patient. We aim to contribute to the body of knowledge surrounding neutrophilic dermatosis and its potential associations. Through this case report, we seek to enhance clinicians' awareness of the diagnostic challenge posed by neutrophilic dermatosis within the context of drug-induced lupus, providing insights into its clinical course and implications for patient care.
Recommended Citation
Olen N, Horsley J, Celik B, Skender J, Niroula S. Hydralazine associated neutrophilic dermatosis: a unique case observing DILE, ANCA vasculitis, and Sweet syndrome. Presented at: American College of Physicians Michigan Chapter and Society of Hospital Medicine 2024 Resident and Medical Student Day; 2024 May 3; Troy, MI
Included in
Internal Medicine Commons, Radiology Commons, Rehabilitation and Therapy Commons, Rheumatology Commons
Comments
American College of Physicians Michigan Chapter and Society of Hospital Medicine Michigan Chapter 2024 Resident and Medical Student Day, May 3, 2024, Troy, MI