Rare Case of Pyodermatitis-Pyostomatitis Vegetans with Concomitant Ulcerative Colitis and Chronic Myelomonocytic Leukemia: A Diagnostic and Therapeutic Challenge
Bildiriler > Tam Metin Bildiri · 2023
Yazarlar: Ayris ÖZTÜRK, Banu YAMAN, Fatih TEKİN, Denis Sabriye BOZER, Işıl TUNA
32nd Congress of the European Academy of Dermatology and Venereology, Almanya, Berlin, Uluslararası, Poster, Tam Metin Bildiri, 2023-2023, cilt -, ss ---
Özet
<p><strong>Introduction & Objectives:</strong></p><p>Pyodermatitis-pyostomatitis vegetans (PD-PSV) is a chronic inflammatory disease that affects the skin and mucous membranes and is usually associated with inflammatory bowel disease (IBD). Mucosal lesions are characterized by small confluent pustules and erosions that follow a specific “snail track” pattern on an erythematous base. Cutaneous lesions manifest as exudative, vesiculopustular, and vegetative plaques that mostly affect the scalp and intertriginous areas.</p><p>In this case report, we present a 27-year-old male patient diagnosed with UC, PD-PSV, and chronic myelomonocytic leukemia (CMML).</p><p><strong>Materials & Methods:</strong></p><p>The patient had been diagnosed with ulcerative colitis (UC) nine months prior to presentation and was admitted to our hospital due to a rash that started in the axillary region and spread to the anterior and posterior surface of the trunk, pubis, and extremities. He had difficulty eating and had a history of diarrhea for several days. For UC, he was started on mesalazine orally at a dose of 1 gram per day.</p><p><strong>Results:</strong></p><p>Physical examination revealed annular, center-crusted, polycyclic erythematous vesicopustules on the scalp, neck, trunk, extremities, axillae, pubis, and inguinal region. Many confluent pustules were observed on the gingiva and palate, which followed a characteristic “snail track” pattern, and numerous confluent pustules on labial mucosa with yellow exudates and hemorrhagic crusts that transformed into vegetative plaques.</p><p>Viral serology and monkeypox tests were negative. Autoimmune panel showed positive ANA and p-ANCA. Imaging studies showed no significant findings. Colonoscopy was consistent with UC. Thrombocytosis and leukocytosis were observed in the peripheral smear, leading to a bone marrow aspiration biopsy and a diagnosis of CMML by Hematology. Histopathologic examination showed mixed inflammatory cell infiltration with eosinophils, with DIF revealing positive IgA, IgG, and C3C. PD-PSV was diagnosed based on characteristic clinical findings, negative tests for other bullous diseases, and compatible histopathology. Treatment with methylprednisolone resulted in a dramatic clinical response within 3 days.</p><p><strong>Conclusion:</strong></p><p>PD-PSV may occur during an exacerbation of IBD or represent subclinical bowel disease. Other bullous disorders with vegetating vesicopustular eruptions should be distinguished from PD-PSV. The diagnosis is based on clinical features, association with IBD, peripheral eosinophilia, and distinctive histologic features. Topical or systemic corticosteroids in combination with immunomodulators used in the treatment of IBD are the most common first- line therapies. This case emphasizes the importance of considering PD-PSV in the differential diagnosis of skin and mucosal lesions in patients with underlying IBD, the concomitant disorders that may accompany PD-PSV, such as myeloproliferative diseases, and the need for multidisciplinary management in patients with other comorbidities.</p>