(Last Updated On: April 2, 2019)

Authors: Carminda P. Rogacion, MD, Emmanuel C. Perez, MD, MPH, FPFCP, FPRA, Johannes F. Dayrit, MD, FPDS

Abstract

Introduction: Behçet’s disease is a chronic multisystem disease that is characterized by vasculitis. Recurrent oral aphthous ulcers, genital ulcers, skin and ocular lesions, and arthritis are the most frequent clinical manifestations. Pathergy may or may not be present. Due to its distinct clinical presentation, knowledge and clinical expertise are needed in order to promptly establish the diagnosis and address the disease. We report two cases of Behcet’s Disease in 2 Filipinos on the basis of history, clinical presentation, and supported by skin punch biopsy findings.

Case Summary: We encountered two patients with Behcet’s disease. Both presented with recurrent oral ulcers and genital ulcers with papulopustules. Case 1 is a 27-year old male who presented with recurrent vasculitis on the lower leg, nodular acne and recurrent apthous ulcers. Skin punch biopsies on a papule and on a purpuric patch showed perivascular and periadnexal inflammatory infiltrate of lymphocytes and neutrophils. Referral to rheumatology confirmed the diagnosis of Behcet’s disease. Case 2 is a 37-year old female, who presented with reccurent oral ulcers, genital ulcers, papustules on the face, nodules on lower extremities, and arthritis. Skin punch biopsies were done on a pustule and a nodule, showing perivascular dermatitis with neutrophils, and lobular panniculitis, respectively. A referral to rheumatology confirmed the diagnosis of Behcet’s disease. Both patients were initially given low doses of oral glucocorticoids. The first patient was given Dapsone 100mg once a day. The second patient was given Colchicine 500mcg once a day. Both patients were noted to have resolution of lesions while under maintenance therapy for several months.

Conclusion: Diagnosis of Behcet’s Disease is made clinically. Pathergy test and skin punch biopsy, though non-specific, support the diagnosis. The clinician must be aware of the constellation of symptoms that characterize the disease since treatment is specific to the clinical features of each patient. This will also prevent systemic complications associated with the disease.  

Citation

Rogacion C, Perez E, Dayrit J. Behcet’s disease in 2 Filipinos: a case report. J Phil Dermatol Soc. 2018; 27(2): 74-9.

 

Keywords

Hemochromatosis, Hereditary Hemochromatosis, Secondary Hemochromatosis, Iron overload disease, HFE gene, Iron.

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