A case of alpha thalassemia trait-associated pyoderma gangrenosum in a three-year-old Filipino male

ABSTRACT

@#INTRODUCTION: Pyoderma gangrenosum is a rare neutrophilic dermatoses in children of unknown etiology. Its occurrence may be associated with a systemic condition. In most cases, diagnosis is late and treatment is sometimes refractory to conventional therapies especially if the underlying disorder is undetected. CASE REPORT A 3-year-old Filipino male presented with a one-year history of extensive ulcers over the nape, chest, back and lower extremities which started as a solitary painful pustule over the right gluteal area. Central healing was observed with cribriform scarring. Patient was previously treated as a case of skin infection without success. Significant laboratory findings showed severe anemia, neutrophilia, increased ferritin and increased ESR. Initial hemoglobin electrophoresis showed normal profile with mild microcytic anemia. Bacterial culture and ANA (anti-nuclear antigen) were negative. A course of oral prednisone (1-2mkd) and dapsone (2mkd) were given for a month with no improvement of the lesions. Folic acid alone was started at 5mg per day for anemia. After 2 weeks, lesions significantly improved. Three years later, upon repeat hemoglobin electrophoresis test, patient was diagnosed with alpha-thalassemia trait. Folic acid was given as mainstay therapy. Lesions healed with characteristic cribriform scarring. CONCLUSION: This is a rare case of a Filipino child who initially manifested with pyoderma gangrenosum and eventually diagnosed with alpha-thalassemia trait. Due to serious cosmetic sequelae of pyoderma gangrenosum, it is important to find and treat the underlying systemic disorder to stop progression of this debilitating and disfiguring dermatosis