Mixed Etiology Hydroxyurea-Related Lower Leg Ulcer that Masqueraded as Sequelae From Sickle Cell Disease and Venous Insufficiency
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Index: WOUNDS. 2012;24(9):254–257.
A 27 year-old African-American man with sickle cell disease (SCD) presented with a complaint of a “painful right leg ulcer” of 3.5 years duration. The wound was located 1 cm above the medial malleolus. The patient was followed by his physician on a regular basis for approximately 1 year using topical wound care modalities, however, his leg ulcer failed to improve. Due to the recalcitrant nature of this condition, the patient was advised to undergo a below-knee amputation in an attempt to prevent osteomyelitis and possible sepsis. Almost 16 months after initial evaluation, the patient consulted a wound care specialist for a second opinion in the hopes of saving his leg. History revealed longstanding SCD with no history of trauma, fever, or signs and symptoms of infection. The patient was taking hydroxyurea (HU) 1000 mg daily for 3 years, due to recurrent painful vaso-occlusive sickle cell crisis.
Physical examination revealed an ulcer measuring 5.25 cm x 3 cm x 0.2 cm with irregular borders, located 1 cm above the medial malleolus of the right leg. The base of the ulcer showed 80% fibrosis and 20% granulation tissue. The lesion was surrounded by a hyper-pigmented area, however, there was no evidence of erythema, purulent discharge, or foul odor, and no bone was exposed (Figures 1 and 2). Increased temperature of the skin around the ulcer was noted with moderate pitting edema of the right leg. The skin was thin, shiny, atrophic, and eczematous. Varicosities, hemosiderosis, and lipodermatosclerosis were apparent. Vascular examination revealed bounding pedal pulses; capillary refill was within normal limits. Neurological examination uncovered normal deep tendon reflexes. Vibratory and sharp, dull sensations were intact. Orthopedic exam revealed normal range of motion at all major joints of the foot and leg. Muscle strength was adequate.
A biopsy revealed evidence of chronic inflammatory cell infiltration without evidence of malignancy or vasculitis. Final diagnosis was HU-related right lower leg ulcer mitigated by sickle cell disease and venous insufficiency.
Wound care included compression therapy, surgical and enzymatic debridement, as well as various antimicrobial agents as required based upon the clinical picture. A wound culture did not reveal growth of any organisms. The patient was medically managed with good hydration and control of anemia. Despite these efforts, however, the ulcer remained recalcitrant. The patient was regularly assessed to search for other etiologies that could cause delayed wound healing. Several months later, HU was discontinued by the patient’s medical doctor due to less-frequent attacks of vaso-occlusive crisis.
Serendipitously, 4 weeks after discontinuation of HU, the right lower leg ulcer dramatically improved with approximately 50% reepithelialzation, (from 5.25 cm x 3 cm x 0.2 cm to 2.5 cm x 1.6 cm x 0.1 cm), with 80% granulation tissue, and 20% fibrotic tissue (Figures 3 and 4). The ulcer completely reepithelialized 7 weeks after discontinuation of HU (Figure 5).
Review of Literature
Sickle cell disease. SCD is a genetic hemolytic disorder.