Leucocyte Adhesion Deficiency-1 Presenting as Leukocytosis and Pyoderma Gangrenosum

ABSTRACT

Leukocyte Adhesion Disorder is a rare autosomal recessive disorder characterized by immunodeficiency caused by compromised neutrophil adhesion and transmigration activity to inflammation sites resulting in recurrent infections, of which type 1 is the most common. We are herewith reporting an interesting case of a 17-year-old female patient who presented with skin lesions over the lower limb for 15-30 days which on further investigating found out to be Pyoderma Gangrenosum in a case of LAD type 1. Patient was managed with antibiotics, steroids, hydration, and supportive care. The lesion responded to steroids and completely vanished with some residual scarring in 3-4months time. Early diagnosis and appropriate line of treatment with oral as well as injectable steroids and antibiotics helped us in combating this rare disease.

Keywords: Leukocyte Adhesion Disorder, LAD Type 1, Pyoderma Gangrenosum, CD18, CD34