PMC4802580_01
Panniculitis of AAT deficiency. A, Tender, ill-defined, edematous, violaceous plaque with flaccid bulla and small central ulceration with serous drainage on upper extremity. B, Progression to atrophic scars after 3 infusions of augmentation therapy in combination with dapsone.
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These symptoms were accompanied by painful, ulcerating plaques of his upper and lower extremities (Fig 1, A). The patient's ulcerated plaques progressed to atrophic scars after 3 infusions of augmentation therapy (Fig 1, B), and the patient's pleural effusions resolved.
Histopathologic features of AAT deficiency-related panniculitis. Septal panniculitis with a lymphocyte predominant inflammatory infiltrate, scattered histiocytes, and septal widening of the subcutaneous fat. No evidence of vasculitis or well-formed granulomas. (right arm; hematoxylin-eosin stain; original magnification: x10.)
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Scattered necrotic keratinocytes were observed in the epidermis and acrosyringia (Fig 2).