CPC01 A puzzling panniculitis

Abstract

Abstract A 51-year-old White woman was seen by dermatology services for a 3-day history of a painful subcutaneous nodule affecting her right distal calf, which was on a background of recurrent erythema nodosum diagnosed in childhood and an admission 6 months prior secondary to neutrophilic panniculitis affecting the right groin. The patient had sustained a traumatic injury to the leg following a fall. She was treated with oral antibiotics for a presumed infected haematoma. As her pain increased, she presented to the emergency department 3 weeks after the fall. On examination, a tender erythematous subcutaneous lesion with central sloughing was noted. She was tachycardic and hypotensive on presentation. Her C-reactive protein was elevated (172 mg L−1), and leucocytosis (15 × 109 cells L−1), with associated neutrophilia and anaemia (9.0 g dL−1), was noted on complete blood count review. Two days later, despite treatment with intravenous (IV) benzylpenicillin, flucloxacillin, clindamycin and topical clobetasol propionate, the lesion enlarged rapidly. Dermatology was consulted. Viral screens, including HIV, herpes simplex virus, Epstein–Barr virus, cytomegalovirus, and hepatitis B and C, were all negative. Serum protein electrophoresis and serum angiotensin-converting enzyme were within normal limits. Her α-1 antitrypsin level was elevated (3.16 g L−1; reference interval 1.1–2.1 g L−1). Her admission was complicated by sepsis, which required escalation of IV antibiotics to meropenem, vancomycin and clindamycin. Punch biopsy revealed epidermal necrosis and ulceration with extensive neutrophilic dermatosis and dermal abscess formation. The inflammation was seen to extend into the subcutis. Findings were reported as consistent with neutrophilic panniculitis. Colchicine was commenced based on ongoing sepsis and anaemia secondary to acute-on-chronic inflammation. Over 2 weeks, the lesion rapidly enlarged, prompting the introduction of oral corticosteroids (0.5 mg kg−1). Adalimumab and doxycycline were further introduced to reduce steroid requirements. Outpatient echocardiogram and colonoscopy revealed no pathology. Reduced levels of α-1 antitrypsin were noted on two occasions (0.89 g L−1 and 0.91 g L−1), coinciding with normal inflammatory markers following her acute illness. To date, no α-1 antitrypsin genetic abnormality has been detected. Panniculitis can be a rare and life-threatening manifestation of α-1 antitrypsin deficiency. Patients presenting with systemic inflammatory response syndrome and panniculitis have a reportedly high mortality risk. No hepatic or respiratory manifestation of α-1 antitrypsin deficiency was seen in this case, highlighting the importance of considering α-1 antitrypsin deficiency in the assessment of panniculitis and rechecking α-1 antitrypsin in this context as it is an acute-phase reactant.