Eccrine gland necrosis allowing presumptive diagnosis of thrombotic vasculopathy in a patient with acute lupus erythematosus, pulmonary alveolar hemorrhage, antiphospholipid antibodies and livedo reticularis with purpura

    Pt was is a 43 year female with a history of hypothyroidism, Raynaud's, Barrett's esophagus, DVT (while on OCPs), recent admission for diffuse alveolar hemorrhage and diagnosed with systemic lupus erythematosus /MCTD overlap characterized by raynaud's, alopecia, puffy hands, GERD, lymphopenia, Smith +, RNP +, chromatin+, low titer anti DNA +, low C4, lupus anticoagulant positive.

    She presented to ER on 10/18 with cough that progressed to hemoptysis. During her admission her CTA was neg for PE, but did show alveolar infiltrates. She became hypoxic and was intubated. BAL revealed alveolar hemorrhage with neg cultures. She was placed on pressors. 

    Intubated 10/20, Pulse steroids 1gm daily for 4 days (started 10/20), Rituxan 375mg/m2 given on 10/21 and cytoxan 750mg/m2 given 10/23. Due to rapid development of livedo with purpura concerning for APS she started pheresis on 10/28 and continued for 5 exchanges. She was extubated on 10/28. She moved to the floor 10/31 and was discharged 11/2. Her ANC never dropped below 1,000. She had her line removed on 10/31. No CNS or no obvious renal involvement. Had menstrual period with some red cells. Spun urine no casts.

    Serologies ANA +, Anti DNA 7, SSA neg RNP >8, SmRNP>8, scl 70 1.6, ACL igg 16 elevation, igm neg, b2 glycoprotein 1 neg, lupus anticoag +, cryos pending. Anti GBM neg.


    The skin biopsy was performed to rule out vasculitis with clinicial suspicion of antiphospholipid-associated thrombotic vasculopathy.