27 Nov 2020

11/2/20: Hansen’s Disease with Lucio Phenomenon

PGY3 Minh Tran presented a case of a 51 year old male with no PMH who presents to the ED with 5 days of LUE pain.  Patient fell on the L arm 2 weeks ago.  Initially, he did not experience any issues, but over the last 5 days, he has noticed skin changes, swelling, pain and ulceration.  Of note, the patient has had chronic ulcerations over bilateral upper extremities for at least 6-10 years.  He notes that he lost his eyebrows and eyelashes around the same time lesions appeared.  Patient also has a R finger ulceration which states is not related to the L arm ulceration.  He thought that was similar to his prior ulcerations; however, the pain is much worse and has not resolved.  ROS also notable for 15 pound weight loss in the last 6 months.  The patient was born in Puebla, Mexico and came to the US >20 years ago.  He has a history of cocaine use about 15 years ago but denies any history of IVDU and skin picking.  Denies any ETOH or tobacco use.  He previously worked in construction as a large machine operator and in building freeways.

Vitals: T 38.7, HR 105, RR 19, BP 104/66, O2 sat 97% on RA

Physical Exam significant for saddle nose, clear cardiac and pulmonary exam, hepatosplenomegaly, alopecia of the eyebrows, LUE edematous with purulent ulcerations with violaceous/purpuric rim and surrounding retiform purpura.  Hemorrhagic bullae distributed throughout.  Chest, abdomen, R hand and LE with scattered ulcerations with crusting.  LE with hyper- and hypopigmented patches.

CBC: WBC 15.4> Hb 8.7/Hct 26.3 <Plt 231(MCV 70.5, RDW 19.6%)

CMP:

Na 117/K 3.7/Cl 81/HCO3 20/BUN 26/Cr 1.01 <Glucose 135

ALP 182>T.protein 7.1/Albumin 2.2/AST 60/ALT 34/Tbili 3.1/Dbili 3.0

Ca 7.7, Mg 1.9, Phos 2.9

Coags: PT 17.4, INR 1.43, PTT 34.8

Infectious workup was initiated given concern for soft tissue infection.  BCx, UA, UCx, Fungal culture, HIV, acute hepatitis panel all negative.  RPR resulted in reactivity with 1:1 titer and FTA-ABS negative.

XR of LUE concerning for gas and soft tissue swelling.  Wound culture obtained which was positive for Group A Strep but negative for AFB and fungal etiologies. 

Rheum workup initiated given the chronicity of ulcerations, with negative ANA, ANCA, dsDNA, SSA/SSB, and Cryoglobulin.

Hematology also consulted given the chronicity of ulcerations for consideration of microangiopathic disease.  APLS labs positive for B2 glycoprotein IgM, Cardiolipin IgM, antiphospholipid IgM and Lupus anticoagulant.  Extensive hypercoagulable workup sent with low levels of Protein C, Protein S, AT-III and FVL assays.  C3 levels were low with normal C4 levels. CH50 sent and resulted in low activity.

Initial derm biopsy done on LUE resulted in epidermal necrosis with neutrophil-rich inflammation and GPC within the dermis.  Repeat derm biopsy on the RUE resulted in perivascular and periadnexal dermatitis with foamy histiocytes and AFB positive bacilli, suggestive of Hansen’s disease.

Treatment: Patient was initially taken to the OR for dermatofasciectomy given concern for acute infection and started on broad spectrum antibiotics.  After derm biopsy resulted in Hansen’s Disease, the patient was initiated on minocycline 100mg qday + Rifampin 600mg qmonth + prednisone 40mg qday.  He stayed in the hospital for skin grafts and was discharged.  On follow up (5 months after initial diagnosis), patient had healthy graft with excellent motion.

Teaching points:

  1. Symptoms of Leprosy: affect the nerves, skin, eyes and lining of the nose (nasal mucosa)
    1. Advanced disease: loss of eyebrows and saddle nose
  2. Transmission: likely respiratory droplets among very close contacts.  Once treated, patient is no longer contagious.
  3. Lucio’s Phenomenon: necrotizing cutaneous vasculitis in multibacillary leprosy or reactive vasculitis resulting in severe necrotic skin lesions (mainly on extremities) from chronic untreated leprosy
    1. 3 criteria: skin ulceration, vascular thrombosis, invasion of blood vessels by Leprosy bacilli
  4. Treatment: Rifampicin 600mg qmonth, dapsone 100mg qday and clofazimine 300mg with prednisone 1mg/kg

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