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Morning Report

Morning Report. Ralph Yachoui 12/8/09. The Case. CC: Skin Rash HPI: 46 y/o hispanic F p/w . 1 month h/o diffuse skin rash, first started on the arms, subsequently spreading to the upper chest, back and limbs. Pruritic and painful

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Morning Report

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  1. Morning Report Ralph Yachoui 12/8/09

  2. The Case • CC: Skin Rash • HPI: 46 y/o hispanic F p/w . 1 month h/o diffuse skin rash, first started on the arms, subsequently spreading to the upper chest, back and limbs. Pruritic and painful . Fever 100.4 on admission; 30lbs weight loss over 2 months

  3. The Case ( continued…) • PMH/PSH: Hypothyroidism • Medications: Levothyroxine • Allergies: NKDA • SH: Denies Etoh, drugs and smoking • FH: No significant family history

  4. Physical examination Fig 1: Numerous erythematous, annular and scaly plaques, some of them coalesced on the back.

  5. Fig 2: Multiple longitudinal bands of melanonychia Physical examination

  6. Physical examination • Per Derm: Rash looks like bullous erythema multiforme. Biopsies obtained • Per Rheum: Rash looks like Subacute cutaneous lupus erythematosus • Remaining of physical was unremarkable

  7. Labs • Hb : 8.5 MCV : 69 • WBC : 1.5 • Plt : 157 • Smear: teardrop cells c/w myelophthisic process

  8. Peripheral smear

  9. Labs . Iron 18 [42-135] Ferritin 360 [7- 160] Transferrin 140 [200-400] . Hb variants: Hb A 95% [ 96-100] Hb A2 6% [ 0- 3.5] Hb F 0 [ <2] Hb S undetected

  10. Labs .LFTs: Alk phos 64 T Bili 2.5 D Bili 0.1 AST 17 ALT 18 . Haptoglobin 23 [ 30- 200] LDH 420 [ 110- 230] . DAT ( Direct Coombs test) :positive IgG : positive

  11. Labs • PTT : 43.3 [ 22- 33] INR : 1 • Mixing studies: NO correction of PTT ! • LA panel : normal • Factor 2-10-9 : normal Factor 8 : 200% [ 60-189] Factor 12 ( Hageman) : 38% [50-150]

  12. Labs • HIV : neg • RPR : neg • Hepatitis panel : neg • Fungal and AFB bld cultures : neg • CRP 0.86 [< 0.5] ESR 68 [<48] • SPEP: Polyclonal hypergammaglobulinemia

  13. Imaging • CT C/A/P : . Right axillary LAD . Scattered ground glass parenchymal opacities bilaterally . Mild bilateral pleural effusion . Top normal spleen size

  14. Biopsy • A 4mm punch biopsy of skin lesion on the back showed perivascular lymphocytic infiltrate and widespread keratinocyte necrosis c/w bullous erythema multiforme

  15. Additional workup • C3 : 50 [ 86-184] • C4 : 27 [ 20-59] • C-ANCA/ P-ANCA : neg • Anti- myeloperoxidase : 10 [> 9 positive] • Anti-ribosomal P : neg • Anti- SCl 70: neg • Ro( SSA) / La (SSB) : neg • RF : neg

  16. Additional workup • Anti- cardiolipin : neg Anti- b2 glycoprotein IgA : 13 [<10] LA ( Hex phas, DRVVT) : neg • ANA : positive homogenous > 640 Speckled 0 Nucleolar 0

  17. Additional workup • ENA SM AB : 1.2 pos RNP AB : 1.2 pos • Anti- ds DNA : 1149 pos DIAGNOSIS: SLE with EM= Rowell syndrome

  18. Rowell syndrome • Original criteria for Rowell syndrome: 1- Lupus erythematosus 2- Erythema multiforme 3- Immunologic abnormalities in the serum: . Speckled pattern of antinuclear antibody (ANA) . Anti-La (SSB) antibody . Positive rheumatoid factor (RF)

  19. Rowell Syndrome (RS) • Since its first description in 1963 by Rowell et al , Rowell Syndrome has been reported in 37 patients, often not respecting the initial criteria…. • Some authors postulate that RS represent a subset of SCLE with targetoid lesions.

  20. Subacute cutaneous lupus erythematosus(SCLE) • First described by Sontheimer et al in 1979 as a distinct subset of cutaneous lupus characterized by: 1- Psoriasiform or annular lesions 2- Mild systemic involvement 3- Anti-Ro (SSA) antibodies 4- Associated with HLA-DR3

  21. SCLE ( psoriasiform lesions ) SCLE

  22. SCLE ( annular lesions) SCLE

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