[CIS PIDD] [cis-pidd] Complicated patient with hypergammaglobulinemia and unknown rash
Shahlaee, Amir
ASHAHLAEE at gru.edu
Thu Nov 13 21:48:03 EST 2014
I have had a difficult time sending HSV PCR from a skin lesion. Any suggestions on where we can send that?
We are in the process of looking at the syphilis question again.
Thanks to both of you.
Sent from my iPhone
On Nov 13, 2014, at 7:29 PM, "Soheil Chegini" <schegini at yahoo.com<mailto:schegini at yahoo.com>> wrote:
The sensitivity of RPR is in excess of 90%, but it may miss up to 10% of cases of syphilis. Was RPR results confirmed by VDRL, TPHA or FTA-ABS?
Soheil Chegini, M.D.
Exton Allergy & Asthma Associates
656 West Lincoln Hwy.
Exton, PA 19341
Phone: (610) 269-3066
Fax: (610) 269-8615
________________________________
From: "Boyce, Thomas G., M.D." <Boyce.Thomas at mayo.edu<mailto:Boyce.Thomas at mayo.edu>>
To: CIS-PIDD <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Sent: Thursday, November 13, 2014 6:36 PM
Subject: RE:[cis-pidd] Complicated patient with hypergammaglobulinemia and unknown rash
any HSV testing by PCR? could have recurrent SJS triggered by HSV.
Thomas G. Boyce, MD, MPH
Pediatric Infectious Diseases and Immunology
Mayo Clinic
email: boyce.thomas at mayo.edu<mailto:boyce.thomas at mayo.edu>
phone: 507-255-8464
fax: 507-255-7767
From: Shahlaee, Amir [mailto:ASHAHLAEE at gru.edu]
Sent: Thursday, November 13, 2014 5:31 PM
To: CIS-PIDD
Subject: [cis-pidd] Complicated patient with hypergammaglobulinemia and unknown rash
I would appreciate any input on this complicated patient who remains a diagnostic dilemma.
His skin findings are much more impressive than I can describe.
Thanks,
Amir Shahlaee
Presentation
>20 year old patient presented to primary MD with complaints of pharyngitis and was treated with Keflex. He had received a course of Bactrim for a diagnosis of OM a week earlier. He subsequently developed a diffuse rash, acute kidney injury (AKI), transaminitis and thrombocytopenia and was transferred to us for a diagnosis of TEN/SJS. His rash had began on his face 3 days after starting Keflex and spread to his extremities.
Admitted to MICU and treated with Vanco/Aztreonam and systemic steroids. Dermatology consult done and biopsy found to be consistent with interface dermatitis with dyskeratotic keratinocytes and a sparse dermal infiltrate. Changes thought to be consistent SJS/TEN or DRESS. Patient discharged after improvement in symptoms.
8 months
Patient readmitted to MICU secondary to rash, UTI, dehydration and transaminitis. Had been treated with Bactrim at outside hospital and developed rash/swelling on face after drug exposure.
Patient also with bilateral pleural effusions.
Treated with steroids and antihistamines and discharged home.
9 months
Patient readmitted to hospital with a RLL necrotizing pneumonia, sepsis, weakness, transaminitis and dehydration again.
CT scan of chest revealed enlarged lymph nodes in hilum, axilla and mediastinum.
BAL refused by patient.
Multiple microabcesses noted in bilateral lower extremities and gluteal area.
Gluteal abscess drained and positive for “staph species”.
CT and MRI scans of lower extremities subsequently revealed sclerotic bone lesions: Biopsy of bone lesions with polytypic plasmacytosis and a single lymphoid aggregate thought to be benign. Also with patchy hypocellularity.
Treated with Vancomycin, Clindamycin and Aztreonam. Was on Zithromax briefly.
Immunoglobulins levels sent that reveal elevated IgG and IgA and a low IgM. Patient also with sporadic lymphopenia. Immunology consulted for first time. Labs outlined in detail below.
Seen by rheumatology who do not believe this is an autoimmune phenomenon. Patient with positive ANA 1:640 in speckled pattern.
11 months
Admitted from immunology clinic for a cohesive, weeping rash that involves the scrotum and inguinal areas in addition to the periumbilical area.
Treated with IV clindamycin
Heme/Onc Consultation done and POEMS syndrome, MGUS or other malignancies ruled out. SPEP and IPEP without evidence of a monoclonal spike.
Discharged home on topical steroids.
11.5 months
Readmitted for exacerbation of his rash. Biopsied by dermatology and found to be consistent with “eczematous dermatitis with impetigination”.
Treated with prednisone and doxycycline with almost complete resolution of rash.
1 year
Significant improvement in rash
Developed condylomas on genitals.
13 months
Worsening of rash
16 months
Noncompliant with follow ups.
Was treated with Augmentin by another physician for worsening of rash with limited response and developed a more generalized erythematous rash.
Returned to clinic this week with a severe exacerbation of his rash.
Physical Exam:
Severe lichenified rash on entire scalp.
Weepy, hyperpigmented rash in inguinal and medial aspect of thigh including on scrotal tissue
Periumbilical extension of rash.
Area behind bilateral ears now healed but with scarred tissue
Labs
Sporadic lymphocytopenia during hospitalizations that has since resolved
Mild anemia
IgA= 591 (range of values observed in this patient: 375-655)
IgG= 3049 (range of values observed in this patient: 1724-3510)
IgM= 28 (range of values observed in this patient: 27-66)
IgE = 124
Blood type A
Anti-B titer of 4
Diphteria 4.6, Tetanus 0.4
IgG 3730 (High)
IgG1 2470 (High)
IgG2 535
IgG3 106
IgG4 7.3
Pneumococcal titers only 5 serotypes less than <1.3
S pneumo type 1
2.0 mcg/mL
S pneumo type 2
1.9 mcg/mL
S pneumo type 3
2.7 mcg/mL
S pneumo type 4
0.6 mcg/mL
S pneumo type 5
8.8 mcg/mL
S pneumo type 8
1.9 mcg/mL
S pneumo type 9N
3.1 mcg/mL
S pneumo type12F
0.6 mcg/mL
S pneumo type 14
1.8 mcg/mL
S pneumo type17F
5.9 mcg/mL
S pneumo type19F
11.5 mcg/mL
S pneumo type 20
2.4 mcg/mL
S pneumo type22F
12.8 mcg/mL
S pneumo type23F
18.9 mcg/mL
S pneumo type 6B
4.6 mcg/mL
S pneumo type10A
5.7 mcg/mL
S pneumo type11A
1.4 mcg/mL
S pneumo type 7F
7.1 mcg/mL
S pneumo type15B
0.6 mcg/mL
S pneumo type18C
<0.2 mcg/mL
S pneumo type19A
4.9 mcg/mL
S pneumo type 9V
7.6 mcg/mL
S pneumo type33F
1.2 mcg/mL
Nitrogen Oxidative Burst Normal
Lymphocyte Subsets (ALC=500 on that day)
%CD3+ 68
CD3+ 278 Cells/mcL (L)
%CD3+4+ 47
CD3+4+ 181 Cells/mcL (L)
%CD3+8+ 19
CD3+8+ 73 Cells/mcL (L)
%CD19+3- 14
CD19+3- 58 Cells/mcL (L)
%CD56+3- 11
CD56+3- 46 Cells/mcL (L)
CD4/CD8 2.48
(ALC=1300 the following day)
%CD3+ 59 (L)
CD3+/mcL. 472 Cells/mcL (L)
%CD3+4+ 39
CD3+4+/mcL 313 Cells/mcL (L)
%CD3+8+ 17
CD3+8+/mcL 136 Cells/mcL (L)
4/8 ratio 2.31
CD11a normal
CD11b normal
CD18 normal
AH50 (Alternate Pathway) 89
Total complete 66
Skin biopsies
Presentation-interface dermatitis with dyskeratotic keratinocytes and a sparse dermal infiltrate. Consistent with EM/SJS vs. DRESS. Few curvilinear structures suggestive of spirochetes.
8 months Bone Marrow Biopsy: Polytypic plasmacytosis, patchy hypocellular marrow
ANA Screen Positive
ANA Pattern Speckled
ANA Titer >=1:640
ANCA MPO AbREF <0.2 U
ANCA PR 3 AbREF 0.4 U
ds-DNA AbREF <12.3 IU/mL
Cytoplasmic Neutrophilic Ab
c-ANCA= Negative (Ref range= Negative)
p-ANCA= Negative (Ref range= Negative)
Borderline equivocal for anti-PR3 antibody by solid-phase immunoassay. Neither cANCA nor pANCA patterns identified by immunofluorescence using ethanol-fixed neutrophils. Results do not appear to be consistent with ANCA-associated vasculitis. Unable to provide further interpretation without clinical information. Correlation with clinical presentation recommended.
HAV IgM Ab Nonreactive
HBsAg Nonreactive
HCV Ab Nonreactive
HBV Core IgM Ab Nonreactive
11 months-Psoriasiform dermatitis possibly with superinfection or impetiginization.
10 months HIV DNA PCR Negative
11 months IFE: No monoclonal IgG, IgA, IgM, kappa or lambda band is identified. IgG and IgA fractions are increased while IgM is decreased.
SPEP: Increased total protein concentration with normal albumin fraction. Diffuse polyclonal hypergammaglobulinemia may reflect chronic infection/inflammation, autoimmune disease, chronic hepatobiliary disease, or some solid tumor states. No monoclonal “spike” is seen. IFE to follow to rule out an occult paraprotein.
Kappa free light chains: 11.6 (elevated)
Lambda free light chains: 4.92 (elevated)
K:L Light Chain Ratio: 2.36 (elevated)
Protime 13.8 second
INR 1.2
APTT 31.7 second
Protein Total 8.8 g/dL (high)
Albumin EP 3.18 g/dL
Alpha 1 Globulin EP 0.30 g/dL
Alpha 2 Globulin EP 0.72 g/dL
Beta Globulin EP 0.99 g/dL
Gamma Globulin EP 3.61 g/dL (high)
B2 Microglobulin 2,127 ng/mL
1 year RPR nonreactive
HTLV ½ Ab Negative
HSV 1 IgG Ab Positive
HSV 2 IgG Ab Negative
HSV ½ IgM Ab Negative
22 months B lymphocyte Panel
CD 19+ 18%
Absolute CD19 166 cells/mcl
CD 19+20+ 18%
CD19+CD10+ 4%
CD19+CD27-CD10-CD38++ 2.8%
CD19+CD27-CD38++IgM++ 2.7%
CD19+CD27-CD21-IgM++ 17.3%
CD19+CD27- 84%
CD19+CD27-CD21+ 60%
CD19+CD27-IgM+IgD+ 58%
CD19+CD27-IgM+IgD+ (of CD27-) 69%
CD19+CD27-CD21- 12.6%
CD19+CD27+ 16%
CD19+CD27+IgM+IgD+ 3%
CD19+CD27+IgM+IgD+ (of CD27+) 16%
CD19+CD27+IgM+IgD- 4.1%
CD19+CD27+IgM+IgD- (of CD27+) 25.7%
CD19+CD27+IgM-IgD- 9.2%
CD19+CD27+IgM+IgD- (of CD27+) 57%
CD19+B220+ 91%
CD19+B220- 9%
CD19+CD5+ 14%
CD19+CD5- 86%
CD19++CD38++CD138++ 0.038%
CD38++CD138++ (of lymphs) 0.061%
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