[PAGID] help with patient

[Mary Beth Hogan]

I have an 8 yr old female child who initially presented with violaceous,
purpuric lesions associated with arthritis, lesion heal rusty.  Most
lesions (Not all episode though) are from the waist down and appear 3
days after illness.  No gross hematuria. Skin biopsy leukocytoclastic
vasculitis with C3 deposition/neutrophils.  Immunoflouresence was
negative for immunoglobulin deposition.    This rash worsens in the
feet/shin with exercise and can lead to something that is "thought to
be" erythema nodosum :painful, tender, discreet,  violaceous etc (never
witnessed by MD).  These episodes have been ongoing over 9 months. 

 

The second rash is associated with sun exposure and is more like a burn
which disappears more quickly than the vasculitis.  

 

She has an intermittent hepatitis "thought to be" autoimmune in nature
and hepatitis B/C studies are negative.  Liver biopsy has not been done.

 

Her cumulative lab data to date is as follows:

 

Labs: Negatives:  ANA, Jo1 antibodies, IgE, RF, hep C surface ab,
SSA/SSB, thyroid studies, ESR, QUIGs, tetanus, diphtheria,
isohemagluttinins:  MRI: no myositis noted

          Positives:  CPK, Aldolase, pANCA (1:320), smooth muscle ab, 

 

Baseline (no lesions):  Negative/normal:     Raji cell, C1q binding
assays. Urine porphyrins, C2, C3, CH50, MBL

                                  Positive:      C4 50(9-36).
Protoporphyrin 45 (0-34), Zinc Protoporphyrin 50 (0-38).

 

No labs have ever been achieved during rashing episodes.

 

Any suggestions for diagnosis or further workup?  Initially it was
thought that she had HSP, but the progression of the disease and lack of
IgA on bx seems to contradict that.  I appreciate your help.

 

 

Mary Beth Hogan, MD

Professor of Pediatrics

Section Chief: Allergy/Immunology/Pulmonary Medicine

University of Nevada School of Medicine

775-784-6522

 

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