[CIS PIDD] [cis-pidd] 13 y/o female with SJS secondary to mycoplasma

[Prescott Atkinson, M.D.]

Hi Sara:

We published a recent case of SJS in a patient with macrolide resistant M. pneumoniae infection (Pediatrics 127(6):e1605-9. PMID: 21536614).  Follow-up of this patient revealed that he is mildly hypogammaglobulinemic.  Since there have been reports of M. pneumoniae actually being detected in the SJS skin lesions of such patients and there are well-described occurrences of septic arthritis with the organism in hypogammaglobulinemic patients, I wonder whether systemic spread of the organism in patients with SJS may also be occurring in the context of a humoral immunodeficiency, perhaps not very severe.  So, a long-winded way of suggesting that you might evaluate antibody production as well as complement in this patient.

Prescott

T. Prescott Atkinson, MD PhD, Professor and Director
Division of Pediatric Allergy, Asthma & Immunology
University of Alabama at Birmingham
Tel:  205-638-9072
Fax:  205-975-7080

From: dmvascon at usp.br [mailto:dmvascon at usp.br]
Sent: Thursday, January 16, 2014 1:07 PM
To: CIS-PIDD
Subject: Re: [cis-pidd] 13 y/o female with SJS secondary to mycoplasma

Dear Sara

I think that in the case of a child presenting HUS it is important to rule out defects of complement pathway (C3, factor I, H, B and CD46) and their phenocopies (antibodies against factor B and H are also associated with HUS). There are some few cases of inborn errors of cobalamin C.
Moreover, besides infectious triggers (E.coli, S. pneumoniae, HIV etc.), autoimmunity (such as SLE, anti phospholipid Ab) are also possibly involved in HUS.
Thinking in the context of M. pneumoniae infection that can induce IgM Ab to I antigen of red blood cells (sometimes with cryoagglutinin features) it is important to think in the possibility of Mycoplasma infection triggering of a new HUS in the patient, besides SJS. It's not uncommon to observe patients with SJS evolving to renal failure due to dehydration or immune complex deposition in renal glomeruli.

Therefore I would look at this patient closely in order to exclude other risk factors to develop "autoimmune" manifestations.

Hope it helps.

Best regards,

Dewton

Dewton de Moraes Vasconcelos, MD, PhD
Department of Dermatology
University of São Paulo School of Medicine
São Paulo, Brazil

________________________________
De: "Sara Anvari," <sanvari at cmh.edu<mailto:sanvari at cmh.edu>>
Para: "CIS-PIDD" <cis-pidd at lists.clinimmsoc.org<mailto:cis-pidd at lists.clinimmsoc.org>>
Enviadas: Quinta-feira, 16 de Janeiro de 2014 13:51:20
Assunto: [cis-pidd] 13 y/o female with SJS secondary to mycoplasma
Dear all,

Wondering if someone out there might have had experience with a case similar to this (see below) and could offer us some additional direction...

We have a 13 y/o female patient who was recently admitted for Stevens Johnson Syndrome that was triggered by a Mycoplasma pneumoniae infection. The patient's past medical history is only significant for an episode of hemolytic uremic syndrome at 4 years of age, otherwise no history of any underlying medical conditions.

The parent was concerned about her daughter's risk of recurrence and was wanted to know if there was a test to identify if she were at higher risk for recurrence of SJS with mycoplasma and if there was anything the patient could take prophylactically to help prevent a recurrence. At this time, we know there is some risk of recurrence of SJS with mycoplasma and we were wondering if anyone may have had a case similar to this and if they could share their insight and experiences with us.

We greatly appreciate any help or insights anyone can offer.

Best regards,

Sara



Sara Anvari, MD
Fellow
Division of Allergy/Asthma/Immunology
Children's Mercy Hospital and Clinics
2401 Gillham Road
Kansas City, MO 64108
Phone: (816) 960-8885
Fax: (816) 960-8888


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