HPI: This almost 12 yo girl has had a recurring facial eruption for ~ 6 months. In her mother's words: "At first it looked like hives. It was itchy and stung. Each day the rash changed in appearance and lasted almost 3-4 weeks. It traveled behind her ears and neck, then on to her hands and arms and finally to her chest and back. The pediatrician put her on oral steroids which did not seem to help at all. We went to 2 dermatologists, 3 pediatricians, and an allergist/immunologist. Their opinions varied from poison ivy, to a virus, to having absolutely no idea. The only thing that seemed to work was hydroxyzine.
The second occurrence happened in February 2010. I gave the hydroxyzine immediately and the symptoms began to disappear within 24 hours.
The next occurrence happened on March 7, 2010. She has had 3 doses of the hydroxyzine and the rash seems to be almost gone.
The patient is on no other medication and has no known allergies. We have racked our brains about everything she eats and all the products we use at home but cannot come up with any rhyme or reason.
Our pediatrician wonders if it is related to the sun......She was outside for recess yesterday and it was the first nice sunny day we have had."
Clinical Photo
Lab: Consider obtaining parvovirus B19-specific antibodies if this has not been done. CBC was done a few months back. This and an ANA panel will be obtained.
Questions: What are your thoughts as to possible diagnoses? The erythema of the cheeks suggests Erythema infectiosum, but this is almost never recurrent.
Diagnosis: This child's case was presented for ideas. She was not seen and her parents have had problems getting an appointment with a pediatric dermatologist. Based on the history and photograph I would consider an atypical erythema infectiosum, urticaria, a collagen vascular disease.
References:
Musiani M, et. al. Recurrent erythema in patients with long-term parvovirus B19 infection. Clin Infect Dis. 2005 Jun 15;40(12):e117-9. Epub 2005 May 11.
Department of Clinical and Experimental Medicine, University of Bologna, Bologna, Italy. monica.musiani@unibo.it
We describe 3 patients with long-term parvovirus B19 infection (defined as detectable parvovirus B19 DNA load for >6 months after the onset of symptoms), which we monitored by serial testing for parvovirus B19 load and the presence of parvovirus B19-specific antibodies in blood. The patients showed recurrent erythema at intervals of several months.
Note: Informed consent to present this patient's history and photograph was obtained from her parents.
It would appear to be a variant of urticaria on the morphology and rapid response to antihistamine. However the trick is to find the trigger factor and that usually involves taking an excellent general and dietary history including unusual food additives and colourants.
ReplyDeletePerhaps a photosensitive dermatitis of some sort, given abrupt onset with exposure to sunlight.
ReplyDeleteI have seen the rash of EI wax and wane over 6 to 8 weeks in the summer months, most likely due to ongoing exposure to sunlight.
The fact that early administration of PO hydroxyzine aborts the rash speaks to an underlying histamine generated eruption. This would also fit with the earlier description of a hive-like eruption and history of pruritis.
In cases of allergic rashes (e.g. recurrent urticaria), I often tell parents that 75% of the time we are unable to ascertain a specific etiologic agent. BT Maurer
I have seen just a few cases of erythema infectiosum (EI) and all of them were typical! (Most probably, I have missed the atypical forms!). As far as I remember, pruritus is not a predominant symptom for EI.
ReplyDeleteSo, I agree with Ian that it seems to be a variant of urticaria and agree with Dr. Maurer that in the majority of cases we cannot find the culprit.
I think errythema infectiosum ( fifth disease) is on top of the list in this patient. Remissions and exacerbations can happen in fifth disease for up to 6 months specially after exercise, fever and sun exposure. Presence of Parvovirus B19-IgM antibodies within 30 days of onset of illness is diagnostic.
ReplyDeleteIf she continued having this problem, SLE should be ruled out.
Khalid Al Hawsawi
Hives like appearence, recurrent nature of rash, spread behind ear and limbs, pruritus and rapid response to Hydroxizine; all goes against EI and most of photosensitivity disorders and favours a diagnosis of URTICARIA.It is quite usual in chronic cases of recurrent usticaria that both Patient and physician are unable to identify a cause.
ReplyDeletein addition to the comments posted, what about hyperimmunoglobulinemia D with periodic fever syndrome, TNF receptor-associated periodic syndrome?
ReplyDeleteI understand that you did not see her, but do you know whether she has any associated symptoms? Dr. H.D., Maine
This is unlikely but is she eating, cutting up mangos? Dr. S.R., CT
ReplyDeleteI have read with great interest the history of patients so well described. A detailed history always is a key to a good precise diagnosis.
ReplyDeleteI would give my diagnosis of Solar urticaria which can be associated with the symptoms as hers.
the fact that it disappears with hydroxyzine also show an histamine induced pathology .
Ofcourse Cutaneous Lupus should be ruled out.
Regards