Saturday, January 29, 2011

Dermatitis and Failure to Thrive

Abstract: 3.5 month old male infant with Failure to Thrive and Dermatitis

HPI: This 3.5 mo old infant male is the product of a normal pregnancy and delivery. He has had a severe dermatitis since shortly after birth. The process is most prevalent on head and neck and torso. He has lost weight on 150 kcal/kg per day. On visits seems happy and content. Three older siblings are all normal.

O/E: Widespread scaly patches on trunk and scalp. Thick cradle cap, greasy scale scalp and face. Background erythema. Red excoriated napkin area.

Photos: Taken when child was 9 weeks old.

Labs: Hi K+ and NA on hospital admission for FTT (but since normalized). Mild eosiniophilia and increased plts. Normal: CBC, ZN, IgE, IgM, IgA, IgG, amino acids. Normal karyotype, negative FISH for Williams Syndrome. Complement levels will be done and hair will be looked at for trichorhexis invagninata.

Diagnosis: Unclear. Initially I thought this child had infantile seborrheic dermatitis, but that usually responds well to treatment. At this point, Leiner's and Netherton's syndromes need to be ruled out. While neglect was initially considered, the child's pediatrician feels at this time that the mother is competent and has raised three other normal children.

Discussion: I saw this child once six weeks ago and because of transportation problems they could not keep f/u appointments. The eruption was originally treated with HC valerate 0.2% cream. Scalp hygiene was discussed. At this point a systemic process is considered.

Questions: In addition to considering Netherton's and complement deficiency syndromes like Leiner's what else would you recommend?

Follow-Up: The patient saw a pediatric gastroenterologist who changed her formula to one that excluded all milk proteins -- the eruption cleared completely in a few days and weight gain ensued. This was a milk allergy that masqueraded as a serious underlying disorder.

Sunday, January 09, 2011

Brachioradial Pruritus

We continue to get questions from readers -- but many do not leave an email address. If you want specific questions answered, I will try to do so but need an email to respond to. As of March 18, 2012, we've had 68 comments to this post -- and some people ask specific questions. If you want a response, you will have to include your email address. Otherwise, we have no mechanism to reply. We do not share email addresses with anyone. We do not bill anyone for anything.

June 15, 2012.  NEW LINK:  WE HAVE POSTED AN INFORMATION SHEET ON DermatologyCentral.  It's a work in process but should be helpful:  Brachioradial Pruritus Resource Page.
I have had an interest in Brachioradial Pruritus (BRP) for over 25 years and published a paper on it in 1985 and co-wrote a chapter on BRP for (Actually, the lion's share of the writing was done by Julianne Mann, then a dermatology resident at Oregon Health Sciences University.) As a result of the chapter, I receive a few requests for information each year. Two came in recently and, with their permission, are presented here. Perhaps, some of our readers have suggestions for management of this annoying and occasionally disabling problem. Note: It is amazing how many comments have been made by people who suffer with BRP. Around 55 at this time. If you wish to leave a comment, and would like us to acknowledge it, please send me your name and email address. It will not appear anywhere on this site; but if you want me to relpy to you I need your contact information. Thank you, DJ Elpern.

1. This is from a 32 y.o. equestrian
I am an otherwise healthy woman desperately searching for a doctor who may be able to discuss some treatment options for my very itchy arms! I am 32 year old, 110 lb, active, female resident of New England and have been suffering with itchy arms (without rash) for at least four years now. When I finally sought treatment- I was referred by my primary MD to a dermatologist who informed me that I had dry, sensitive skin, and prescribed a topical cream and an oral steroid - I carefully followed his advice for 2 years - with absolutely no relief from the itch. The only thing that relieved the symptoms (which flare more intensely in the evening hours) was ice packs. I was often woken from sleep by the itch.

The itch occurs in cyclical pattern- symptomatic for months, followed by a month or two of none or very mild symptoms, then followed by another flare. it is present in sunny months as well as winter.

Finally, frustrated by the unresolved symptoms, at my own expense, I had one visit with a dermatologist in a nearby large city who very quickly diagnosed brachioradial pruritus and prescribed topical Capsaicin. Capsaicin works - sort of. But, it is a total pain in the butt for minimal relief. I am seeking alternative treatment.....acupuncture, chiropractic, anything ... ? Do I need an X-ray? What do I do? I am on my own out here in a sea of medical professionals who seem unwilling to take this condition seriously or look for possible causes that may be an underlying cause for this condition of itchy arms....

It may also be of interest that I have a large amount of muscle/ligament type tension in my neck and shoulders- it's pretty severe, some of this tension is a manifestation of stress, some results from my very physical occupation as an Equestrian.... I am personally tempted to think that all the tension in my neck and shoulders may have something to do with the arm itch - but this conflicts with the cyclical nature of the itch. Has the tension is chronic?
To me, BRP it's a big deal!

2. This is from a 37 yo health care professional:
I have suffered with this for no less than 5 years. Until about a year ago it was sporadic, but has been substantially worse and constant for the last year or so. Nights are particularly difficult, as the itching becomes intolerable and uncontrollable. I have recently found some comfort with ice packs, but naturally having to traipse off to the kitchen several times a night is inconvenient and exhausting. The most recent prescriptions I have tried are 10mg cetirizide for daytime and 25mg doxepin (1-2 at bedtime), and also betamethosone cream. The cetirizide helps with other allergy issues I have, but doesn't offer any relief to the itchy arms. Also, the doxepin helps me get a few solid hours of sleep, if I take 2, but, as with hydroxizine, I feel "hungover" the next morning... AND insatiably hungry.
I do LOVE the sun, and have always found that I feel better through the cold months if I go tanning a couple times a week. In the winter months, I spend a bit of time tanning prior to spending any significant time outside in the summer. I have fair skin, and it helps prevent burning before spending a day at the beach. (which I do, ALL day, as often as possible.) I do not, however notice any increase or decrease in the itching based on exposure to sun/tanning. In the last year, however, I HAVE starting being a bit more careful and use a minimum of SPF 30 during peak hours at the beach. Here is a recent picture:
(Editor's note: This shows some typical findings. The skin looks a bit dry and lichenified and there is evidence of excoriation. Not all cases show these changes, some are more subtle.)

I have seen several doctors, including a dermatologist, regarding this debilitating itch and NOTHING has helped enough to warrant the side effects. I cannot seem to get anyone to understand that it isn't like a skin itch, it feels like its UNDER my skin. I scratch so much, especially at night, that I bleed. I am not a crazy, irrational person.... but the loss of sleep and inability to find anything that relieves this itching probably makes me seem as though I am.

Comment: Both of these patients appear to have chronic BRP. I see a similar patient once or twice a year. While most patients with BRP have episodic, relatively easy to treat disease, there are a few patients who have disabling symptoms. There is an aphorism, "It is often more important to treat the patient who has the disease than the disease the patient has." I think this applies to persons with chronic BRP. In a real way, these are orphan patients. Few dermatologists have the expertise, and fewer the time, to adequately evaluate and treat these patient. If you have suggestions, they would be most appreciated.

Actual Use
Most patients with BRP will get some relief from ice packs.  3M makes a reusable pack thatsells in the U.S. for $2.50.  This is an inexpensive way to start treating BRP if you have not used ice already.  You could use this 2 - 3 times a day for 15 - 20 minutes or whatever works best for you.