Wednesday, March 10, 2010

Case for Diagnosis

Abstract:  11 y.o. girl with 6 month history of facial eruption
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.

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.
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.

Wednesday, March 03, 2010

An Orphan Patient

Abstract:  44 yo man with a 10 year history of a progressive and disabling dermatitis if the feet.
HPI:  This 44 yo professional was first seen 10 years ago with a dermatitis of both feet and nails.  KOH prep from toe nails was positive for hyphae and he was treated with 3 months of Lamisil p.o.  Nails and feet improved at that time.  He was next seen in 2004 with dermatitis of both feet located on plantar areas which was predominantly hyperkeratotic with areas of excoriation.  He had developed a cellulitis of the right leg which required hospitalization.  KOH from affected aeas was negative in 2004.  Treated with betamethasone diproprionate 0.05% ointment and wet compresses and was "80%" improved in two weeks.  At that time a diagnosis of "keratoderma" and possible "dyshidrosis" was considered.  The process recurred and he asked his PCP to place him on prednisone which was done and seemed to help for a while.  From 2004 - 2010 he saw a number of other dermatologists and podiatrists both locally and at a large university center where a number of other therapies were tried, including Castelanni's paint.  None worked for very long and he was seen back at my office in March 2010.  The patient is at the end of his wits with this.  It dominates his life and is the cause of pain which interferes with his ability to stand at work.

O/E:  March 1, 2010:  Symmetrical hyperkeratosis of the plantar aspects of both feet with areas of excoriation.  Nails look normal.  Palms normal.  KOH prep from plantar dermatosis is negative for hyphae and a fungal culture was plated.

Photos March 2010:

Diagnosis:  Is this keratoderma, tylosis or an unusual contact dermatitis? Could this have begun with tinea pedis nine years ago or was than an incidental finding?

Plan:  Patch testing needs to be considered to r/o occult contact.  I doubt biopsy will help.  Will start therapy with Salex Cream (6% salycilic acid) as we await fungal culture.

Questions:  Does anyone have strong feelings about a diagnosis here?  If so, what therapy should be tried? 

1. Shelley WB, Shelley ED.  The orphan patient. N Engl J Med. 1988 Mar 10;318(10):646. In this important letter to the NEJM, the Shelleys define the orphan as an individual “with a unique, inchoate, baffling and often disabling disease and yet clearly not discernable in the medical literature.”  While the patient described here is not strictly an "orphan patient" his 10 year unsuccessful quest for control or cure, puts him in that unfortunate category.  Your help will be appreciated.

2. Brian Maurer sent us an important review of "Shoe Dermatitis" by Robert Adams which appeared in California Medicine in 1972.  It is still valuable.