Two year old boy with papular eruption since birth.
HPI: The patient is a 2-3/4 year old child who presents for
evaluation of lesions on the torso and face, hat have been present since
birth. Initially, the lesions were on
his left TMJ area and they have generalized since then.
The child has had many ear infections and now has
tubes. Otherwise, he has been
healthy. On direct questioning, his
mother states that the lesions become red after a bath.
O/E: The examination shows a
healthy-appearing boy. He has tan macules on the left TMJ, on the chest, the
back, and a few lesions on the legs. The
lesion measure 2-4 mm in diameter. After
stroking, they appeared to urticate.
Clinical Photos:
IMPRESSION: This is most likely maculopapular cutaneous mastocytosis, otherwise known as urticaria
pigmentosa.
Discussion: The child is very
apprehensive and the question is whether a biopsy should be done or should one
observe him. He seems healthy and at present
not troubled by his lesions.
Reference:
1. Severity of cutaneous findings predict the presence of systemic
symptoms in pediatric maculopapular cutaneous mastocytosis.
Barnes M1, Van L, DeLong L, Lawley LP. Pediatr Dermatol. 2014
May-Jun;31(3):271-5. Abstract: Although
the prognosis of maculopapular cutaneous mastocytosis (MPCM), also referred to
as urticaria pigmentosa, is often benign, clinicians lack evidence to reliably
predict those at risk of associated systemic manifestations. We sought to
elucidate clinical markers of disease severity to provide better treatment and
prognostic information for individuals with MPCM. A retrospective chart review
querying characteristics of children diagnosed with MPCM in the Emory
Dermatology Clinic was performed. Follow-up was obtained through a clinical
encounter or telephone interview. Linear regression was used to determine
predictors of the number of MPCM-related systemic symptoms. Of 67 subjects, 57%
were male, and the mean age of onset was 4.5 months. The maximum number of MPCM
lesions was 1 to 10 in 16%, 11 to 30 in 33%, 31 to 50 in 25%, 51 to 100 in 6%,
and more than 100 in 20% of subjects. For their MPCM lesions, 46% of subjects
reported itching, 34% flushing, and 25% blistering. Reported systemic symptoms
included diarrhea (22%), abdominal pain (15%), wheezing or dyspnea (13%),
vomiting (10%), bone pain (10%), headaches (8%), cough (10%), rhinorrhea (8%),
irritability (6%), and anaphylaxis (1.5%). In a multivariate linear regression
analysis, the maximum number of MPCM lesions (p = 0.02) and the number of skin
symptoms (p < 0.01) were statistically significant predictors of the number
of systemic symptoms, controlling for age of onset, body sites involved, and sex.
The correlation between cutaneous findings and symptomatology could aid
clinicians in identifying individuals with MPCM who might warrant systemic
evaluation and therapy.
2. Review Article: Mast Cells, Mastocytosis, and Related Disorders
2. Review Article: Mast Cells, Mastocytosis, and Related Disorders
Theoharis C. Theoharides, Ph.D., M.D., Peter Valent, M.D.,
and Cem Akin, M.D., Ph.D.
N Engl J Med 2015; 373:163-172July 9, 2015
Dr. Theoharides reports receiving royalties from a patent
(US 8,268,365 B2) related to an antiinflammatory composition for treating brain
inflammation, licensed to Algonot (a portion of the proceeds is given to Tufts
University under an agreement and another portion is given to to
AutismFreeBrain, a nonprofit company for autism research). He also reports
holding a patent (US 7,906,153 B2) related to mast cells, antiinflammatory
agents, multiple sclerosis, central nervous system disorders, and a mixture of
flavonoids and olive extracts, a patent (US 7,799,766 B2) related to the
treatment of hormonally dependent cancers, and a patent (US 6,689,748 B1)
related to a method of treating mast-cell activation–induced diseases with a
proteoglycan that is licensed to Algonot. Dr. Valent reports receiving grant
support from Ariad Pharmaceuticals, Celgene, Bristol-Myers Squibb, Pfizer,
Novartis, and Blueprint Medicines. Dr. Akin reports receiving consulting fees
from Novartis, Patara Pharma, and Blueprint Medicines and royalties from a
patent (WO2003065986 A2) related to the Laboratory of Allergic Diseases 2
mast-cell line. No other potential conflict of interest relevant to this
article was reported.
(I can send a pdf of this article to anyone who asks. DJE)
3. Guidelines for the Diagnosis and Treatment of Cutaneous
Mastocytosis in Children
Mariana Castells, MD,* Dean D. Metcalfe, MD,** and Luis Escribano,
MD, Am J Clin Dermatol. 2011 Aug 1; 12(4): 259–270.
This excellent article is available Free Full Text online.
from Brian Maurer, PA-C: In the realm of general pediatric practice, urticaria pigmentosa is a clinical diagnosis. I seldom refer, and never obtain labs or biopsy. As there is no effective treatment, reassurance goes a long way.
ReplyDeletefrom Yoon Cohen, D.O (Dr. Cohen is a fellow in Pediatric Dermatology).: This topic came up on our monthly journal club last month.
ReplyDeleteThe diagnosis is usually made clinically, and we usually reassure the parents.
If there are multiple lesions, or experiences systemic symptoms, we order CBC and tryptase. CBC to screen mast-cell leukemia (although it is rare, it occur within <5%). If tryptase is greater than 20, we refer to Hem/Onc for the further evaluation, otherwise we follow the patient regularly.
As for the management, we reassure the parents if it is solitary or asymptomatic, however, if there are multiple lesions or symptomatic, we tend to treat as the following:
For mild symptoms, we advise the parent to give non-sedative antihistamine such as Zyrtec day time
For moderate symptoms, we add sedative antihistamine such Benadryl or hydroxyzine at night to the day time regimen
For severe symptoms, we uses a low dosage of doxepin and prescribe EpiPen. If parents are worry about doxepin, we recommended both H1 and blockers.
I also included a patient handout and the article we reviewed!
Hope this helps....a little for the kid
I agree with all that has been said. Has anyone ever needed to use mast-cell stabilizers for this because antihistamines did not work?
DeleteWith my limited training in peds derm, I haven't observed this medication been used for pts with mastocytoma/UP in our derm dept yet, of course, which does not mean they have not used in the past. If pts fail up to Doxepin with tryptase greater than 20, we usually refer those pts to Hem/Onc for the further work-up and management. I recently read a related article that discusses on the management of mastocytosis. The article notes that Cromolyn sodium can be helpful especially in pts with GI symptoms.
Delete(http://www.bloodjournal.org/content/bloodjournal/121/16/3085.full.pdf).
From Dr. Khalifa Sharquie of Baghdad, Iraq: Mastocytosis not uncommon problem among Irarqi children and usually of good prognosis and disappear after years .No needs for investigation apart from biopsy as a support for family and treatment is only supportive
ReplyDeleteThe kids are very resilient and rarely have systemic symptoms. Gets better over time.
ReplyDelete