Friday, November 06, 2015

Macular and Papular Eruption in a Young Child

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.  

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
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.


  1. 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.

  2. from Yoon Cohen, D.O (Dr. Cohen is a fellow in Pediatric Dermatology).: This topic came up on our monthly journal club last month.

    The 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

    1. I agree with all that has been said. Has anyone ever needed to use mast-cell stabilizers for this because antihistamines did not work?

    2. With 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.

  3. 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

  4. The kids are very resilient and rarely have systemic symptoms. Gets better over time.


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