Disease Information for Urticaria pigmentosa

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Clinical Manifestations
Signs & Symptoms
Brown skin discoloration
Darier's sign/hive with scratch/papule
Excoriations
Flushing
Hemorrhagic rash
Hemorrhagic/Confluent ecchymotic rash
Increased pigmentation/tanning/hyperpigmented
Itching after hot bath
Pruritic/itching rash
Pruritis Itching
Rash
Rash/Characteristic pattern/distribution
Subepidermal Blisters
Urticaria, nonallergic/symptomatic
Chronic Diarrhea in a Child
Clinical Presentation & Variations
Presentation/ Papular Rash Face Upper Arms
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Self limited usually
Course/Usually spontaneous remission/2-3 years
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Children/all
Population/Infant
Laboratory Tests
Abnormal Lab Findings - Decreased
Complement (total CH50) level (Lab)
Abnormal Lab Findings - Increased
Beta Tryptase, Serum (Lab)
CarboxyPeptidase A (CPA)
Heparin serum level/intrinsic (Lab)
Histamine (Lab)
Tryptase, serum (Lab)
URINE Histamine
URINE Histamine metabolites
Diagnostic Test Results
Pathology
BX/Skin biopsy/Abnormal
BX/Skin Mast cells/clustered
PATH/Mast cell tissue infiltrates
Associated Diseases & Rule outs
Rule Outs
Anaphylaxis, generalized
Angioedema/angioneurotic edema
Carcinoid syndrome/metastatic carcinoid
Smallpox
Urticaria/hives
Associated Disease & Complications
Chronic hives/urticaria
Dermatitis/Chronic rash
Dermatographism (Darier's Sign)
Hyperpigmentation
Mastocytosis, systemic
Telangiectasia
Urticaria pigmentosa
Urticaria/hives
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Mast cell involvement/disorder (ex)
Pathophysiology
Pathophysiology/Basophile degranulation process
Pathophysiology/Histamine cellular release
Pathophysiology/Mast cell degranulation/activation
Process
PROCESS/Benign condition (ex)
PROCESS/Bimodal age distribution/incidence
PROCESS/Hypertrophic/Hyperplastic disorder (ex)
Synonyms
Synonym
Cutaneous Mastocytoses Maculopapular, Cutaneous Mastocytosis Maculopapular, Localised cutaneous mastocytosis, Localized cutaneous mastocytosis, Maculopapular Cutaneous Mastocytoses, Maculopapular Cutaneous Mastocytosis, pigmentation congenital urticaria pigmentosa, pigmentosa urticaria, UP MPCM, urticaria pigmentosa, Urticaria pigmentosa (disorder), Urticaria Pigmentosa Maculopapular Cutaneous Mastocytosis, Synonym/Localized infantile mastocytosis, Synonym/Mastocytosis, infantile, Synonym/Nettleships/E disease type 1, Synonym/Urticaria perstans hemorrhagica, Synonym/Xanthalasmoidea
Treatment
Drug Therapy - Indication
RX/Diphenhydramine (Benadryl)
RX/Oral Cromolyn (cromoglycate)
Surgical Procedures or Treatments
SX/Biopsy
Definition

The most common form of mastocytosis. It occurs primarily in children and is typically characterized by multiple persistent small, reddish-brown, hyperpigmented, pruritic macules and papules. They are located most commonly on the trunk, but seen also on the extremities, head, and neck. (Dorland, 27th ed)

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External Links Related to Urticaria pigmentosa
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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