Causes of Mastocytosis
List of causes of Mastocytosis
Following is a list of causes or underlying conditions
(see also Misdiagnosis of underlying causes of Mastocytosis)
that could possibly cause Mastocytosis includes:
Mastocytosis Causes: Book Excerpts
Related information on causes of Mastocytosis:
As with all medical conditions,
there may be many causal factors.
Further relevant information on causes of Mastocytosis may be found in:
Causes of Mastocytosis: Online Medical Books
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Review excerpts from medical books online, free, without registration,
for more information about the causes of Mastocytosis.
Urticaria:
Differential Diagnosis
(In a Page: Signs and Symptoms)
-
Idiopathic urticaria without angioedema
–Most common diagnosis in patients with hives
–Often related to food or drug allergies, bites, or stings
–25% of patients with one episode will progress to chronic urticaria
-
Chronic urticaria
–Idiopathic in 50% of cases
–Chronic idiopathic urticaria spontaneously
resolves within 2 years in 80% of patients
–Criterion for chronic urticaria is duration of more than 6 weeks
-
Occult infection (e.g., sinusitis, oral infection, cholecystitis, vaginitis, prostatitis, hepatitis, HIV, tinea manus or pedis)
-
Malignancy
-
Thyroid disease
-
Drugs (e.g., radiocontrast media, penicillin, salicylates, benzoates, azo dyes)
–May result in life-threatening episodes of urticaria and acute angioedema that can lead to anaphylaxis -
Urticaria secondary to physical stimuli [e.g., exercise (cholinergic), vibratory pressure, sun exposure (solar urticaria), cold exposure]
–Dermographism occurs in 5% of the population; manifests as a physical urticaria that arises in the distribution line of a scratch or rubbed skin area -
Hereditary or acquired deficiency of complement factor C1
–Generally appears as episodic angioedema in the absence of urticaria
–Only in the absence of urticaria should hereditary or acquired complement deficiency be considered
-
Angioedema-urticaria-eosinophilia syndrome
–Associated with elevated serum IgE, fever, and fluid retention during an acute attack -
Urticarial vasculitis
–Presents as urticaria that lasts longer than 12–24 hours
–Associated with autoimmune disease (e.g., systemic lupus erythematosus)
-
Cutaneous mastocytosis/urticaria pigmentosa
» READ BOOK EXCERPT ONLINE »
Source: In a Page: Signs and Symptoms, 2004
Urticaria:
Differential Diagnosis
(In A Page: Pediatric Signs and Symptoms)
- Urticaria
–Epidemiology: Lifetime incidence 20%; most cases resolve within 48 hours; chronic
>6 weeks
–Pathophysiology: Hypersensitivity reaction: allergens (IgE-mediated, prior sensitization), complement, and other cytokines activate mast cells and basophils to release histamine (also kinins, prostaglandins, serotonin) with plasma extravasation; wheals/hives: dermis edema
–Triggers: Most cases are idiopathic
–IgE-mediated: Insects (bees, wasps, scorpions, spiders, jellyfish), foods (eggs, shellfish, tree nuts, peanuts, tomatoes), drugs (penicillins, cephalosporins, NSAIDs, barbiturates, amphetamines, insulin, blood products), pollen, danders, food additives
–Non-IgE-mediated: Infections (strep, EBV; hepatitis A, B, and C; adenovirus, enterovirus; fleas, mites), drugs (opiates, acetylsalicylic acid, local anesthetics), physical (exercise, cold/heat, UV light, water, pressure), contrast dyes, latex
-
Chronic urticaria: Associated with collagen vascular diseases (SLE, cryoglobulinemia), inflammatory bowel disease, malignancy, thyroiditis, hyperthyroidism, Behçet disease, vasculitis
-
Angioedema: 50% of urticaria cases; subcutaneous and mucous membrane edema
-
Anaphylaxis (IgE-mediated)
–Most potent foods: Peanuts, fish
–Mortality: 100–500 deaths/year in U.S.
–Associated shock has a poor prognosis
-
Hereditary angioedema
–High mortality
–Most cases are autosomal dominant
–C1 esterase inhibitor deficiency
–Recurrent episodes of edema (face, upper
airway, extremities)
–Triggers: Trauma, surgery
–Unresponsive to epinephrine, antihistamines
-
Others: Erythema multiforme, mastocytosis, guttate psoriasis, flushing, cellulitis
» READ BOOK EXCERPT ONLINE »
Source: In A Page: Pediatric Signs and Symptoms, 2007
Urticaria [Hives]:
Medical causes
(Handbook of Signs & Symptoms (Third Edition))
Anaphylaxis
Anaphylaxis — an acute reaction — is marked by the rapid eruption of diffuse urticaria and angioedema, with wheals ranging from pinpoint to palm-size or larger. Lesions are usually pruritic and stinging; paresthesia commonly precedes their eruption. Other acute findings include profound anxiety; weakness; diaphoresis; sneezing; shortness of breath; profuse rhinorrhea; nasal congestion; dysphagia; and warm, moist skin.
Hereditary angioedema
With hereditary angioedema — an autosomal dominant disorder — cutaneous involvement is manifested by nonpitting, nonpruritic edema of an extremity or the face. Respiratory mucosal involvement can produce life-threatening acute laryngeal edema.
Lyme disease
Although not diagnostic of Lyme disease — a tick-borne disease — urticaria may result from the characteristic skin lesion (erythema chronicum migrans). Later effects include constant malaise and fatigue, intermittent headache, fever, chills, lymphadenopathy, neurologic and cardiac abnormalities, and arthritis.
Other causes
Drugs
Drugs that can produce urticaria include aspirin, codeine, dextrans, immune serums, insulin, morphine, penicillin, quinine, sulfonamides, and vaccines.
Radiographic contrast medium
Radiographic contrast medium, especially when administered I.V., commonly produces urticaria.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Signs & Symptoms (Third Edition), 2006
Cutaneous larva migrans:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
Under favorable conditions — warmth, moisture, sandy soil — hookworm or roundworm ova present in feces of affected animals (such as dogs and cats) and hatch into larvae, which can then burrow into human skin on contact. After penetrating its host, the larva becomes trapped under the skin, unable to reach the intestines to complete its normal life cycle.
The parasite then begins to move, producing the peculiar, tunnel-like lesions that are alternately meandering and linear, reflecting the nematode’s persistent and unsuccessful attempts to escape its host.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Urticaria and angioedema:
Causes and incidence
(Professional Guide to Diseases (Eighth Edition))
Urticaria and angioedema are common allergic reactions that may occur in 20% of the general population. The causes of these reactions include allergy to drugs, foods, insect bites and stings and, occasionally, inhalant allergens (animal dander and cosmetics) that provoke an immunoglobulin (Ig) E-mediated response to protein allergens. However, certain drugs may cause urticaria without an IgE response. When urticaria and angioedema are part of an anaphylactic reaction, they almost always persist long after the systemic response has subsided. This occurs because circulation to the skin is the last to be restored after an allergic reaction, which results in slow histamine reabsorption at the reaction site.
Nonallergic urticaria and angioedema are also related to histamine release. External physical stimuli, such as cold (usually in young adults), heat, water, or sunlight, may also provoke urticaria and angioedema. Dermographism urticaria, which develops after stroking or scratching of the skin, occurs in as much as 20% of the population. Such urticaria develops with varying pressure, usually under tight clothing, and is aggravated by scratching.
Several different mechanisms and underlying disorders may provoke urticaria and angioedema. These include IgE-induced release of mediators from cutaneous mast cells; binding of IgG or IgM to antigen, resulting in complement activation; and such disorders as localized or secondary infections (such as respiratory infection), neoplastic diseases (such as Hodgkin’s disease), connective tissue diseases (such as systemic lupus erythematosus), collagen vascular diseases, and psychogenic diseases.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Diseases (Eighth Edition), 2005
Urticaria [Hives]:
Medical causes
(Professional Guide to Signs & Symptoms (Fifth Edition))
Anaphylaxis
This life-threatening reaction is marked by the rapid eruption of diffuse urticaria and angioedema, with wheals ranging from pinpoint to palm-size or larger. Lesions are usually pruritic and stinging and preceded by paresthesia. Other acute findings include profound anxiety, weakness, diaphoresis, sneezing, shortness of breath, profuse rhinorrhea, nasal congestion, dysphagia, and warm, moist skin.
Lyme disease
Urticaria may result from the characteristic skin lesion (erythema chronicum migrans) produced by this tick-borne disease. Later effects include constant malaise and fatigue, intermittent headache, fever, chills, lymphadenopathy, neurologic and cardiac abnormalities, and arthritis.
Other causes
Drugs
Many drugs can produce urticaria. Among the most common are aspirin, atropine, codeine, dextrans, immune serums, insulin, morphine, penicillin, quinine, sulfonamides, and vaccines. In addition, radiographic contrast media commonly produce urticaria, especially when administered I.V.
» READ BOOK EXCERPT ONLINE »
Source: Professional Guide to Signs & Symptoms (Fifth Edition), 2006
Urticaria/Angioedema:
Differential Overview
(Field Guide to Bedside Diagnosis)
❑ Ingestants
❑ Drugs
❑ Inhalants
❑ Hymenoptera venom
❑ Latex sensitivity
❑ Dermatographism
❑ Pressure urticaria
❑ Cholinergic urticaria
❑ Cold urticaria
❑ Solar urticaria
❑ Infection
❑ Urticarial vasculitis
❑ Hereditary angioedema
❑ Mastocytosis
» READ BOOK EXCERPT ONLINE »
Source: Field Guide to Bedside Diagnosis, 2007
Urticaria and angioedema:
Causes
(Handbook of Diseases)
Urticaria and angioedema are common allergic reactions. Causes include allergy to drugs, foods, insect stings and, occasionally, inhalants, such as animal dander and cosmetics, that provoke an immunoglobulin (Ig) E-mediated response to protein allergens. However, certain drugs may cause urticaria without an IgE response.
When urticaria and angioedema are part of an anaphylactic reaction, they almost always persist long after the systemic response has subsided. This occurs because circulation to the skin is inhibited after an allergic reaction, which results in slow histamine reabsorption at the reaction site. Nonallergic urticaria and angioedema are probably also related to histamine release.
External physical stimuli, such as cold (usually in young adults), heat, water, or sunlight, may provoke urticaria and angioedema. Dermographism urticaria develops with varying pressure, usually under tight clothing, and is aggravated by scratching.
Several different mechanisms and underlying disorders may provoke urticaria and angioedema. These include IgE-induced release of mediators from cutaneous mast cells; binding of IgG or IgM, resulting in complement activation; localized or secondary infections such as respiratory infection; neoplastic diseases such as Hodgkin’s disease; connective tissue diseases such as systemic lupus erythematosus; collagen vascular diseases; and psychogenic diseases.
» READ BOOK EXCERPT ONLINE »
Source: Handbook of Diseases, 2003
Urticaria:
Medical causes
(Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series)
Anaphylaxis
An acute reaction, anaphylaxis is marked by the rapid eruption of diffuse urticaria and angioedema, with wheals ranging from pinpoint to palm-size or larger. Lesions are usually pruritic and stinging; paresthesia commonly precedes their eruption. Other acute findings include profound anxiety, weakness, diaphoresis, sneezing, shortness of breath, profuse rhinorrhea, nasal congestion, dysphagia, and warm, moist skin.
Hereditary angioedema
An autosomal dominant disorder, cutaneous involvement is manifested by nonpitting, nonpruritic edema of an extremity or the face. Respiratory mucosal involvement can produce life-threatening acute laryngeal edema.
Lyme disease
Although not diagnostic of this tick-borne disease, urticaria may result from the characteristic skin lesion (erythema chronicum migrans). Later effects include constant malaise and fatigue, intermittent headache, fever, chills, lymphadenopathy, neurologic and cardiac abnormalities, and arthritis.
Other causes
Drugs
Many drugs can cause urticaria; the most common include aspirin, atropine, codeine, dextran, immune serums, insulin, morphine, penicillin, quinine, sulfonamides, and vaccines. In addition, radiographic contrast medium commonly produces urticaria, especially when administered intravenously.
» READ BOOK EXCERPT ONLINE »
Source: Alarming Signs and Symptoms: Lippincott Manual of Nursing Practice Series, 2007
Urticaria:
Medical causes
(Signs & Symptoms: A 2-in-1 Reference for Nurses)
Anaphylaxis
Anaphylaxis is marked by the rapid eruption of diffuse urticaria and angioedema, with wheals ranging from pinpoint to palm-size or larger. Lesions are usually pruritic and stinging; paresthesia commonly precedes their eruption. Other acute findings include profound anxiety; weakness; diaphoresis; sneezing; shortness of breath; profuse rhinorrhea; nasal congestion; dysphagia; and warm, moist skin.
Hereditary angioedema
Hereditary angioedema is an autosomal dominant disorder in which cutaneous involvement is manifested by nonpitting, nonpruritic edema of an extremity or the face. Respiratory mucosal involvement can produce life-threatening acute laryngeal edema.
Lyme disease
Although not diagnostic of this tick-borne disease, urticaria may result from the characteristic skin lesion (erythema chronicum migrans). Later effects of Lyme disease include constant malaise and fatigue, intermittent headache, fever, chills, lymphadenopathy, neurologic and cardiac abnormalities, and arthritis.
Other causes
Drugs
Many drugs can produce urticaria. Among the most common are aspirin, atropine, codeine, dextrans, immune serums, insulin, morphine, penicillin, quinine, sulfonamides, and vaccines. In addition, radiographic contrast medium commonly produces urticaria, especially when administered I.V.
» READ BOOK EXCERPT ONLINE »
Source: Signs & Symptoms: A 2-in-1 Reference for Nurses, 2007
Urticaria [Hives]:
Medical causes
(Nursing: Interpreting Signs and Symptoms)
Anaphylaxis.Anaphylaxis—an acute allergic reaction—is marked by the rapid eruption of diffuse urticaria and angioedema, with wheals ranging from pinpoint to palm-size or larger. Lesions are usually pruritic and stinging; paresthesia commonly precedes their eruption. Other acute findings include profound anxiety, weakness, diaphoresis, sneezing, shortness of breath, profuse rhinorrhea, nasal congestion, dysphagia, and warm, moist skin.
Hereditary angioedema.With hereditary angioedema, cutaneous involvement is manifested by nonpitting, nonpruritic edema of an extremity or the face. Respiratory mucosal involvement can produce life-threatening acute laryngeal edema.
Lyme disease.Although not diagnostic of Lyme disease, urticaria may result from the characteristic skin lesion (erythema chronicum migrans). Later effects include constant malaise and fatigue, intermittent headache, fever, chills, lymphadenopathy, neurologic and cardiac abnormalities, and arthritis.
Other causes
Drugs.Drugs that can produce urticaria include aspirin, codeine, dextrans, immune serums, insulin, morphine, penicillin, quinine, sulfonamides, and vaccines.
Radiographic contrast medium.Radiographic contrast medium, especially when administered I.V., commonly produces urticaria.
» READ BOOK EXCERPT ONLINE »
Source: Nursing: Interpreting Signs and Symptoms, 2007
Urticaria:
Urticaria - pathophysiology
(The 5-Minute Pediatric Consult)
- Immune-mediated:
- Antigen is cross-linked to IgE on a mast cell.
- This causes mast cell activation leading to the release of vasoactive mediators, such as histamine, leukotrienes, prostaglandin D2, platelet-activating factor, and other vasoactive mediators.
- These vasoactive mediators cause pruritus, vasodilatation, and capillary leak, which lead to the characteristic findings.
- Common triggers include some medications such as penicillins, foods such as milk or eggs, and envenomations.
- Non–immune-mediated:
- Degranulation of mast cells secondary to other non-IgE reactions such as physical changes, chemicals, some medications such as beta-lactams and sulfa-containing drugs and some foods
- Autoimmune-mediated:
- Degranulation of mast cells caused by cross-linking of IgE by IgG, or IgG binding to the high-affinity IgE (FcεRI) receptor on mast cells
Urticaria - etiology
Acute Urticaria
- Viral infections are thought to make up ~80% of all cases of acute urticaria. Most commonly isolated causes include the following viruses:
- Epstein–Barr
- Coxsackie A and B
- Hepatitis A, B, and C
- Parasitic infections
- Bacterial infections (especially group A Strep)
- Medications: Most frequently reported include the following:
- Radiocontrast
- Foods
- Transfusion of blood products
- Food additives and dyes
- Natural remedies including cranberry, feverfew, glucosamine, and ginger
- Insect venom including bees, wasps, hornets
Chronic Urticaria
- Idiopathic: Majority have unknown cause, but many feel that an association with an autoimmune mechanism is likely
- Physical (~20–30%):
- Dermatographism (9%): Stroking of skin causes linear urticaria at site of contact.
- Cholinergic (5%): Diffuse erythema and elevated but pale urticarial lesions; intense pruritus. Associated with sweating reflex, so often associated with overheating or exertion. May be worsened in combination with other triggers in specific combinations
- Cold (3%): Urticarial lesions present at areas of skin exposed to low temperatures; has a familial and nonhereditary form
- Delayed pressure/vibratory: Deep or prolonged pressure on skin produces significant urticaria and often angioedema. Vibratory urticaria is a form of delayed pressure urticaria caused by repetitive vibration (e.g., use of a jack-hammer).
- Mast cell disease:
- Urticaria pigmentosa: Excessive number of mast cells in skin, bone marrow, lymph nodes, and other tissues. Flares characterized by pruritus, flushing, tachycardia, nausea, and vomiting
- Systemic mastocytosis
- Systemic disease:
- Rheumatologic:
- Urticarial vasculitis: Erythematous wheals that resemble urticaria but histologically appear as leukocytoclastic vasculitis. Often presents with systemic symptoms and lasts for >24 hours
- Muckle–Wells syndrome: Chronic recurrent urticaria, deafness, amyloidosis, and arthritis
- Neoplasms
- Infections: Parasites especially noted to cause chronic urticaria
- Autoimmune: Antibodies to IgE or IgE receptor (FcεRI)
» READ BOOK EXCERPT ONLINE »
Source: The 5-Minute Pediatric Consult, 2008
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