Sec 25 Skin Manifestations of Bone Marrow or Blood Chemistry Disorders Flashcards by Charlene DPA

Skin lesions that bear a clinical and/or histopathologic resemblance to lymphoma

Cutaneous pseudolymphoma

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Relatively dense lymphoid infiltrate in the reticular dermis usually B-cell rich resembling lymphoma

Cutaneous lymphoid hyperplasia

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Medications that may induce CLH

Phenytoin
Carbamazepine
Phenobarbital
B blockers
Calcium channel blockers
ACE inhibitors
Allopurinol
D-penicillamine
Penicillin
Mexiletine chloride
Cyclosporine
Histamine inhibitors

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B and T cell

reticular dermis

Cutaneous lymphoid hyperplasia

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B and T cell
subcutis
lymphadenopathy

Kimura disease

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B and T cell
reticular dermis
eosinophilia

Angiolymphoid hyperplasia with eosinophilia

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B and T cell
subcutis
POEMS syndrome, lymphadenopathy

Castleman disease

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T cell

papillary dermis and epidermis

Pseudomycosis fungoides

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T cell
papillary dermis and epidermis
contact allergens

Lymphomatoid contact dermatitis

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T cell

perivascular and periadnexal dermis

Lymphocytic infiltration of the skin (Jessner’s)

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Lymphoma B symptoms

fever of unknown origin
unexplained weight loss
night sweats
fatigue
malaise

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Presents most commonly as a solitary nodule or as a localized array of nodules, plaques or papules on the head, neck, extremities, breasts and genitalia with doughy to firm consistency

Cutaneous lymphoid hyperplasia

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Syndrome caused by anticonvulsants such as phenytoin which presents with fever, lymphadenopathy, hepatosplenomegaly, arthralgia, eosinophilia and generalized macules and papules or nodules

Hydantoin-associated pseudolymphoma syndrome

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Presents as a unilateral eruption of angiomatous papules on the extremities with dense lymphoid infiltrate associated with histiocytes, plasma cells and prominent thickened capillaries

Acral pseudolymphomatous angiokeratoma of children

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Cutaneous pseudolymphoma of mixed cell or large forms of primary B-cell lymphoma

Large cell lymphocytoma

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Reveals a dense, nodular or diffuse lymphoid infiltrate in the reticular dermis tends to be top heavy and taper to the lower dermis; epidermis is normal and separated from infiltrate with narrow grenz zone

Cutaneous lymphoid hyperplasia

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Infiltrate consists of predominantly small lymphocytes with an admixture of large lymphoid cells, histiocytes and eosinophils

Cutaneous lymphoid hyperplasia

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Present in germinal centers that contain phagocytized debris from apoptotic lymphoid cells

Tingible-body macrophages

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Defining immunophenotypic feature of CLH

Small, nongerminal center B cells and plasma cells are polytypic

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CLH with presence of a dominant B-cell clone

Clonal CLH

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Disorders that can contain dense cutaneous infiltrates of both T cells and B cells

Lymphoid keratosis
Pseudolymphomatous folliculitis
Systemic immunoglobulin IgG4-related plasmacytic syndrome
CD4+ small/medium pleomorphic T cell lymphoma
Cutaneous lymphoid hyperplasia

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Progonis: CLH

Resolve sponataneously or persist indefinitely

Regress with biopsy

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First line treatment: CLH

Excision
Topical corticosteroids (mid to high potent twice daily)
Intralesional corticosteroids (5-40 mg/ml, 1ml monthly)
Systemic antibiotics (minocyline, cephalexin)
Topical tacrolimus

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Second line treatment: CLH

Cryotherapy
G-Aminolevulinic acid PDT
Laser therapy (PDL)
Hydroxychloroquine
Systemic corticosteroids
Local radiation therapy

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Presents as a solitary or multiple nodules in the subcutis, represents a florid, subcutaneously deep seated form of disease like CLH which is more common in Asian men with peripheral eosinophilia and regional lymphadenopathy

Kimura disease

Presents with multiple, smaller, more superficial intradermal papulonodules that are usually unilateral which are malformation of blood vessels caused by and underlying arteriovenous shunt which is more common in women

Angiolymphoid hyperplasia with eosinophilia

Histopath: Hyperplasia of small blood vessels lined by plump endothelial cells protruding into the lumen with vascular hyperplasia with thickened vascular walls, lymphocytic infiltrate and prominent eosinophilia

Angiolymphoid hyperplasia with eosinophilia

Complication of Kimura disease

Lichen amyloidosis | Nephrotic syndrome

First line treatment of Kimura disease and Angiolymphoid hyperplasia

``` Excision Topical corticosteroids (high potency 2x/day) Intralesional corticosteroids (5-40 mg/ml, 1 ml monthly) ```

Also known as Angiofollicular lymphoid hyperplasia or Giant lymph node hyperplasia

Castleman disease

4 Subtypes of Castleman disease

1. Hyaline-vascular 2. Plasma cell 3. HHV-8 associated 4. Multicentric, NOS

POEMS Syndrome

``` Polyneuropathy Organomegaly Endocrinopathy M protein Skin changes ```

Presents as an isolated mediastinal mass can be nodal or extranodal associated with polyneuropathy, organomegaly, endocrinopathy, presence of M protein, hyperpigmentation and hypertrichosis

Castleman disease

Histopathology: Exhibits small, concentrically whorled, lymphoid follicles surrounded by small lymphocytes arranged in a concentric, onionskin pattern with extensive proliferation of capillaries in between follicles

Castleman disease - Hyaline-vascular

Histopathology: Exhibits large, hyperplastic secondary lymphoid follicles associated with highly vascular interfollicular zone rich in plasma cells

Castleman disease - Plasma cell

Complications: Castleman disease

``` Paraneoplastic pemphigus Plane xanthoma Vasculitis Peliosis hapitis Lymphoma Follicular dendritic cell sarcoma ```

Prognosis: Castleman disease

Favorable for localized, extranodal

Treatment: Castleman disease

``` Excision (first line) Second line: Radiation therapy Chemotherapy Rituximab +/- chemotherapy or thalidomide Anti-IL-6 or anti-IL-6 antibody Bortezomib ```

Present as one or few plaques on trunk or extremities with history of associated disease or medication

Pseudomycosis fungoides

Medications associated with Pseudomycosis fungoides

``` Hydantoin Carbamazepine Propyl valerate Imatinib mesylate Dexchlorpheniramine maleate Antihistamines ```

Histopathology: Papillary dermal, band-like infiltrate containing mostly small and medium-sized atypical lymphocytes with clefted and cerebriform nuclei, no epidermal-dermal junction obscured, no grenz zone, minimal epidermotropism

Pseudomycosis fungoides

Treatment: Pseudomycosis fungoides

``` First line: Drug discontinuation Treatment of underlying disorder Topical corticosteroids UVB and narrowband UVB phototherapy Psoralen plus UVA phototherapy Second line: Systemic corticosteroids (60/40/20 mg PO taper) ```

Presents as generalized red scaly papules and plaques that may become confluent with resultant exfoliative erythroderma associated with pruritus in adults of both genders

Lymphomatoid contact dermatitis

Allergens responsible in Lymphomatoid contact dermatitis

``` Phosphorus sesquisulfide Para-tertiary butyl phenol formaldehyde resin Ethylenediamine dihydrochloride N-isopropyl-N-phenyl-p-phenylenediamine Benzydamine hydrochloride Teak wood Cobalt naphthalene Gold Nickel Para-phenylenediamine ```

Histopathology: Exhibits superficial lymphocytic dermatitis that contains foci of spongiosis simulating the appearance of cutaneous T-cell lymphomas with edema in the papillary dermis

Lymphomatoid contact dermatitis

Treatment: Lymphomatoid contact dermatitis

``` First line: Elimination of responsible allergen Topical corticosteroids Second line: Topical tacrolimus and pimecrolimus ```

Presents as one or more erythematous plaques or nodules generally localized to one or few sites on the face, neck and upper trunk or arms in middle-aged adults some with photoexacerbation

Lymphocytic infiltration of the skin

Histopathology: Reveals a superficial and deep perivascular and variably periadnexal infiltrate of small mature lymphocytes with histiocytes, plasma cells and plasmacytoid macrophages

Lymphocytic infiltration of the skin

Prognosis: Lymphocytic infiltration of the skin

Chronic coursee | Spontaneous remission and eventual resolution

Treatment: Lymphocytic infiltration of the skin (first line)

Topical corticosteroids Topical tacrolimus and pimecrolimus Photoprotection Intralesional steroid injection (5-40mg/ml, 1 ml monthly)

Treatment: Lymphocytic infiltration of the skin (second line)

Hydroxycholoroquine (100-200 mg PO daily) Systemic corticosteroids (60/40/20 taper 5 days each) Auranofin (3 mg PO daily) Acitretin (25-50 mg PO daily) Thalidomide (100mg PO daily) Pulse dye laser (595 nm)

They are antigen presenting cells that interact with T cells

Dendritic cells

Has a long cytoplasmic projections and large almost kidney-shaped nucleus with characterisic Birbeck granule, a tennis racket-shaped structure involved in pinocytosis and receptor-mediated endocytosis

Langerhan cell

Receptor that permits internalization of antigen into Birbeck granules and presentation of antigen at the cell surface and most specific marker for Langerhan cells

Langerin (CD207)

LCH prototype of acute disseminated multisystemic form in infants or newborns and if untreated, fatal

Letterer-Siwe disease

Chronic progressive multifocal form of LCH beginning in childhood

Hand-Schuller-Christian disease

Localized benign form of LCH

Eosinophilic granuloma

Benign self-healing variant of LCH

Hashimoto-Pritzker disease

Presumptive diagnosis of LCH

Light morphologic characteristics

Designated diagnosis of LCH

Light morphologic characteristics + 2 or more supplemental positive results to stains for adenosine triphosphatase, S100 protein, a-D-mannosidase, and peanut lectin

Definitive diagnosis of LCH

Light morphologic characteristics + Birbeck granules in the lesional cell visible with EM and/or positive results on staining for CD1a antigen on the lesional cell

Classification of LCH

Single-system Disease - Localized Single-system Disease - Multiple site Multisystem Disease - Low risk group Multisystem Disease - High risk group

Monostotic bone involvement Isolated skin involvement Solitary lymph node involvement

Single-system Disease - Localized

Polyostotic bone involvement Multifocal bone disease Multiple lymph node involvement

Single-system Disease - Multiple site

Disseminated disease with involvement of skin, bone, lymph node, or pituitary

Multisystem Disease - Low risk group

Disseminated disease with involvement of hematopoietic system, lungs, liver and/or spleen

Multisystem Disease - High risk group

Presents with small translucent papule 1-2 mm in diameter slightly raised rose-yellow usually on the trunk or scalp which may show scaling and can become ulcerated and crusted

Langerhans-Cell Histiocytosis

Characterized by the eruption of multiple or solitary elevated form red-brown nodules or flesh-red lesions similar to infantile angiomas which ulcerate easily growing in size then forming crusts then shed leaving whitish atrophic scars

Hashimoto-Pritzker disease

T or F: Oral manifestations may be the first sign of LCH

True

T or F: Nail changes are unfavorable prognostic sign

True

Nail changes in LCH

``` Fragile lamina Paronychia Subungual pustules Nail fold destruction Onycholysis Subungual hyperkeratosis Longitudinal grooving Pigmented and purpuric striae of nail bed ```

T or F: Bone lesions are the most frequent manifestation of LCH

True | 80%, seen alone in 50-60% of cases

Part of the skull most often involved in LCH with lead to limited osteolytic foci that merge to form typical "map" lesions

Temporoparietal region

Involvement of this bone is responsible for exophthalmos

Retro-ocular bone involvement

Chest radiograph finding in LCH

Classic "honeycomb" appearance or micronodular pattern

Most common lymph nodes affected in LCH

Cervical lymph nodes

Present in more than 50% of cases and occurs more often in patients with involvement of the skull and orbits

Diabetes insipidus

First sign of CNS involvement in LCH

Cerebellar manifestations

Most common intracranial manifestation in LCH

Hypothalamic pituitary region infiltration (10-15% - associated with diabetes insipidus and anterior pituitary hormone deficiency)

Second most common intracranial manifestation in LCH

Neurodegenerative LCH

Type of reaction where extensive epidermotropism, lichenoid infiltration of LCH cells in the upper dermis.

Proliferative reaction

Type of reaction which consists of aggregatio of LCH cells with multinucleated histiocytes and a varying number of eosinophils

Granulomatous reaction

Type of reaction which consists of mainly HSC and numerous foam cells intermingled with LCH cells and eosinophils

Xanthomatous reaction

Expression of this is an indicator of good prognosis and limited disease in LCH

E-cadherin

Most important predictor of poor outcome in LCH

Organ dysfunction

Treatment for Single-system Disease (skin)

``` Observation (children) Nitrogen mustard (adult) ```

Treatment for Single-system Disease (bone)

Excision - accessible

Represents a broad group of different disorders by proliferation of histiocytes other than Langerhans cells

Non-Langerhans cell histiocytosis

Laboratory investigations for LCH

``` CBC Coagulation profile Serum protein electrophoresis ESR CRP level Serum glucose level LFT Electrolyte levls T and B cell counts T cell subset analysis Urinalysis (urine osmalality test) ```

Treatment for Single-system Disease (bone) in children

Indomethacin

Treatment for Multisystem Disease LCH

Vinblastine (0.1-0.2 mg/kg) +/- Glucocorticoids

Treatment for Multisystem Disease LCH if monotherapy is not effective

Vincristine Cyclophosphamide Doxorubicin Chlorambucil

Treatment for refractory and advanced Multisystem Disease LCH

Cyclosporin Interferon-a2 2-chlorodeoxyadenosine (Cladribine)

Accounts for 80-90% of Non-Langerhans cell histiocytosis

Juvenile xanthogranuloma

NLCH that appears within the first year of life which is a benign self-healing disorder characterized by asymptomatic yellowish papulonodular lesions of the skin without metabolic disorders

Juvenile xanthogranuloma

Variant of JXG rare with less than 20 cases onset on first year of life more common in males with lesions ranging from 2-12mm in diameter with generalized distribution no tendecy to merge into plaques rapidly become xanthomatous

Papular xanthoma

Rare NLCH with approximately 40 cases may start at any age with asymptomatic eruption of round or oval papules that are firm, or dark red and range from 3 to 10mm appearing in successive crops usually in hundreds and symmetrically distributed involving mucous membranes

Generalized eruptive histiocytosis

Rare NLCH with 30 cases onset is 5-34 months considered a limited form of GEH with asymptomatic slightly raised round or oval orange-red or red-brown lesions 2-8mm in diameter on upper face (eyelids, forehead, cheeks)

Benign cephalic histiocytosis

Exceptional form of NLCH in few children and adults which is a progressive disease with no tendency to involute spontaneously which is a proliferation of mature spindle-shaped cells

Progressive nodular histiocytosis

Rare benign NLCH with 100 cases more frequent in males onset is before 25 years associated with diabetes insipidus with cutaneous manifestations consisting of hundreds of papules that are red-brown then become yellowish symmetrically involve the eyelids, trunk, face and proximal extremities and in flexures and folds

Xanthoma disseminatum

Rare MLCH 60 cases variant of XD with visceral manifestations and progressive

Erdheim-Chester disease

Uncommon NLCH more than 100 cases purely cutaneous Caucasian women 40 years of age more commonly affected which may represent an abnormal histiocytic reaction like trauma (solitary forms) or internal malignancy and autoimmune disease (diffuse forms)

Multicentric reticulohistiocytosis

NLCH described in 60 cases with no gender predilection onset from 17 to 85 years with lesions consisting of indurated papulonodules varying in color from red-orange to violaceous or yellow slowly enlarging into plaques with well-demarcared edges with central atrophy and telangiectasia and linked to paraproteinemia (90%)

Necrobiotic xanthogranuloma

Relatively rare benign self-limited NLCH with approximately 365 cases with worldwide distribution increased in blacks no gender predilection and any age group confined mainly to cervical lymph nodes

Sinus histiocytosis with massive lymphadenopathy

JXG form which is characterized by numerous firm hemispheric lesions 2-5 mm in diameter irregularly scattered throughout the skin but are located mainly on upper body

Papular JXG

JXG form which is less frequent occurs as few nodules generally round 10-20mm in diameter, translucent and show telangiectasia

Nodular JXG

Lesions of JXG greater than 2cm

Giant juvenile xanthogranuloma

Group of JXG lesions with a tendency to coalesce into a plaque

JXG en plaque

2 new variants of JXG

Blueberry muffin baby - bluish papules and nodules | Multiple lichenoid JXG - hundreds of discrete papules

Most typical extracutaneous finding in JXG

Ocular involvement

Extremely rare extracutaneous manifestation of papular form with CNS involvement and related to systemic lesions

Juvenile chronic myelogenous leukemia

Histopathology: Nonepidermotropic histiocytic infiltrate lacking Langerhans granules with monomorphic nonlipid-containing histiocytic infiltrate occupying upper half (early) and foam cells, FB giant cells, and Touton giant cell (mature)

Juvenile xanthogranuloma

Complication: Juvenile xanthogranuloma

Severe secondary glaucoma

Prognosis: Juvenile xanthogranuloma

Flatten with time Evolves separately - different stages Good prognosis

Histopathology: Dense, monomorphous nonxanthomatous histiocytic infiltrate (containing nucleus with scanty chromatin and abundant light poorly limited cytoplasm) in the papillary and midportion of the dermis with few lymphocytes

Generalized eruptive histiocytosis | Benign cephalic histiocytosis

Electron microscopy: Tumor cells tend to contain a large number of dense and regularly laminated bodies clustered together with occasional worm-like bodies

Generalized eruptive histiocytosis

Electron microscopy: Many coated vesicles with diameter ranging from 500-1500 nm with clusters of comma-shaped bodies

Benign cephalic histiocytosis

Prognosis: GEH/BCH

Regress spontaneously

Presents with yellow to orange papules 2-10mm in diameter that are randomly scattered over the body without localization in the flexural areas

Progressive nodular histiocytosis - superficial papules

Presents with deep dermal nodules ranging from 1 to 5 cm without overlying telangiectasia mainly on the trunk

Progressive nodular histiocytosis - deep nodules

Histopathology: Xanthogranuloma with predominance of storiform spindle-shaped histiocytes that are positive for macrophage/dendritic cell line markers (CD68) and negative for CD1a and S100

Progressive nodular histiocytosis

Prognosis: Progressive nodular histiocytosis

Progresses but good health

Most common sign in Erdheim-Chester disease

Chronic bone pain

Associated with multiple myeloma, Waldenstrom macroglobulinemia and monoclonal gammopathy

Xanthoma disseminatum

Histopathology: Mixture of histiocytes, foam cells, inflammatory cells and later, foam cells and Touton giant cells with siderosis observed

Xanthoma disseminatum

Histopathology: Shows an infiltrate of lipid-laden histiocytes intermingled with Touton giant cells and eosinophils surrounded by fibrosis

Erdheim-Chester disease

Complications: Xanthoma disseminatum

``` Conjunctival inflammation Respiratory obstruction Siezures Diabetes insipidus Growth retardation ```

Clinical variants of Xanthoma disseminatum

1. Self-healing form 2. Persistent form 3. Progressive form

Associated with cutaneous and mucosal eruption with severe arthropathy and other visceral symptoms with papulonodular lesions (few mm to 2 cm) round, translucent and yellow-rose or yellow-brown grouping into plaques with cobblestone appearance that do not ulcerate preferential to fingers, palms and back of fingers arranged on the nail folds (coral beads)

Multicentric reticulohistiocytosis

MRH that is characterized by a single firm rapidly growing nodule varying in color from yellow-brown to dark red most commonly on the head which may be preceded by trauma

Solitary cutaneous reticulohistiocytosis

MRH that is purely cutaneous with eruption of form, smooth asymptomatic pinkish yellowish (early) to red-brown (older) papulonodular lesions 3-10 mm in diameter scattered diffusely

Diffuse cutaneous reticulohistiocytosis

Initial sign of Multicentric reticulohistiocytosis

Severe chronic diffuse polyarthritis with arthralgia

MRH characterized by familial occurrence and typical ocular involvement (glaucoma, cataract, uveitis)

Familial histiocytic dermatoarthritis

Histopathology: Composed of histiocytes and lymphocytes then older lesions showing presence of numerous large mononucleated histiocytes with abundance of eosinophilic homogenous cytoplasm containing fine granules that has a ground-glass appearance

Multicentric reticulohistiocytosis

Prognosis: Multicentric reticulohistiocytosis

Favorable for purely cutaneous forms (involute spontaneously)

Associated with myeloma, arthropathy, hypertension, neuropathy, neoplastic syndrome, primary biliary cirrhosis and Graves' disease

Necrobiotic xanthogranuloma

Histopathology: Granulomatous infiltrate involving the whole dermis and subcutis composed of mixture of lymphocytes epithelioid cells, foam cells, and Touton giant cells with areas of severe clearly defined necrosis

Necrobiotic xanthogranuloma

Complications: Necrobiotic xanthogranuloma

``` Lagophthalmos Conjunctivitis Keratitis Scleritis Uveitis Corneal ulceration Loss of ocular function ```

Prognosis: Necrobiotic xanthogranuloma

Chronic progressive course difficult to predict

Presents with yellowish macules and patches reddish-borwn papules and plaques and nodules that may become ulcerated or eroded accompanied by fever

Sinus histiocytosis with massive lymphadenopathy

Histopathology: Diffuse dermal infiltrate composed predominantly of histiocytes with large vesicular nuclei and abundant pale cytoplasm with emperipolesis (phagocytosis of leukocytes, lymphocytes)

Sinus histiocytosis with massive lymphadenopathy

Prognosis: Sinus histiocytosis with massive lymphadenopathy

Benign course with spontaneous regression

NLCH which require treatment

Xanthoma disseminatum Erdheim-Chester disease Multicentric reticulohistiocytosis Necrobiotic xanthogranuloma

Group of disorders characterized by an abnormal accumulation of mast cells

Mastocytosis

Type III tyrosine kinase receptor product of proto-oncogene located on chromosome 4q12 expressed on mast cells and others

KIT (CD117)

Cytokine that shares a number of signal transduction pathways wth SCF but minimal direct effect in human mast cell proliferation

IL-3

Cytokine that enhances mast cell function when added to matures cultures

IL-4

Cytokine that has been shown to increase mast cell mediator concentration

IL-6

Cytokine that increase the number of mast cell in culture

IL-9

Cytokine that inhibits mast cell proliferation and influcence mast cell phenotype and function

IFN-g

Criteria: Systemic Mastocytosis

Major -Multifocal dense infiltrates of mast cells in BM and/or other extracutaneous organs Major -More than 25% of the mast cells in BM aspirate smears or tissue biopsy sections are spindle shaped or display atypical morphology -Detection of a codon 816 c-kit point mutation in blood, BM or lesional tissue -Mast cell in BM, blood or other lesional tissue expressing CD25 or CD2 -Baseline total tryptase level greater than 20 ng/ml

Mastocytosis unique to adults

Systemic mastocytosis with an associated clonal hematologic nonmast cell lineage disease

Hematologic disorders associated with SM-AHNMD

Myeloproliferative disorders | Myelodysplastic disorders

Patients with this type of Mastocytosis have impaired liver function, hypersplenism and malabsorption, have increasing mast cell numbers and are difficult to manage clinically

Aggressive systemic mastocytosis

Mastocytosis that is characterized by multiorgan failure and BM smears mast cells represent greater than 20% of the nucleated cell population

Mast cell leukemia

Mastocytosis that is rare, with locally destructive growth and with distant spread with mast cells that are highly atypical and immature

Mast cell sarcoma

Most common skin manifestation of Cutaneous mastocytosis

Urticaria pigmentosa

Appear as tan to brown papules or macules, from 1.0-2.5 that present at birth or arise during infancy on trunk sparing central facem scalp, palms and soles

Urticaria pigmentosa (children)

Appear as reddish-brown macules and papules with variable hyperpigmentation and fine telangiectasias on trunk and proximal extremities

Urticaria pigmentosa (adults)

Tan-brown nodules that occur in 10-35% of children on distal extremities with onset before 6 months of age with associated flushing and hypotension if with trauma

Mastocytoma

Scratching or rubbing lesions of Cutaneous mastocytoma leading to urtication and erythema

Darier's sign

Mastocytosis that is seen almost exclusively in infants although it may persist into adult life

Diffuse cutaneous mastocytoma

Rare and seen almost exclusively in adults appearing as telangeictatic macules with irregular borders

Telangiectasis macularis eruptiva perstans

Mast cell mediators

``` Preformed -Histamine -Heparin -Tryptase -Chymase Newly synthesized -Leukotrienes -Prostagladins Cytokines -Stem cell factor -TNF-a -TGF-b -IL-5 -IL-6 -GM-CSF ```

T or F: Symptoms of mastocytosis can be exacerbated by exercise, heat or local trauma`

True

Stains for detecting mast cells

Toluidine Giemsa CD117 (KIT)

Elevated in patient with SM

a-tryptase

Can be detected in both mastocytosis and anaphylactic reactions

b-tryptase

Total (a and b) serum typtase levels

>20 ng/ml - abnormal 20-75 ng/ml - evidence of SM >75 ng/ml - systemic involvement

Major urinary metabolite of histamine

1,4-methylimidazole acetic acid

Next most common urinary metabolite

Methyhistamine

Treatment: Cutaneous mastocytosis (first line)

Emollients | H1 +/- H2 antihistamines

Treatment: Cutaneous mastocytosis (second line)

``` Topical glucocorticoids Calcineurin inhibitors Psoralen + UVA PDL (TMEP) Leukotriene antagonists Oral cromolyn ```

Treatment: Cutaneous mastocytosis (third line)

Intralesional corticosteroids Surgical excision (mastocytoma) Glucocorticoids

Treatment: Noncutaneous mastocytosis - Gastrointestinal

``` 1st H2 antihistamines Cromolyn 2nd PPI Leukotriene antagonist Anticholinergics 3rd Glucocorticoids ```

Treatment: Noncutaneous mastocytosis - Cardiovascular

``` 1st H1 and H2 antihistamines Subcutaneous epinephrine (anaphylaxis) 2nd Glucocorticoids (prophylaxis) ```

Treatment: Noncutaneous mastocytosis - Musculoskeletal

``` 1st Calcium supplements Vitamin D 2nd Bisphosphonates NSAIDs (with caution) 3rd Local radiation ```

Treatment: Noncutaneous mastocytosis - Hematologic

Systemic chemotherapy (appropriate)

Characterized by vascular contraction (leading to slowing of blood flow), platelet adhesion, and activation and subsequent development of plug at site of injury

Primary hemostasis

Promotes vascular integrity when primary hemostasis is not sufficient and when larger vessels are involved.

Secondary hemostasis

Maintains by vasoconstriction in secondary hemostatis

Prostaglandins Serotonin Thromboxane

Sec 25 Skin Manifestations of Bone Marrow or Blood Chemistry Disorders Flashcards

(181 cards)