monocyte/macrophage disorders northeast regional medical center/kcom
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Monocyte/Macrophage Disorders
Northeast Regional Medical Center/KCOM
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Granuloma Annulare
Localized
Generalized
Macular
Deep
Perforating
In HIV
In Lymphoma
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Granuloma Annulare
Common, Idiopathic, all races
50% patients IgM and C3 in vessels
LCV changes sometimes seen
Suggests Ab mediated vasculitis
Common in HIV patients
EBV sometimes found
Occurs in resolved lesions Zoster
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GA - Histology
Classic – histiocytes palisading around “necrobiotic” collagen. Granulomas located in the upper dermis with perivascular lymphocytic infiltrateNecrobiosis – “altered” collagen, paler grayer hue, fragmented, haphazardly arranged, more compact.Mucin prominent in older lesions.
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GA- Histology
Interstitial – diffuse dermal infiltrate between collagen bundles consisting of histiocytes, monocytes, neutrophils.
“Skip” areas of normal dermis seen.
Interstitial mucin often seen.
May be adjacent to classic granulomas
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Interstitial GA
Upper dermis
“Skip areas”
Mucin
Deep dermis, subQ
No “skip” areas
No mucin
NLD
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Localized GA
Young adults
Acral
Annular, scalloped
White or pink flat topped papules spread peripherally
75% clear in 2 yrs
25% last 8 yrs
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Diffuse GA
MC women past middle age
Diabetes reported in 20% cases
MC neck, upper trunk, shoulders
MC form of GA seen in HIV.
Clears spontaneously in 3-4 years.
Difficult to treat.
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Subcutaneous GA
Aka Deep, Pseudorheumatoid Nodule
MC children, boys > girls 2:1
MC ages 5-12.
Acral distribution
History of trauma preceding lesion
Asymptomatic but often an extensive workup is done to rule out JRA.
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Perforating GA
MC dorsum of hands
Papules with central keratotic core
Core represents transdermal elimination of degenerated or “necrobiotic” material in center of palisaded histiocytes.
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GA in HIV disease
GA may occur at all phases of HIV disease.
Typically papular lesions
60% Diffuse, 40% Localized
Photodistributed and perforating lesions may occur
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GA and Lymphoma
Rare
Atypical presentation:
Facial or Palmar
Painful
Any type of lymphoma can occur.
Lymphoma may occur before or after the GA.
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GA- Treatment
Biopsy, IL, Cryo, topical Vit. E, Excision
GENERALIZED: Problematic
Oral steroids, high dose but high relapse rate – diabetes complicates
Dapsone, Nicotinomide, SSKI, Cyclosporine, Accutane.
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Annular Elastolytic Giant Cell Granuloma of Meischer/Actinic
Granuloma of O’Brien
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Annular Elastolytic Giant Cell Granuloma of Meischer/Actinic
Granuloma of O’Brien
Variants of GA.
AEGCG – solitary atrophic thin yellow plaque on the forehead, NLD-like.
AGOB – Photo- distribution, papules and plaques
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Histo: Like GA, but with Giant Cells, Elastophagocytosis
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Histo: Like GA, but with Giant Cells, Elastophagocytosis
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Histo: Like GA, but with Giant
Cells, Elastophago-
cytosis
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Photoexacerbated GA
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Granuloma Mulitforme of Leiker
Similar histology to AEGCG & AGOB
Only Central Africa, Adults > 40 yrs old.
Upper Trunk and Arms
Begin as small papules, expand into round or oval plaques 15cm wide and as much as 4mm in height.
Must rule out tuberculoid leprosy.
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Granuloma Mulitforme of Leiker
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Sarcoidosis
Multisystem Disease
Lungs, lymph nodes, skin and eyes MC.
10x more frequent in blacks in US
Women under age 40
Irish, African, Afro-Caribbean.
Presence inversely proportional to the incidence of TB and/or Leprosy.
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Sarcoidosis
Etiology unknown
HLA-A1 – Lofgren’s syndrome
HLA-B13 – Chronic & Persistent form
HLA-B8
HLA-DR3
Final common pathway is granuloma formation
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NON-CASEATING GRANULOMAS COMPOSED OF EPITHELIOID CELLS AND OCCASIONAL LANGERHAN’S GIANT CELLS
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“NAKED” GRANULOMAS
“NAKED” meanse a sparse rather than a dense infiltrate. Lymphocytes, macrophages & fibroblasts may occur
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Asteroid Body inside a multinucleated giant cell
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SCHAUMANN OR CONCHOIDAL BODIES ARE COMPOSED OF CALCIUM CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)
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Sarcoidosis AKA….
Besnier-Boeck-Schaumann Disease
Boeck’s sarcoid
Besnier’s lupus pernio
Schaumann’s benign lymphogranulomatosis
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Sarcoid Skin Involvement
Anywhere from 9% to 37% of cases.
2 types: specific and non-specific
Specific: granulomas on biopsy
Non-Specific: reactive, Erythema Nodosum
Skin findings may occur before, during or after systemic findings.
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Sarcoid – like syphillis, mimics many other dz’sPapules, nodules, plaques.
Subcutaneous nodules.
Scar sarcoid, erythroderma.
Ulcerations, verrucous.
Ichthyosiform, hypomelanotic
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Papular Sarcoid
MC form
AKA Miliary Sarcoid
Face, eyelids, neck, shoulders
May involute to macules
Ddx: syringomas
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Papular Sarcoid
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Papular Sarcoid
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Papular Sarcoid
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Papular Sarcoid
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Annular SarcoidosisCentral clearing Hypo-pigment-ationAtrophyScarringFavor head & neckAssoc. with chronic sarcoidosis
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Annular Sarcoidosis
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Hypopigmented Sarcoid
May be the earliest sign of sarcoidosis in blacks.
MC extremities
Visually macular, but often have a palpable dermal or subQ component in center of lesion
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Hypopigmented Sarcoid
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Lupus Pernio
Violaceous
Nose, cheeks, lips
Forehead, ears
43% associated with punched out bone lesions.
37% Ocular lesions
Nasal perforation
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Punched-Out Lytic lesions, Bone Cysts
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Ulcerative Sarcoidosis
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Lupus Pernio
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Lupus Pernio
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Lupus Pernio
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Lupus Pernio
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Darier-Roussy Sarcoid5% or fewer of patients with sarcoidosis have subcutaneous nodules.
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Darier-Roussy (SubQ)
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Scar Sarcoid
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Scar Sarcoid
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Erythrodermic Sarcoid
Extremely Rare
Begins as erythematous patches that become confluent.
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Ichthyosiform Sarcoid
Legs
Arms
No palpable component
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Ichthyosiform Sarcoid
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Alopecia
Occurs in 2 settings;
1) Existing plaques extend onto scalp.
--leads to permanent scarring.
2) Macular lesions appear on scalp resembling Alopecia Areata
--may be permanent or reversible
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Morpheaform Sarcoid
Rare
Dermal Fibrosis
Simulates Morphea
Antimalarials may help.
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Morpheaform Sarcoid
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Morpheaform Sarcoid
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Mucosal Sarcoid
Pinhead sized papules
Grouped or fused together to form a plaque.
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Erythema Nodosum in Sarcoid
MC nonspecific cutaneous finding in sarcoidosis
Young females
Anterior shins
Good prognosis
Lofgren’s Syndrome = fever, arthralgias, hilar adenopathy, fatigue, EN
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Systemic Sarcoidosis
MC – Lungs
Ocular 20-30%
Bones & Liver 20%, elevated Alk Phos.
Renal, Hypercalcemia
Heart, CNS, Spleen
Elevated ACE levels to follow disease activity only.
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Heerfort’s Syndrome
Parotid gland enlargement
Lacrimal gland enlargement
Uveitis
Fever
Sarcoidosis
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Mikulicz’s Syndrome
Sarcoidosis with enlargement of the;
Lacrimal glands
Submaxillary and Parotid glands.
Problematic: numerous conditions involving enlarged partoid glands have since been named after Dr. Mikulicz.
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CXR- Hilar Adenopathy
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Sarcoidosis in Fingers
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Sarcoidosis in Fingers
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CNS
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Candle-wax drippings – granulomatous uveitis
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Sarcoid - Treatment
Systemic Corticosteroids
Antimalarials
Methotrexate
Thalidomide
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Non-X HistocytosesJuvenile Xanthogranuloma
Benign Cephalic Histiocytosis
Solitary/Multicentric Reticulohistiocytosis
Generalized Eruptive Histiocytoma
Necrobiotic Xanthogranuloma
Xanthoma Disseminatum
Papular Xanthoma
Indeterminate Cell Histiocytosis
Progressive Nodular Histiocytoma
Hereditary Progressive Mucinous Histiocytosis
Rosai-Dorfman Disease
Sea-Blue Histiocytosis
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Juvenile Xanthogranuloma (JXG)
MC Non-Langerhans’ histiocytosis
1st year of life, usu. white males
80% are solitary, well demarcated, firm, rubbery red to pink with yellow tinge
Regress in 3-6 years with atrophy.
Ocular involvement rare, MC iris
Assoc. with NF-1 and JCML
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JXG Histopathology
Non-encapsulatedInfiltrate in the upper and mid reticular dermisMononuclear cells with abundant amphophilic cytoplasm that is poorly lipidized or vacuolated.
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MULTINUCLEATED “FOAM” CELLS aka TOUTON GIANT CELLS ALONG WITH EOS, NEUTS, LYMPHS.
STAINS:
+ CD1
+ FACTOR
XIIIa
- S100
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Benign Cephalic Histiocytosis
Rare
Males 2:1, Onset 6-12 months of age
Begins on head, cheeks, spreads to neck and upper trunk
Multiple reddish yellow papules 2-3mm, may coalesce into a reticulate pattern.
Involute over 2 to 8 years with atrophy
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BENIGN CEPHALIC HISTIOCYTOSIS
DIFFUSE DERMAL INFILTRATION OF NON-LIPIDIZED HISTIOCYTIC CELLS, S-100 NEGATIVE
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Reticulohistiocytosis
Solitary form – aka Reticulohistiocytic Granuloma or Reticulohistiocytoma
Solitary form has no systemic involvement
Multicentric form – aka Multicentric Reticulohistiocytosis
Underlying malignancy in 30%
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Reticulohistiocytic Granuloma
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Reticulohistiocytic Granuloma: Multinucleate Giant Cells, Histiocytes, Lymphocytes with some stroma fibrosis
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Multicentric Reticulohistiocytosis
Multisystem disease, 5th decade, F>M.
90% Face & hands, red-brown papules and nodules
Paronychia: “coral bead” appearance
Joints symmetrically involved with mutilating arthritis, telescoping shortening of digits, doigts en lorgnette, opera-glass fingers, RF is negative
1/3 have high cholesterol, xanthelasma
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“Coral Bead” Paronychia
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Classic Ground Glass Touton Giant Cells, PAS +
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90% Face & Hands
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Tx: Multicentric Reticulohisticytosis
Treatment is problematic because mutilating arthritis requires immunosuppressive therapy.
Immunosuppressive therapy can worsen underlying malignancies
Prednisone, Antimalarials, MTX, Cytoxan, PUVA, Nitrogen mustard.
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Generalized Eruptive Histiocytoma
Widespread symmetric papules, trunk and proximal extremities, come in cropsProgressive development of new lesions over several years with eventual spontaneous involution to hyper-pigmented maculesFlesh, brown or violaceous papulesControversy: is this just xanthoma disseminatum? MRH? Indeterminate cell histiocytosis?
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Generalized Eruptive Histiocytoma
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GENERALIZED ERUPTIVE HISTIOCYTOMA:
DERMAL INFILTRATE OF NON-LIPIDIZED MONONUCLEAR HISTIOCYTES, S-100 IS NEGATIVE
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GENERALIZED ERUPTIVE HISTIOCYTOMA:
DERMAL INFILTRATE OF NON-LIPIDIZED MONONUCLEAR HISTIOCYTES, S-100 IS NEGATIVE
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Necrobiotic Xanthogranuloma (NXG)Multisystem disease of older adults
Characteristic periorbital yellow plaques that resemble xanthelasmas except that they are deep, firm, indurated and may extend into the orbit
Trunk & proximal extremity lesions are orange-red plaques with an active red border and an atrophic border with superficial telangiectiasias.
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NXG: Periorbital yellow plaques that resemble xanthelasmas except that they are deep, firm, indurated, may involve the orbit
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NXG: Trunk & proximal extremity lesions are orange-red plaques w/ active red border & an
atrophic border with superficial telangiectasias
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NXG: conjunctivitis, keratitis, scleritis, uveitis, iritis, ectropion or proptosis
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NXG: Process extends into the fat, obliterating fat lobules. Extensive zones of degenerated collagen or “necrobiosis” surrounded by palisaded macrophages.
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NXG: Foam Cells with abundant infiltrate of lymphocytes, plasma cells
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NXG: Cholesterol Clefts
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NXG and Malignancy
80% IgG monoclonal paraproteinemia (Kappa)
Bone marrow may show plasmacytosis, anemia, leukopenia, myeloma, myelodysplastic syndromes.
Cause unknown, course progressive
Treat aimed at paraproteinemia: Melaphan, Chlorambucil, Corticosteroids, Plasmapheresis, Alpha Interferon-2b
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Xanthoma Disseminatum
Serum lipids are normal, MC young malesMucocutaneous, discreet, disseminatedIntertriginous distributionDiabetes Insipidus 40% due to xanthomatous infiltration of the pituitary gland.Chronic and Benign, may persist, may involute spontaneously after some years
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XD - Periorbital
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XD - Axillary
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XD - Pathology
Xanthoma Cells
Eosinophilic Histiocytes
Numerous Touton giant cells
Inflammatory cell infiltrate usually present.
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Papular Xanthoma
Small yellowish papules
Localized or generalized
No tendency to merge into plaques
Aggregates of foam cells in the dermis without a cellular or histiocytic phase
Absence of inflammatory cells.
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Indeterminate Cell Histiocytosis
Dermal precursors of Langerhan’s cells
S-100 positive
CD1 positive
NO BIRBECK GRANULES!
Chronic without spontaneous involution
No systemic involvement
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Progressive Nodular Histiocytosis
Superficial papules & deeper nodules
Diffuse, symmetrical, non-flexural.
Larger lesions may ulcerate, become painful
Face lesions may coalesce into leonine facies
General health is good
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Progressive Nodular Histiocytosis
Histo: DF-like, few Toutons, lacks the PAS+ ground glass giant cells of MRH.
Stains positive for Vimentin, CD68, Factor XIIIa
Stains negative for S-100 and CD34
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Hereditary Progressive Mucinous Histiocytosis in
WomenAD or X-linkedFew to numerous flesh to red-brown papules up to 5mm in diameterFace, arms, forearms, hands, legsOnset 2nd decadeSlow progression, no tendency to spontaneous involution, no systemic involvement
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Hereditary Progressive Mucinous Histiocytosis in
Women
May histologically differentiate from other non-X histiocytoses as follows:
Familial pattern
Abundant mucin + Alcian blue staining
Lack of lipidized and multinucleated cells
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Rosai-Dorfman Disease
Aka Sinus Histiocytosis with Massive LymphadenopathyOnset 1st or 2nd decade of lifeFever, massive cervical LAD, polyclonal hyperglobulinemia, leukocytosis, anemia, elevated SED rate.Males and blacks MC.Skin involvement in 43% of casesMost patients with skin lesions are > age 40
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Rosai-Dorfman Disease
Isolated or disseminated yellow-brown papules or nodules, or macular erythema. Large annular lesions resembling GA may occur.HHV-6 identified in numerous reports.May clear spontaneouslySkin biopsy non-specific unless emperipolesis is present but lymph node pathology is characteristic…..
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Rosai-Dorfman Disease – LN Biopsy
Expansion of the sinuses by large foamy histiocytes admixed with plasma cellsCD4, Factor XIIIa and S-100 positiveNo Birbeck granules
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RDD - Emperipolesis – Histiocytes engulf plasma cells and lymphocytes
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RDD - Emperipolesis
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RDD - Treatment
Radiation
Chemotherapy
Systemic corticosteroids
Thalidomide
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Sea-Blue Histiocytosis
Familial or Acquired
Characteristic and diagnostic cell is a histiocyte containing cytoplasmic granules that stain as follows:
Blue-green with Geimsa
Blue with May-Gruenwald
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Sea-Blue Histiocytosis
Lesions include papules, eyelid swelling and patchy gray pigmentation of the face and upper trunk.
Infiltrates marrow, spleen, liver, lymph nodes, lungs and skin in some cases.
Similar findings seen in patients with Myelogenous leukemia and Neimann-Pick Disease, and following prolonged use of IV fat supplementation
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Sea-Blue Histiocytosis – Bone Marrow
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X-type Histiocytoses
Hashimoto-Pritzker aka Congenital Self-Healing
Reticulohistiocytois
Histiocytosis X Aka Letterer-SiweAka Hand-Schuller ChristianAka Eosinophilic Granuloma
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Hashimoto-Pritzker
Onset: birth or very soon thereafter
Solitary or multinodular
Red, brown, pink or dusky
Lesions > 1 cm characteristically ulcerate as they resolve
Asymptomatic, resolves in 8 to 24 weeks
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Hashimoto-Pritzker
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Hashimoto-Pritzker Before and After
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Hashimoto-Pritzker
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Hashimoto-Pritzker
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EM: 10-25% of cells have Langerhans’ cell granules, but this does not distinguish
Hashimoto-Pritzker from Histiocytosis X.
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H&E: large mononuclear cells & multinucleated giant cells with ground glass or foamy cytoplasm
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S-100 stain CD1a stain
HASHIMOTO-PRITZKER
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H-P MANAGEMENT
Must rule out Histiocytosis-X as both present similarly
Rule out systemic involvement with physical exam, CBC, LFT, Bone survey.
If any of the above are abnormal, consider liver-spleen scan and bone marrow biopsy.
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Histiocytosis X
Proliferation of Langerhans’ cellsMC-Bone, Skin, Lymph, Lungs, Liver and Spleen, Endocrine glands, CNS.Children age 1-4 years oldLymphs are clonal, but not as atypical appearing as lymphoma cells – debate as to whether this is neoplastic v. reactive
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Histiocytosis X
RESTRICTED TYPES:A) Biopsy proven skin rash without other
involvementB) Monostotic lesions, with or without
diabetes insipidus, LAD or rashC) Polyostotic lesions with or without
diabetes insipidus, LAD or rash.
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Histiocytosis X
EXTENSIVE TYPE:A) Visceral involvement with or without
bone lesions, diabetes insipidus, LAD or rash but WITHOUTsigns of organ dysfunction of lung, liver or hematopoetic system
B) Visceral involvement with or without bone lesions, diabetes insipidus, LAD or rash but WITH signs of organ dysfunction.
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Histiocytosis X Distribution
MC is Letterer-Siwe: Tiny red, red-brown or yellow papules that are widespread but favor the intertriginous areas, behind ears and scalp.Lesions may erode or weep.In children, LS distribution is assoc. with multisystem disease, but in adults 25% have disease limited to skin only.
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Histicytosis X - scalp
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Often mistaken for SD, but focal hemorrhage is present
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Often mistaken for SD, but focal hemorrhage is present
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Histiocytosis X - TX
Skin only: topical steroids, nitrogen mustard, PUVA, Interferon Alpha.extensive disease but without organ dysfunction: oral corticosteroids Extensive disease with orgain dysfunction: Vinblastine, Cyclosporine, Radiation. Refractory: 2-chlorodeoxyadenosine
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SLICK RICK SAYS: “DON’T FORGET TO TURN IN YOUR TEST QUESTIONS”
THE END