Monocyte macrophage disorders
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Monocyte/Macrophage Disorders. Northeast Regional Medical Center/KCOM. Granuloma Annulare. Localized Generalized Macular Deep Perforating In HIV In Lymphoma. Granuloma Annulare. Common, Idiopathic, all races 50% patients IgM and C3 in vessels LCV changes sometimes seen

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Monocyte macrophage disorders l.jpg

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 infiltrate

  • Necrobiosis – “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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Upper dermis

“Skip areas”

Mucin

Deep dermis, subQ

No “skip” areas

No mucin

Interstitial GA

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

  • Variants of GA.

  • AEGCG – solitary atrophic thin yellow plaque on the forehead, NLD-like.

  • AGOB – Photo- distribution, papules and plaques





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Photoexacerbated GA Granuloma of O’Brien


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Granuloma Mulitforme of Leiker Granuloma of O’Brien

  • 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 Granuloma of O’Brien


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Sarcoidosis Granuloma of O’Brien

  • 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 Granuloma of O’Brien

  • 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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“NAKED” GRANULOMAS OCCASIONAL LANGERHAN’S GIANT CELLS

“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 OCCASIONAL LANGERHAN’S GIANT CELLS


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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…. CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Besnier-Boeck-Schaumann Disease

  • Boeck’s sarcoid

  • Besnier’s lupus pernio

  • Schaumann’s benign lymphogranulomatosis


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Sarcoid Skin Involvement CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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’s CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Papules, nodules, plaques.

  • Subcutaneous nodules.

  • Scar sarcoid, erythroderma.

  • Ulcerations, verrucous.

  • Ichthyosiform, hypomelanotic


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Papular Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • MC form

  • AKA Miliary Sarcoid

  • Face, eyelids, neck, shoulders

  • May involute to macules

  • Ddx: syringomas


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Papular Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Papular Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Papular Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Papular Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Annular Sarcoidosis CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Central clearing

  • Hypo-pigment-ation

  • Atrophy

  • Scarring

  • Favor head & neck

  • Assoc. with chronic sarcoidosis


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Annular Sarcoidosis CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Hypopigmented Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Lupus CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Ulcerative Sarcoidosis CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Lupus Pernio CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Lupus Pernio CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Lupus Pernio CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Lupus Pernio CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Darier-Roussy Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 5% or fewer of patients with sarcoidosis have subcutaneous nodules.


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Darier-Roussy (SubQ) CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Scar Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Scar Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Erythrodermic Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Extremely Rare

  • Begins as erythematous patches that become confluent.


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Ichthyosiform Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Legs

  • Arms

  • No palpable component


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Ichthyosiform Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Alopecia CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Rare

  • Dermal Fibrosis

  • Simulates Morphea

  • Antimalarials may help.


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Morpheaform Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Morpheaform Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Mucosal Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Pinhead sized papules

  • Grouped or fused together to form a plaque.


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Erythema Nodosum in Sarcoid CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Parotid gland enlargement

  • Lacrimal gland enlargement

  • Uveitis

  • Fever

  • Sarcoidosis


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Mikulicz’s Syndrome CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Sarcoidosis in Fingers CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Sarcoidosis in Fingers CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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CNS CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Candle-wax drippings – granulomatous uveitis CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)


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Sarcoid - Treatment CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Systemic Corticosteroids

  • Antimalarials

  • Methotrexate

  • Thalidomide


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Non-X Histocytoses CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Juvenile 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) CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • 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 CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

  • Non-encapsulated

  • Infiltrate in the upper and mid reticular dermis

  • Mononuclear 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 WITH EOS, NEUTS, LYMPHS.

  • 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 WITH EOS, NEUTS, LYMPHS.

DIFFUSE DERMAL INFILTRATION OF NON-LIPIDIZED HISTIOCYTIC CELLS, S-100 NEGATIVE


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Reticulohistiocytosis WITH EOS, NEUTS, LYMPHS.

  • 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 WITH EOS, NEUTS, LYMPHS.


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Reticulohistiocytic Granuloma: Multinucleate Giant Cells, Histiocytes, Lymphocytes with some stroma fibrosis


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Multicentric Reticulohistiocytosis Histiocytes, Lymphocytes with some stroma fibrosis

  • 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 Histiocytes, Lymphocytes with some stroma fibrosis


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Classic Ground Glass Touton Giant Cells, PAS + Histiocytes, Lymphocytes with some stroma fibrosis


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90% Face & Hands Histiocytes, Lymphocytes with some stroma fibrosis


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Tx: Multicentric Reticulohisticytosis Histiocytes, Lymphocytes with some stroma fibrosis

  • 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 Histiocytes, Lymphocytes with some stroma fibrosis

  • Widespread symmetric papules, trunk and proximal extremities, come in crops

  • Progressive development of new lesions over several years with eventual spontaneous involution to hyper-pigmented macules

  • Flesh, brown or violaceous papules

  • Controversy: is this just xanthoma disseminatum? MRH? Indeterminate cell histiocytosis?


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Generalized Eruptive Histiocytoma Histiocytes, Lymphocytes with some stroma fibrosis


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GENERALIZED ERUPTIVE HISTIOCYTOMA: Histiocytes, Lymphocytes with some stroma fibrosis

DERMAL INFILTRATE OF NON-LIPIDIZED MONONUCLEAR HISTIOCYTES, S-100 IS NEGATIVE


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GENERALIZED ERUPTIVE HISTIOCYTOMA: Histiocytes, Lymphocytes with some stroma fibrosis

DERMAL INFILTRATE OF NON-LIPIDIZED MONONUCLEAR HISTIOCYTES, S-100 IS NEGATIVE


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Necrobiotic Xanthogranuloma (NXG) Histiocytes, Lymphocytes with some stroma fibrosis

  • 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: Process extends into the fat, obliterating fat lobules. Extensive zones of degenerated collagen or “necrobiosis” surrounded by palisaded macrophages.




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NXG and Malignancy plasma cells

  • 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 plasma cells

  • Serum lipids are normal, MC young males

  • Mucocutaneous, discreet, disseminated

  • Intertriginous distribution

  • Diabetes 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 plasma cells


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XD - Axillary plasma cells


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XD - Pathology plasma cells

  • Xanthoma Cells

  • Eosinophilic Histiocytes

  • Numerous Touton giant cells

  • Inflammatory cell infiltrate usually present.


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Papular Xanthoma plasma cells

  • 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 plasma cells

  • 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 plasma cells

  • 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 plasma cells

  • 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 Women plasma cells

  • AD or X-linked

  • Few to numerous flesh to red-brown papules up to 5mm in diameter

  • Face, arms, forearms, hands, legs

  • Onset 2nd decade

  • Slow progression, no tendency to spontaneous involution, no systemic involvement


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Hereditary Progressive Mucinous Histiocytosis in Women plasma cells

  • 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 plasma cells

  • Aka Sinus Histiocytosis with Massive Lymphadenopathy

  • Onset 1st or 2nd decade of life

  • Fever, massive cervical LAD, polyclonal hyperglobulinemia, leukocytosis, anemia, elevated SED rate.

  • Males and blacks MC.

  • Skin involvement in 43% of cases

  • Most patients with skin lesions are > age 40


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Rosai-Dorfman Disease plasma cells

  • 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 spontaneously

  • Skin biopsy non-specific unless emperipolesis is present but lymph node pathology is characteristic…..


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Rosai-Dorfman Disease – LN Biopsy plasma cells

  • Expansion of the sinuses by large foamy histiocytes admixed with plasma cells

  • CD4, Factor XIIIa and S-100 positive

  • No Birbeck granules



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RDD - Emperipolesis lymphocytes


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RDD - Treatment lymphocytes

  • Radiation

  • Chemotherapy

  • Systemic corticosteroids

  • Thalidomide


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Sea-Blue Histiocytosis lymphocytes

  • 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 lymphocytes

  • 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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X-type Histiocytoses lymphocytes

  • Hashimoto-Pritzker

    • aka Congenital Self-Healing Reticulohistiocytois

  • Histiocytosis X

    • Aka Letterer-Siwe

    • Aka Hand-Schuller Christian

    • Aka Eosinophilic Granuloma


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Hashimoto-Pritzker lymphocytes

  • 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 lymphocytes



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Hashimoto-Pritzker lymphocytes


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Hashimoto-Pritzker lymphocytes


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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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HASHIMOTO-PRITZKER with ground glass or foamy cytoplasm

S-100 stain

CD1a stain


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H-P MANAGEMENT with ground glass or foamy cytoplasm

  • 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 with ground glass or foamy cytoplasm

  • Proliferation of Langerhans’ cells

  • MC-Bone, Skin, Lymph, Lungs, Liver and Spleen, Endocrine glands, CNS.

  • Children age 1-4 years old

  • Lymphs 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 with ground glass or foamy cytoplasm

  • RESTRICTED TYPES:

    • A) Biopsy proven skin rash without other involvement

    • B) Monostotic lesions, with or without diabetes insipidus, LAD or rash

    • C) Polyostotic lesions with or without diabetes insipidus, LAD or rash.


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Histiocytosis X with ground glass or foamy cytoplasm

  • 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 with ground glass or foamy cytoplasm

  • 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 with ground glass or foamy cytoplasm


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Often mistaken for SD, but focal hemorrhage is present with ground glass or foamy cytoplasm


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Often mistaken for SD, but focal hemorrhage is present with ground glass or foamy cytoplasm


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Histiocytosis X - TX with ground glass or foamy cytoplasm

  • 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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THE END with ground glass or foamy cytoplasm

SLICK RICK SAYS: “DON’T FORGET TO TURN IN YOUR TEST QUESTIONS”


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