Monocyte/Macrophage Disorders Northeast Regional Medical Center/KCOMSlide 2
Granuloma Annulare Localized Generalized Macular Deep Perforating In HIV In LymphomaSlide 3
Granuloma Annulare Common, Idiopathic, all races half patients IgM and C3 in vessels LCV changes in some cases seen Suggests Ab interceded vasculitis Common in HIV patients EBV at times discovered Occurs in determined injuries ZosterSlide 4
GA - Histology Classic – histiocytes palisading around "necrobiotic" collagen. Granulomas situated in the upper dermis with perivascular lymphocytic penetrate Necrobiosis – "adjusted" collagen, paler grayer tint, divided, aimlessly masterminded, more minimized. Mucin unmistakable in more seasoned injuries.Slide 5
GA-Histology Interstitial – diffuse dermal invade between collagen groups comprising of histiocytes, monocytes, neutrophils. "Skip" territories of typical dermis seen. Interstitial mucin frequently seen. May be adjoining great granulomasSlide 6
Upper dermis "Skip regions" Mucin Deep dermis, subQ No "skip" territories No mucin Interstitial GA NLDSlide 13
Localized GA Young grown-ups Acral Annular, scalloped White or pink level topped papules spread incidentally 75% clear in 2 yrs 25% last 8 yrsSlide 19
Diffuse GA MC ladies past middle age Diabetes reported in 20% cases MC neck, upper trunk, shoulders MC type of GA found in HIV. Clears suddenly in 3-4 years. Hard to treat.Slide 23
Subcutaneous GA Aka Deep, Pseudorheumatoid Nodule MC kids, young men > young ladies 2:1 MC ages 5-12. Acral dissemination History of injury going before sore Asymptomatic however regularly a broad workup is done to preclude JRA.Slide 25
Perforating GA MC dorsum of hands Papules with focal keratotic center Core speaks to transdermal end of deteriorated or "necrobiotic" material in focus of palisaded histiocytes.Slide 27
GA in HIV sickness GA may happen at all periods of HIV malady. Regularly papular injuries 60% Diffuse, 40% Localized Photodistributed and puncturing sores may happenSlide 28
GA and Lymphoma Rare Atypical presentation: Facial or Palmar Painful Any kind of lymphoma can happen. Lymphoma may happen before or after the GA.Slide 29
GA-Treatment Biopsy, IL, Cryo, topical Vit. E, Excision GENERALIZED: Problematic Oral steroids, high dosage yet high backslide rate – diabetes confuses Dapsone, Nicotinomide, SSKI, Cyclosporine, Accutane.Slide 30
Annular Elastolytic Giant Cell Granuloma of Meischer/Actinic Granuloma of O\'BrienSlide 31
Annular Elastolytic Giant Cell Granuloma of Meischer/Actinic Granuloma of O\'Brien Variants of GA. AEGCG – lone atrophic flimsy yellow plaque on the temple, NLD-like. AGOB – Photo-appropriation, papules and plaquesSlide 32
Histo: Like GA, however with Giant Cells, ElastophagocytosisSlide 33
Histo: Like GA, yet with Giant Cells, ElastophagocytosisSlide 34
Histo: Like GA, yet with Giant Cells, Elastophago-cytosisSlide 35
Photoexacerbated GASlide 36
Granuloma Mulitforme of Leiker Similar histology to AEGCG & AGOB Only Central Africa, Adults > 40 yrs old. Upper Trunk and Arms Begin as little papules, venture into round or oval plaques 15cm wide and as much as 4mm in tallness. Must preclude tuberculoid sickness.Slide 37
Granuloma Mulitforme of LeikerSlide 38
Sarcoidosis Multisystem Disease Lungs, lymph hubs, skin and eyes MC. 10x more successive in blacks in US Women under age 40 Irish, African, Afro-Caribbean. Nearness conversely corresponding to the occurrence of TB and/or Leprosy.Slide 39
Sarcoidosis Etiology obscure HLA-A1 – Lofgren\'s disorder HLA-B13 – Chronic & Persistent structure HLA-B8 HLA-DR3 Final regular pathway is granuloma developmentSlide 40
NON-CASEATING GRANULOMAS COMPOSED OF EPITHELIOID CELLS AND OCCASIONAL LANGERHAN\'S GIANT CELLSSlide 41
"Exposed" GRANULOMAS "Stripped" meanse a scanty as opposed to a thick penetrate. Lymphocytes, macrophages & fibroblasts may happenSlide 43
Asteroid Body inside a multinucleated mammoth cellSlide 44
SCHAUMANN OR CONCHOIDAL BODIES ARE COMPOSED OF CALCIUM CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)Slide 45
Sarcoidosis AKA… . Besnier-Boeck-Schaumann Disease Boeck\'s sarcoid Besnier\'s lupus pernio Schaumann\'s generous lymphogranulomatosisSlide 46
Sarcoid Skin Involvement Anywhere from 9% to 37% of cases. 2 sorts: particular and non-particular Specific: granulomas on biopsy Non-Specific: receptive, Erythema Nodosum Skin discoveries may happen some time recently, amid or after systemic discoveries.Slide 47
Sarcoid – like syphillis, mirrors numerous other dz\'s Papules, knobs, plaques. Subcutaneous knobs. Scar sarcoid, erythroderma. Ulcerations, verrucous. Ichthyosiform, hypomelanoticSlide 48
Papular Sarcoid MC structure AKA Miliary Sarcoid Face, eyelids, neck, shoulders May involute to macules Ddx: syringomasSlide 49
Papular SarcoidSlide 50
Papular SarcoidSlide 51
Papular SarcoidSlide 52
Papular SarcoidSlide 53
Annular Sarcoidosis Central clearing Hypo-shade ation Atrophy Scarring Favor head & neck Assoc. with incessant sarcoidosisSlide 54
Annular SarcoidosisSlide 55
Hypopigmented Sarcoid May be the most punctual indication of sarcoidosis in blacks. MC limits Visually macular, however regularly have a discernable dermal or subQ part in focal point of soreSlide 56
Hypopigmented SarcoidSlide 57
Lupus Pernio Violaceous Nose, cheeks, lips Forehead, ears 43% connected with punched out bone sores. 37% Ocular sores Nasal puncturingSlide 58
Punched-Out Lytic injuries, Bone CystsSlide 59
Ulcerative SarcoidosisSlide 60
Lupus PernioSlide 61
Lupus PernioSlide 62
Lupus PernioSlide 63
Lupus PernioSlide 64
Darier-Roussy Sarcoid 5% or less of patients with sarcoidosis have subcutaneous knobs.Slide 65
Darier-Roussy (SubQ)Slide 66
Scar SarcoidSlide 67
Scar SarcoidSlide 68
Erythrodermic Sarcoid Extremely Rare Begins as erythematous patches that get to be intersecting.Slide 69
Ichthyosiform Sarcoid Legs Arms No discernable partSlide 70
Ichthyosiform SarcoidSlide 71
Alopecia Occurs in 2 settings; 1) Existing plaques broaden onto scalp. - prompts changeless scarring. 2) Macular injuries show up on scalp looking like Alopecia Areata - might be lasting or reversibleSlide 72
Morpheaform Sarcoid Rare Dermal Fibrosis Simulates Morphea Antimalarials may offer assistance.Slide 73
Morpheaform SarcoidSlide 74
Morpheaform SarcoidSlide 75
Mucosal Sarcoid Pinhead estimated papules Grouped or melded to shape a plaque.Slide 76
Erythema Nodosum in Sarcoid MC nonspecific cutaneous finding in sarcoidosis Young females Anterior shins Good guess Lofgren\'s Syndrome = fever, arthralgias, hilar adenopathy, weakness, ENSlide 77
Systemic Sarcoidosis MC – Lungs Ocular 20-30% Bones & Liver 20%, hoisted Alk Phos. Renal, Hypercalcemia Heart, CNS, Spleen Elevated ACE levels to take after ailment action as it were.Slide 78
Heerfort\'s Syndrome Parotid organ development Lacrimal organ growth Uveitis Fever SarcoidosisSlide 79
Mikulicz\'s Syndrome Sarcoidosis with augmentation of the; Lacrimal organs Submaxillary and Parotid organs. Dangerous: various conditions including augmented partoid organs have since been named after Dr. Mikulicz.Slide 80
CXR-Hilar AdenopathySlide 81
Sarcoidosis in FingersSlide 82
Sarcoidosis in FingersSlide 83
Candle-wax drippings – granulomatous uveitisSlide 85
Sarcoid - Treatment Systemic Corticosteroids Antimalarials Methotrexate ThalidomideSlide 86
Non-X Histocytoses 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 HistiocytosisSlide 87
Juvenile Xanthogranuloma (JXG) MC Non-Langerhans\' histiocytosis 1 st year of life, usu. white guys 80% are singular, all around divided, firm, rubbery red to pink with yellow tinge Regress in 3-6 years with decay. Visual association uncommon, MC iris Assoc. with NF-1 and JCMLSlide 89
JXG Histopathology Non-embodied Infiltrate in the upper and mid reticular dermis Mononuclear cells with bottomless amphophilic cytoplasm that is inadequately lipidized or vacuolated.Slide 90
MULTINUCLEATED "Froth" CELLS otherwise known as TOUTON GIANT CELLS ALONG WITH EOS, NEUTS, LYMPHS. STAINS: + CD1 + FACTOR XIIIa - S100Slide 91
Benign Cephalic Histiocytosis Rare Males 2:1, Onset 6-12 months of age Begins on head, cheeks, spreads to neck and upper trunk Multiple rosy yellow papules 2-3mm, may blend into a reticulate example. Involute more than 2 to 8 years with decaySlide 92
BENIGN CEPHALIC HISTIOCYTOSIS DIFFUSE DERMAL INFILTRATION OF NON-LIPIDIZED HISTIOCYTIC CELLS, S-100 NEGATIVESlide 93
Reticulohistiocytosis Solitary structure – otherwise known as Reticulohistiocytic Granuloma or Reticulohistiocytoma Solitary structure has no systemic inclusion Multicentric structure – otherwise known as Multicentric Reticulohistiocytosis Underlying threat in 30%Slide 94
Reticulohistiocytic GranulomaSlide 95
Reticulohistiocytic Granuloma: Multinucleate Giant Cells, Histiocytes, Lymphocytes with some stroma fibrosisSlide 96
Multicentric Reticulohistiocytosis Multisystem illness, 5 th d
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