Monocyte/Macrophage Disorders

1. Monocyte/Macrophage Disorders Northeast Regional Medical Center/KCOM

2. Granuloma Annulare • Localized • Generalized • Macular • Deep • Perforating • In HIV • In Lymphoma

3. 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

4. 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.

5. 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

6. Upper dermis “Skip areas” Mucin Deep dermis, subQ No “skip” areas No mucin Interstitial GA NLD

13. Localized GA • Young adults • Acral • Annular, scalloped • White or pink flat topped papules spread peripherally • 75% clear in 2 yrs • 25% last 8 yrs

19. 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.

23. 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.

25. 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.

27. 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

28. GA and Lymphoma • Rare • Atypical presentation: • Facial or Palmar • Painful • Any type of lymphoma can occur. • Lymphoma may occur before or after the GA.

29. 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.

30. Annular Elastolytic Giant Cell Granuloma of Meischer/Actinic Granuloma of O’Brien

31. 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

32. Histo: Like GA, but with Giant Cells, Elastophagocytosis

33. Histo: Like GA, but with Giant Cells, Elastophagocytosis

34. Histo: Like GA, but with Giant Cells, Elastophago-cytosis

35. Photoexacerbated GA

36. 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.

37. Granuloma Mulitforme of Leiker

38. 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.

39. Sarcoidosis • Etiology unknown • HLA-A1 – Lofgren’s syndrome • HLA-B13 – Chronic & Persistent form • HLA-B8 • HLA-DR3 • Final common pathway is granuloma formation

40. NON-CASEATING GRANULOMAS COMPOSED OF EPITHELIOID CELLS AND OCCASIONAL LANGERHAN’S GIANT CELLS

41. “NAKED” GRANULOMAS “NAKED” meanse a sparse rather than a dense infiltrate. Lymphocytes, macrophages & fibroblasts may occur

43. Asteroid Body inside a multinucleated giant cell

44. SCHAUMANN OR CONCHOIDAL BODIES ARE COMPOSED OF CALCIUM CARBONATE. THEY ARE EASILY MISSED (LEFT) IF NOT VIEWED UNDER POLARIZED LIGHT (RIGHT)

45. Sarcoidosis AKA…. • Besnier-Boeck-Schaumann Disease • Boeck’s sarcoid • Besnier’s lupus pernio • Schaumann’s benign lymphogranulomatosis

46. 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.

47. Sarcoid – like syphillis, mimics many other dz’s • Papules, nodules, plaques. • Subcutaneous nodules. • Scar sarcoid, erythroderma. • Ulcerations, verrucous. • Ichthyosiform, hypomelanotic

48. Papular Sarcoid • MC form • AKA Miliary Sarcoid • Face, eyelids, neck, shoulders • May involute to macules • Ddx: syringomas

49. Papular Sarcoid

50. Papular Sarcoid