1 Non‐infectious granulomatous dermatoses Adriano Piris, MD • Co‐director, Mihm Cutaneous Pathology Consultative Service Co director, Mihm Cutaneous Pathology Consultative Service Department of Dermatology, Brigham & Women’s Hospital • Stewart Rahr‐MRA Young Investigator Melanoma Research Alliance, Washington, D.C. • Consultant Staff Department of Pathology, Dana Farber Cancer Institute Non Non-infectious granulomas infectious granulomas Sarcoidosis Sarcoidosis Granuloma annulare Granuloma annulare Rheumatoid nodule Rheumatoid nodule Palisaded neutrophilic Palisaded neutrophilic and granulomatous and granulomatous Necrobiosis lipoidica Necrobiosis lipoidica Elastolytic granuloma Elastolytic granuloma Necrobiotic Necrobiotic xanthogranuloma xanthogranuloma dermatitis dermatitis Crohn’s disease Crohn’s disease Sarcoidosis Sarcoidosis • Systemic disease of unknown etiology • Non-caseating granulomas Non caseating granulomas Third and fourth decades Third and fourth decades Female predominance Female predominance African americans African americans Cutaneous lesions in sarcoidosis Cutaneous lesions in sarcoidosis • Papules, plaques and nodules Papules, plaques and nodules • Lupus pernio Lupus pernio • Verrucous Verrucous • Hypopigmented Hypopigmented Al i Al i • Subcutaneous nodules Subcutaneous nodules • Lichenoid Lichenoid • Erythrodermic Erythrodermic • Vasculitic (rare variant) Vasculitic (rare variant) • Alopecic Alopecic • Morpheaphorm Morpheaphorm • Ichthyosiform Ichthyosiform • Atrophic Atrophic • Ulcerative Ulcerative Courtesy of Dr Linda Wang Courtesy of Dr Linda Wang Courtesy of Dr Linda Wang Courtesy of Dr Linda Wang
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Non infectious granulomatous dermatosespathology.massgeneral.org/HMS/Poznan/pdf/Piris Granulomatous...Non‐infectious granulomatous dermatoses ... Group of disorders in which the
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• Presence of foreign bodies does not rule out the diagnosis
Differential diagnosis
Infectious granulomata
(use special stains!)
Foreign body granulomata
Crohn’s disease
(it may be the first manifestation)
Interstitial and Palisading Granulomatous Dermatitis
Group of disorders in which the unifying histiologic feature is histiocytic infiltrationhistiologic feature is histiocytic infiltration with variable alteration of the connective tissue framework
NecrobiosisNecrobiosis
Areas of altered dermal connectiveAreas of altered dermal connectivetissue with loss of definition of tissue with loss of definition of collagen bundles and alteration in collagen bundles and alteration in st i i (H&E)st i i (H&E)staining (H&E)staining (H&E)
Degeneration and loss of collagen/elasticDegeneration and loss of collagen/elasticfibers and connective tissue cellsfibers and connective tissue cells
Replacement by mucin, fibrin, lipids, sclerosisReplacement by mucin, fibrin, lipids, sclerosis
Interstitial and Palisading Granulomatous Dermatitis
• Granuloma annulare
• Elastolytic granuloma
• Necrobiosis lipoidica
• Rh t id d l• Rheumatoid nodule
• Necrobiotic xanthogranuloma
• Palisaded neutrophilic and granulomatous dermatitis
Courtesy of Dr Linda WangCourtesy of Dr Linda Wang
Patient BH
A 74 year old woman with arterial hypertension on an ACE inhibitor developed a plaque on the ankle
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Necrobiosis lipoidica
• Entire dermis usually involved by zones or layers
• Layers include fibrosis alternating with areas of altered collagen, with scattered lymphocytes and histiocytes, and cellular debris, especially around altered collagen
• Multinucleate giant cells and foamy macrophages scattered throughout lesion
• 20% show necrobiosis of the subcutaneous septa, a form of panniculitis
• Foci of mucin not present
• NL diffuse, involving entire dermis; GA focal or multifocal
Palisaded neutrophilic and granulomatous dermatitis Palisaded neutrophilic and granulomatous dermatitis associated with systemic diseasesassociated with systemic diseases