sarcoidosis

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cutaneous sarcoidosis by Dr. Deepak Kumar

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Presentation Transcript

SARCOIDOSIS : 

SARCOIDOSIS By: Dr. Deepak Kumar

INTRODUCTION : 

INTRODUCTION Sarcoidosis (Boeck disease) is a multisystem granulomatous disease of unknown cause. The lung is the most commonly affected organ, but the skin is frequently involved. Occur at any age (20-40 years) Male : female ratio 1:1 Black (females) are more susceptible SARCOIDOSIS

MAIN FEATURES : 

MAIN FEATURES Generalized Affect any organ of the body (except adrenal gland) Similar histological pattern in all affected organs Kveim test is positive in active cases SARCOIDOSIS

PATHOPHYSIOLOGY : 

PATHOPHYSIOLOGY SARCOIDOSIS

GRANULOMA : 

GRANULOMA SARCOIDOSIS

GRANULOMA : 

GRANULOMA SARCOIDOSIS

GRANULOMA : 

GRANULOMA SARCOIDOSIS

GRANULOMAdifferentials : 

GRANULOMAdifferentials TUBERCULOSIS OF SKIN SARCOIDOSIS

GRANULOMA differentials : 

GRANULOMA differentials SARCOIDOSIS TUBERCULOSIS OF SKIN

GRANULOMA differentials : 

GRANULOMA differentials LUPUS VULGARIS SARCOIDOSIS SARCOIDOSIS

GRANULOMAdifferentials : 

GRANULOMAdifferentials TUBERCULOID LEPROSY SARCOIDOSIS SARCOIDOSIS

GRANULOMAdifferentials : 

GRANULOMAdifferentials TUBERCULOID LEPROSY SARCOIDOSIS SARCOIDOSIS

GRANULOMAdifferentials : 

GRANULOMAdifferentials CUTANEOUS LEISHMANIASIS SARCOIDOSIS

GRANULOMAdifferentials : 

GRANULOMAdifferentials FOREIGN BODY GRANULOMA SARCOIDOSIS

AFFECTED ORGANS : 

AFFECTED ORGANS Lungs (90%) Lymph nodes (75%) Liver (60%) Spleen (40%) Skin (25%) Eyes (25%) Musculoskeletal (25%) CNS (5%) Heart (5%) Endocrine system GIT system SARCOIDOSIS

CUTANEOUS SARCOIDOSIS : 

CUTANEOUS SARCOIDOSIS 25% to 35% systemic sarcoidosis Can occur without systemic disease Cutaneous Lesions Specific Granulomas on biopsy Non-specific Reactive SARCOIDOSIS

CLASSIFICATION OF CUTANEOUS SARCOIDOSIS : 

CLASSIFICATION OF CUTANEOUS SARCOIDOSIS SARCOIDOSIS

CLASSICAL FORMSERYTHEMA NODOSUM : 

CLASSICAL FORMSERYTHEMA NODOSUM Sudden onset Painful erythematous nodules Site: shin, knee, ankle Young women Acute and benign stage Involute spontaneously in 2 years Löfgren syndrome Hilar lymphadenopathy Iridocyclitis Erythema nodosum SARCOIDOSIS

CLASSICAL FORMSMACULOPAPULAR FORM : 

CLASSICAL FORMSMACULOPAPULAR FORM Transient maculopapular eruptions Site: Trunk Diascopy Yellow discoloration Lupoid grains SARCOIDOSIS

CLASSICAL FORMSMACULOPAPULAR FORMdifferentials : 

CLASSICAL FORMSMACULOPAPULAR FORMdifferentials ROSACEA SARCOIDOSIS

CLASSICAL FORMSPAPULAR FORM : 

CLASSICAL FORMSPAPULAR FORM Painless,1 to 5 mm First yellowish-brown, later brownish-red Site: Face and extensor aspects of the limbs Merging into an erythematous plaque Diascopy Lupoid grains Favourable prognosis SARCOIDOSIS

CLASSICAL FORMSPAPULAR FORMdifferentials : 

CLASSICAL FORMSPAPULAR FORMdifferentials ACNE AGMINATA SARCOIDOSIS

CLASSICAL FORMSPAPULAR FORMdifferentials : 

CLASSICAL FORMSPAPULAR FORMdifferentials PAPULAR SECONDARY SYPHILIS SARCOIDOSIS

CLASSICAL FORMSNODULAR FORM : 

CLASSICAL FORMSNODULAR FORM Larger than 5 mm Yellowish-red at first, later violaceous Soft and round Proximal limbs, trunk and face SARCOIDOSIS

CLASSICAL FORMSANNULAR FORM : 

CLASSICAL FORMSANNULAR FORM Peripheral evolution and central clearing Centre depigmented and scarred Forehead, face and neck SARCOIDOSIS

CLASSICAL FORMSANGIOLUPOID FORM : 

CLASSICAL FORMSANGIOLUPOID FORM Rare but characteristic Soft and hemispherical nodule Orange-red or reddish-brown colour Site: Side of the bridge of the nose Spontaneous resolution SARCOIDOSIS

CLASSICAL FORMSSUBCUTANEOUS FORM : 

CLASSICAL FORMSSUBCUTANEOUS FORM Painless persistent nodules Site: Extremities Rare Accompanied by Hilar lymphadenopathy SARCOIDOSIS

CLASSICAL FORMSSUBCUTANEOUS FORMDIFFERENTIALS : 

CLASSICAL FORMSSUBCUTANEOUS FORMDIFFERENTIALS TUBERCULOID LEPROSY SARCOIDOSIS

CLASSICAL FORMSSUBCUTANEOUS FORMDIFFERENTIALS : 

LYMPHOCYTOMA CUTIS SARCOIDOSIS CLASSICAL FORMSSUBCUTANEOUS FORMDIFFERENTIALS

CLASSICAL FORMSSUBCUTANEOUS FORMDIFFERENTIALS : 

LUPUS VULGARIS SARCOIDOSIS CLASSICAL FORMSSUBCUTANEOUS FORMDIFFERENTIALS

CLASSICAL FORMSPLAQUE FORM : 

CLASSICAL FORMSPLAQUE FORM Site: Limbs, shoulders, buttocks and thighs Diffuse Persistent SARCOIDOSIS

CLASSICAL FORMSPLAQUE FORMDIFFERENTIALS : 

CLASSICAL FORMSPLAQUE FORMDIFFERENTIALS TERTIARY SYPHILIS SARCOIDOSIS

CLASSICAL FORMSPLAQUE FORMDIFFERENTIALS : 

PSORIASIS SARCOIDOSIS CLASSICAL FORMSPLAQUE FORMDIFFERENTIALS

CLASSICAL FORMSPLAQUE FORMDIFFERENTIALS : 

NECROBIOSIS LIPOIDICA SARCOIDOSIS CLASSICAL FORMSPLAQUE FORMDIFFERENTIALS

CLASSICAL FORMSLUPUS PERNIO : 

CLASSICAL FORMSLUPUS PERNIO Common in women Symmetrical bluish-red and dusky violaceous nodules and plaques Site: Nose, cheeks, ears, fingers, hands and Toes Typical appearance on diascopy Nasal involvement Swelling, ulceration of nasal vestibule Present with difficulty in breathing Lesions seldom resolve SARCOIDOSIS

CLASSICAL FORMSLUPUS PERNIODIFFERENTIALS : 

CLASSICAL FORMSLUPUS PERNIODIFFERENTIALS ROSACEAL RHINOPHYMA SARCOIDOSIS

CLASSICAL FORMSSCAR SARCOIDOSIS : 

CLASSICAL FORMSSCAR SARCOIDOSIS Long-standing scars Inflamed and infiltrated Purplish-red lesions later turn brown Common site knees Histology is typical of sarcoisosis SARCOIDOSIS

CLASSICAL FORMSSCAR SARCOIDOSISDIFFERENTIALS : 

CLASSICAL FORMSSCAR SARCOIDOSISDIFFERENTIALS SARCOIDOSIS KELOID

ATYPICAL FORMS : 

ATYPICAL FORMS HYPOPIGMENTATION Macular Site: limbs Not anaesthetic Histology Non caseating granuloma Melanocyte degeneration ERYTHRODERMIC SARCOIDOSIS Extremely rare Red scaling patches merge into brownish-red sheets Lymphadenopathy SARCOIDOSIS

ATYPICAL FORMS : 

ATYPICAL FORMS MUCOSAL INVOLVEMENT Nasal mucosa Buccal lesions or tongue involvement Plaques, diffuse pale yellow Ulceration NAIL INVOLVEMENT Rare Thickening, fragility, ridging, pitting, atrophy Accompanied by Bone cysts in terminal phalanx Lupus pernio SARCOIDOSIS

CLASSIFICATION OF PULMONARY SARCOISOSIS : 

CLASSIFICATION OF PULMONARY SARCOISOSIS STAGE 0 Initial normal film STAGE 1 Symmetrical hilar adenopathy STAGE 2 Adenopathy with pulmonary opacities SARCOIDOSIS

CLASSIFICATION OF PULMONARY SARCOISOSIS : 

CLASSIFICATION OF PULMONARY SARCOISOSIS STAGE 3 Pulmonary opacities without hilar adenopathy STAGE 4 Pulmonary fibrosis SARCOIDOSIS

EYE INVOLVEMENT : 

EYE INVOLVEMENT Uveitis Conjunctivitis Lacrimal gland involvement Heerfordt’s syndrome Uveitis Parotid gland enlargement Cranial nerve palsies SARCOIDOSIS

LIVER AND SPLEEN INVOLVEMENT : 

LIVER AND SPLEEN INVOLVEMENT Granulomatous infiltration Hepatosplenomegaly SARCOIDOSIS

NERVOUS SYSTEM INVOLVEMENT : 

NERVOUS SYSTEM INVOLVEMENT Aseptic Meningitis Space occupying lesion Cranial neuropathy Peripheral neuropathy SARCOIDOSIS

NERVOUS SYSTEM INVOLVEMENT : 

SARCOIDOSIS NERVOUS SYSTEM INVOLVEMENT

MUSCULOSKELETAL SYSTEM : 

MUSCULOSKELETAL SYSTEM Bone cysts Nodules in muscles SARCOIDOSIS

INVESTIGATIONS : 

INVESTIGATIONS Radiology Kveim skin test Histopathology Biochemistry S. calcium Gamma globulins Monitor disease activity ACE level Radioactive gallium-67 uptake SARCOIDOSIS

DIAGNOSIS : 

DIAGNOSIS SARCOIDOSIS

TREATMENT : 

TREATMENT TOPICAL THERAPY High-potency topical corticosteroids Intralesional triamcinolone Tacrolimus PUVA in hypopigmentation Cosmetic camouflage in lupus pernio SARCOIDOSIS

TREATMENT : 

TREATMENT SYSTEMIC THERAPY Corticosteroids Prednisolone daily 40 mg Maintenance 15 mg alternate Methotrexate Chlorambucil Azathioprine PULSED-DYE LASER Lupus pernio UVA1 (340–440 NM) Plaque sarcoid SARCOIDOSIS

PROGNOSIS : 

PROGNOSIS Better Females Less severe pulmonary disease Positive tuberculin test Normal globulin levels HLA-B8 Mortality 3–6% Cause of death Renal involvement Pulmonary disease SARCOIDOSIS

Slide 53: 

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