Algorithmic approach in the diagnosis of inflammatory dermatosess

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An approach to diagnose inflammatory dermatoses, derived from Weedon and Philip MacKee textbooks on Dermatopathology.

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A logical approach in the diagnosis of inflammatory dermatoses:

A logical approach in the diagnosis of inflammatory dermatoses Brig Asher Ahmed Mashhood MBBS, FCPS, Clinical Fellowship in Dermatopathology (UK) Consultant Dermatologist CMH Peshawar Acknowledgement: The histopathology pics are taken from Histopathology of Skin by Philip MacKee

Normal histology of Skin:

Normal histology of Skin

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Stratum cornium

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Stratum lucidum

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Stratum granulosum

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Stratum spinosum

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Stratum spinosum(prickle cell layer) Intercellular junctions

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Stratum basale melanocytes

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RETE RIDGES DERMAL PAPILLAE

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Papillary dermis Reticular dermis Fibroblasts

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Approach is to identify 1. TISSUE REACTION PATTERN 2. DISTRIBUTION PATTERNS OF INFLAMMATORY INFILTRATE this crude “algorithmic approach” is probably the best one to make sense of the wide range of skin diseases

TISSUE REACTION PATTERN:

TISSUE REACTION PATTERN Weedon uses 6 MAJOR patterns

Major tissue reaction patterns:

Major tissue reaction patterns 1. Lichenoid (basal cell damage; interface dermatitis) 2. Psoriasiform (regular epidermal hyperplasia) 3. Spongiotic intraepidermal , intercellular oedema 4. Vesiculobullous 5. Granulomatous 6. Vasculopathic

(1) Lichenoid reaction pattern (“interface dermatitis”):

(1) Lichenoid reaction pattern (“interface dermatitis”) Lichenoid Dermatitis Interface Dermatitis

Lichenoid Dermatoses Characterized by basal cell degeneration and band-like lymphohistocytic infiltrate in upper dermis hugging and often obscuring dermoepidermal interface. :

Lichenoid Dermatoses Characterized by basal cell degeneration and band-like lymphohistocytic infiltrate in upper dermis hugging and often obscuring dermoepidermal interface. Causes LP LPP Lichen Striatus Lichen Nitidus Ashy Dermatosis FDE

Interface Dermatitis :

Interface Dermatitis

Interface Dermatitis Refers to basal cell vacuolization/degeneration often accompanied by single cell keratinocyte apoptosis. :

Interface Dermatitis Refers to basal cell vacuolization/degeneration often accompanied by single cell keratinocyte apoptosis. Causes Lichenoid Dermatoses EM/SJ Syndrome / TEN LE Poikloderma Disease PLC and PLEVA

(2) Psoriasiform Pattern:

(2) Psoriasiform Pattern Psoriasiform : Epidermal hyperlasia in which there is regular elongation of rete ridges Psoriasis: Confluent parakeratotic scales including neutrophilic abscesses (Munro’s abscess)

Psoriasiform dermatitis:

Psoriasiform dermatitis

Psoriasiform Epidermal Acanthosis:

Psoriasiform Epidermal Acanthosis Elongated rete ridges of about equal length that alternate with long dermal papillae to form a strikingly undulate pattern. Even Uneven Regular Irregular

Psoriasiform hyperplasia:

Psoriasiform hyperplasia D/D Psoriasis Prurigo, lichenification Keratodermas, epidermal naevi, seb K Warts and molluscum

(3) Spongiotic (“eczematous”) pattern:

(3) Spongiotic (“eczematous”) pattern Intraepidermal intercellular oedema ( spongiosis ) May form microscopic foci or grossly visible vesicles

Spongiosis:

Spongiosis Intercellular oedema Separation of spinous cells Intercellular bridges conspicuous Sprinkling of inflammatory cells (exocytosis)

Conditions featuring spongiosis:

Conditions featuring spongiosis Eczemas Pityriasis rosea Superficial fungal infection Lichen striatus Insect bite reaction Prurigo nodularis Polymorphic eruption of pregnancy Acral papular eruption of childhood Parapsoriasis

Diseases featuring eosinophilic spongiosis:

Diseases featuring eosinophilic spongiosis Insect bite reaction Atopic/ contact dermatitis Drug reactions Polymorphic eruption of pregnancy

Diseases featuring neutrophilic spongiosis:

Diseases featuring neutrophilic spongiosis Pustular psoriasis AGEP Dermatophytosis Paederus dermatitis Seborrhoeic eczema Infantile acropustulosis

(4) Vesiculobullous Pattern:

(4) Vesiculobullous Pattern Anatomical level of split Subcorneal , Stratum spinosum , suprabasal , sub-epidermal Underlying mechanism exaggerated spongiosis intracellular odema (ballooning) due to viral inclusions e.g. HSV Immunobullous-acantholysis Nature of inflammatory infiltrate Cell poor; Cell rich Immunofluorescence: often required to confirm diagnosis

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Subcorneal Pustules

Subcorneal Blister :

Subcorneal Blister D/D Pemphigus foliaceus, Subcorneal pustular dermatosis, pustular psoriasis, bullous impetigo

Pemphigus foliaceus: close-up view of crusted erosions. :

Pemphigus foliaceus : close-up view of crusted erosions.

Intraepidermal blister e.g. Subacute eczema, viral infections:

Intraepidermal blister e.g. Subacute eczema, viral infections

Acute Eczema:

Acute Eczema

Suprabasal blister :

Suprabasal blister PV, Paraneoplastic pemphigus, Hailey-Hailey; Darier’s, Grover’s.

ACANTHOLYSIS:

ACANTHOLYSIS D/D Pemphigus, Dariers, Hailey-hailey, Grovers, viral infections Tombstones

Pemphigus vulgaris:

Pemphigus vulgaris

Vesiculobullous: Subepidermal:

Vesiculobullous: Subepidermal

Subepidermal blister with predominaly eosinophils e.g. Pemphigoid, herpes gestationis :

Subepidermal blister with predominaly eosinophils e.g. Pemphigoid , herpes gestationis

The blister cavity contains a heavy eosinophil infiltrate. :

T he blister cavity contains a heavy eosinophil infiltrate.

Herpes gastationis:

Herpes gastationis

Subepidermal blister with predominaly neutrophils e.g. Linear IgA disease, DH, Bullous LE, Sweets, EBA :

Subepidermal blister with predominaly neutrophils e.g. Linear IgA disease, DH, Bullous LE, Sweets, EBA

Linear IgA disease: floor of the blister showing an intense neutrophil infiltrate. :

Linear IgA disease : floor of the blister showing an intense neutrophil infiltrate.

Linear IgA disease of childhood (CBDC):

Linear IgA disease of childhood (CBDC)

Dermatitis herpetiformis: Papilliary tip neutrophil microabscesses. :

Dermatitis herpetiformis : Papilliary tip neutrophil microabscesses .

Dermatitis Herpetiformis:

Dermatitis Herpetiformis

. Cell-free subepidermal blister: EB,porphyria, burns, TEN, suction blister, bullous amyloid, cryotherapy, diabetic bulla :

. C ell-free subepidermal blister : EB, porphyria , burns, TEN, suction blister, bullous amyloid, cryotherapy , diabetic bulla

(5) Granulomatous Pattern:

(5) Granulomatous Pattern Sarcoidal Tuberculoid Necrobiotic Suppurative Foreign body Miscellaneous

Sarcoidal “Naked” Granulomas:

Sarcoidal “Naked” Granulomas Epithelioid cells Discrete giant cells (often foreign body type) Few lymphocytes and plasma cells Sarcoidosis and Foreign body granuloma

Tuberculoid Granulomas:

Tuberculoid Granulomas Tuberculosis Tuberculids Tuberculoid Leprosy Leishmaniasis Rosacea Perioral dermatitis Acne agminata Crohn’s Disease

Tuberculoid Granulomas:

Tuberculoid Granulomas Confluence Prominent rim of lymphocytes and plasma cells Langhan -type giant cells +/- Caseation

Suppurative Granulomas:

Suppurative Granulomas Infectious Atypical myocbacteria Deep fungal infections Non Infections Superficial granulomatous pyoderma gangrenosum

Suppurative (Pustulating) granulomas:

Suppurative (Pustulating) granulomas Epithelioid granulomas with central collections of neutrophils

Necrobiotic Granulomas:

Necrobiotic Granulomas Granuloma annulare Necrobiosis lipoidica Granuloma multiforme Rheumatoid nodules Actinic granuloma

Necrobiotic Granulomas:

Necrobiotic Granulomas Altered dermal connective tissue Palisading of epithelioid cells Rimed by lymphocytes +/- Multinucleate giant cells

(6) Vasculitis and vasculopathy :

(6) Vasculitis and vasculopathy Vasculitis is a term applied to inflammation and some form of vessel wall damage (necrosis or fibrinoid change). Plus/ minus extravasated RBC, perivascular infiltrate and karyorrhexis .

Leukocytoclastic vasculitis: the blood vessels show florid fibrinoid necrosis and intense inflammation. :

Leukocytoclastic vasculitis : the blood vessels show florid fibrinoid necrosis and intense inflammation.

Leukocytoclastic vasculitis :

Leukocytoclastic vasculitis

DISTRIBUTION PATTERNS OF INLFAMMATORY INFILTRATE:

DISTRIBUTION PATTERNS OF INLFAMMATORY INFILTRATE Weedon uses 4 patterns :- 1. Superficial perivascular 2. Superficial and deep dermal perivascular inflammation 3. Folliculitis and perifolliculitis 4. Panniculitis

(1) Superficial perivascular inflammatory infiltrate:

(1) Superficial perivascular inflammatory infiltrate Usually associated with spongiotic , psoriasiform or lichenoid reaction. Occurs independent of any other H/P change in; drug reactions, dermatophytosis , viral examthems , chronic urticaria

Erythema annulare:

Erythema annulare

Superficial Perivascular infiltrate e.g. Erythema annulare centrifugum :

Superficial Perivascular infiltrate e.g. Erythema annulare centrifugum

Pigmented purpuric dermatoses: there is an upper dermal heavy perivascular lymphocytic infiltrate. :

Pigmented purpuric dermatoses : there is an upper dermal heavy perivascular lymphocytic infiltrate.

(2) Superficial and deep perivascular infiltrate:

(2) Superficial and deep perivascular infiltrate Polymorphic Light reactions (PLE) Pseudolymphoma / Jessner’s Syphilis ) Lupus erythematosus I nsect bite reactions

Jessner’s:

Jessner’s

Superficial and deep perivascular infiltrate e.g. Lymphocytic infiltrate of Jessner :

Superficial and deep perivascular infiltrate e.g. Lymphocytic infiltrate of Jessner

Chronic insect bite reactions: a superficial and deep perivascular infiltrate is seen. The infiltrate is usually wedge-shaped and have significant eosinophils. :

Chronic insect bite reactions : a superficial and deep perivascular infiltrate is seen. The infiltrate is usually wedge-shaped and have significant eosinophils .

Tumid LE: The epidermis is spared and there is a superficial and deep perivascular infiltrate and periadenexal infiltrate with intradermal mucin.:

Tumid LE : The epidermis is spared and there is a superficial and deep perivascular infiltrate and periadenexal infiltrate with intradermal mucin .

Secondary Syphilis: The appearance is that of diffuse psoriasiform hyperplasia and superficial & deep perivascular infiltrate with prominent plasma cells and endovasculitis.:

Secondary Syphilis : The appearance is that of diffuse psoriasiform hyperplasia and superficial & deep perivascular infiltrate with prominent plasma cells and endovasculitis.

(3) Folliculitis and Perifolliculitis:

(3) Folliculitis and Perifolliculitis Superficial vs deep Infective vs non-infective

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PATHOLOGY OF FURUNCLE AND CARBUNCLE

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Folliculitis Demodex folliculitis

Carbuncle:

Carbuncle

D/D Folliculitis:

D/D Folliculitis Infective Staph aureus Pseudomonas Gram-negative Pityrosporum folliculitis Acne vulgaris and Rosacea (acute and chronic) Perifolliculitis capitis Non-infective Physical trauma Pseudofolliculitis Contact with mineral oils, tars etc Perforating folliculitis Follicular mucinosis

Perifolliculitis (inflammation of skin around hair follicles):

Perifolliculitis (inflammation of skin around hair follicles)

D/D of Perifolliculitis:

D/D of Perifolliculitis Keratosis pilaris Follicular eczema Eosinophilic folliculitis ( Ofuji’s disease) Majocchi’s granuloma Lichen planopilaris DLE Alopecia Areaate Traction alopecia

(4) Paniculitis:

(4) Paniculitis Septal Erythema nodosum Lobular Enzymic (pancreatic), α 1-antitrypsin deficiency, Subcutaneous fat necrosis of the newborn Mixed panniculitis Lupus erythematous profundus , infective panniculitis Panniculitis with vasculitis PAN, thrombophlebitis, erythema induratum

Septal paniculitis e.g. erythema nodosum :

Septal paniculitis e.g. erythema nodosum

Lobular paniculitis e.g. Subcutaneous fat necrosis of the newborn :

Lobular paniculitis e.g. Subcutaneous fat necrosis of the newborn

CONCLUSION 10 patterns for all inflammatory dermatoses:

CONCLUSION 10 patterns for all inflammatory dermatoses Major reaction pattern 1. Lichenoid 2 . Psoriasiform 3 . Spongiotic 4 . Vesiculobullous 5. Granulomatous 6. Vasculopathic Minor reaction pattern 1. Superficial perivascular 2. Superficial and deep perivascular 3. Folliculitis and perifolliculitis 4. Panniculitis

THANK YOU:

THANK YOU

Minor Reaction Patterns:

Minor Reaction Patterns Hyperkeratosis Parakeratosis Hypergranulosis Epidermolytic hyperkeratosis Acantholytic Dyskeratosis Cornoid lamellation Papillomatosis, Follicular Plugging Transepidermal elimination Epidermal atrophy and necrosis

HYPERKERATOSIS:

HYPERKERATOSIS D/D Ichthyosis, Keratodermas, PRP, Warts, Seb K, Epidermal naevus, Cut horn etc.

PARAKERATOSIS:

PARAKERATOSIS D/D Psoriasis, PRP, P. rosea, PLC, Actinic Keratosis, ILVEN etc

Papillomatosis :

Papillomatosis D/D Viral wart Epidermal Naevus Sebaceous Naevus Seborrhoeic keratosis Acrokeratosis verruciformis

HYPERGRANULOSIS:

HYPERGRANULOSIS D/D Keratodermas, chronic licenification, lichen planus

Epidermolytic Hyperkeratosis :

Epidermolytic Hyperkeratosis D/D of epidermolytic hyperkeratosis CBIE Linear verrucous epidermal naevus Epidermolytic keratoderma Epidermolytic acanthoma

Coronoid Lamella :

Coronoid Lamella D/D of Coronoid lamella : Porokeratosis, Seb K, Wart. AK, BCC/SCC

FOLLICULAR PLUGGING :

FOLLICULAR PLUGGING DLE, LSA, KP, Lichen planopilaris

Transepidermal elimination:

Transepidermal elimination Reactive perforating collagenosis Perforating serpiginous elastosis. Perforating folliculitis Kyrle’s disease Granuloma annulare PXE Lichen nitidus Chondrodermatitis nodularis

EPIDERMAL ATROPHY:

EPIDERMAL ATROPHY D/D Scleroderma, LSA, LE, LP AtrophodermaIatrogenic

EPIDERMAL CELL NECROSIS:

EPIDERMAL CELL NECROSIS D/D Erythema Multiforme, SJ Syndrome, TEN, graft vs host disease, Pemphigus paraneoplastica, necrolytic migratory erythema

Acantholytic Dyskeratoses :

Acantholytic Dyskeratoses Grains: Flat oval cells with cigar shaped nuclei in the St corneum

CORPS RONDS:

CORPS RONDS D/D Dariers disease, Grovers diasese

Non-infective folliculitis:

Non-infective folliculitis

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PATHOLOGY OF SUPERFICIAL AND DEEP FOLLICULITIS

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