Inflammatory skin disorders

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Inflammatory skin disorders, also inflammatory skin diseases, are a significant part of dermatopathology. They lead to trepidation among pathologists that don't see lots of skin. Non-inflammatory skin disease is covered in dermatologic neoplasms and non-malignant skin disease.

Classification

  • Bullous.
  • Interface.
  • Nodular & diffuse.
  • Spongiotic.
  • Vasculitis.
  • Perivascular.
  • Panniculitis.
  • Psoriasiform.

Tabular comparison of inflammatory skin disease (adapted from Brinster[1][2]):

Pattern Key histologic feature Subclassifications Example Image
Bullous large "empty spaces" -subcorneal
-suprabasillar
-subepidermal 		-pemphigus foliaceus
-pemphigus vulgaris
-dermatitis herpetiformis 		(WC)
Interface inflammation at DE junction -vacuolar (minimal)
-lichenoid (band-like) 		-erythema multiforme, SLE
-lichen planus
Nodular & diffuse intradermal inflammatory infiltrate - nodular and/or diffuse -neutrophic
-lymphocytic
-plasmic
-eosinophilic
-histocytic 		-follicular occlusion triad, ruptured cyst/follicle
-CTCL, reactive
-plasma cell neoplasm, syphilis
-eosinophilic cellulitis, Kimura disease
-granuloma annulare, sarcoidosis, TB 		(WC)
Spongiotic small empty spaces between keratinocytes - can see squamous bridges (best seen at high power); +/- quasi-microvacuolar appearance -acute
-subacute
-chronic 		-poison ivy
-nummular dermatitis
-atopic dermatitis 		severe (WC), mild (WC)
Vasculitis inflammation of vessel wall/vessel was destruction -small vessel
-medium vessel
-large vessel 		-leukocytoclastic vasculitis
-PAN
-giant cell arteritis 		(WC)
Perivascular inflammation around vessels -neutrophilic
-lymphocytic
-mastocytic
-eosinophilic 		-cellulitis
-viral exanthem, Rx reaction
-mastocytosis
-insect bite, Rx reaction 		(WC)
Panniculitis inflammation of adipose tissue -septal
-lobular 		-erythema nodosum, scleroderma panniculitis
-erythema induratum, infection
Psoriasiform epidermal thickening
and long rete ridges 		-regular
-irregular 		-psoriasis
-lichen simplex chronicus 		(WC)

Notes:

  • DE junction = dermal-epidermal junction.
  • The "empty space" in bullous disease in situ is filled with fluid.

Non-specific patterns

Psoriasiform pattern

General

  • Can be subclassified.

DDx:

  • Psoriasis vulgaris (most common).
  • Psoriatic arthritis.
  • Drug-induced.
  • Dermatomycosis (fungal infection).
  • Others.

Microscopic

Features:[3][1]

  • Regular epidermal thickening - as very long rete ridges (described as "test tube-morphology") - key feature.
    • Epidermis between rete ridges thin ("thinning of suprapapillary plate").
  • Parakeratosis.
  • Thin/absent granular layer.
  • Dilated blood vessels in superficial dermis.
  • Neutrophil clusters (Munro microabscess).

Specific diseases

Seborrheic dermatitis

General

  • Very common.

Microscopic

Features:[4]

  • Spongiosis (epidermal edema).
  • Acanthosis (epidermal thickening).
  • "Follicular lipping" = parakeratosis with neutrophils.
  • Perivascular neutrophils and lymphocytes.

Lupus erythematosus

General

Microscopic

Features - classic:

  • Lymphocytic interface dermatitis.[5]
  • Basal layer vacuolation.[6]
  • Intradermal mucin.
  • Thin epidermis.
  • Compact stratum corneum.

DDx:

Images:

Discoid lupus erythematosus

General

Microscopic

Features:[7]

  • Lymphocytic interface dermatitis with basal layer vacuolation and keratinocyte apoptosis.
  • Deep dermal lymphocytes.
  • Basket weave stratum corneum.
  • Dermal mucin.
  • Thick dermal-epidermal basement membrane.

DDx:

Image:

Dermatomyositis

For the muscle manifestations see: Neuromuscular_pathology#Dermatomyositis.

General

  • Complement mediated disease - membrane attack complex.
  • Usually middle age.
  • Associated skin rash is common.
    • May precede or follow muscle pathology.
  • Associated with malignancy in approximately 10% of cases.[8]

Clinical:

  • Jo-1 +ve.

Gross

  • Have lesions on the knuckles - Gottron's papules.

Microsopic

Features:

  • Vacuolar interface dermatitis (lymphocytic inflammation at the dermal-epidermal junction).
  • +/-Loss of rete ridges.

DDx:

  • Lupus.

Lichen planus

Should not be confused with lichen planus-like keratosis.

General

Etiology:

  • Autoimmune disease, T-cell–mediated.[11]

Clinical:[12]

  • 6 Ps: pruritic (itchy), purple, polygonal, planar papules and plaques.

Note:

  • Clinically, lichen planus and lichen sclerosus are similar. The main difference is lichen planus involves mucous membranes.[13]

Gross

  • Wickham striae = white lines/dots.
    • Due to hypergranulosis.

Microscopic

Features:[14][15]

  • Loss of basal cells (stratum basale) -- key feature.
  • Loss of rete ridges/formation of pointed rete ridges "sawtoothing".
  • Interface dermatitis (lymphocytes at dermal-epidermal junction).
  • Hypergranulosis; stratum granulosum thickened (grossly seen as "Wickham stria" = white lines).
  • Hyperkeratosis; stratum corneum thickened.
  • Necrotic basal cell in dermis = colloid bodies = Civatte bodies.[16]

DDx:

Notes:

Images:

Sign out

FORESKIN, CIRCUMCISION:
- LICHEN PLANUS.

Lichen sclerosus

Main article: Lichen sclerosus

Microscopic

Features:[18]

  • Subepithelial fibrosis - key feature.

Psoriasis

General

  • Other considerations:
    • Drug-induced psoriasis.[21]

Clinical

  • Auspitz sign = pin-point bleeding on removal of scale.
  • Koebner phenomenon = lesions form at site of trauma.

Common locations:[19]

  • Knees, elbows, scalp, glands penis.

Microscopic

Features:[19]

  • Acanthosis + long rete ridges (psoriasiform hyperplasia).
  • Parakeratosis.
  • Dilated vessels in superficial dermis (give rise to Auspitz sign).
  • Spongiform pustules = PMNs in stratum spinosum.
  • PMNs in parakeratotic stratum corneum (Munro microabscess).
  • Thinning of the suprapapillary plate.
    • The epidermis between rete ridges is thinner than in the normal adjacent skin - key feature.

DDx:

Note:

Stains

Lichen striatus

General

Features:[22]

  • Benign, self-limited.

Clinical:

Clinical image:

Microscopic

Features:

  • "Ball and glove" pattern:
    • Rete ridges (glove) surround nexts of lymphocytes (ball).
  • Hyperkeratosis.
  • Parakeratosis.

DDx:

Images:

Lichen aureus

General

  • Rare.

Clinical:[23]

  • Plaques + minimal scale - erythematous/golden.

Microscopic

Features:[23]

  • Lymphocyte-predominant perivascular pattern.
    • May have features suggestive of vacuolar interface pattern.
  • Fibrosis of superficial papillary dermis.

Images:

Granuloma faciale

General

Microscopic

Features:[25]

  • Neutrophilic vasculitis - often subtle:
    • Neutrophils within blood vessel walls.
    • Vessels destruction (fibrinoid necrosis or nuclear dust).
  • Mixed inflammatory superficial dermal infiltrate.
    • Eosinophils, neutrophils, plasma cells, histiocytes.
  • Lesion separated from the (normal) epidermis (Grenz zone).[24]

DDx of chronic fibrosing leukocytoclastic vasculitis:[25]

  • Granuloma faciale.
  • Erythema elevatum diutinum - lacks Grenz zone.
  • Inflammatory pseudotumour.

Image:

Atopic dermatitis

  • AKA atopic eczema, AKA infantile eczema.

General

  • Very common - especially in children.[26]
    • Most children outgrow the condition.

Microscopic

Features:

  • Spongiotic dermatitis.

DDx:

Images:

Sweet syndrome

General

  • Uncommon.

Microscopic

Features:

  • Nodular and diffuse pattern - neutrophils predominant - without pus.

DDx rips:

  • Rheumatoid neutrophilic dermatitis.
  • Bowel-associated dermatosis-arthritis syndrome.
  • Pyoderma gangrenosum.
  • Sweet syndrome.

Image:

Granuloma inguinale

Not to be confused with granuloma annulare.
  • Previously known as donovanosis.

General

  • Infection caused by Klebsiella granulomatis - Gram-negative, rod-shaped bacteria.
    • Used to be known as Calymmatobacterium granulomatis.[28]

Location:

Clinical:[29]

  • Sexually transmitted.
  • Nodule or papule developing into a painless ulcer in the inguinal region.
  • No lymphadenopathy (unlike syphilis).

Microscopic

Features:

  • Donovan bodies - key feature:
    • Rod-shaped bodies within histiocytes.

DDx:

Image:

Stains

Pyoderma gangrenosum

General

  • May be seen in IBD (UC and CD) - approximately 30% of cases.[30]

Clinical

  • Pathergy = minor trauma (to the skin) results in a non-healing lesion.[31]

Gross

  • Skin ulceration - classically on the legs.[30]

Microscopic

Features:[30]

  • Early: lymphocytes cells only in the dermis - often perivascular & vascular.
    • +/-Fibrinoid necrosis of vessels at edge of lesion.
  • Late: abscess formation (neutrophils).

DDx:

Image:

Necrobiotic xanthogranuloma

  • Abbreviated NXG.

General

  • Usually elderly.[32]

Gross

Features:

  • Periorbital - classic location.[33]

Microscopic

Features:[32]

  • "Necrobiotic granuloma":
    • Palisading granuloma with a core collagen-like material.
  • +/-Cholesterol clefts - common.
  • +/-Touton giant cells.
  • +/-Atypical foreign body-type giant cells +/- asteroid bodies.

DDx:

Image:

DDx for pattern

Spongiotic dermatitides

DDx:[1]

Psoriasiform dermatitides

Regular psoriasiform dermatitis

DDx:[1]

  • Psoriasis vulgaris.
  • Dermatophyte infection.
  • Pityriasis rubra pilaris.

Irregular psoriasiform dermatitis

Interface dermatitides

Vacuolar interface dermatitides

DDx:[1]

Others:[1]

Additional:[34]

  • Phototoxic dermatitis.
  • Acute radiation dermatitis.
  • Erythema multiforme-like drug eruption.
  • Lichen sclerosis et atrophicus.
  • Erythema dyschromicum perstans (ashy dermatosis).

Super lame mnemonic Danny G & SLE:

  • Dermatomyositis, GVHD, SLE, Lichen sclerosus, Erythema multiforme.

Lichenoid interface dermatitides

DDx:[1]

Additional:[34]

Bullous disease

Main article: Bullous disease

Subcorneal bullous disorders

DDx with acantholysis:[1]

DDx without acantholysis:DDx:[1]

Suprabasilar bullous disorders

DDx:[1]

Memory device - PhD + Grover = Pemphigus vulgaris, Hailey-Hailey, Darier, Grover.

Subepidermal bullous disorders

DDx:[1]

Others:

Mnemonic DELPHI:

  • Dermatitis herpetiformis.
  • Epidermolysis bullosa acquisita.
  • Bullous lupus erythematosis.
  • Pemphigoid, bullous.
  • Herpes gestationis - rare autoimmune bullous dermatosis of pregnancy, not related to HSV.[35]
  • Linear IgA disease.

Dermatitides with perivascular inflammation

Lymphocytes

Lymphocytes (tightly cuffed):[1]

  • Infections.
  • Medications.
  • Malignancy.
  • Vitiligo (with absent melanocytes).
  • Scleroderma/morphea.

Lymphocytes & plasma cells +/-eosinophils:

  • Erythema chronicum migrans (Lyme disease).

Lymphocytes (not tightly cuffed):

  • Viral exanthems.
  • Drug eruptions.

Lymphocytes & eosinophils:

  • Drug reaction.
  • Insect bite.
  • Dermatophyte infection.

A simple memory device - 6 Ls:[36]

  • Lymphoma.
  • Pseudolymphoma (cutaneous lymphoid hyperplasia, lymphocytoma cutis).
  • Lupus erythematosus.
  • Lymphocytic infiltrate of Jessner / tumid lupus erythematosus.
  • Polymorphous light eruption.
  • Lyme disease.

Neutrophils

DDx:[1]

  • Cellulitis.
  • Neutrophilic eccrine hidradenitis (idiopathic palmar-plantar hidradenitis)

With eosinophils:

  • Urticaria.

Eosinophils

DDx:[1]

  • Hypersensitivity reaction/drug reaction.
  • Insect bite.
  • Bullous pemphigoid, urticarial.

Mast cells

Vasculitis

Main article: Vasculitides

General DDx:

  • Infection.
  • Autoimmune.
  • Malignancy.
  • Toxic (medications).

Small vessel:

Medium vessel:

Medium vessels often with small vessel involvement:

Large vessel:

Nodular and diffuse dermatitides

Neutrophils

With pus (suppurative)

Neutrophils only:

  • Follicular occlusion triad.
  • Ruptured cyst/follicle.

Neutrophils and eosinophils:

  • Halogenoderma.
  • Blastomycosis-like pyoderma (pyoderma vegetans) - due to bacterial infection, usu. S. aureus.

Without pus

With vasculitis:

Without vasculitis:

Lymphocytes

Plasma cells

Eosinophils

Histiocytes

Granulomatous

Sarcoidal
  • Sarcoidosis - have few lymphoid cells around them; thus called "naked granulomas".[2]
Tuberculoid
  • Tuberculosis.
  • Tertiary syphilis - usu. abundant plasma cells.
  • Cutaneous leishmaniasis.
Foreign body-type granulomas
Palisaded granumolas

Blue granuloma:

Red granuloma:

See also

References

  1. ↑ 1.00 1.01 1.02 1.03 1.04 1.05 1.06 1.07 1.08 1.09 1.10 1.11 1.12 1.13 Brinster NK (March 2008). "Dermatopathology for the surgical pathologist: a pattern based approach to the diagnosis of inflammatory skin disorders (part I)". Adv Anat Pathol 15 (2): 76–96. doi:10.1097/PAP.0b013e3181664e8d. PMID 18418089.
  2. ↑ 2.0 2.1 Brinster, NK. (Nov 2008). "Dermatopathology for the surgical pathologist: a pattern-based approach to the diagnosis of inflammatory skin disorders (part II).". Adv Anat Pathol 15 (6): 350-69. doi:10.1097/PAP.0b013e31818b1ac6. PMID 18948765.
  3. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1191. ISBN 978-1416031215.
  4. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1191. ISBN 978-1416031215.
  5. ↑ JAH. 20 February 2009.
  6. ↑ Crowson AN, Magro CM, Mihm MC (April 2008). "Interface dermatitis". Arch. Pathol. Lab. Med. 132 (4): 652–66. PMID 18384217. http://www.archivesofpathology.org/doi/full/10.1043/1543-2165%282008%29132%5B652%3AID%5D2.0.CO%3B2.
  7. ↑ URL: http://www.dermpedia.org/baby-dermpedia-for-beginners/discoid-lupus-erythematosus. Accessed on: 7 March 2012.
  8. ↑ Chen YJ, Wu CY, Huang YL, Wang CB, Shen JL, Chang YT (2010). "Cancer risks of dermatomyositis and polymyositis: a nationwide cohort study in Taiwan". Arthritis Res. Ther. 12 (2): R70. doi:10.1186/ar2987. PMC 2888225. PMID 20398365. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2888225/.
  9. ↑ Teichman, JM.; Sea, J.; Thompson, IM.; Elston, DM. (Jan 2010). "Noninfectious penile lesions.". Am Fam Physician 81 (2): 167-74. PMID 20082512.
  10. ↑ Gupta, R.; Bansal, B.; Singh, S.; Yadav, I.; Gupta, K.; Kudesia, M. (2009). "Lichen planus of uterine cervix - the first report of a novel site of occurrence: a case report.". Cases J 2: 9306. doi:10.1186/1757-1626-2-9306. PMID 20062629.
  11. ↑ URL: http://emedicine.medscape.com/article/1078327-overview. Accessed on: 11 September 2010.
  12. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1191. ISBN 978-1416031215.
  13. ↑ McPherson, T.; Cooper, S.. "Vulval lichen sclerosus and lichen planus.". Dermatol Ther 23 (5): 523-32. doi:10.1111/j.1529-8019.2010.01355.x. PMID 20868406.
  14. ↑ URL: http://emedicine.medscape.com/article/1078327-overview. Accessed on: 11 September 2010.
  15. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1192. ISBN 978-1416031215.
  16. ↑ Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 604. ISBN 978-1416054542.
  17. ↑ URL: http://www.careforumwales.org/cell-carcinoma/histopathologic-terminology.html. Accessed on: 28 August 2011.
  18. ↑ Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 1065-6. ISBN 0-7216-0187-1.
  19. ↑ 19.0 19.1 19.2 Mitchell, Richard; Kumar, Vinay; Fausto, Nelson; Abbas, Abul K.; Aster, Jon (2011). Pocket Companion to Robbins & Cotran Pathologic Basis of Disease (8th ed.). Elsevier Saunders. pp. 603. ISBN 978-1416054542.
  20. ↑ URL: http://medical-dictionary.thefreedictionary.com/spondylosis. Accessed on: 28 August 2011.
  21. ↑ Milavec-Puretić, V.; Mance, M.; Ceović, R.; Lipozenčić, J. (Mar 2011). "Drug induced psoriasis.". Acta Dermatovenerol Croat 19 (1): 39-42. PMID 21489366.
  22. ↑ 22.0 22.1 Taniguchi Abagge, K.; Parolin Marinoni, L.; Giraldi, S.; Carvalho, VO.; de Oliveira Santini, C.; Favre, H.. "Lichen striatus: description of 89 cases in children.". Pediatr Dermatol 21 (4): 440-3. doi:10.1111/j.0736-8046.2004.21403.x. PMID 15283785.
  23. ↑ 23.0 23.1 Hazan, C.; Fangman, B.; Cohen, D. (2007). "Lichen aureus.". Dermatol Online J 13 (1): 23. PMID 17511956.
  24. ↑ 24.0 24.1 URL: http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=1289066309. Accessed on: 23 September 2011.
  25. ↑ 25.0 25.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 198-9. ISBN 978-0443066542.
  26. ↑ URL: http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001856/. Accessed on: 28 December 2011.
  27. ↑ URL: http://dermatology.cdlib.org/94/NYU/Apr2002/7.html. Accessed on: 4 January 2012.
  28. ↑ O'Farrell N (December 2002). "Donovanosis". Sex Transm Infect 78 (6): 452–7. PMC 1758360. PMID 12473810. http://sti.bmj.com/cgi/pmidlookup?view=long&pmid=12473810.
  29. ↑ Bezerra, SM.; Jardim, MM.; Silva, VB. (Jun 2011). "Granuloma inguinale (Donovanosis).". An Bras Dermatol 86 (3): 585-6. PMID 21738983.
  30. ↑ 30.0 30.1 30.2 Brooklyn, T.; Dunnill, G.; Probert, C. (Jul 2006). "Diagnosis and treatment of pyoderma gangrenosum.". BMJ 333 (7560): 181-4. doi:10.1136/bmj.333.7560.181. PMC 1513476. PMID 16858047. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1513476/.
  31. ↑ Wong, WW.; Machado, GR.; Hill, ME.. "Pyoderma gangrenosum: the great pretender and a challenging diagnosis.". J Cutan Med Surg 15 (6): 322-8. PMID 22202507.
  32. ↑ 32.0 32.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 629. ISBN 978-0443066542.
  33. ↑ Fernández-Herrera, J.; Pedraz, J. (Jun 2007). "Necrobiotic xanthogranuloma.". Semin Cutan Med Surg 26 (2): 108-13. doi:10.1016/j.sder.2007.02.008. PMID 17544963.
  34. ↑ 34.0 34.1 Alsaad, KO.; Ghazarian, D. (Dec 2005). "My approach to superficial inflammatory dermatoses.". J Clin Pathol 58 (12): 1233-41. doi:10.1136/jcp.2005.027151. PMC 1770784. PMID 16311340. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1770784/.
  35. ↑ URL: http://emedicine.medscape.com/article/1063499-overview. Accessed on: 23 September 2011.
  36. ↑ URL: http://www.dermpathmd.com/mnemonics/mnemonics_dermatopathology.htm. Accessed on: 23 September 2011.

External links

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