Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Pathophysiology | Etiology | Clinical features | Diagnosis | Laboratory | Prognostic factors | Case reports | Treatment | Clinical images | Microscopic (histologic) description | Microscopic (histologic) images | Virtual slides | Immunofluorescence description | Positive stains | Electron microscopy description | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Aghighi M, Motaparthi K. Chronic cutaneous lupus. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorlupussle.html. Accessed May 18th, 2022.
Definition / general
- Lupus erythematosus is a multisystem autoimmune disease that involves the skin and internal organs
- Cutaneous lupus erythematosus is categorized as acute, subacute and chronic
Essential features
- Most common sites for discoid lupus erythematous are face, scalp and conchal bowl
- Discoid lupus erythematous presents with erythematous and scaly plaques with hyperpigmentation; scalp involvement may lead to scarring, dyspigmentation and alopecia
- Microscopic features of discoid lupus erythematous include epidermal atrophy, effacement of rete ridges, hyperparakeratosis, follicular keratotic plugging and basement membrane thickening with vacuolar interface change and keratinocyte apoptosis
Terminology
- Lupus erythematous (LE)
- Systemic lupus erythematous (SLE)
- Discoid lupus erythematous (DLE)
- Tumid lupus erythematosus
- Chilblain lupus erythematosus
- Lupus panniculitis
- Lupus profundus
ICD coding
- ICD-10: L93.0 - Discoid lupus erythematosus
Epidemiology
- Mostly young women between 20 and 30
- More common in African Americans than in Caucasians
Sites
- Discoid lupus erythematosus (DLE): face, scalp, conchal bowl
- Other variants of chronic cutaneous lupus erythematosus
- Tumid lupus erythematosus: upper trunk, lateral face
- Chilblain lupus erythematosus: acral
- Lupus panniculitis: upper extremities, upper trunk
Pathophysiology
- In patients with defects in innate immunity and opsonization, UV radiation causes apoptosis; subsequent phagocytosis leads to recognition of self antigens and autoimmunity
- Activated immune reaction is a result of interaction between hereditary predisposition and environmental or infectious agents
- MHC II may identify self antigens which show cross reactivity with pathogens
- HLA-B8, HLA-DR3, HLA-DR2, HLA-A1, HLA-B15, HLA-DRw6 are strongly associated with lupus (Arthritis Res Ther 2015;17:182)
Etiology
- Sun exposure (Int J Womens Dermatol 2019;5:320)
- Smoking is associated with discoid lupus erythematosus and tumid lupus erythematosus (Clin Cosmet Investig Dermatol 2019;12:707)
Clinical features
- Discoid lupus erythematosus: erythematous and scaly plaques with hyperpigmentation; scalp involvement often results in scarring, dyspigmentation and alopecia (Postepy Dermatol Alergol 2019;36:739)
- Tumid lupus erythematosus: erythematous plaques without scale or dyspigmentation
- Chilblain lupus erythematosus: violaceous patches or plaques incited or exacerbated by cold exposure
- Lupus panniculitis and profundus: tender subcutaneous nodules with or without epidermal or pigmentary changes
Diagnosis
- Blood draw
- Skin biopsy
Laboratory
- Localized discoid lupus erythematosus: 5% of cases associated with systemic lupus erythematosus (Int J Womens Dermatol 2017;3:S62)
- Generalized discoid lupus erythematosus: 30% of cases associated with systemic lupus erythematosus
- Tumid lupus erythematosus: no association with systemic lupus erythematosus
- In association with systemic disease: urinalysis, serum creatinine for renal disease, CBC / differential for anemia, leukopenia, lymphopenia, thrombocytopenia (Dtsch Arztebl Int 2015;112:423)
Prognostic factors
- Discoid lupus erythematosus is associated with low mortality but significant impact on quality of life due to dysesthesia, pruritus, scarring alopecia and dyspigmentation
- Early treatment helps to reduce scarring and atrophy (J Am Acad Dermatol 2011;64:849)
Case reports
- 19 year old man with lupus panniculitis (Medicine (Baltimore) 2016;95:e3429)
- 35 year old man with ankylosing spondylitis and discoid lupus erythematosus (North Clin Istanb 2019;6:412)
- 40 year old man with squamous cell carcinoma of the lower lip and discoid lupus erythematosus (Our Dermatol Online 2019;10:376)
- 54 year old woman with oral sarcoid granuloma associated with discoid lupus erythematosus (J Oral Diag 2017;02:e20170028)
- 57 year old African American woman with comedonal variant of chronic cutaneous lupus erythematosus (JAAD Case Rep 2019;5:801)
- 57 year old woman with sarcoidosis and chronic cutaneous lupus erythematosus (BMC Dermatol 2016;16:14)
Treatment
- Antimalarials such as hydroxychloroquine, acitretin and topical steroids (Cochrane Database Syst Rev 2017;5:CD002954, Am J Clin Dermatol 2016;17:135)
- Direct sunlight avoidance
Clinical images
Microscopic (histologic) description
- Discoid lupus erythematosus
- Epidermal atrophy or hypertrophy, attenuation (flattening) of rete ridges
- Hyperkeratosis and follicular keratotic plugging
- Basement membrane thickening
- Vacuolar degeneration with keratinocyte apoptosis in the basilar layer
- Cell poor infiltrate in the papillary dermis in most cases
- Lichenoid (band-like) infiltrate composed of lymphocytes in some cases
- Dilated blood vessels, edema and pigmentary incontinence in dermis
- Dense superficial and deep lymphocytic infiltrate around the adnexae and vessels
- Also increased plasma cells
- Increased mucin in dermis
- Pilosebaceous unit damage and broad zones of dermal fibrosis (Int J Womens Dermatol 2017;3:S62)
- Tumid lupus erythematous
- Increased mucin in dermis
- Lymphocytic infiltrate in superficial and deep dermis, around the adnexae and vessels
- Chilblain lupus erythematosus
- Vacuolar interface change
- Papillary dermal edema
- Lymphocytic infiltrate in superficial and deep dermis and surrounding eccrine glands
- Variable vasculitic or vasculopathic changes (Br J Dermatol 2020;183:729)
- Lupus panniculitis and profundus
- Lupus profundus
- Hyperkeratosis and acanthosis
- Vacuolar interface change
- Increased mucin in dermis
- Lymphocytic infiltrate in superficial and deep dermis, around the adnexae and vessels
- Lupus panniculitis and profundus
- Lymphocytes, plasma cells, neutrophils with karyorrhectic debris and rare eosinophils in subcutaneous fat lobules and septa
- Hyaline fat necrosis
- Lupus profundus
Microscopic (histologic) images
Virtual slides
Immunofluorescence description
- Positive lupus band test: IgM is most common, while IgG is most specific and C3 may also be paired with IgM (PLoS One 2013;8:e70983)
- Granular IgM is continuous in sun exposed skin and interrupted in sun protected skin
- In patients with systemic lupus, lupus band is identified in lesional and nonlesional skin, while in cutaneous lupus, immunoreactant deposition is only identified in lesional skin
- However, lupus band can be observed in sun exposed skin of healthy patients; specificity is increased in sun protected skin
- Possibility of in vivo antinuclear antibody in patients who have systemic lupus erythematosus (Ann Rheum Dis 1990;49:163)
- IgM also highlights cytoid bodies in cutaneous lupus erythematosus (J Cutan Pathol 2007;34:481)
Positive stains
- Alcian blue or colloidal iron highlights interstitial and periadnexal mucin deposition
- Periodic acid Schiff (PAS) and collagen IV highlight basement membrane thickening in discoid lupus erythematosus
- CD123 in discoid lupus erythematosus and chilblain lupus erythematosus (Int J Trichology 2020;12:62)
- More CD123 positive plasmacytoid dendritic cells in dermis and fewer in epidermis of discoid lupus erythematosus compared with dermatomyositis (J Cutan Pathol 2008;35:452)
- CD123 fails to distinguish chilblain lupus erythematosus from idiopathic perniosis (Am J Dermatopathol 2018;40:265)
Electron microscopy description
- Subendothelial, subepithelial and mesangial deposits in discoid lupus erythematosus (Front Immunol 2016;7:647)
Sample pathology report
- Scalp, biopsy:
- Discoid lupus erythematosus (see comment)
- Comment: There is epidermal atrophy and follicular ostia are dilated and contain keratotic plugs. There is vacuolar degeneration in the basilar layer along with basement membrane thickening. There is a superficial and deep perivascular and periadnexal lymphocytic infiltrate with plasma cells. Dermal mucin is increased.
- Central chest, biopsy:
- Tumid lupus erythematosus (see comment)
- Comment: There is a superficial and deep perivascular and periadnexal lymphocytic infiltrate with plasma cells. Dermal mucin is increased.
- Shoulder, biopsy:
- Lupus profundus (see comment)
- Comment: There is epidermal hyperkeratosis and acanthosis. There is vacuolar degeneration in the basilar layer. Dermal mucin is increased. There is a superficial and deep perivascular, periadnexal and subcutaneous lymphocytic infiltrate with plasma cells. There is hyaline fat necrosis with karyorrhexis.
Differential diagnosis
- Discoid lupus erythematosus:
- Lymphocytic infiltrate of Jessner:
- Variant of tumid lupus erythematosus
- Erythematous annular plaques that heal without scarring or dyspigmentation
- Superficial and deep perivascular and periadnexal lymphocytic infiltrate with plasma cells
- Absence of epidermal changes and mucin in dermis is differentiating feature from discoid lupus erythematosus
- Lymphocytic infiltrate of Jessner:
- Lichen planus variants:
- Hypertrophic lichen planus:
- Violaceus, hyperkeratotic and lichenified plaques on extensor surfaces
- Parakeratosis, hypergranulosis and pseudoepitheliomatous hyperplasia
- Lichenoid interface dermatitis limited to base of rete ridges
- Differentiated from hypertrophic (verrucous) discoid lupus erythematosus by presence of eosinophils and absence of periadnexal and deep perivascular inflammation
- Lichen planopilaris:
- Perifollicular scale, erythema and scarring alopecia
- Lichenoid interface tissue reaction affects infundibulum and isthmus, with or without involvement of the epidermis
- Differentiated from discoid lupus erythematosus by absence of deep and perieccrine inflammation and mucin
- Lupus erythematosus and lichen planus overlap:
- Differentiated from discoid lupus erythematosus based on combination of histopathologic and immunopathologic features
- Epidermal changes typical of lichen planus along with deep and periadnexal inflammation
- Immunofluorescence findings of lupus erythematosus and lichen planus
- Hypertrophic lichen planus:
- Rosacea:
- Presents with erythematous papules and pustules and involvement of nasolabial folds
- No pigmentary changes, scarring or atrophy
- Lymphohistiocytic perifolliculitis, with variable perifollicular and intrafollicular neutrophils
- Lupus panniculitis:
- Subcutaneous panniculitis-like T cell lymphoma (SPTCL):
- Atypical T cells in subcutis which rim adipocytes
- Typically lacks plasma cells, eosinophils or neutrophils
- Bean bag cells: histiocytes which demonstrate erythrophagocytosis and cytophagocytosis
- Monoclonal T cell receptor gene rearrangement
- Positive for CD8, TCR beta F1 and cytotoxic markers
- Negative for CD4, TCR delta, CD56 and EBER
- Subcutaneous panniculitis-like T cell lymphoma (SPTCL):
- Chilblain lupus erythematosus:
- Perniosis:
- Painful erythematous or edematous nodules on acral skin following exposure to cold
- Clinical context variable: chronic cutaneous lupus erythematosus (CCLE) without systemic disease, chilblain lupus erythematosus in the context of systemic lupus erythematosus, idiopathic perniosis and COVID-19
- Vacuolar interface change is common
- Marked subepidermal edema
- Superficial and deep perivascular and perieccrine lymphocytic infiltrate
- Vasculitis variable
- Interstitial fibrin and increased mucin favor chilblain lupus erythematosus over idiopathic perniosis (Am J Dermatopathol 2018;40:265)
- Perniosis:
- Tumid lupus erythematosus:
- Polymorphous light eruption:
- Pruritic papules, vesicles and plaques with photodistribution
- Marked papillary dermal edema
- Superficial and deep perivascular lymphocytic infiltrate
- Distinguished from tumid lupus by marked edema, absence of mucin and lack of significant periadnexal inflammation
- Polymorphous light eruption:
Additional references
Board review style question #1
A 37 year old woman presents with erythematous plaques without dyspigmentation or scale on her upper trunk. The biopsy demonstrates a perivascular and periadnexal lymphocytic infiltrate without epidermal changes. Which of the following statements is correct?
- Edema is typically prominent
- The histopathologic findings include prominent keratinocyte necrosis in the basal layer of the epidermis and follicular epithelium
- These findings represent polymorphous light eruption
- These findings represent tumid lupus erythematous
- This condition is associated with systemic lupus erythematosus
Board review style answer #1
D. The cutaneous findings represent tumid lupus erythematosus
Comment Here
Reference: Chronic cutaneous lupus
Comment Here
Reference: Chronic cutaneous lupus
Board review style question #2
A 32 year old woman presents with hyperpigmented plaques on the conchal bowl and a scarring alopecia. Skin biopsy revealed an atrophic epidermis, basement membrane thickening with vacuolar interface change and increased mucin in dermis. There is a superficial and deep and perieccrine lymphocytic infiltrate. Which of the following is the best diagnosis?
- Discoid lupus erythematosus
- Lichen planopilaris
- Lymphocytic Infiltrate of Jessner
- Polymorphous light eruption
- Rosacea
Board review style answer #2