Skin nontumor
Dermal perivascular and vasculopathic reaction patterns
Erythema elevatum diutinum


Minor changes: 5 January 2021

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Kiran Motaparthi, M.D.
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Cite this page: Motaparthi K. Erythema elevatum diutinum. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorerythemaelevatum.html. Accessed January 18th, 2021.
Definition / general
  • Rare, chronic fibrosing vasculitis associated with a variety of systemic diseases
Essential features
  • Red-brown plaques and nodules on extensor and acral surfaces
  • Histopathology reflects spectrum of overlapping findings dependent on stage of disease
    • Early: neutrophilic infiltrate and leukocytoclastic vasculitis
    • Established: mixed infiltrate with granulomas, granulation and fibrosis
    • Late: prominent fibrosis obscures sparse neutrophils; xanthomatization
ICD coding
  • ICD-10: L95.1 - erythema elevatum diutinum
Epidemiology
  • Rare
  • Most commonly affects adults between 30 - 60 years of age but earlier onset observed in association with human immunodeficiency virus (HIV) infection
  • No sex or racial predilection (J Am Acad Dermatol 1992;26:38)
Sites
Pathophysiology
Etiology
  • Unknown
Clinical features
  • Erythematous or purpuric patches or thin, soft plaques in early disease
  • Red-brown, violaceous or yellow-brown indurated papules, plaques or nodules in established or late disease; lesions may be asymptomatic or associated with pruritus or dysesthesia (J Am Acad Dermatol 1992;26:38)
  • Bulky nodules may be associated with underlying HIV infection (J Cutan Pathol 2018;45:94)
  • Annular plaques simulating granuloma annulare are rare (J Cutan Pathol 2019;46:97)
  • Vesiculobullous presentations are also described (Clin Exp Dermatol 2017;42:777, Indian J Dermatol 2020;65:164)
  • Associated disorders and systemic manifestations:
    • Infections: HIV, hepatitis B, hepatitis C and Streptococcus
    • Gastrointestinal: inflammatory bowel disease and celiac disease
    • Hematologic: IgA monoclonal gammopathy, paraproteinemia, Waldenström macroglobulinemia, lymphoma
    • Autoimmune connective tissue: granulomatosis with polyangiitis, dermatomyositis, systemic lupus erythematosus, rheumatoid arthritis, relapsing polychondritis
    • Ocular: keratitis, scleritis, uveitis, blindness
    • Other: pregnancy, solid organ malignancy (J Eur Acad Dermatol Venereol 2014;28:1594)
Diagnosis
  • Skin biopsy with histologic examination of tissue
Laboratory
  • Given the associated disorders, the following laboratory evaluation is recommended:
    • Complete blood count
    • HIV and hepatitis B and C serologies
    • Serum protein electrophoresis or immunofixation
    • Antistreptolysin O
    • Antinuclear antibody
    • Antineutrophil cytoplasmic antibodies (ANCA) testing
Prognostic factors
Case reports
Treatment
  • Oral dapsone monotherapy is effective in 80% of cases, particularly in early disease
  • Alternative medical treatments can be combined with dapsone for refractory cases
    • Topical, intralesional or oral corticosteroids
    • Sulfonamides: sulfasalazine, sulfapyridine
    • Antimicrobials: tetracyclines, macrolides
    • Colchicine
  • Medical therapy including dapsone ineffective for chronic lesions with prominent fibrosis
Clinical images

Contributed by Kiran Motaparthi, M.D.
Plaque on the elbow

Plaque on the elbow

Nodules on the shin and foot

Nodules on the shin and foot

Nodule on the hand

Nodule on the hand

Microscopic (histologic) description
  • Histopathologic features vary based on the stage of disease
    • Early:
      • Leukocytoclastic vasculitis with fibrinoid necrosis of small vessel walls, along with predominantly neutrophilic dermal infiltrate
      • Prominent dermal edema may simulate Sweet syndrome or result in vesiculation (J Am Acad Dermatol 1992;26:38)
    • Established:
      • Polymorphous infiltrate includes neutrophils but also lymphocytes, histiocytes, plasma cells and eosinophils
      • Granulation tissue
      • Perivascular fibrosis in onion skinning pattern
      • Granuloma formation is variable (Clin Exp Dermatol 1992;17:87)
    • Late:
Microscopic (histologic) images

Contributed by Kiran Motaparthi, M.D.
Neutrophilic infiltrate

Neutrophilic infiltrate

Nodular mixed infiltrate

Nodular mixed infiltrate

Dilated vessels and prominent fibrosis

Dilated vessels and prominent fibrosis

Small vessel vasculitis

Small vessel vasculitis

Storiform fibrosis

Storiform fibrosis

Virtual slides

Images hosted on other servers:

Erythema elevatum diutinum

Immunofluorescence description
Negative stains
Electron microscopy description
Sample pathology report
  • Skin, extensor forearm, punch biopsy:
    • Erythema elevatum diutinum
Differential diagnosis
Board review style question #1

    A 34 year old man presents with multiple firm red-brown nodules distributed over the acral and extensor surfaces. Which of the following features is observed in all stages of this disease?

  1. Cholesterol clefts
  2. Dermal edema
  3. Fibrosis
  4. Granulomas
  5. Neutrophils
Board review style answer #1
E. Neutrophils. This is erythema elevatum diutinum.

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Reference: Erythema elevatum diutinum
Board review style question #2

    Which of the following disorders demonstrates overlapping histopathologic features with early erythema elevatum diutinum?

  1. Palisaded neutrophilic granulomatous dermatitis
  2. Rheumatoid nodule
  3. Sclerotic fibroma
  4. Sweet syndrome
  5. Tuberous xanthoma
Board review style answer #2
D. Sweet syndrome

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Reference: Erythema elevatum diutinum
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