Skin nontumor
Chronic purpuric skin eruptions
Pigmented purpuric dermatosis (PPD)


Topic Completed: 27 March 2020

Minor changes: 27 March 2020

Copyright: 2020, PathologyOutlines.com, Inc.

PubMed Search: Pigmented purpuric dermatosis [TIAB] pathology


Sepideh Nikki Asadbeigi, M.D.
Shabnam Momtahen, M.D.
Page views in 2020 to date: 531
Cite this page: Asadbeigi S, Momtahen S. Pigmented purpuric dermatosis (PPD). PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skinnontumorpigmentedpurpuricdermatosis.html. Accessed May 24th, 2020.
Definition / general
  • Chronic purpuric skin disorders characterized by macules, patches and petechiae on a brown, red or yellow base resulting from hemosiderin deposition of erythrocyte extravasation
Essential features
  • Chronic purpuric skin disorders
  • Perivascular lymphocytic infiltrate along with endothelial cell damage and erythrocyte extravasation
  • Variable pigmentation resulting from the hemosiderin deposition
Terminology
ICD coding
  • ICD-10: L81.7 - Pigmented purpuric dermatosis
  • ICD-11: EF40.0 - Capillaritis
Epidemiology
Sites
Pathophysiology
  • Capillaritis in the dermis with possible concomitant venous hypertension leading to endothelial cell dysfunction and extravasation of red blood cells (StatPearls Publishing 2019)
  • Three suggested pathogenetic mechanisms include: disturbed humoral immunity, cellular immune reaction and capillary fragility
Etiology
Clinical features
  • Different subtypes mainly distinguished with clinical presentations (StatPearls Publishing 2019)
    • Schamberg disease (progressive pigmentary purpura)
      • Most common type, pinpoint to pinhead reddish puncta known as “grains of cayenne pepper,” purpuric macules forming patches, bilateral with a chronic course and numerous exacerbations and remissions
    • Purpura annularis telangiectodes of Majocchi disease
      • Annular patches with telangiectasias and central atrophy
      • Linear or arciform patches may be present, bilateral with a chronic course, relapses and remissions
    • Pigmented purpuric lichenoid dermatitis of Gougerot and Blum
      • Lichenoid and violaceous plaques with superimposed purpura, one to several centimeters in diameter, pruritic, may resemble Kaposi sarcoma, bilateral, chronic
    • Lichen aureus
      • Rusty yellow or gold lichenoid papules, patches and plaques, unilateral, acute onset, persistent course
    • Eczematid-like purpura of Doucas and Kapetanakis
      • Eczematous features, with scale overlying purpuric and petechial macules, pruritic with mild lichenification, acute onset and fluctuating course
    • Itching purpura or disseminated pruriginous angiodermatitis
      • Orange to brown purpuric macules with acute onset and chronic course
    • Unilateral linear capillaritis or segmental pigmented purpura and quadrantic capillaropathy
      • Linear, dermatomal or segmental distribution with self resolving course
    • Granulomatous pigmented purpura
      • Red to brown purpuric macules or papules, mostly involving knee and ankles, with a persistent course, more often in Asians
  • Pigmented purpuric dermatosis like variant of mycosis fungoides: can overlap or mycosis fungoides can evolve from pigmented purpuric dermatosis (Am J Dermatopathol 1997;19:108, Cutis 2014;94:297)
  • This form shows large areas of confluence, violaceous hue and pruritus usually lasting more than one year
Diagnosis
  • Diagnosis is often made by clinical presentation
  • Biopsy is used for confirmation
  • Blood work up and coagulation screening to exclude other conditions
Laboratory
  • No associated laboratory abnormalities
Prognostic factors
  • Variable progression and resolution course
  • Pigmented purpuric dermatosis with monoclonal lymphocytic infiltrate: extensive disease, approximately 40% can have clinical and pathologic features of mycosis fungoides (Am J Clin Pathol 2007;128:218)
  • Progression to mycosis fungoides rarely reported (Am J Dermatopathol 1997;19:108)
Case reports
Treatment
Clinical images

Images hosted on other servers:

Schamberg disease

Purpura annularis telangiectodes

Diet induced pigmented purpuric dermatosis

Pigmented purpuric dermatosis of Gougerot-Blum

Microscopic (histologic) description
  • Common histologic features in all subtypes: (StatPearls Publishing 2019)
    • Perivascular lymphocytic infiltrate: CD4+ lymphocytes, occasional CD1a dendritic cells and macrophages around superficial small blood vessels
    • Erythrocyte extravasations
    • Hemosiderin deposition and variable pigmentation
    • Mild epidermal spongiosis and lymphocyte exocytosis; present in all subtypes except lichen aureus
  • Possible associated features:
    • Epidermis: focal parakeratosis, basal layer vacuolar degeneration
    • Dermis: interface changes
    • Vascular: endothelial edema, proliferation with narrowing of vessel lumen, focal karyorrhectic debris
  • Progressive pigmentary dermatosis (Schamberg disease): (Dermatol Clin 1985;3:165)
    • Variable degree of epidermal spongiosis
  • Pigmented purpuric lichenoid dermatitis of Gougerot and Blum:
    • Dermatitis with band-like infiltrate in the upper dermis ± epidermal spongiosis
  • Itching purpura:
    • Marked spongiosis and neutrophils, hemosiderin can be minimal in early lesions
  • Eczematoid purpura of Doucas and Kapetanakis: (Ann Dermatol 2015;27:404)
    • Epidermal spongiosis
  • Lichen aureus: (Br J Dermatol 1985;112:307)
    • Lichenoid lymphocytic inflammation at the dermal / epidermal junction with Grenz zone
  • Granulomatous pigmented purpura: (Am J Dermatopathol 2012;34:746, J Dermatol 1996;23:551)
    • Dense granulomatous infiltrate in the papillary dermis, monocyte infiltrate, thickened capillaries and hemosiderin deposition
  • Pigmented purpura-like variant of mycosis fungoides: (Cutis 2014;94:297, Am J Clin Pathol 2007;128:218)
    • Prominent cytologic atypia of lymphocytes infiltrating into epidermis, Pautrier microabscesses
Microscopic (histologic) images

Contributed by Sepideh N. Asadbeigi, M.D. and Shabnam Momtahen, M.D.

Perivascular lymphocytes

Vacuolization


Erythrocyte extravasation

Hemosiderin deposition

Immunofluorescence description
Positive stains
Molecular / cytogenetics description
Sample pathology report
  • Skin, left lower leg, punch biopsy:
    • Pigmented purpuric dermatoses (see comment)
    • Comment: The histopathologic examination shows mild spongiosis with vacuolization of the basal layer of epidermis. There is cuffing of the small vessels in the papillary dermis by lymphocytes. Erythrocyte extravasation and superficial hemosiderin deposition in the dermis is present. The overall histologic features are consistent with pigmented purpuric dermatoses.
Differential diagnosis
Board review style question #1

    A 38 year old woman presents with a 2 week history of bilateral red macules on the shins. Physical examination reveals pinpoint petechia resembling grains of cayenne pepper on her legs. Past medical history is unremarkable. CBC and coagulation studies are within normal limits. The biopsy shows a superficial perivascular lymphocytic infiltrate with no luminal wall fibrin. Mild epidermal spongiosis, erythrocyte extravasation and siderophages are present. What is the most compatible diagnosis is this patient?

  1. Drug eruption
  2. Cutaneous vasculitis
  3. Schamberg disease
  4. Mycosis fungoides
Board review answer #1
C. Schamberg disease.

Reference: Pigmented purpuric dermatosis (PPD)

Comment Here
Board review style question #2
    Which of the following is true about pigmented purpuric dermatoses?

  1. Early stages of mycosis fungoides have histologic overlap
  2. Fibrinoid necrosis of the vessel wall is a characteristic feature
  3. Hyperplasia of endothelial cells and an increase in superficial vessels are frequently observed
  4. A skin biopsy is necessary to distinguish multiple variants
Board review answer #2
A. Early stages of mycosis fungoides have histologic overlap with pigmented purpuric dermatoses. Fibrinoid necrosis of the vessel wall is a feature of cutaneous vasculitis and should not be present in pigmented purpuric dermatoses. Hyperplasia of endothelial cells and an increase in superficial vessels are seen in stasis dermatitis which should be considered in differential diagnosis. Clinical presentation is commonly used to distinguish multiple variants of pigmented purpuric dermatoses.

Reference: Pigmented purpuric dermatosis (PPD)

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