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Pigmented Purpuric Dermatoses (PPD) or Capillaritis

Pathogenesis
  • Diseases w/petechial hemorrhage 2nd to minimal inflammation of capillaries in dermis. Unknown reason for inflammation.
  • No systemic association. No coagulopathy association. Idiopathic.
Differential Diagnosis
  • Cutaneous small vessel vasculitis: to distinguish from lichenoid variant
  • Capillaritis: mycosis fungoides; ACD; non-allergic reactions to topical Rx; Rx eruptions; RA; LE; hypergammaglobulinemic purpura of Waldenström
  • Dermal hemorrhage in venous hypertension (more diffuse hemosiderin deposits)
Treatment
  • Tx often not effective. Counselling pt about benign & chronic / relapsing course of disease.
  • Compression stockings
  • Topical: CS if pruritus or erythema (also IL CS but risk of atrophy & doesn’t prevent new lesions)
  • Phototherapy: PUVA or NB-UVB for more extensive disease
  • Systemic: pentoxifylline (400 mg TID); ascorbic acid (500mg BID) + rutoside (50mg BID) also reported as treatment; griseofulvin 500-750 mg DIE / colchicine 0,5mg BID / minocycline (few reports showed benefit)

Epidemiology
  • Most common form
  • Adults: 50 yo M > F (more common in adults than children)
  • Most common PPD in children
Location
  • Rarely, familial
  • Lower legs > thighs, buttocks, trunk, arms
Clinical features
  • Chronic, no symptoms
  • Yellow-reddish-brown patches in crops with petechiae (cayenne pepper) and irregular borders
Pathology

PPD:

  • RBC extravasation
  • Endothelial swelling
  • Perivascular lymphocytic infiltrate
  • Hemosiderin in macrophages

Epidemiology
  • Ø common
  • F > M
  • Adolescents / young adults
  • Rarely, familial
Location
  • Trunk, proximal lower extremities
Clinical features
  • Chronic, ø sx
  • 1-3 cm annular plaques +/- slow expansion
  • Punctate petechiae & telangiectasias at borders
Pathology

PPD:

  • RBC extravasation
  • Endothelial swelling
  • Perivascular lymphocytic infiltrate
  • Hemosiderin in macrophages

Epidemiology
  • Rare
  • Mid-aged / older men
Location
  • Lower extremities
Clinical features
  • Pruritic
  • Petechial or purpuric macules, papules, patches
  • Presence of scales
Pathology
  • PPD + spongiosis w/parakeratosis

Epidemiology
  • Rare
  • Mid-aged / older men
Location
  • Lower extremities
Clinical features
  • Chronic, +/- pruritus
  • Mix of 2 lesions: Schamberg + purpuric lichenoid papules
Pathology
  • PPD + lichenoid infiltrate + spongiosis w/parakeratosis
 

Epidemiology
  • Rare
  • Young adults (Males)
Location
  • Lower extremities (perforator vein)
Clinical features
  • Solitary patch
  • Golden-rust-purple-brown color
Pathology
  • PPD + lichenoid infiltrate

Epidemiology
  • Rare
  • Children and adolescents
  • Most favorable prognosis
Location
  • One extremity
Clinical features
  • Linear and unilateral
  • Lesions similar to Schamberg or lichen aureus
  • Spontaneous resolution
Pathology
  • PPD

Epidemiology
  • Very rare
  • Middle age
  • Asians
Location
  • Lower extremities
Clinical features
  • Brown patch with hemorrhagic papules
Pathology
  • Hemorrhagic granulomatous inflammation in upper dermis