Skin nontumor
Dermal perivascular and vasculopathic reaction patterns
Pigmented purpuric dermatosis
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Last author update: 27 March 2020
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PubMed Search: Pigmented purpuric dermatosis [TIAB] pathology
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 | Immunofluorescence description | Positive stains | Molecular / cytogenetics 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: Asadbeigi S, Momtahen S. Pigmented purpuric dermatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorpigmentedpurpuricdermatosis.html. Accessed June 8th, 2023.
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
- Pigmented purpuric dermatitis
- Pigment purpuric eruptions (Dermatol Clin 1985;3:165)
- Purpura simplex (J Am Acad Dermatol 1991;25:642)
- Capillaritis
- Inflammatory purpura without vasculitis
Epidemiology
- Rare
- Generally occurs in adults but can involve children (Pediatr Dermatol 2001;18:299)
- M:F = 1.1 (J Cutan Pathol 2018;45:325, Ann Dermatol 2015;27:404)
- Idiopathic, not associated with coagulopathies or thrombocytopenia
Sites
- Lower extremities > upper extremities > trunk (Ann Dermatol 2015;27:404)
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
- Unknown
- Rare familial cases
- Potential contributors: Systemic diseases, drugs, localized infection, diabetes mellitus, venous stasis, venous hypertension, capillary fragility, aberrant cell mediated immunity, contact allergens, hyperlipidemia (J Am Acad Dermatol 1991;25:642, J Am Acad Dermatol 2005;53:168, J Am Acad Dermatol 2002;46:942, Am J Clin Dermatol 2014;15:65, Am J Clin Dermatol 2017;18:383, Dermatol Clin 1985;3:165, Pediatr Dermatol 2001;18:299, Cutan Ocul Toxicol 2019;38:258, Dermatol Online J 2014;21)
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
- Schamberg disease (progressive pigmentary purpura)
- 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
- 31 year old man with mimicker of mycosis fungoides (Indian Dermatol Online J 2016;7:183)
- 45 year old woman with segmental lichen aureus (An Bras Dermatol 2017;92:704)
- 61 year old woman with pigmented purpuric lichenoid dermatosis of Gougerot-Blum (Ann Dermatol 2018;30:245)
- 61 year old Japanese woman with zosteriform pigmented purpuric dermatosis (Arch Dermatol 2007;143:1599)
- 68 year old woman with granulomatous pigmented purpuric dermatitis and Sjögren syndrome (Acta Derm Venereol 2013;93:95)
Treatment
- Treatment only in patients with associated symptoms or cosmetic concerns (StatPearls Publishing 2019)
- First line: discontinuation of potential contributing factors, topical steroid and antihistamines, topical tacrolimus or pimecrolimus
- Second line: phototherapy (Dermatology 2009;219:184, J Eur Acad Dermatol Venereol 2011;25:603)
- Other treatments: pentoxifylline, minocycline, methotrexate, colchicine (J Eur Acad Dermatol Venereol 2006;20:1378, StatPearls Publishing 2019)
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
- Direct immunofluorescence: deposition of fibrinogen, IgM or C3 in superficial dermal vessels (J Am Acad Dermatol 1991;25:642)
Positive stains
- Perls’ Prussian Blue Stain, Fontana-Masson stain: iron (hemosiderin)
- Mycosis fungoides / pigmented purpuric dermatosis overlap: reduction of CD7 and L selectin / CD62L (Am J Clin Pathol 2007;128:218)
Molecular / cytogenetics description
- Two principal categories: monoclonal and polyclonal (Am J Clin Pathol 2007;128:218)
- Can be a form of cutaneous T cell lymphoid dyscrasia based on monoclonality, preservation of persistent T cell clones and loss of pan T cell marker (Am J Clin Pathol 2007;128:218)
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
- Stasis dermatitis:
- Increase in superficial vessels without significant perivascular lymphocytic infiltrate
- Leukocytoclastic vasculitis / cutaneous vasculitis:
- Erythrocyte extravasation with vascular destruction, leukocytoclasia, fibrinoid necrosis, vascular luminal wall fibrin or thrombi in the vascular lumen (J Eur Acad Dermatol Venereol 2001;15:15)
- Mycosis fungoides:
- Large groups of lymphocytes in the upper stratum spinosum, intraepidermal lymphocytes, lymphocyte atypia, no papillary dermal edema
- T cell rearrangement study can help to distinguish (Am J Dermatopathol 1997;19:108)
- Eczematous dermatitis:
- Spongiotic dermatitis without any characteristic features of pigmented purpuric dermatosis, eosinophils are usually present
- Kaposi sarcoma:
- Vascular proliferation, dermal spindle cells and vascular slits (Cutis 1983;31:406)
Additional references
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?
- Drug eruption
- Cutaneous vasculitis
- Schamberg disease
- Mycosis fungoides
Board review style answer #1
Board review style question #2
- Which of the following is true about pigmented purpuric dermatoses?
- Early stages of mycosis fungoides have histologic overlap
- Fibrinoid necrosis of the vessel wall is a characteristic feature
- Hyperplasia of endothelial cells and an increase in superficial vessels are frequently observed
- A skin biopsy is necessary to distinguish multiple variants
Board review style 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.
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Reference: Pigmented purpuric dermatosis (PPD)
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Reference: Pigmented purpuric dermatosis (PPD)
