143: Pigmented Purpuric Dermatoses Flashcards
What are the common characteristics of pigmented purpuric dermatoses?
Pigmented purpuric dermatoses are characterized by petechiae, pigmentation, and occasionally telangiectasia. They are most commonly located on the lower extremities but can also involve the trunk and upper extremities.
What is the clinical course of pigmented purpuric dermatoses?
Pigmented purpuric dermatoses are generally benign and asymptomatic but tend to run a chronic course with flares and remissions.
What is the most common subtype of pigmented purpuric dermatosis in children?
The most common subtype of pigmented purpuric dermatosis in children is Progressive Pigmentary Dermatosis (Schamberg disease), although it is more common overall in adults with peak incidence in the fifth decade.
How does Purpura Annularis Telangiectodes (Majocchi purpura) present clinically?
Purpura Annularis Telangiectodes (Majocchi purpura) is characterized by annular patches of follicular and punctate reddish-brown macules with telangiectases and purpura on the lower extremities. Individual lesions begin as punctate telangiectatic macules that extend peripherally with central hypopigmentation or slight atrophy. Lesions may be solitary or multiple in number and are generally asymptomatic, lasting several months with flares and remissions.
What are the histopathologic features shared by the subtypes of pigmented purpuric dermatoses?
The subtypes of pigmented purpuric dermatoses share the following common histopathologic features: superficial lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition.
Where are pigmented purpuric eruptions most commonly located?
On the lower extremities, but may also involve the trunk and upper extremities.
In which demographic do pigmented purpuric dermatoses occur more frequently?
They occur more frequently in males, except for the Majocchi subtype, which is seen more frequently in females.
What characterizes Purpura Annularis Telangiectodes (Majocchi purpura)?
Annular patches of follicular and punctate reddish-brown macules with telangiectases and purpura on the lower extremities.
What is the typical presentation of Progressive Pigmentary Dermatosis (Schamberg disease)?
Irregularly shaped reddish-brown patches with ‘pin-head’ sized reddish puncta resembling grains of cayenne pepper.
What is the characteristic feature of Eczematid-like purpura of Doucas and Kapetanakis?
Asymptomatic seasonal eruption occurring in the spring and summer, characterized by mild scaling overlying pinpoint erythematous lesions.
What is the most likely diagnosis for a 45-year-old male with reddish-brown patches on his lower extremities resembling grains of cayenne pepper?
The most likely diagnosis is Schamberg disease (Progressive Pigmentary Dermatosis). It is chronic with flares and remissions occurring indefinitely.
What subtype of pigmented purpuric dermatoses is characterized by annular patches of reddish-brown macules with telangiectases, and what is its typical duration?
This is Purpura Annularis Telangiectodes (Majocchi Purpura). It generally lasts several months with flares and remissions.
What are the common histopathologic features shared by all subtypes of pigmented purpuric dermatoses?
The common histopathologic features include superficial lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition.
What subtype of pigmented purpuric lesions is more common in females?
Majocchi Purpura is more common in females and is characterized by annular patches with telangiectases and purpura, often involving the lower extremities.
What subtype is characterized by reddish-brown patches resembling grains of cayenne pepper, and what is its most common site?
This is Schamberg Disease (Progressive Pigmentary Dermatosis), and its most common site is the lower extremities.
What are the common clinical features of Progressive Pigmentary Dermatosis (Schamberg disease)?
- Irregularly shaped reddish-brown patches with ‘pin-head’ sized reddish puncta resembling grains of cayenne pepper.
- Most common in children but overall more common in adults, peaking in the fifth decade.
- Insidious development, usually asymptomatic.
- Commonly located on the lower extremities, but can also involve the trunk and upper extremities.
- Chronic course with flares and remissions occurring indefinitely.
How does Purpura Annularis Telangiectodes (Majocchi purpura) present clinically?
- Characterized by annular patches of follicular and punctate reddish-brown macules with telangiectases and purpura on the lower extremities.
- Lesions begin as punctate telangiectatic macules that extend peripherally with central hypopigmentation or slight atrophy.
- Lesions may be solitary or multiple in number.
- Generally asymptomatic, lasting several months with flares and remissions.
- Most commonly presents in young adult females.
What is the epidemiological trend of Pigmented Purpuric Dermatoses (PPDs) in terms of age and gender?
- PPDs may present at any age but are most common in middle age.
- The granulomatous variant is reported more commonly in patients of Asian descent.
- PPDs occur more frequently in males, except for the Majocchi subtype, which is seen more frequently in females.
What distinguishes Pigmented Purpuric Lichenoid Dermatosis of Gougerot and Blum from other PPDs?
- It presents as reddish-brown round or polygonal lichenoid papules and plaques with a background of purpura or telangiectases.
- The term ‘lichenoid’ describes the clinical appearance rather than the underlying histology.
- Most commonly found on the lower extremities and runs a chronic course.
What are the clinical features of Lichen aureus?
Lichen aureus presents with localized and persistent lesions with circumscribed macules or papules that are distinctive gold, rust, or orange in color. Lesions are generally asymptomatic but can be intensely pruritic. Most commonly localized to one lower extremity, but other body sites can be involved. Predilection for young adult males with peak incidence in the second and third decades. Chronic course with stable or slowly progressive lesions. Histology shows a dense, band-like lichenoid infiltrate of inflammatory cells.
What distinguishes Unilateral linear capillarity (Segmental Pigmented Purpura) from other types of pigmented purpura?
Unilateral linear capillarity is distinguished by its linear or segmental distribution and has a favorable prognosis with spontaneous resolution occurring more commonly than in other subtypes.
What are the histopathologic features of Granulomatous pigmented purpura?
Granulomatous pigmented purpura is characterized by classic histopathologic feature of PPD combined with granulomatous infiltrate. Granulomatous infiltrate is most commonly seen in the papillary dermis but may also be found in the mid to deep dermis, separate from a more superficially located lichenoid infiltrate.
What is the relationship between Pigmented purpuric dermatosis and Mycosis fungoides?
The relationship between Pigmented purpuric dermatosis and Mycosis fungoides includes: Mycosis fungoides mimicking pigmented purpura clinically, pigmented purpura evolving into Mycosis fungoides, pigmented purpura that simulates Mycosis fungoides histologically, and T-cell monoclonality of PPD is most likely to predict progression to Mycosis fungoides.
What is a common association with Granulomatous pigmented purpura?
Hyperlipidemia is a relatively common association.
What is the typical course of Lichen aureus?
It runs a chronic course with stable or slowly progressive lesions.
What is the peak incidence age for young adult males with Lichen aureus?
In the second and third decades of life.
What subtype of pigmented purpuric dermatoses is characterized by seasonal eruptions, and how does it resolve?
This is Eczematid-like Purpura of Doucas and Kapetanakis. It spreads rapidly over 15-30 days and fades without treatment over months to years, though recurrence is possible.
What subtype of pigmented purpuric dermatoses is characterized by localized, gold-colored macules on one lower extremity?
This is Lichen Aureus. Its histological feature includes a dense, band-like lichenoid infiltrate of inflammatory cells.
What subtype of pigmented purpuric dermatoses is characterized by disseminated orange-brown lesions with severe pruritus?
This is Itching Purpura (Disseminated Pruriginous Angiodermatitis). It has a chronic course but spontaneous remissions are possible.
What subtype of pigmented purpuric dermatoses is characterized by linear purpuric lesions on one lower extremity?
This is Unilateral Linear Capillaritis (Segmental Pigmented Purpura). It has a favorable prognosis with spontaneous resolution occurring more commonly than in other subtypes.
What subtype of pigmented purpuric dermatoses is characterized by purpuric and brown macules on the lower extremities in a patient with hyperlipidemia?
This is Granulomatous Pigmented Purpura. Its histological hallmark includes a granulomatous infiltrate in the papillary dermis.
What is the most important histological feature to distinguish early mycosis fungoides from inflammatory mimickers?
The most important feature is lymphocytes with extremely convoluted, medium to large nuclei that are single or clustered in the epidermis.
What subtype of pigmented purpuric lesions is associated with hyperlipidemia, and what is its histological hallmark?
Granulomatous Pigmented Purpura is associated with hyperlipidemia, and its histological hallmark is a granulomatous infiltrate in the papillary dermis.
