22 - 143 - PIGMENTED PURPURIC DERMATOSES

Question 1

Common histopathologic features of PPD

Answer 1

superficial lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition

Question 2

Pigmented purpuric eruptions (also called pigmented purpuric dermatoses [PPDs]) are a group of dermatoses that are characterized by

Answer 2

PPT

petechiae, pigmentation, and, occasionally, telangiectasia.

Question 3

most common of the PPDs to occur in children

Answer 3

PROGRESSIVE PIGMENTARY DERMATOSIS SCHAMBERG DISEASE

Question 4

irregularly shaped reddish-brown patches with “pin head sized reddish puncta, closely resembling grains of cayenne pepper” over the legs

Answer 4

PROGRESSIVE PIGMENTARY DERMATOSIS (SCHAMBERG DISEASE)

Question 5

annular patches of follicular and punctate reddish-brown macules with telangiectasias and purpura on the lower extremities

Answer 5

PURPURA ANNULARIS TELANGIECTODES (MAJOCCHI PURPURA)

Question 6

This subtype of PPD is characterized by its distinctive annular pattern

Answer 6

PURPURA ANNULARIS TELANGIECTODES (MAJOCCHI PURPURA)

Question 7

Individual lesions begin as punctate telangiectatic macules that extend peripherally with central hypopigmentation or slight atrophy.

Answer 7

PURPURA ANNULARIS TELANGIECTODES (MAJOCCHI PURPURA)

Question 8

As opposed to other subtypes of PPDs, this type presents most commonly in young adult females.

Answer 8

Majocchi Purpura

Question 9

Question 10

Answer 10

Schamberg/ Progressive Pigmentary Dermatosis

Question 11

Answer 11

Majocchi Purpura/ Purpura Annularis telangiectoides

Question 12

Answer 12

Gougerot and Blum/ Pigmented Purpuric Lichenoid Dermatosis

Question 13

Answer 13

Doucas and Kapetanakis/ Eczematoid-like purpura

Question 14

This subtype is clinically distinguished by the presence of reddish-brown round or polygonal lichenoid papules and plaques, with a background of purpura or telangiectasias.

Answer 14

PIGMENTED PURPURIC LICHENOID DERMATOSIS OF GOUGEROT AND BLUM

Question 15

T/F: Lichenoid describes this clinical appearance of lichenoid papules and plaques rather than the underlying histology in PIGMENTED PURPURIC LICHENOID DERMATOSIS OF GOUGEROT AND BLUM

Answer 15

True

Question 16

mild scaling overlying pinpoint erythematous macules and patches with associated pruritus

Answer 16

ECZEMATID-LIKE PURPURA OF DOUCAS AND KAPETANAKIS

Question 17

Histopathologically, spongiosis is present, in addition to the classic histopathologic features of PPD.

Answer 17

ECZEMATID-LIKE PURPURA OF DOUCAS AND KAPETANAKIS

Question 18

This subtype spreads rapidly over a period of 15 to 30 days and will subsequently fade without treatment over several months to years, although recurrence is possible.

Answer 18

ECZEMATID-LIKE PURPURA OF DOUCAS AND KAPETANAKIS

Question 19

lichen refers to both its clinical and histopathologic features in this type of PPD

Answer 19

LICHEN AUREUS

Question 20

This PPD subtype presents with more localized and persistent lesions with circumscribed macules or papules that are a distinctive gold, rust, or orange color

Answer 20

Lichen Aureus

Question 21

presents acutely with widely disseminated orange-brown to purpuric lesions associated with severe pruritus

Answer 21

ITCHING PURPURA (DISSEMINATED PRURIGINOUS ANGIODERMATITIS)

Question 22

The lesions first appear on the dorsal feet or lower extremities and then spread upward, sometimes with involvement of the trunk. Purpuric lesions are more apparent along the waistline, axilla, antecubital and popliteal fossae. Although this subtype of PPD has a chronic course, spontaneous remissions are possible.

Answer 22

ITCHING PURPURA (DISSEMINATED PRURIGINOUS ANGIODERMATITIS)

Question 23

This PPD is clinically distinguished by its linear or segmental distribution.

It tends to have a favorable prognosis, with spontaneous resolution occurring more commonly than in the other subtypes of PPDs.

Answer 23

UNILATERAL LINEAR CAPILLARITIS (SEGMENTAL PIGMENTED PURPURA)

Question 24

In addition to the classic histopathologic features of a PPD, a granulomatous infiltrate is present.

The granulomatous infiltrate is most commonly located in the papillary dermis, but may be in the mid to deep dermis separate from a more superficially located lichenoid infiltrate.

Answer 24

GRANULOMATOUS PIGMENTED PURPURA

Question 25

Hyperlipidemia is a relatively common association in this subtype

Answer 25

GRANULOMATOUS PIGMENTED PURPURA

Question 26

3 proposed pathogenesis of PPD

Answer 26

1. disturbance or weakness of cutaneous blood vessels, leading to capillary fragility and erythrocyte extravasation
2. PPDs develop from a humoral immune response
3. result of a cellular immune response

Question 27

Question 28

Question 29

Question 30

Clinical course and prognosis of PPDs

Answer 30

benign and commonly asymptomatic. In general, PPDs are chronic with flares and remissions.

Question 31

Exceptions to the chronic course of PPD include which subtypes?

Answer 31

unilateral linear capillaritis and drug-induced PPDs, which tend to have shorter clinical courses and more favorable overall prognoses.

Question 32

Oral treatment options to PPD

Answer 32

• Oral bioflavonoid (rutoside, 50 mg twice daily) and ascorbic acid (500 mg twice daily)
• calcium dobesilate, 500 mg twice daily
• Griseofulvin, 500 mg to 750 mg daily,
• Colchicine, 0.5 mg twice daily
• pentoxifylline 400 mg TID x 2 months
• topical betamethasone dipropionate cream, 0.05% twice daily, for 2 months
• immunosuppresants (methotrexate, systemic steroids, cyclosporine)

Question 33

common initial therapies in PPD

Answer 33

• topical steroids
• antihistamines

Others:
- compression stockings (treat associated venous insufficiency)
- topical pimecrolimus
- intralesional steroids