Chapter 143 - Pigmented Purpuric Dermatoses

Question 1

PPD is characterized by (3)

Answer 1

Petechiae
Pigmentation
Telangiectasia

Question 2

Common histopathologic features of PPDs

Answer 2

Superficial lymphocytic infiltrate
Erythrocyte extravasation
Hemosiderin deposition

Question 3

Ppds occur more frequently in males, except for ____, which occur more commonly in females

Answer 3

Majocchi purpura (purpura annularis telangiectodes)

Question 4

Most common of PPDs to occur in children

Answer 4

Schamberg disease

Progressive Pigmentary Dermatosis

Question 5

It is an eruption of irregularly shaped reddish brown patches with pin head sized reddish puncta resembling cayenne pepper

Answer 5

Schamberg disease

Progressive Pigmentary Dermatosis

Question 6

It presents with annular patches of follicular and punctate reddish brown macules with telangiectasias and purpura on lower extremities

Answer 6

Majocchi purpura

Purpura annularis Telangiectodes

Question 7

It is characterized by presence of reddish brown round or polygonal lichenoid papules and plaques, with a background of purprura or telangiectasia

Answer 7

Pigmented purpuric lichenoid dermatosis of Gougerot and Blum

Question 8

It can be mistaken for Kaposi sarcoma

Answer 8

Pigmented purpuric lichenoid dermatosis of Gougerot and Blum

Question 9

Asymptomatic, seasonal eruption occurring in spring and summer with mild scaling overlying pinpoint erythematous macules and patches with associated pruritus

Answer 9

Eczematid-like Purpura of Doucas and Kapetanakis

Question 10

Aside from common histopathologic features like superficial lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition, spngiosis can also be observed in

Answer 10

Eczematid-like purpura of Doucas and Kapetanakis

Question 11

All PPDs have abrupt presentation, except for

Answer 11

Schamberg disease

PPD Lichenoid of Gougerot and Blum

Question 12

Presents with more localized and persistent lesions with circumscribed macules or papules that are a distinctive gold, rust, or orange color common in young adult males

Answer 12

Lichen aureus/Lichen purpuricus

Question 13

Often localized to one lower extremity with segmental distribution

Answer 13

Lichen aureus

Question 14

Presents with disseminated orange-brown to purpuric lesions associated with severe pruritus

Answer 14

Itching purpura (disseminated pruriginous angiodermatitis)

Question 15

PPDs with linear and pseudodermatomal pattern

Answer 15

Unilateral linear capillaritis

Segmental pigmented purpura

Question 16

Subtypes of unilateral linear capillaritis

Answer 16

Quadrantic capillaropathy

Segmenta pigmented purpura

Question 17

Spontaneous resolution occurs more commonly than in other subtypes of PPD

Answer 17

Unilateral Linear capillaritis

Question 18

Common subtype in Asian descent

Answer 18

Granulomatous variant of PPDs

Question 19

Granulomatous variant of PPD has been relatively associated with

Answer 19

Hyperlipidemia

Question 20

Most likely to predict progression to MF

Answer 20

T cell monoclonality

Question 21

The most important feature to distinguish early MF from others is with the presence of

Answer 21

Lymphocytes with extremely convoluted, medium to large nuclei, single or clustered in epidermis and in small sheets in the dermis

Question 22

Important role in the pathogenesis of PPD

Answer 22

Cellular reaction especially with Langerhans cells

Question 23

In early Schamberg disease, the ff cell types (2) predominates

Answer 23

CD4+ cells

CD1a dendritic cells

Question 24

3 theories on pathogenesis of PPDs

Answer 24

1. Disturbance or weakness of cutaneous blood vessels
2. Humoral immune response (C3,C1q,IgM, or IgA)
3. Cellular immune response
   (Lymphocytes, macrophages, Langerhans cells)

Question 25

Majority of PPD cases are ___ (etiology)

Answer 25

Idiopathic

Question 26

Diagnosis is gemrally made ___ and supported by ____

Answer 26

Clinically; histopath exam

Question 27

Measurement of capillary fragility by application of sphygmomanometer

Answer 27

Hess test

Question 28

Increased capillary fragility is consistently seen in PPD | True or False

Answer 28

False

Question 29

Hemosiderin can be highlighted by ____ stain

Answer 29

Prussian blue

Question 30

In older lesions, extravasated RBCs may no longer be present | True or False

Answer 30

True

Question 31

PPD is a true capillaritis | True or False

Answer 31

False | Due to lack of fibrin in luminal walls and absence of thrombi

Question 32

Subtype of PPD with granulomatous infiltrate in the papillary dermis, or in mid to deep dermis, which is separated from a more superficial lichenoid infiltrate

Answer 32

Granulomatous PPD

Question 33

Variant of PPD, on HPx presents as band like mononuclear infiltrate in upper dermis separated from epidermis by thin rin of uninvolved collagen

Answer 33

Lichen aureus

Question 34

Variant of PPDs with shorter course and more favorable overall prognosis

Answer 34

Unilateral linear capillaritis | Drug-induced PPDs

Question 35

Common initial therapies for PPD

Answer 35

Topical steroids | antihistamines

Question 36

Treatment with ___ BiD for ____ weeks cleared lichen aureus lesions

Answer 36

Topical pimecrolimus; 10

Question 37

___ given for 4 weeks lead to clearance in all lesions with maintenance of remission 3 months after treatment for chronic PPD

Answer 37

Oral bioflavanoid (Rutoside 50mg BID) + Ascorbic acid 500mg BiD

Question 38

Used for recalcitrant Schamberg disease

Answer 38

Colchicine 0.5mg BID

Question 39

MOA of Pentoxifylline 400mg TiD for PPD

Answer 39

Inhibits T cell adherence to endothelial cells and keratinocytes

Question 40

Immunosuppressants (oral corticosteroids, MTX, cyclosporine) are rarely indictaed for the benign nature of this condition TRUE or FALSE

Answer 40

True

Question 41

Used for Majocchi purpura

Answer 41

MTX 15 mg weekly for 4 weeks

Question 42

MOA of PUVA in treating PPD

Answer 42

Inhibits T cell intereukin 2 production leading to resolution of perivascular lymphocytic infiltrate

Question 43

Affects both immunomodulation and vascular destruction

Answer 43

PDT

Question 44

Other treatments for PPD

Answer 44

Ca dobesilate 500mg BiD for 3 mos, sustained for 1 year Griseofulvin 500-750mg OD for 1 month Minocycline

Question 45

Sucessful clearance of Schamberg using a ___ nm PDL after 5 monthly treatments

Answer 45

595 nm

Question 46

Drug-induced PPD are more likely to present in lower extremities True or False

Answer 46

False, more likely to be generalized

Question 47

Lab testing is required for diagnosis | True or False

Answer 47

False, not required

Question 48

Rule out other causes of purpura

Answer 48

Coagulation studies

Question 49

Used to confirm diagnosis of Drug-induced PPD

Answer 49

Rechallenge

Question 50

___ &; ___ account for lesions localizing in lower extremities

Answer 50

Gravity and increased venous pressure

Question 51

Itching purpura are more apparemt along (4)

Answer 51

Waistline Axilla Antecubital Popliteal fossa

Question 52

Suspicious for MF: (4)

Answer 52

PPDs with large areas of confluence Reticular arrangement Superimposed violaceous hue Pruritus present/relapsing for years

Question 53

Possible agents that cause contact dermatitis leading to PPDs (8)

Answer 53

``` Benzoyl peroxide Black rubber Cobalt Disperse dyes Epoxy resin Eutectic mixture of local anesthetics Methylmethacrylate PPD ```

Question 54

Hess test is reliable as capillary fragility is consistently seen in PPD True or False

Answer 54

False, not reliable, not consistent

Question 55

MOA of PUVA for PPD

Answer 55

Inh T cell IL2 production, resolution of perivascular infiltrate

Question 56

For immunomodulation and vascular destruction

Answer 56

Photodynamic therapy