Chapter 143 - Pigmented Purpuric Dermatoses Flashcards
PPD is characterized by (3)
Petechiae
Pigmentation
Telangiectasia
Common histopathologic features of PPDs
Superficial lymphocytic infiltrate
Erythrocyte extravasation
Hemosiderin deposition
Ppds occur more frequently in males, except for ____, which occur more commonly in females
Majocchi purpura (purpura annularis telangiectodes)
Most common of PPDs to occur in children
Schamberg disease
Progressive Pigmentary Dermatosis
It is an eruption of irregularly shaped reddish brown patches with pin head sized reddish puncta resembling cayenne pepper
Schamberg disease
Progressive Pigmentary Dermatosis
It presents with annular patches of follicular and punctate reddish brown macules with telangiectasias and purpura on lower extremities
Majocchi purpura
Purpura annularis Telangiectodes
It is characterized by presence of reddish brown round or polygonal lichenoid papules and plaques, with a background of purprura or telangiectasia
Pigmented purpuric lichenoid dermatosis of Gougerot and Blum
It can be mistaken for Kaposi sarcoma
Pigmented purpuric lichenoid dermatosis of Gougerot and Blum
Asymptomatic, seasonal eruption occurring in spring and summer with mild scaling overlying pinpoint erythematous macules and patches with associated pruritus
Eczematid-like Purpura of Doucas and Kapetanakis
Aside from common histopathologic features like superficial lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition, spngiosis can also be observed in
Eczematid-like purpura of Doucas and Kapetanakis
All PPDs have abrupt presentation, except for
Schamberg disease
PPD Lichenoid of Gougerot and Blum
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
Lichen aureus/Lichen purpuricus
Often localized to one lower extremity with segmental distribution
Lichen aureus
Presents with disseminated orange-brown to purpuric lesions associated with severe pruritus
Itching purpura (disseminated pruriginous angiodermatitis)
PPDs with linear and pseudodermatomal pattern
Unilateral linear capillaritis
Segmental pigmented purpura
Subtypes of unilateral linear capillaritis
Quadrantic capillaropathy
Segmenta pigmented purpura
Spontaneous resolution occurs more commonly than in other subtypes of PPD
Unilateral Linear capillaritis
Common subtype in Asian descent
Granulomatous variant of PPDs
Granulomatous variant of PPD has been relatively associated with
Hyperlipidemia
Most likely to predict progression to MF
T cell monoclonality
The most important feature to distinguish early MF from others is with the presence of
Lymphocytes with extremely convoluted, medium to large nuclei, single or clustered in epidermis and in small sheets in the dermis
Important role in the pathogenesis of PPD
Cellular reaction especially with Langerhans cells
In early Schamberg disease, the ff cell types (2) predominates
CD4+ cells
CD1a dendritic cells
3 theories on pathogenesis of PPDs
- Disturbance or weakness of cutaneous blood vessels
- Humoral immune response (C3,C1q,IgM, or IgA)
- Cellular immune response
(Lymphocytes, macrophages, Langerhans cells)
Majority of PPD cases are ___ (etiology)
Idiopathic
Diagnosis is gemrally made ___ and supported by ____
Clinically; histopath exam
Measurement of capillary fragility by application of sphygmomanometer
Hess test
Increased capillary fragility is consistently seen in PPD
True or False
False
Hemosiderin can be highlighted by ____ stain
Prussian blue
In older lesions, extravasated RBCs may no longer be present
True or False
True
PPD is a true capillaritis
True or False
False
Due to lack of fibrin in luminal walls and absence of thrombi
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
Granulomatous PPD
Variant of PPD, on HPx presents as band like mononuclear infiltrate in upper dermis separated from epidermis by thin rin of uninvolved collagen
Lichen aureus
Variant of PPDs with shorter course and more favorable overall prognosis
Unilateral linear capillaritis
Drug-induced PPDs
Common initial therapies for PPD
Topical steroids
antihistamines
Treatment with ___ BiD for ____ weeks cleared lichen aureus lesions
Topical pimecrolimus; 10
___ given for 4 weeks lead to clearance in all lesions with maintenance of remission 3 months after treatment for chronic PPD
Oral bioflavanoid (Rutoside 50mg BID) + Ascorbic acid 500mg BiD
Used for recalcitrant Schamberg disease
Colchicine 0.5mg BID
MOA of Pentoxifylline 400mg TiD for PPD
Inhibits T cell adherence to endothelial cells and keratinocytes
Immunosuppressants (oral corticosteroids, MTX, cyclosporine) are rarely indictaed for the benign nature of this condition
TRUE or FALSE
True
Used for Majocchi purpura
MTX 15 mg weekly for 4 weeks
MOA of PUVA in treating PPD
Inhibits T cell intereukin 2 production leading to resolution of perivascular lymphocytic infiltrate
Affects both immunomodulation and vascular destruction
PDT
Other treatments for PPD
Ca dobesilate 500mg BiD for 3 mos, sustained for 1 year
Griseofulvin 500-750mg OD for 1 month
Minocycline
Sucessful clearance of Schamberg using a ___ nm PDL after 5 monthly treatments
595 nm
Drug-induced PPD are more likely to present in lower extremities
True or False
False, more likely to be generalized
Lab testing is required for diagnosis
True or False
False, not required
Rule out other causes of purpura
Coagulation studies
Used to confirm diagnosis of Drug-induced PPD
Rechallenge
___ &; ___ account for lesions localizing in lower extremities
Gravity and increased venous pressure
Itching purpura are more apparemt along (4)
Waistline
Axilla
Antecubital
Popliteal fossa
Suspicious for MF: (4)
PPDs with large areas of confluence
Reticular arrangement
Superimposed violaceous hue
Pruritus present/relapsing for years
Possible agents that cause contact dermatitis leading to PPDs (8)
Benzoyl peroxide Black rubber Cobalt Disperse dyes Epoxy resin Eutectic mixture of local anesthetics Methylmethacrylate PPD
Hess test is reliable as capillary fragility is consistently seen in PPD
True or False
False, not reliable, not consistent
MOA of PUVA for PPD
Inh T cell IL2 production, resolution of perivascular infiltrate
For immunomodulation and vascular destruction
Photodynamic therapy
