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Pigmented purpuric dermatoses (syn. capillaritis)
CREATED: 22ND MAY 2015 | LAST UPDATED: 27TH JANUARY 2017
Introduction
The pigmented purpuric dermatoses (syn. capillaritis) are a Related chapters
group of chronic skin conditions of mostly unknown
aetiology that have a very distinctive clinical appearance. Vasculitis
They are characterised by extravasation of erythrocytes in
the skin with marked haemosiderin deposition, resulting in
many tiny red lesions described as cayenne pepper spots,
which group together to form brown-red patches.
This chapter is set out as follows:
Aetiology
Clinical findings
Images
Investigations
Management
Aetiology
The cause is unknown
Gravity is an important factor and in some cases exercise may be a provoking factor
Medications have been associated in 14% of cases - many drugs have been implicated
Clinical findings
Several types of capillaritis have been defined, of which some are named after the dermatologist who first
described them. Not all presentations of capillaritis will fit neatly into one of these groups.
Schamberg disease (syn. progressive pigmented purpura)
Is the most common type of capillaritis, and tends to arise in young adults
It is generally asymptomatic
Distribution
Although most common on the lower legs, it can arise on any part of the body
Morphology
Crops of brown-orange patches with cayenne pepper spots on their borders
The rash is usually irregularly distributed with few or many patches
Itching purpura
This has a similar appearance to Schamberg disease but has a more rapid onset, is more itchy, and more
extensive
Majocchi's purpura (purpura annularis telangiectodes)
Although this predominantly affects young patients, any age can be affected
Distribution
Although most common on the lower legs, it can arise on any part of the body
Morphology
Clinically there are few to many, 1-3 cm patches-plaques that are usually annular from the onset, and
which gradually enlarge
 The lesions are composed of telangiectases and haemosiderin staining, may be purple, yellow or brown
and may contain cayenne pepper spots

Lichen aureus

Is more common in children and young adults

It is often described as itchy, but can be asymptomatic
Distribution
Lesions tend to be unilateral and solitary
They are located more frequently on the lower extremities, but can also affect the upper extremities,
hands, trunk and eyelids
When multiple, they have a linear arrangement with or without segmental and zosteriform distribution
Morphology
A rusty-golden coloured patch, which may or may not contain purpuric dots

Gougerot-Blum syndrome (pigmented purpuric lichenoid dermatosis)

This form of capillaritis is less common

It most commonly affects males age 40-60 years
The rash, which predominantly affects the lower legs, is composed of lichenoid papules that become confluent
and thickened. Lesions may itch

Differential diagnosis of the pigmented purpuric dermatoses

Thrombocytopenia
Poikiloderma (various types)
Affected patches of skin have a striking appearance with telangiectasia, atrophy and a combination of
hyper- and hypopigmentation
Most forms of poikiloderma are benign, however patients with more extensive skin change should be
considered for referral to exclude poikilodermatous mycoses fungoides (a rare form of cutaneous
T-cell lymphoma), which in its early stages can look like one of the pigmented purpuric dermatoses
Vasculitis - causes palpable purpura. Refer to the related chapter on vasculitis

Images
Please click on images to enlarge or download. The PCDS would like to thank Dermatoweb, DermQuest (Galderma),
and others who have contributed images. All named individuals and organisations maintain copyright for the relevant
images. This website is non-profit and holds the images for educational purposes only. Any image downloaded must
only be used for teaching purposes and not for commercial use. Notice and credit must be given to the PCDS or other
named contributor. Please follow this link if you have any high-quality images that you can contribute to the website.

Figure: 1

Schamberg disease

Figure: 2

Same patient

Crops of brown-orange patches with cayenne pepper spots on

their borders
Figure: 3

As above

Figure: 4

Schamberg disease

Arrows denote cayenne pepper-like spots

Figure: 5

Majocchi's purpura

Figure: 6

Same patient as above

Figure: 7

Majocchi's purpura

Figure: 8

Same patient as above

Figure: 9

Majocchi's purpura - generalised

Figure: 10

Majocchi's purpura of the wrist

Figure: 11

Lichen aureus

Figure: 12

Lichen aureus
Figure: 13

Lichen aureus

Figure: 14

Itching purpura

Figure: 15

Same patient as above

Figure: 16

As above: post-treatment with Eumovate cream

Figure: 17

Pigmented purpuric dermatoses of the axilla -

undetermined

Figure: 18

Same patient as above

Figure: 19

Gougerot-Blum syndrome
Figure: 20

Poikiloderma

Areas of telangiectasia, atrophy and a combination of hyper-

and hypopigmentation

Figure: 21

Poikilodermatous mycoses fungoides

 Investigations
Patient require a FBC to exclude thrombocytopenia
A biopsy is not usually required, but if taken shows a perivascular infiltrate of lymphocytes and macrophages,
which is centred on the superficial small blood vessels of the skin
When the rash is atypical and possibly suggestive of poikilodermatous mycoses fungoides, one or more
incisional biopsies will be required because as with other types of cutaneous T-cell lymphoma the
histopathological features are not always apparent in the early stages

Management
There is no known cure for most cases of capillaritis. It can disappear within a few weeks, recur from time to time, or
frequently persist for years.

If a medication could be the cause consider discontinuing the drug if feasible

Emollients may have a soothing effect if the skin is sore or itchy
Short-term topical steroids may benefit some patients in the earlier stages when there is perhaps more in the
way of inflammation of the vessels
If the lower leg is affected consider graduated compression elastic hosiery

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