22 Part 22
::
Vascular Diseases
Figure 143-3 Pigmented purpuric dermatosis: Majocchi
disease. Multiple nonpalpable, nonblanching purpuric
lesions arranged in annular configurations and associated
with tiny telangiectasias. Note brownish discoloration of
older lesions.
clinically by mild scaling overlying pinpoint ery-
thematous macules and patches with associated
pruritus. Lichenification can occur from repeated
scratching. Histopathologically, spongiosis is pres-
ent, in addition to the classic histopathologic fea-
tures of PPD. 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.
LICHEN AUREUS
In 1958, Martin first reported this subtype of PPD
under the term lichen purpuricus, which was later
named lichen aureus in 1960 by Calnan to emphasize
its vivid yellow-orange color.9,10 As opposed to pig-
mented purpuric lichenoid dermatosis of Gougerot
and Blum, where lichenoid refers to the clinical mor-
phology of the lesions, in lichen aureus, lichen refers
to both its clinical and histopathologic features. This
2592 Figure 143-4 Lichenoid dermatosis of Gougerot and Blum.
(Used with permission from Dr. April Armstrong.)
Kang_CH143_p2590-2598.indd 2592
Figure 143-5 Lichen aureus. (Used with permission from
Dr. Ashley Crew.)
subtype presents with more localized and persistent
lesions with circumscribed macules or papules that
are a distinctive gold, rust, or orange color (Fig. 143-5).
Histologically a dense, band-like lichenoid infiltrate
of inflammatory cells is seen. The lesions are gener-
ally asymptomatic but at times are intensely pruritic.
They are most commonly localized to one lower
extremity, but other body sites can be involved, and a
segmental distribution has been reported.11,12 This dis-
order has a predilection for young adult males, with
a peak incidence in the second and third decades. It
runs a chronic course, with stable or slowly progress-
ing lesions.
ITCHING PURPURA
(DISSEMINATED
PRURIGINOUS
ANGIODERMATITIS)
Itching purpura, also known as disseminated prurigi-
nous angiodermatitis, presents acutely with widely
disseminated orange-brown to purpuric lesions asso-
ciated with severe pruritus.13,14 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 waist-
line, axilla, antecubital and popliteal fossae. Although
this subtype of PPD has a chronic course, spontaneous
remissions are possible.
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 UNILATERAL LINEAR
CAPILLARITIS (SEGMENTAL
PIGMENTED PURPURA)
In 1990, a transient PPD in a segmental distribution
on the lower trunk of a middle-aged woman was
reported. Later, Riordan and colleagues reported PPDs
in 4 young men with linear and pseudodermatomal
patterns, which they termed unilateral linear capillaritis.15
Both “segmental pigmented purpura” and “qua-
drantic capillaropathy” are considered subtypes of
unilateral linear capillaritis.16,17 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.
GRANULOMATOUS
PIGMENTED PURPURA
The first report of the granulomatous variant of PPDs
was in 1996 by Saito and Matsuoka and since then
more than 17 cases have been reported.18-20 It is most
common in middle age and has been reported more
commonly in patients of Asian descent.1,20 Clinically,
the lesions appear similar to other PPDs with purpuric
and brown macules developing most commonly on
the lower extremities and dorsal feet. This subtype is
distinguished by its histopathologic findings. In addi-
tion to the classic histopathologic features of a PPD,
a granulomatous infiltrate is present. The granuloma-
tous infiltrate is most commonly located in the pap-
illary dermis, but may be in the mid to deep dermis
separate from a more superficially located lichenoid
infiltrate. Systemic granulomatous disorders and
infectious processes (mycobacterial and deep fungal)
must be ruled out in these patients. Hyperlipidemia is
a relatively common association with granulomatous
pigmented purpura with 9 of 17 reported cases show-
ing elevated cholesterol levels in a review from 2014.19
PIGMENTED PURPURIC
DERMATOSIS/MYCOSIS
FUNGOIDES OVERLAP
In 1988, Barnhill and Braverman reported the first
cases of pigmented purpura-like eruptions progressing
to mycosis fungoides.21 Furthermore, the first patient
diagnosed with lichen aureus in the United States
was later diagnosed with mycosis fungoides (MF;
see Chap. 119). Some evidence supports the idea that
lichenoid variants of PPD may be precursors of MF,
with similar histologic findings and clonal popula-
tions of lymphocytes.22-29 Although there is no clear
Kang_CH143_p2590-2598.indd 2593
connection between these 2 diseases, and the occur-
rence of MF in the setting of a PPD is rare, 3 different
22
relationships have been reported: MF mimicking pig-
mented purpura clinically, pigmented purpura evolv-
ing into MF, and pigmented purpura that simulates
MF histologically.
In a study of T-cell clonality and markers, T-cell
monoclonality of PPD was most likely to predict pro-
gression to MF, whereas the absence of certain T-cell
markers was a less reliable predictor.30 It is impor-
tant to note that both MF and PPD can display clon-
ality. Histologic clues of MF are subtle and include
greater lymphoid atypia in the intraepidermal lym-
phocytes compared to dermal lymphocytes, large
Chapter 143 :: Pigmented Purpuric Dermatoses
intraepidermal groups of lymphocytes anywhere in
the epidermis, or many lymphocytes in the spinous
layer.30,31 Santucci and colleagues suggested that to
distinguish early MF from its inflammatory mimick-
ers the most important feature is lymphocytes with
extremely convoluted, medium to large nuclei, that
are single or clustered in the epidermis and in small
sheets in the dermis.32 Ackerman compared the his-
tologic features of lichenoid purpuric eruptions
with plaque stage MF and noted many similarities,
concluding that it may be impossible to differentiate
these two on a histologic basis alone.33
The differentiation between PPD and MF can be
difficult and requires the integration of clinical, histo-
logic and immunophenotypic information.30,33 PPDs
with large areas of confluence, reticular arrange-
ments, a superimposed violaceous hue, or pruritus
that has been present or relapsing for several years are
suspicious for MF. There is a predominance in adult
males.21,34 For selected patients, long-term followup is
needed to monitor for evolution into MF, even though
the overall incidence of MF occurring in association
with PPD is rare.
ETIOLOGY AND
PATHOGENESIS
There are 3 different views on the pathogenesis of
PPDs. The first is that there is a disturbance or weak-
ness of cutaneous blood vessels, leading to capillary
fragility and erythrocyte extravasation. This pro-
posed mechanism, however, does not account for the
inflammatory infiltrate that is also seen in these erup-
tions. The second theory is that PPDs develop from a
humoral immune response. This theory is supported
by direct immunofluorescence studies showing vas-
cular deposition of C3, C1q, immunoglobulin M, or
immunoglobulin A.35,36 The final theory is that PPDs
develop as a result of a cellular immune response.37,38
This theory suggests that the inflammatory infiltrate,
consisting of lymphocytes, macrophages, and Langer-
hans cells, leads to vascular fragility and subsequent
extravasation of erythrocytes. Aiba and Tagami used
immunohistologic studies in 8 cases of Schamberg dis-
ease to demonstrate that the dermal infiltrate was pre- 2593
dominantly composed of helper-inducer T-cells and
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