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 Figure 143-5 Lichen aureus. (Used with permission from
older lesions. Dr. Ashley Crew.)

clinically by mild scaling overlying pinpoint ery- subtype presents with more localized and persistent
thematous macules and patches with associated lesions with circumscribed macules or papules that
pruritus. Lichenification can occur from repeated are a distinctive gold, rust, or orange color (Fig. 143-5).
scratching. Histopathologically, spongiosis is pres- Histologically a dense, band-like lichenoid infiltrate
ent, in addition to the classic histopathologic fea- of inflammatory cells is seen. The lesions are gener-
tures of PPD. This subtype spreads rapidly over a ally asymptomatic but at times are intensely pruritic.
period of 15 to 30 days and will subsequently fade They are most commonly localized to one lower
without treatment over several months to years, extremity, but other body sites can be involved, and a
although recurrence is possible. 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-
LICHEN AUREUS ing lesions.

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 ITCHING PURPURA
its vivid yellow-orange color.9,10 As opposed to pig-
mented purpuric lichenoid dermatosis of Gougerot (DISSEMINATED
and Blum, where lichenoid refers to the clinical mor-
phology of the lesions, in lichen aureus, lichen refers
PRURIGINOUS
to both its clinical and histopathologic features. This 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
2592 Figure 143-4 Lichenoid dermatosis of Gougerot and Blum. this subtype of PPD has a chronic course, spontaneous
(Used with permission from Dr. April Armstrong.) remissions are possible.

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 UNILATERAL LINEAR
connection between these 2 diseases, and the occur-
rence of MF in the setting of a PPD is rare, 3 different
22
CAPILLARITIS (SEGMENTAL relationships have been reported: MF mimicking pig-
mented purpura clinically, pigmented purpura evolv-
PIGMENTED PURPURA) ing into MF, and pigmented purpura that simulates
MF histologically.
In 1990, a transient PPD in a segmental distribution In a study of T-cell clonality and markers, T-cell
on the lower trunk of a middle-aged woman was monoclonality of PPD was most likely to predict pro-
reported. Later, Riordan and colleagues reported PPDs gression to MF, whereas the absence of certain T-cell
in 4 young men with linear and pseudodermatomal markers was a less reliable predictor.30 It is impor-
patterns, which they termed unilateral linear capillaritis.15 tant to note that both MF and PPD can display clon-
Both “segmental pigmented purpura” and “qua- ality. Histologic clues of MF are subtle and include
drantic capillaropathy” are considered subtypes of greater lymphoid atypia in the intraepidermal lym-
unilateral linear capillaritis.16,17 This PPD is clinically phocytes compared to dermal lymphocytes, large

Chapter 143 :: Pigmented Purpuric Dermatoses

distinguished by its linear or segmental distribution. It intraepidermal groups of lymphocytes anywhere in
tends to have a favorable prognosis, with spontaneous the epidermis, or many lymphocytes in the spinous
resolution occurring more commonly than in the other layer.30,31 Santucci and colleagues suggested that to
subtypes of PPDs. 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
GRANULOMATOUS sheets in the dermis.32 Ackerman compared the his-
tologic features of lichenoid purpuric eruptions
PIGMENTED PURPURA with plaque stage MF and noted many similarities,
concluding that it may be impossible to differentiate
The first report of the granulomatous variant of PPDs these two on a histologic basis alone.33
was in 1996 by Saito and Matsuoka and since then The differentiation between PPD and MF can be
more than 17 cases have been reported.18-20 It is most difficult and requires the integration of clinical, histo-
common in middle age and has been reported more logic and immunophenotypic information.30,33 PPDs
commonly in patients of Asian descent.1,20 Clinically, with large areas of confluence, reticular arrange-
the lesions appear similar to other PPDs with purpuric ments, a superimposed violaceous hue, or pruritus
and brown macules developing most commonly on that has been present or relapsing for several years are
the lower extremities and dorsal feet. This subtype is suspicious for MF. There is a predominance in adult
distinguished by its histopathologic findings. In addi- males.21,34 For selected patients, long-term followup is
tion to the classic histopathologic features of a PPD, needed to monitor for evolution into MF, even though
a granulomatous infiltrate is present. The granuloma- the overall incidence of MF occurring in association
tous infiltrate is most commonly located in the pap- with PPD is rare.
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) ETIOLOGY AND
must be ruled out in these patients. Hyperlipidemia is
a relatively common association with granulomatous
PATHOGENESIS
pigmented purpura with 9 of 17 reported cases show- There are 3 different views on the pathogenesis of
ing elevated cholesterol levels in a review from 2014.19 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-
PIGMENTED PURPURIC posed mechanism, however, does not account for the
inflammatory infiltrate that is also seen in these erup-
DERMATOSIS/MYCOSIS tions. The second theory is that PPDs develop from a
humoral immune response. This theory is supported
FUNGOIDES OVERLAP by direct immunofluorescence studies showing vas-
cular deposition of C3, C1q, immunoglobulin M, or
In 1988, Barnhill and Braverman reported the first immunoglobulin A.35,36 The final theory is that PPDs
cases of pigmented purpura-like eruptions progressing develop as a result of a cellular immune response.37,38
to mycosis fungoides.21 Furthermore, the first patient This theory suggests that the inflammatory infiltrate,
diagnosed with lichen aureus in the United States consisting of lymphocytes, macrophages, and Langer-
was later diagnosed with mycosis fungoides (MF; hans cells, leads to vascular fragility and subsequent
see Chap. 119). Some evidence supports the idea that extravasation of erythrocytes. Aiba and Tagami used
lichenoid variants of PPD may be precursors of MF, immunohistologic studies in 8 cases of Schamberg dis-
with similar histologic findings and clonal popula- ease to demonstrate that the dermal infiltrate was pre- 2593
tions of lymphocytes.22-29 Although there is no clear dominantly composed of helper-inducer T-cells and

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