A group of dermatoses characterized by petechiae, pigmentation, and occasionally, telangiectasia.
Found most commonly on the lower extremities; however, lesions may involve the upper body but rarely become generalized.
Benign, generally asymptomatic eruptions that tend to be chronic with flares and remissions.
Common histopathologic features include a superficial lymphocytic infiltrate, erythrocyte extravasation, and hemosiderin deposition.
Clinical variation between eruptions led to their subclassification into eponymic groups; however, frequent overlap may make differentiation difficult.
Pigmented purpuric eruptions (also called pigmented purpuric dermatoses [PPDs]) are a group of dermatoses that are characterized by petechiae, pigmentation, and, occasionally, telangiectasia. They are most commonly located on the lower extremities; however, lesions may involve the trunk and upper extremities, or, rarely, become generalized. They are subdivided into clinical subcategories, which include progressive pigmentary dermatosis (Schamberg disease), purpura annularis telangiectodes (Majocchi purpura), pigmented purpuric lichenoid dermatosis of Gougerot and Blum, eczematid-like purpura of Doucas and Kapetanakis, and lichen aureus (Table 143-1). These dermatoses are benign and generally asymptomatic, but tend to run a chronic course with flares and remissions. Despite differing clinical presentations, the subtypes of PPDs share common histopathologic features, including a superficial lymphocytic infiltrate, erythrocyte extravasation and hemosiderin deposition.
Table 143-1Subtypes of Pigmented Purpuric Dermatoses ||Download (.pdf) Table 143-1 Subtypes of Pigmented Purpuric Dermatoses
| ||SCHAMBERG ||MAJOCCHI ||GOUGEROT AND BLUM ||DOUCAS AND KAPETANAKIS ||ITCHING PURPURA ||LICHEN AUREUS |
|Average age of presentation ||40s ||30s ||40s ||40s ||50s ||20s-30s |
|Sex ||M > F ||F > M ||M > F ||M > F ||M > F ||M > F |
|Onset ||Insidious ||Abrupt ||Insidious ||Abrupt ||Abrupt ||Abrupt |
|Primary lesion ||Red-brown macule with pinpoint petechiae (“cayenne pepper”) ||Annular plaques with border of telangiectasia and purpura ||Lichenoid papule ||Red-brown macule + scale ||Red-brown macule ||Orange-brown papule or plaque |
PPDs are relatively uncommon. They may present at any age, including in children, and are most common in middle age (see Table 143-1). There is no ethnic predisposition, although the granulomatous variant has been reported more commonly in patients of Asian descent.1 In general, PPDs occur more frequently in males, except for the Majocchi subtype, which is seen more frequently in females.2
PROGRESSIVE PIGMENTARY DERMATOSIS (SCHAMBERG DISEASE)
Schamberg first described an eruption of irregularly shaped reddish-brown patches with “pin head sized reddish puncta, closely resembling grains of cayenne pepper” over the legs of a 15-year-old boy in 1901.3 Schamberg disease is the most common of the PPDs to occur in children. It is, however, more common overall in adults with a peak incidence in the fifth decade.4 The lesions are insidious in their development and are usually asymptomatic. The lower extremities are the most common site of involvement. Lesions can involve the trunk or the upper extremities (Figs. ...