Table 2.
The clinical and paraclinical characteristics of PPLD and the 4 most common differential diagnoses
| Majocchi disease | Schamberg disease | PPLD | Eczematoid-like purpura of Doucas and Kapetanakis | Lichen purpuricus (aureus) | |
|---|---|---|---|---|---|
| Sex | Predominantly seen in females | Predominantly seen in males | Predominantly seen in males | Predominantly seen in males | Both sexes |
| Frequency | Rare | Most common type of PPD | Rare | Uncommon | Rare |
| Peak incidence | Children and young adults | Adolescents and young adults | Middle-aged | Middle-aged | Children and young adults |
| Skin distribution | Begins on the lower limb symmetrically and then extends to the trunk and upper extremities | Most frequently bilaterally on the tibial regions, but may be unilateral or involve the thighs, buttocks, trunk, or upper extremities | Predilection for the legs, and rarely on the trunk and thighs | Legs with progression to the thighs, trunk, and upper extremities | Lesions frequently occur bilaterally on the lower limbs, although can be unilateral and may affect the trunk and upper limbs; unlike other forms of PPD, the lesions of lichen aureus may also occur in a dermatomal distribution, or can follow the distribution of veins or arteries |
| Clinical presentation | Variable number of annular erythematous plaques and patches, often with central clearing and atrophy | Discrete reddish-brown patches that are bordered from red to brown; nonpalpable, pinpoint puncta; Cayenne pepper- like lesions | Minute, lichenoid papules that tend to fuse into plaques of various hues | Lesions are extensive and patients typically complain of severe pruritus | Presents with yellowish or red papules or patches which may either itch or be asymptomatic |
| Histology | Perivascular and predominantly band-like infiltrate of lymphocytes that extend to the overlying epidermis, showing vacuolar alteration of the basal layer and spongiosis; hemosiderin-laden macrophages, and extravasation of red blood cells within the lichenoid infiltrate | Perivascular infiltrate of mononuclear cells in the upper dermis, endothelial cell swelling, extravasated red blood cells, and hemosiderin-laden macrophages | Perivascular infiltrate of lymphocytes which is lichenoid and macrophages centered on the superficial small blood vessels of the skin with endothelial cell swelling and narrowing of lumina | Spongiosis with inflammation of the epidermis | Perivascular lymphohistiocytic infiltrate in a band-like pattern; hemosiderin-laden macrophages, and extravasation of red blood cells; the epidermis is unaffected |
| Remission | Common | Common | Often chronic course but spontaneous remission may occur | Spontaneous remission may occur, but recurrences may occur | Spontaneous remission may occur, but recurrences may occur |
| Treatment | The disorder is benign and self-limiting; treatment is not effective and the lesions may last several months to years | Systemic steroids have been reported to result in clearance; ascorbic acid and antihistamines have been used with limited success; PUVA therapy is beneficial; narrow band UVB | Difficult; topical and systemic steroids, elastic stockings, antipruritic topical preparations, systemic antihistamines, PUVA, griseofulvin, cyclosporin A, bioflavonoids, and ascorbic acid have been suggested | Topical corticosteroids and antihistamines | Oral corticosteroids; may resolve spontaneously |