Purpura pigmentosa progressive L81.7

Spectrum of clinically different, inflammatory, mostly chronic and relapsing, little symptomatic, hemorrhagic-pigmentary diseases of the skin (no systemic involvement), which are characterized in a fresh relapse phase by petechiae and later by yellow-orange-brownish foci.

The distal lower legs are preferentially affected, in very pronounced cases also the thigh, trunk and upper extremity.

Pathogenesis is unclear. Allergic late type reactions (type IV reactions) caused by systemic or external drugs, by infections but also by food are discussed. In particular, the following triggers are mentioned:

• Drugs: statins (13%), beta blockers (10%), benzodiazepines (diazepam), meprobamate, diuretics (furosemide), isotretinoin and others.
• infections: respiratory infections
• food additives
• chronic venous insufficiency (CVI)
• house dust
• cryoglobulinemia type III
• Contact allergens (dyeing or bleaching agents in cotton textiles).

In larger studies (Kim et al. 2015), associated diseases were described as: hypertension (16%), diabetes mellitus (10%). To what extent these "concomitant diseases" are significant for the pathogenesis of the clinical picture remains open.

Start is possible at any age. Particularly in younger and middle-aged adults; occasionally also in children < 10 years of age. Preferably occurring in the male sex.

Schamberg's disease (about 60% of all cases of PPP) occurs as a relapsing, clinically variable clinical picture, the appearance of which depends on its acuity and relapse frequency. With high relapse activity, reddish-brownish, frayed, reddish-brownish patches reminiscent of terrazzo floors are more likely to impress. With prolonged persistence, an increasing, rich, inhomogeneously fractured brown tone sets in.

The patches are usually symmetrically distributed, varying in size (measuring from 0.3 cm to 10-20 cm), usually indistinctly circumscribed, sometimes very discrete, asymptomatic (pruritus is usually absent) yellow, yellow-brown, or reddish-brown patches that usually present more intensely on the distal lower legs relative to the proximal portions. Included in these areas are 0.1-0.2 cm, light or brown-red, non-anemic spot enhancements in irregular distribution and density. The surface of the skin lesions is usually smooth, rarely atrophic.

Fine lamellar scaling may occur; this may result in aneczematous picture(eczematid-like purpura, also known as "blue jeans dermatitis").

Lichenoid aspects may also result(Gougerot-Blum type; especially in older men), as well as apparently purely teleangiectatic forms.

To what extent the anular teleangiectatic form described by Majocchi(purpura anularis teleangiectodes) requires a special position (especially in young women; spontaneous healing) remains open.

This statement is also valid for the unilateral lichen aureus(occurring as a localized, sometimes also as a strict dermatosis).

Bulky, band-like, subepidermal, rarely perivascular (vessels are cuffed), accentuated, lymphohistiocytic infiltrate. In the "eczematid-like purpura" variant, there is minor acanthosis with discrete focal epidermotropy. Erythrocyte diapedesis especially in the stratum papillare and hemosiderin deposits in the dermis. In the lichenoid variant (Gougerot-Blum type), a lichenoid pattern with vacuolated degeneration of basal epithelial cells may also be histologically detectable.

Clinical Differential Diagnoses:

• Leukocytoclastic vasculitis: Usually acute; hardly any extensive discoloration of the skin; histology is diagnostic.
• Urticaria pigmentosa: Permanently present; no extensive discolorations; Darier's sign is positive; histology is diagnostic.
• Purpura jaune d'ocre: pigmentation due to congestion in chronic venous insufficiency or in simple (heat- or cardiac-related) congestive edema.
• Sarcoidosis of the skin: small nodular (plaque-like) disseminated form. Histology with evidence of sarcoid infiltrates is diagnostic.

Histologic differential diagnoses:

• Lichenoid or fixed drug reactions.
• pigmented-purpuric form of mycosis fungoides
• stasis dermatitis (simple, non-inflammatory erythrocyte extravasations)

Avoidance of the triggering factors.

Discontinue or convert triggering medications.

Chronic bacterial infections (focus search) are to be sanitized.

Positive effects of such therapy measures can only be expected after weeks or months .

Symptomatic therapy is in the foreground.

Itching or burning sensations are very rare. In these cases, therapy is attempted with cooling lotion alba or ethanolic zinc oxide shaking mixture R292, if necessary with the addition of 2-5% polidocanol as polidocanol cream 2-5% or polidocanol-zinc oxide shaking mixture 3- or 5%, or 1% menthol cream(menthol cream 1%).

Otherwise, intermittent use of glucocorticoid-containing externals, e.g. hydrocortisone creams (Hydrogalen, hydrocortisone cream 0.5-2.0%) or prednicarbate cream.

Systemic glucocorticoids such as prednisolone (e.g. Solu Decortin H) 20-40 mg/day are only indicated in extended cases; gradual dose reduction according to the clinical findings.

Vaso-sealing drugs such as vitamin C 2x500 mg/day p.o. or rutoside (e.g. rutinion 3 times/day 1-2 tbl. p.o.) should be tried.

Successes with PUVA therapy have been reported.

Different courses are possible. Mostly chronic-recurrent, not rarely progressive course. If caused by medication, healing after discontinuation of the medication. The Majocchi variant tends to heal spontaneously. Purpura pigmentosa progressiva of older men, tends to be markedly chronic (relapsing course for years).

1. Basak PY, Ergin S (2001) should pentoxifylline be regarded as an effective treatment for Schamberg's disease? J Am Acad Dermatol 44: 548-549
2. Dhali TK et al (2015) Phototherapy as an effective treatment for Majocchi's disease--case report. An Bras Dermatol 90:96-99
3. Filo V et al (2001) Unilateral progressive pigmented capillaropathy (Schamberg's disease?) of the arm. Br J Dermatol 144: 190-191
4. Kalinke DU, Wüthrich B (1999) Purpura pigmentosa progressiva in cryoglobulinemia type III and tartrazine intolerance. dermatologist 50: 47-51
5. Kanwar AJ, Thami GP (1999) Familial Schamberg's disease. Dermatology 198: 175-176
6. Kim DH et al (2015) Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis. Ann Dermatol 27:404-410
7. Satoh T et al (2002) Chronic pigmented purpura associated with odontogenic infection. J Am Acad Dermatol 46: 942-944
8. Schamberg JF (1901) A peculiar progressive pigmentary disease of the skin. Br J Dermatol 13: 1-5
9. Schober SM egt al (2014) Early treatment with rutoside and ascorbic acid is highly effective for progressive pigmented purpuric dermatosis. J Dtsch Dermatol Ges 12:1112-1119
10. Simon M et al (1986) PUVA therapy of eczematide-type purpura. Act Dermatol 12: 100-102 Torrelo A et al. (2003) Schamberg's purpura in children: a review of 13 cases. J Am Acad Dermatol 48: 31-33
11. Fence H (1987) Haemorrhagic pigmentary dermatoses. Z Hautkr 62: 1485-1491