Purpura pigmentosa progressive L81.7

Last updated on: 29.10.2020

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Synonym(s)

Adalin-Purpura; Adalin purpura and eczematide-like purpura; Angiodermatitis disseminated pruriginous; Capillaritis haemorrhagica maculosa; Carbamide purpura; Carbromal rash; chronic pigmented purpura; Chronic pigment purpura; Dermatosis pigmentaria progressiva; Dermatosis progressive pigmented; Eccematid-like purpura; eczematidal purpura; eczematide-like purpura; Epidemic purple-lichenoid dermatitis; essential familial telangiectasia; essential telangiectasia; Majocchi disease; Majocchi purpura; Majocchi syndrome; Mons pubis; Mons pubis dermatitis; pigmented purpuric dermatoses; Pigment purpura; Pigment purpura progressive; PPP; progressive pigmented dermatosis; progressive pigment purpura; purple itching; Purpura Adaline Purpura; Purpura anularis teleangiectodes; Purpura eczematid; Purpura mons pubis; purpura porphyrica; Schamberg M.; Schamberg Syndrome

History
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Pubic mountain, 1901

Definition
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Spectrum of clinically different, inflammatory, mostly chronic and intermittent, asymptomatic, haemorrhagic-pigmentary skin diseases (not a systemic disease), which are characterized by petechiae in a fresh intermittent phase and later by yellow-orange-brownish foci. Preferably the distal lower legs are affected, in very pronounced cases also thighs, trunk and upper extremities.

Etiopathogenesis
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Pathogenesis is unclear. Triggering by drugs, infections and other factors are discussed, especially:

  • 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) the following were described as associated diseases: hypertension (16%), diabetes mellitus (10%). The extent to which these concomitant diseases are significant for the pathogenesis of the disease remains open.

Manifestation
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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.

Localization
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Preferably occurring on the lower legs. Infestation of thighs, stomach, arms is possible.

Clinical features
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Schamberg's disease (about 60% of all cases of PPP) occurs as an intermittent, clinically variable disease pattern whose appearance depends on its acuteity and frequency of attacks.

It is impressive, symmetrically distributed, differently sized (measuring from 0.3 cm to 10-20 cm), mostly blurred, sometimes very discreet, asymptomatic (itching is usually absent) yellow, yellow-brown or red-brown spots, which are usually more prominent on the distal lower legs in relation to the proximal parts. Included in these areas are 0.1-0.2 cm large, light or brown-red, non-anaemic spot reinforcements in irregular distribution and density. The surface of the skin lesions is usually smooth, also atrophic.

More rarely a fine-lamellar scaling is detectable; this can result in an eczematous impression ( eczematide-like purpura, also known as blue jeans dermatitis).

Also lichenoid aspects may result(type Gougerot-Blum; 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 treatment (especially in young women; spontaneous healing) remains open.

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

Histology
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Shaky, band-shaped, subepidermal, less frequently perivascular (vessels are enclosed like manschettes) accentuated, lymphohistiocytic infiltrate. In the variant "eczematid-like purpura" there is slight acanthosis with discrete focal epidermotropy. Erythrocyte diapedesis particularly in the papillary stratum and hemosiderin deposits in the dermis. In the lichenoid variant (type Gougerot-Blum) a lichenoid pattern with vacuole degeneration of basal epithelial cells may also be histologically detectable.

Differential diagnosis
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Clinical differential diagnosis:

  • Leucocytoclastic vasculitis: Mostly acute event, hardly any extensive discoloration of the skin; histology is diagnostic.
  • Urticaria pigmentosa: Permanently present; no extensive discoloration; the Darier sign is positive; histology is diagnostic
  • Purpura jaune d'ocre: pigmentation due to congestion in chronic venous insufficiency or in simple (heat or cardiac) congestive oedema.

Histological differential diagnoses:

General therapy
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Avoid the triggering factors. Reduce or convert the triggering medication. Effects can only be expected after weeks or months.

External therapy
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Symptomatic therapy is in the foreground. Very rare are itching or burning sensations. In these cases, therapy is attempted with cooling lotio alba or ethanolic zinc oxide shaking mixture R292, possibly 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, glucocorticoid-containing topical preparations are applied at intervals, e.g. with hydrocortisone (hydrogals, hydrocortisone cream 0.5-2.0%) or prednicarbate (e.g. Dermatop Fatty Ointment).

Internal therapy
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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.

Progression/forecast
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Different courses are possible. Mostly chronic-recurrent, progressive course. If caused by medication, healing after discontinuation of the medication. The Majocchi variant tends to heal spontaneously. The Purpura pigmentosa progressiva of older men, tends to pronounced chronicity (intermittent course for years).

Literature
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  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

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Last updated on: 29.10.2020