Synonyms: progressive pigmented purpuric dermatitis, Gougerot-Blum capillaritis, itching purpura, pigmented purpuric eruption

Schamberg's disease was first described by Schamberg in 1901 in a 15 year-old boy. It represents benign dermatoses with purpura due to leaking from capillaries close to the skin surface. It most often affects the legs and spreads slowly. The discoloration is brown/orange due to haemosiderin deposition in the skin.

Pigmented purpuric reactions have five disease types:^1,2

• Progressive pigmentary purpura or Schamberg's disease.
• Pigmented purpuric lichenoid dermatitis of Gougerot and Blum - red/brown papules and plaques in men - which responds to psoralen combined with ultraviolet A (PUVA) treatment.
• Purpura annularis telangiectodes - rare, with a preponderance in young females and manifests as annular erythematous plaques and patches.
• Eczematoid-like purpura of Doucas and Kapetanakis - occurs in men, with bilateral intensely itchy lesions on legs.
• Lichen aureus - a localised persistent form of pigmented purpuric dermatitis.³

There is clinical and histological overlap between these and they may actually represent variable presentations of the same disease process.

Epidemiology

The condition is rare. One study, in a UK hospital-based dermatology practice serving a population of 300,000, identified only ten cases of purpuric pigmented dermatosis (of which Schamberg's disease is only one type) during a 10-month period.⁴ It is more common in males and can occur at any age. It has been stated as the most common cause of petechiae in children¹ - however, it is thought that this may not actually be the case.⁵ There has been a case report of four family members with Schamberg's disease suggesting a possible genetic link.⁶

Aetiology^1,2

The underlying cause is not known. However, the following have been postulated:

• Recent viral infection.
• Hypersensitivity to an unknown causal agent.
• Aberrant cell-mediated immunity (perivascular infiltrate has specific types of CD cells only).
• Associated with certain medications - thiamine, aspirin, chlordiazepoxide and paracetamol.⁷ It has also been reported with bezafibrate.⁷

Presentation

There are no symptoms apart from itching and patients note their skin looks blotchy. For some this is enough to cause psychological distress. However, some patients have reported pains in their limbs - which may be coincidental.

Lesions are most commonly on the lower limbs bilaterally but can occur anywhere or be unilateral. A case involving the genitals has been reported.

The lesions consist of:

• Asymmetrical brown/orange patches.
• Non-blanchable purpura.
• Petechiae called "cayenne pepper" spots (develop at the edge of old lesions).

Patterns can vary, e.g. annular, linear. There may also be associated lichenification, scaling and pruritic marks.

Differential diagnosis^1,2

Other causes of purpura:

• Vasculitis, e.g. leukocytoclastic vasculitis.
• T-cell lymphoma (especially if presenting in young males).¹
• Drug eruption.
• Trauma.
• Self-induced purpura.
• Mycosis fungoides.⁸
• Primary benign hypergammaglobulinaemic purpura of Waldenström.⁹

Investigations

• Blood tests - including platelets and clotting - are usually normal.
• Autoantibody screen and hepatitis serology should be performed.
• Skin biopsy - histology reveals a capillaritis of dermal vessels. Other changes that may be seen include perivascular inflammatory infiltrate, endothelial hypertrophy with extravasation of blood cells and haemosiderin-laden macrophages.¹
• Examination of the skin using a dermatoscope may be helpful.⁴

Associated diseases²

• Diabetes mellitus
• Rheumatoid arthritis
• Systemic lupus erythematosus
• Thyroid abnormalities
• Hepatic disease
• Porphyria
• Malignancies
• Dyslipidaemias

Management

Any suspected precipitants should be withdrawn.

• Itching - treat with mild topical corticosteroid or antihistamines.
• One study has reported good results with narrow band ultraviolet light.¹⁰
• Another study reported the successful use of aminaphtone, a drug normally used in other venous conditions such as chronic venous congestion of the lower limbs.¹¹
• Superimposed infection - will need antibiotics.
• Systemic steroids - no real evidence of their benefit.¹

Other tried treatments include vitamin C supplements, laser therapy and wearing support hosiery to prevent venous stasis.² There is no evidence of definite benefit of the former two. Immunosuppressants have also been used.² Psoralen therapy has been used and the results look promising.^2,12

Prognosis

Schamberg's disease usually runs a chronic course with frequent exacerbations and remissions.The rash may be present for many years with slow extension. Lesions may occasionally disappear spontaneously.

There are three reports of patients with Schamberg's disease developing T-cell lymphoma.¹

Document references

1. Tristani-Firouzi P, Meadows KP, Vanderhooft S; Pigmented purpuric eruptions of childhood: a series of cases and review of literature.; Pediatr Dermatol. 2001 Jul-Aug;18(4):299-304. [abstract]
2. Sardana K, Sarkar R, Sehgal VN; Pigmented purpuric dermatoses: an overview.; Int J Dermatol. 2004 Jul;43(7):482-8. [abstract]
3. Kim MJ, Kim BY, Park KC, et al; A case of childhood lichen aureus. Ann Dermatol. 2009 Nov;21(4):393-5. Epub 2009 Nov 30. [abstract]
4. Mehregan D et al; Pigmented Purpuric Dermatitis, Medscape, Jan 2010
5. Torrelo A, Requena C, Mediero IG, et al; Schamberg's purpura in children: a review of 13 cases.; J Am Acad Dermatol. 2003 Jan;48(1):31-3. [abstract]
6. Sethuraman G, Sugandhan S, Bansal A, et al; Familial pigmented purpuric dermatoses.; J Dermatol. 2006 Sep;33(9):639-41. [abstract]
7. Nishioka K, Katayama I, Masuzawa M, et al; Drug-induced chronic pigmented purpura.; J Dermatol. 1989 Jun;16(3):220-2. [abstract]
8. Ugajin T, Satoh T, Yokozeki H, et al; Mycosis fungoides presenting as pigmented purpuric eruption. Eur J Dermatol. 2005 Nov-Dec;15(6):489-91. [abstract]
9. Nikam BP, Singh NJ, Shetty DD; Primary benign hypergammaglobulinemic purpura of Waldenstrom masquerading as Indian J Dermatol Venereol Leprol. 2011 Mar-Apr;77(2):205-8.
10. Fathy H, Abdelgaber S; Treatment of pigmented purpuric dermatoses with narrow-band UVB: a report of six J Eur Acad Dermatol Venereol. 2011 May;25(5):603-6. doi: [abstract]
11. de Godoy JM, Batigalia F; Aminaphtone in the control of Schamberg's disease. Thromb J. 2009 Jun 11;7:8. [abstract]
12. Seckin D, Yazici Z, Senol A, et al; A case of Schamberg's disease responding dramatically to PUVA treatment. Photodermatol Photoimmunol Photomed. 2008 Apr;24(2):95-6. [abstract]

Internet and further reading

• Capillaritis, DermNet NZ
• Ho C, Yan P, Mistry N, et al; Dermacase: Can you identify this condition? 4. Progressive pigmented purpuric Can Fam Physician. 2010 Oct;56(10):1023, 1024-5.