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Schamberg's Disease
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 discolouration is brown/orange due to haemosiderin deposition in the skin.
Pigmented purpuric reactions have six disease types: [1]
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.
Itching purpura of Lowenthal (disseminated pruriginous angiodermatitis) - rare (like Schamberg's
disease) but accompanied by itching.
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 and there are few epidemiological studies in the literature. One study of patients attending
an Indian outpatient clinic over an 18-month period found that there were 100 cases of pigmented purpuric
dermatosis in a total of 55,323 patients (0.18%). 95 of these patients had Schamberg's disease. The male-tofemale ratio was 3.8:1. The age range was from 11-66 years with a mean of 34 years. [2] It is a common cause of
petechiae in children. [3] There has been a case report of four family members with Schamberg's disease,
suggesting a possible genetic link. [4]
Aetiology [1] [5] [6]
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. It has also
been reported with bezafibrate and amlodipine. [7]
Presentation [6]
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.
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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 - eg, annular, linear. There may also be associated lichenification, scaling and pruritic marks.
Differential diagnosis [5] [8]
Other causes of purpura:
Vasculitis - eg, leukocytoclastic vasculitis.
T-cell lymphoma (especially if presenting in young males).
Drug eruption.
Trauma.
Self-induced purpura.
Mycosis fungoides. [9]
Primary benign hypergammaglobulinaemic purpura of Waldenström. [10]
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. [11]
Examination of the skin using a dermatoscope may be helpful although there are limited reports in the
literature. [12]
Associated diseases [1] [5]
Diabetes mellitus.
Rheumatoid arthritis.
Systemic lupus erythematosus.
Thyroid abnormalities.
Hepatic disease, including hepatitis B.
Porphyria.
Malignancies.
Dyslipidaemias.
Management [1] [6]
Any suspected precipitants should be withdrawn.
Itching - treat with mild topical corticosteroid or antihistamines.
Good results have been obtained with narrow-band ultraviolet light.
One study reported the successful use of aminaphtone, a drug normally used in other venous
conditions such as chronic venous congestion of the lower limbs. [13]
Superimposed infection - will need antibiotics.
Systemic steroids provide some benefits but these are outweighed by the risks of systemic sideeffects.
Advanced fluorescent technology has produced some promising cosmetic results. [14]
Other tried treatments include vitamin C supplements, laser therapy and wearing support hosiery to prevent
venous stasis. There is no evidence of definite benefit of the former two. Immunosuppressants have also been
used. [5] PUVA therapy has been used and the results look promising. [15]
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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.
The development of T-cell lymphoma in patients with Schamberg's disease has been reported. [16]
Further reading & references
Schamberg Disease Photograph; Dermnet Skin Disease Atlas, 2011
1. Kim DH, Seo SH, Ahn HH, et al; Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis. Ann
Dermatol. 2015 Aug;27(4):404-10. doi: 10.5021/ad.2015.27.4.404. Epub 2015 Jul 29.
2. Sharma L, Gupta S; Clinicoepidemiological study of pigmented purpuric dermatoses. Indian Dermatol Online J. 2012
Jan;3(1):17-20. doi: 10.4103/2229-5178.93486.
3. Mallory S et al; Illustrated Manual of Pediatric Dermatology, 2005.
4. Sethuraman G, Sugandhan S, Bansal A, et al; Familial pigmented purpuric dermatoses. J Dermatol. 2006 Sep;33(9):63941.
5. Sardana K, Sarkar R, Sehgal VN; Pigmented purpuric dermatoses: an overview; Int J Dermatol. 2004 Jul;43(7):482-8.
6. Capillaritis; DermNet NZ
7. Schetz D, Kocic I; Anew adverse drug reaction - Schamberg's disease caused by amlodipine administration - a case
report. Br J Clin Pharmacol. 2015 Aug 10. doi: 10.1111/bcp.12742.
8. 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.
9. 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.
10. Nikam BP, Singh NJ, Shetty DD; Primary benign hypergammaglobulinemic purpura of Waldenstrom masquerading as
disseminated Schamberg's purpura. Indian J Dermatol Venereol Leprol. 2011 Mar-Apr;77(2):205-8.
11. Billings S et al; Inflammatory Dermatopathology: APathologist's Survival Guide, 2010.
12. Khopkar U; Dermoscopy and Trichoscopy in Diseases of the Brown Skin, 2013.
13. de Godoy JM, Batigalia F; Aminaphtone in the control of Schamberg's disease. Thromb J. 2009 Jun 11;7:8.
14. Manolakos DA, Weiss J, Glick B, et al; Treatment of Schamberg's disease with advanced fluorescence technology. J Drugs
Dermatol. 2012 Apr;11(4):528-9.
15. Seckin D, Yazici Z, Senol A, et al; Acase of Schamberg's disease responding dramatically to PUVAtreatment.
Photodermatol Photoimmunol Photomed. 2008 Apr;24(2):95-6.
16. Shen Aet al ; Capillaritis as a potential harbinger of cutaneous T-cell lymphoma, 2004.
Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical
conditions. EMIS has used all reasonable care in compiling the information but make no warranty as to its
accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions.
For details see our conditions.
Original Author:
Dr Gurvinder Rull
Current Version:
Dr Laurence Knott
Peer Reviewer:
Dr Helen Huins
Document ID:
1723 (v23)
Last Checked:
14/09/2015
Next Review:
12/09/2020
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