Medscape is available in 5 Language Editions – Choose your Edition here.


Pigmented Purpuric Dermatitis

  • Author: Darius Mehregan, MD; Chief Editor: Dirk M Elston, MD  more...
Updated: Mar 31, 2016


The pigmented purpuric dermatoses are a group of chronic diseases of mostly unknown etiology that have a very distinctive clinical appearance. They are characterized by extravasation of erythrocytes in the skin with marked hemosiderin deposition.

A number of clinical patterns of pigmented purpuric dermatoses or capillaritis are recognized that may represent different presentations of the same disorder; however, this generally does not influence the treatment or the prognosis. They all show a similar histologic appearance. The term pigmented purpuric dermatoses includes Schamberg disease (ie, progressive pigmentary dermatosis), purpura annularis telangiectodes (Majocchi disease),[1] lichen aureus, itching purpura, eczematidlike purpura of Doucas and Kapetanakis, and the pigmented purpuric lichenoid dermatosis of Gougerot and Blum. Many consider itching purpura and eczematidlike purpura to be variants of Schamberg disease.



The etiology is unknown. Several cofactors have been reported that appear to influence disease presentation, including hypertension, diabetes mellitus, venous stasis, strenuous exercise, gravitational dependency, capillary fragility, focal infections, and chemical ingestion.[2] Histologically, a perivascular T-cell lymphocytic infiltrate is centered on the superficial small blood vessels of the skin, which show signs of endothelial cell swelling and narrowing of the lumen. Extravasation of red blood cells with marked hemosiderin deposition in macrophages is also found, and a rare granulomatous variant of chronic pigmented dermatosis has been reported.




United States

Pigmented purpuric dermatoses are common.


During a 10-month period, the author's United Kingdom hospital-based dermatology practice, which serves a population of 300,000 persons, identified only 10 such cases. Five cases were diagnosed as having lichen aureus, and the remainder had more extensive capillaritis.


Persons of any race can be affected by pigmented purpuric dermatoses.


Pigmented purpuric dermatoses usually occur more frequently in men than in women. However, purpura annularis telangiectodes of Majocchi is seen more frequently in women.


Schamberg disease may occur in persons of any age.

Itching purpura and the dermatosis of Gougerot and Blum mainly affect middle-aged men.

Lichen aureus and Majocchi disease are predominantly diseases of children or young adults.

Contributor Information and Disclosures

Darius Mehregan, MD Associate Professor, Hermann Pinkus Chairman of Dermatology, Department of Dermatology, Wayne State University School of Medicine; Clinical Associate Professor of Pathology, University of Toledo College of Medicine; Dermatopathologist, Pinkus Dermatopathology Laboratory; Consulting Staff, Department of Dermatology, J Dingell Veterans Affairs Medical Center

Darius Mehregan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society of Dermatopathology, International Society of Dermatology, International Society of Dermatopathology, Phi Beta Kappa, Society for Investigative Dermatology

Disclosure: Nothing to disclose.


Jennifer Michelle Heyl, MD Resident Physician, Department of Dermatology, Wayne State University School of Medicine

Disclosure: Nothing to disclose.

Rahil M Dharia Wayne State University School of Medicine

Rahil M Dharia is a member of the following medical societies: American Association of Physicians of Indian Origin, American Medical Association, American Medical Student Association/Foundation, Association of Students for Hinduism Awareness, Michigan Association of Physicians of Indian Heritage

Disclosure: Nothing to disclose.

Specialty Editor Board

Richard P Vinson, MD Assistant Clinical Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Texas Medical Association, Association of Military Dermatologists, Texas Dermatological Society

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jean-Hilaire Saurat, MD Chair, Professor, Department of Dermatology, University of Geneva, Switzerland

Jean-Hilaire Saurat, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous authors, John D Wilkinson, MD, MBBS, MRCS, FRCP, and Cedric C Banfield, BSc, MSc, MBBS, MRCP(UK), to the development and writing of this article.

  1. Hoesly FJ, Huerter CJ, Shehan JM. Purpura annularis telangiectodes of Majocchi: case report and review of the literature. Int J Dermatol. 2009 Oct. 48(10):1129-33. [Medline].

  2. Kim DH, Seo SH, Ahn HH, Kye YC, Choi JE. Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis. Ann Dermatol. 2015 Aug. 27 (4):404-10. [Medline].

  3. Yanez S, Val-Bernal JF. Purpuric generalized lichen nitidus: an unusual eruption simulating pigmented purpuric dermatosis. Dermatology. 2004. 208(2):167-70. [Medline].

  4. Alexandrescu DT, Dutcher JP, O'Boyle K, et al. Fatal intra-alveolar hemorrhage after rituximab in a patient with non-Hodgkin lymphoma. Leuk Lymphoma. 2004 Nov. 45(11):2321-5. [Medline].

  5. Barnhill RL, Braverman IM. Progression of pigmented purpura-like eruptions to mycosis fungoides: report of three cases. J Am Acad Dermatol. 1988 Jul. 19(1 Pt 1):25-31. [Medline].

  6. Hanna S, Walsh N, D'Intino Y, Langley RG. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatr Dermatol. 2006 Jul-Aug. 23(4):350-4. [Medline].

  7. Puddu P, Ferranti G, Frezzolini A, et al. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoideswith autoimmune purpura. J Am Acad Dermatol. 1999 Feb. 40(2 Pt 2):298-9. [Medline].

  8. Toro JR, Sander CA, LeBoit PE. Persistent pigmented purpuric dermatitis and mycosis fungoides: simulant, precursor, or both? A study by light microscopy and molecular methods. Am J Dermatopathol. 1997 Apr. 19(2):108-18. [Medline].

  9. Waltermann K, Marsch WCh, Kreft B. [Bufexamac-induced pigmented purpuric eruption]. Hautarzt. 2009 May. 60(5):424-7. [Medline].

  10. Tsao H, Lerner LH. Pigmented purpuric eruption associated with injection medroxyprogesterone acetate. J Am Acad Dermatol. 2000 Aug. 43(2 Pt 1):308-10. [Medline].

  11. Adams BB, Gadenne AS. Glipizide-induced pigmented purpuric dermatosis. J Am Acad Dermatol. 1999 Nov. 41(5 Pt 2):827-9. [Medline].

  12. Pantanowitz L, Dezube BJ, Pinkus GS, Tahan SR. Histological characterization of regression in acquired immunodeficiency syndrome-related Kaposi's sarcoma. J Cutan Pathol. 2004 Jan. 31(1):26-34. [Medline].

  13. Buckthal-McCuin J, Mutasim DF. Macular arteritis mimicking pigmented purpuric dermatosis in a 6-year-old caucasian girl. Pediatr Dermatol. 2009 Jan-Feb. 26(1):93-5. [Medline].

  14. Zaballos P, Puig S, Malvehy J. Dermoscopy of pigmented purpuric dermatoses (lichen aureus): a useful tool for clinical diagnosis. Arch Dermatol. 2004 Oct. 140(10):1290-1. [Medline].

  15. Magro CM, Schaefer JT, Crowson AN, Li J, Morrison C. Pigmented purpuric dermatosis: classification by phenotypic and molecular profiles. Am J Clin Pathol. 2007 Aug. 128(2):218-29. [Medline].

  16. Wong WR, Kuo TT, Chen MJ, Chan HL. Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases. Br J Dermatol. 2001 Jul. 145(1):162-4. [Medline].

  17. Kerns MJ, Mallatt BD, Shamma HN. Granulomatous pigmented purpura: an unusual histological variant. Am J Dermatopathol. 2009 Feb. 31(1):77-80. [Medline].

  18. Kim SK, Kim EH, Kim YC. Treatment of Pigmented Purpuric Dermatosis with Topical Photodynamic Therapy. Dermatology. 2009 Jul 8. [Medline].

  19. Wong WK, Ratnam KV. A report of two cases of pigmented purpuric dermatoses treated with PUVA therapy. Acta Derm Venereol. 1991. 71(1):68-70. [Medline].

  20. Seckin D, Yazici Z, Senol A, Demircay Z. A case of Schamberg's disease responding dramatically to PUVA treatment. Photodermatol Photoimmunol Photomed. 2008 Apr. 24(2):95-6. [Medline].

  21. Lasocki AL, Kelly RI. Narrowband UVB therapy as an effective treatment for Schamberg's disease. Australas J Dermatol. 2008 Feb. 49(1):16-8. [Medline].

  22. Dhali TK, Chahar M, Haroon MA. Phototherapy as an effective treatment for Majocchi's disease--case report. An Bras Dermatol. 2015 Jan-Feb. 90 (1):96-9. [Medline].

  23. Tamaki K, Yasaka N, Osada A, et al. Successful treatment of pigmented purpuric dermatosis with griseofulvin. Br J Dermatol. 1995 Jan. 132(1):159-60. [Medline].

  24. Okada K, Ishikawa O, Miyachi Y. Purpura pigmentosa chronica successfully treated with oral cyclosporin A. Br J Dermatol. 1996 Jan. 134(1):180-1. [Medline].

  25. Laufer F. The treatment of progressive pigmented purpura with ascorbic acid and a bioflavonoid rutoside. J Drugs Dermatol. 2006 Mar. 5(3):290-3. [Medline].

Pigmented purpuric dermatitis affecting the trunk. Some of the lesions show the characteristic orange-brown, speckled, cayenne pepper–like discoloration that is the hallmark clinical sign of a capillaritis. Men are more frequently affected than women. If the lesions are pruritic, then the term itching purpura is sometimes used. Early cutaneous T-cell lymphoma, purpuric clothing contact dermatitis, and drug hypersensitivity reactions should be considered in the differential diagnosis.
Lichen aureus is the name given to localized pigmented purpuric dermatitis or capillaritis. In this patient, the skin on the extensor surface of the elbow is affected.
Histologic features of a skin biopsy sample obtained from a patient with lichen aureus shows extravasation of erythrocytes and a perivascular T-cell infiltrate.
Endothelial cell swelling is a histologic feature of capillaritis. This biopsy sample was obtained from a patient with lichen aureus.
Hemosiderin deposition is seen in dermal macrophages in this biopsy sample obtained from a patient with lichen aureus.
Capillaritis affecting the lower legs is known as Schamberg disease. In Schamberg disease, irregular plaques and patches of orange-brown pigmentation develop on the lower limbs.
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.