Pigmented Purpuric Dermatitis 

  • Author: Darius Mehregan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 19, 2010
 

Background

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), 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.

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Pathophysiology

The etiology is unknown. Venous hypertension, exercise, and gravitational dependency are important cofactors that appear to influence disease presentation. 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.

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Epidemiology

Frequency

United States

Pigmented purpuric dermatoses are common.

International

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.

Mortality/Morbidity

Typically, the condition is asymptomatic, but pruritus may sometimes be a prominent feature in some cases, especially in patients with itching purpura or eczematidlike purpura of Doucas and Kapetanakis. These diseases have no systemic findings.

Race

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

Sex

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

Age

  • 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.
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Contributor Information and Disclosures
Author

Darius Mehregan, MD  Associate Professor, Hermann Pinkus Chairman of Dermatology, Department of Dermatology, Wayne State University; Clinical Associate Professor of Pathology, University of Toledo; Dermatopathologist, Pinkus 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, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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, American Dermatological Association, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University 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, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

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, and Texas Medical Association

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

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

Disclosure: Nothing to disclose.

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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.
 
 
 
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