eMedicine Specialties > Dermatology > Diseases of the Vessels

Pigmented Purpuric Dermatitis

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
Coauthor(s): Jennifer Michelle Heyl, MD, Resident Physician, Department of Dermatology, Wayne State University School of Medicine
Contributor Information and Disclosures

Updated: Jan 19, 2010

Introduction

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.

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.

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.

Clinical

History

Patients complain about the appearance of their skin.

  • In Schamberg disease, irregular plaques and patches of orange-brown pigmentation develop on the lower limbs. The lesions are chronic and persist for years. With time, many of the lesions tend to extend and may become darker brown in color, but some may spontaneously clear.
  • In itching purpura, the lesions are much more extensive, and patients typically complain of severe pruritus.

Physical

The hallmark of a pigmented purpuric dermatosis is its characteristic orange-brown, speckled, cayenne pepper–like discoloration.

  • The lower limbs are affected in Schamberg disease, whereas itching purpura is characterized by more generalized skin involvement.
  • In lichen aureus, the eruption is usually a solitary lesion or a localized group of golden brown lesions that may affect any part of the body; however, the leg is the most commonly affected area. Linear or segmental forms of lichen aureus have been reported.
  • Majocchi disease is characterized by small annular plaques of purpura that contain prominent telangiectasias.
  • Pigmented purpura with lichenoid-type skin change is yet another clinical variant, which Gougerot and Blum first reported. Lesions appear similar to those of Schamberg disease in association with red-brown lichenoid papules.
  • Note the clinical images below.

  • Pigmented purpuric dermatitis affecting the trunk...

    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.

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    Pigmented purpuric dermatitis affecting the trunk...

    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 pigm...

    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.

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    Lichen aureus is the name given to localized pigm...

    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.


  • Capillaritis affecting the lower legs is known as...

    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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    Capillaritis affecting the lower legs is known as...

    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.

Causes

The cause of pigmented purpuric dermatoses is unknown. Rare familial cases of Schamberg disease and Majocchi disease have been reported in the literature, implying a genetic cause in a minority of patients.

More on Pigmented Purpuric Dermatitis

Overview: Pigmented Purpuric Dermatitis
Differential Diagnoses & Workup: Pigmented Purpuric Dermatitis
Treatment & Medication: Pigmented Purpuric Dermatitis
Follow-up: Pigmented Purpuric Dermatitis
Multimedia: Pigmented Purpuric Dermatitis
References
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References

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Further Reading

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Keywords

pigmented purpuric dermatitis, pigmented purpuric dermatosis, pigmented purpuric dermatoses, capillaritis, benign pigmented purpura, pigmented purpuric eruptions, Schamberg disease, progressive pigmentary dermatosis, itching purpura of Loewenthal, eczematid-like purpura of Doucas and Kapetanakis, pigmented purpuric lichenoid dermatosis of Gougerot and Blum, lichen aureus, purpura annularis telangiectoides, purpura annularis telangiectodes, purpura simplex, Majocchi disease

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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.

Medical Editor

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.

Pharmacy Editor

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.

Managing Editor

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.

CME Editor

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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