Pigmented Purpuric Dermatitis Clinical Presentation

  • Author: Darius Mehregan, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 27, 2012
 

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

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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 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, 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, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

References

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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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