Pigmented Purpuric Dermatitis

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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),[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.

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.

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

History

Patients complain about the appearance of their skin.

Physical

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

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.

Laboratory Studies

Imaging Studies

Other Tests

Procedures

Histologic Findings

Histologically, a perivascular infiltrate of lymphocytes and macrophages is centered on the superficial small blood vessels of the skin. Signs of endothelial cell swelling and narrowing of lumina may be seen, as demonstrated in the image below.


View Image

Endothelial cell swelling is a histologic feature of capillaritis. This biopsy sample was obtained from a patient with lichen aureus.

The infiltrate is composed of predominantly CD4+ lymphocytes along with occasional CD1a+ dendritic cells. Plasma cells and neutrophils are occasionally present; the latter is not uncommon in lesions of itching purpura. Extravasation of red blood cells with marked hemosiderin deposition in macrophages is typically seen, as demonstrated in the image below. However, the degree of hemosiderin deposition may be variable, and it can be minimal in early lesions of itching purpura.


View Image

Hemosiderin deposition is seen in dermal macrophages in this biopsy sample obtained from a patient with lichen aureus.

Histochemical staining with Perls stain and Fontana-Masson stain, to demonstrate iron (hemosiderin) and exclude melanin pigment respectively, may be helpful. Hemosiderin deposition in the dermis is more superficial in pigmented purpuric dermatitis than that seen in stasis dermatitis, which is a useful differentiating feature. Mild epidermal spongiosis and exocytosis of lymphocytes may be seen in all variants except lichen aureus, which, in general, tends to show a bandlike infiltrate separated from the epidermis by a thin rim of uninvolved collagen.

Kerns et al described an unusual variant of pigmented purpuric dermatoses, granulomatous pigmented purpura, in a 42-year-old white woman, and Wong et al reported 2 cases of a similar variant.[15, 16]

Medical Care

No medical intervention is of proven benefit for the treatment of the pigmented purpuric dermatoses.

Medication Summary

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Hydrocortisone topical (Westcort)

Clinical Context:  An adrenocorticosteroid derivative suitable for application to skin or external mucous membranes. Has mineralocorticoid and glucocorticoid effects, resulting in relief of pruritus.

Clobetasol (Temovate)

Clinical Context:  Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.

Betamethasone topical (Diprolene, Betatrex)

Clinical Context:  For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.

Class Summary

These agents are effective in relieving pruritus. These agents have anti-inflammatory properties and cause profound and varied metabolic effects. They modify the body's immune response to diverse stimuli.

Diphenhydramine (Benadryl, Benylin, Diphen, AllerMax)

Clinical Context:  For symptomatic relief of pruritus caused by release of histamine in inflammatory reactions.

Class Summary

These agents may treat itching by blocking effects of endogenously released histamine.

Further Outpatient Care

Prognosis

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

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 Editors

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

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

Disclosure: Nothing to disclose.

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory

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

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

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.

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.

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.

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.