A 38-year-old woman presents with a rash affecting her legs. She first noticed the rash, which has been slowly increasing in size, ~2 years ago. Prior therapy with miconazole cream proved ineffectual. The patient denies itching or other localized symptoms. The patient’s medical history is negative for diabetes, eczema, and thyroid disease. Examination reveals well-demarcated, erythematous macules of her lower legs interspersed with brownish pigmentation.
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Pigmented purpuric dermatosis is a generic term used to describe a variety of chronic skin conditions characterized by petechiae and pigmented macules.1 Several variants are recognized, many of which are named after the clinician who first described them. Examples include are Schamberg disease (also termed progressive pigmented purpuric dermatosis), Majocchi disease (purpura annularis telangiectodes), pigmented purpuric lichenoid dermatosis of Gougerot-Blumand, and Doucas-Kapetanakis eczematid-like purpura. Schamberg disease is the most common type, named after Jay Frank Schamberg who published a case series on this condition in 1901.2
Schamberg disease affects the lower extremities and is more common in males than females. The condition is usually asymptomatic and is characterized by petechiae, purpura, and increased skin pigmentation. The pigmentation results from hemosiderin deposition secondary to extravasation of red blood cells from leaking capillaries.3 Reddish-brown discolorations along the border of older lesions are referred to as cayenne pepper spots.3
Most cases are idiopathic although gravitational influence, alcohol, and certain medications may be contributing factors.4 Schamberg disease is often treatment-resistant; early treatment with topical corticosteroids may minimize underlying inflammation. Improvement has been reported with colchicine, pentoxifylline, and fractional non-ablative laser.5-7
Amy DePalma, MSN, NP-C is a nurse practitioner on staff at the DermDox Center for Dermatology in Gilbert, Pennsylvania.
Stephen Schleicher, MD, is director of the DermDox Center for Dermatology in Pennsylvania, as well as an associate professor of medicine at Commonwealth Medical College and a clinical instructor of dermatology at Arcadia University and Kings College.
- Kim DH, Seo SH, Ahn HH, Kye YC, Choi JE. Characteristics and clinical manifestations of pigmented purpuric dermatosis. Ann Dermatol. 2015;27(4):404-410.
- Saxena SK, Spangler M, Mikkilineni A. Schamberg’s Disease. J Urgent Care Med. Accessed November 17, 2020. https://www.jucm.com/schambergs-disease/.
- Sardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: an overview. Int J Dermatol. 2004;43(7):482-488.
- Zaldivar Fujigaki JL, Anjum F. Schamberg disease. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2020.
- Cavalcante MLLL, Masuda PY, Brito FF, Pinto ACVD, Itimura G, Nunes AJF. Schamberg’s disease: case report with therapeutic success by using colchicine. An Bras Dermatol. 2017;92(2):246-248.
- Kano Y, Hirayama K, Orihara M, Shiohara T. Successful treatment of Schamberg’s disease with pentoxifylline. J Am Acad Dermatol. 1997;36(5 Pt 2):827-830.
- Hilerowicz Y, Sprecher E, Gat A, Artzi O. Successful treatment of Schamberg’s disease with fractional non-ablative 1540 nm erbium:glass laser. J Cosmet Laser Ther. 2018;20(5):265-268.