Pigmented Purpuric Dermatitis Workup

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

Laboratory Studies

  • A complete blood cell count is necessary to exclude thrombocytopenia, and coagulation screening helps to exclude other possible causes of purpura.
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Imaging Studies

  • Dermoscopy has been reported to be a useful tool for assisting the clinical diagnosis of pigmented purpuric dermatoses.[13]
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Other Tests

  • Capillary fragility may be assessed by the Hess test.
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Procedures

  • A skin biopsy helps to confirm the diagnosis of a pigmented purpuric eruption and aids in excluding cutaneous T-cell lymphoma, which in its early stages may closely mimic a pigmented purpuric dermatitis both clinically and histologically.[14]
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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.

Endothelial cell swelling is a histologic feature 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.

Hemosiderin deposition is seen in dermal macrophagHemosiderin 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]

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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

  1. Hoesly FJ, Huerter CJ, Shehan JM. Purpura annularis telangiectodes of Majocchi: case report and review of the literature. Int J Dermatol. Oct 2009;48(10):1129-33. [Medline].

  2. Yanez S, Val-Bernal JF. Purpuric generalized lichen nitidus: an unusual eruption simulating pigmented purpuric dermatosis. Dermatology. 2004;208(2):167-70. [Medline].

  3. Alexandrescu DT, Dutcher JP, O'Boyle K, et al. Fatal intra-alveolar hemorrhage after rituximab in a patient with non-Hodgkin lymphoma. Leuk Lymphoma. Nov 2004;45(11):2321-5. [Medline].

  4. Barnhill RL, Braverman IM. Progression of pigmented purpura-like eruptions to mycosis fungoides: report of three cases. J Am Acad Dermatol. Jul 1988;19(1 Pt 1):25-31. [Medline].

  5. Hanna S, Walsh N, D'Intino Y, Langley RG. Mycosis fungoides presenting as pigmented purpuric dermatitis. Pediatr Dermatol. Jul-Aug 2006;23(4):350-4. [Medline].

  6. Puddu P, Ferranti G, Frezzolini A, et al. Pigmented purpura-like eruption as cutaneous sign of mycosis fungoideswith autoimmune purpura. J Am Acad Dermatol. Feb 1999;40(2 Pt 2):298-9. [Medline].

  7. Toro JR, Sander CA, LeBoit PE. Persistent pigmented purpuric dermatitis and mycosis fungoides: simulant, precursor, or both? A study by light microscopy and molecular methods. Am J Dermatopathol. Apr 1997;19(2):108-18. [Medline].

  8. Waltermann K, Marsch WCh, Kreft B. [Bufexamac-induced pigmented purpuric eruption]. Hautarzt. May 2009;60(5):424-7. [Medline].

  9. Tsao H, Lerner LH. Pigmented purpuric eruption associated with injection medroxyprogesterone acetate. J Am Acad Dermatol. Aug 2000;43(2 Pt 1):308-10. [Medline].

  10. Adams BB, Gadenne AS. Glipizide-induced pigmented purpuric dermatosis. J Am Acad Dermatol. Nov 1999;41(5 Pt 2):827-9. [Medline].

  11. Pantanowitz L, Dezube BJ, Pinkus GS, Tahan SR. Histological characterization of regression in acquired immunodeficiency syndrome-related Kaposi's sarcoma. J Cutan Pathol. Jan 2004;31(1):26-34. [Medline].

  12. Buckthal-McCuin J, Mutasim DF. Macular arteritis mimicking pigmented purpuric dermatosis in a 6-year-old caucasian girl. Pediatr Dermatol. Jan-Feb 2009;26(1):93-5. [Medline].

  13. Zaballos P, Puig S, Malvehy J. Dermoscopy of pigmented purpuric dermatoses (lichen aureus): a useful tool for clinical diagnosis. Arch Dermatol. Oct 2004;140(10):1290-1. [Medline].

  14. [Best Evidence] Magro CM, Schaefer JT, Crowson AN, Li J, Morrison C. Pigmented purpuric dermatosis: classification by phenotypic and molecular profiles. Am J Clin Pathol. Aug 2007;128(2):218-29. [Medline].

  15. Wong WR, Kuo TT, Chen MJ, Chan HL. Granulomatous variant of chronic pigmented purpuric dermatosis: report of two cases. Br J Dermatol. Jul 2001;145(1):162-4. [Medline].

  16. Kerns MJ, Mallatt BD, Shamma HN. Granulomatous pigmented purpura: an unusual histological variant. Am J Dermatopathol. Feb 2009;31(1):77-80. [Medline].

  17. Kim SK, Kim EH, Kim YC. Treatment of Pigmented Purpuric Dermatosis with Topical Photodynamic Therapy. Dermatology. Jul 8 2009;[Medline].

  18. Wong WK, Ratnam KV. A report of two cases of pigmented purpuric dermatoses treated with PUVA therapy. Acta Derm Venereol. 1991;71(1):68-70. [Medline].

  19. Seckin D, Yazici Z, Senol A, Demircay Z. A case of Schamberg's disease responding dramatically to PUVA treatment. Photodermatol Photoimmunol Photomed. Apr 2008;24(2):95-6. [Medline].

  20. Lasocki AL, Kelly RI. Narrowband UVB therapy as an effective treatment for Schamberg's disease. Australas J Dermatol. Feb 2008;49(1):16-8. [Medline].

  21. Tamaki K, Yasaka N, Osada A, et al. Successful treatment of pigmented purpuric dermatosis with griseofulvin. Br J Dermatol. Jan 1995;132(1):159-60. [Medline].

  22. Okada K, Ishikawa O, Miyachi Y. Purpura pigmentosa chronica successfully treated with oral cyclosporin A. Br J Dermatol. Jan 1996;134(1):180-1. [Medline].

  23. Laufer F. The treatment of progressive pigmented purpura with ascorbic acid and a bioflavonoid rutoside. J Drugs Dermatol. Mar 2006;5(3):290-3. [Medline].

  24. Aiba S, Tagami H. Immunohistologic studies in Schamberg's disease. Evidence for cellular immune reaction in lesional skin. Arch Dermatol. Jul 1988;124(7):1058-62. [Medline].

  25. Basak PY, Ergin S. Should pentoxifylline be regarded as an effective treatment for Schamberg's disease?. J Am Acad Dermatol. Mar 2001;44(3):548-9. [Medline].

  26. Burrows NP, Jones RR. Cell adhesion molecule expression in capillaritis. J Am Acad Dermatol. Nov 1994;31(5 Pt 1):826. [Medline].

  27. Champion RH, Burton JL, Burns DA. Purpura. In: Textbook of Dermatology. 6th ed. London, England: Blackwell Science; 1998:. 2149-52.

  28. Farrokhzad S, Champion RH. Pigmented purpuric dermatoses. Dermatologica. 1970;140(1):45-53. [Medline].

  29. Ghersetich I, Lotti T, Bacci S, et al. Cell infiltrate in progressive pigmented purpura (Schamberg's disease): immunophenotype, adhesion receptors, and intercellular relationships. Int J Dermatol. Dec 1995;34(12):846-50. [Medline].

  30. Kwon SJ, Lee CW. Figurate purpuric eruptions on the trunk: acetaminophen-induced rashes. J Dermatol. Nov 1998;25(11):756-8. [Medline].

  31. Okada K, Ishikawa O, Miyachi Y. Purpura pigmentosa chronica successfully treated with oral cyclosporin A. Br J Dermatol. Jan 1996;134(1):180-1. [Medline].

  32. Saito R, Matsuoka Y. Granulomatous pigmented purpuric dermatosis. J Dermatol. Aug 1996;23(8):551-5. [Medline].

  33. Sardana K, Sarkar R, Sehgal VN. Pigmented purpuric dermatoses: an overview. Int J Dermatol. Jul 2004;43(7):482-8. [Medline].

  34. Simon M Jr, Heese A, Gotz A. Immunopathological investigations in purpura pigmentosa chronica. Acta Derm Venereol. 1989;69(2):101-4. [Medline].

 
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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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