This group of diseases have in common a purpuric appearance usually occurring on the lower extremities of younger adults. At least four major variants have been described. Under the microscope, these lesions share similar features. This disease can sometimes mimic Kaposi's sarcoma or even Mycosis fungoides, prompting a biopsy. The pathologist can easily distinguish this disorder from these other malignancies.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Progressive pigmented purpura INCIDENCE Common AGE RANGE-MEDIAN Younger adults
DISEASE ASSOCIATIONS CHARACTERIZATION Isolated reports following exposure Dyes
PATHOGENESIS CHARACTERIZATION GENERAL Several mechanisms have been proposed but no single theory has explained all of the changes
Delayed humoral immunity
Perforater vein incompetence
T-CELL LYMPHOID DYSCRASIA
- Lichen aureus: clinicopathologic features, natural history, and relationship to mycosis fungoides.
Department of Dermatology, Medical University of Graz, Graz, Austria.
- Arch Dermatol. 2008 Sep;144(9):1169-73. Abstract quote
BACKGROUND: A possible association between lichen aureus (LA) and mycosis fungoides (MF) has been suggested in the past. We evaluated the clinicopathologic features of LA and its relationship to MF. Data from 23 patients with a clinicopathologic diagnosis of LA were reviewed.
OBSERVATIONS: Lesions were asymmetrically localized on 1 area of the body (mostly 1 extremity) and were characterized histologically by dense, bandlike lymphocytic infiltrates. A monoclonal T-cell population was detected in half of the cases. After a mean follow-up of 102.1 months, 14 patients had no sign of skin disease, 7 patients had unmodified skin lesions, and 2 other patients with unmodified skin lesions had died of unrelated conditions. Treatment modalities did not affect the outcome. There was no relationship between the presence or absence of monoclonality and patient status at follow-up assessments.
Conclusion Patients with classic lesions of LA do not show progression to MF.
- Pigmented purpuric dermatosis: classification by phenotypic and molecular profiles.
Department of Pathology and Laboratory Medicine, Weill Medical College of Cornell University New York Presbyterian Hospital-Cornell Campus, New York, NY 10021, USA.
- Am J Clin Pathol. 2007 Aug;128(2):218-29. Abstract quote
The categorization of pigmented purpuric dermatosis (PPD) as a form of cutaneous lymphoid dyscrasia has been suggested. Phenotypic and molecular studies were done on 43 patients with PPD. The molecular studies used a capillary gel electrophoresis T-cell receptor beta multiplex polymerase chain reaction assay.
There were 2 principal categories: polyclonal PPD represented by 22 cases and monoclonal variants comprising 21 cases. Monoclonal cases had extensive skin lesions. An identical restricted T-cell repertoire independent of time and location was observed. Approximately 40% of the monoclonal cases had clinical and pathologic features of mycosis fungoides (MF). In the polyclonal variant, disease outside the lower extremities was uncommon; there were no patients with MF. Striking reductions in CD7 and CD62L were seen in both groups.
PPD is a form of cutaneous T-cell lymphoid dyscrasia, based on the frequency of monoclonality, the preservation of persistent T-cell clonotypes, and extent of pan-T-cell marker loss. Stratification of lesions of PPD according to the molecular profile may be of significant value prognostically and influence therapeutic intervention.
GROSS APPEARANCE/CLINICAL VARIANTS CHARACTERIZATION GENERAL
Progressive pigmentary dermatosis (Schamberg's disease)
Eczematid-like purpura of Doucas and Kapetanakis
Itching purpura of Loewenthal
Dissemintated prurignous angiodermatitis
Most common type
Acute onset and self-limited
Punctate purpuric macules forming confluent patches
Symmetrical in pretibial region
Familial cases rarely
Unilateral cases rarely
Purpura annularis telangiectodes of Majocchi Annular patches with perifollicular, red punctate lesions and telangiectasia Pigmented purpuric lichenoid dermatosis of Gougerot and Blum
Lichenoid papular coalesce to form plaque-like lesions
Symmetrical on lower legs
Grouped macules or lichenoid papules with rusty, golden, or purplish color
Lower legs common
Also trunk or upper extremity
Schamberg's purpura in children: A review of 13 cases.
Torrelo A, Requena C, Mediero IG, Zambrano A.
Departments of Dermatology and Pathology Hospital del Nino Jesus.
J Am Acad Dermatol 2003 Jan;48(1):31-3 Abstract quote
BACKGROUND: Schamberg's purpura (progressive pigmentary dermatosis or pigmented purpuric dermatosis) is uncommonly described in preadolescent children. Patients and Methods: A retrospective review of cases of Schamberg's purpura with onset before 10 years of age was undertaken. Clinical and histopathologic data, as well as analytical studies including hemogram, erythrocyte sedimentation rate, serum chemistry, urinalysis, cryoglobulins, and screening for thrombotic and clotting disorders were recorded.
RESULTS: A total of 13 patients (3 male and 10 female) from 1 to 9 years of age (median, 5 years) had the typical cutaneous features of Schamberg's purpura. The lower limbs were affected in all patients. Lesions were unilateral in 3 cases. In 4 patients, lesions faded within 1 to 4 years, and in 1 patient lesions improved after 6 years. In 8 patients lesions persisted 1 to 7 years after. Laboratory studies were normal in all patients.
CONCLUSION: Schamberg's purpura is a chronic benign form of pigmented purpura which is occasionally seen before puberty.
Chronic vulvar purpura: persistent pigmented purpuric dermatitis (lichen aureus) of the vulva or plasma cell (Zoon's) vulvitis?
Li Q, Leopold K, Carlson JA.
Division of Dermatopathology, and Department of Obstetrics and Gynecology, Albany Medical College, Albany, NY, USA.
J Cutan Pathol. 2003 Oct;30(9):572-6. Abstract quote
BACKGROUND: Lichen aureus is localized variant of persistent pigmented purpuric dermatitis that typically affects the legs and can be associated with delayed hypersensitivity reactions or vascular abnormalities. Plasma cell vulvitis (Zoon's vulvitis) is a rare condition that frequently contains hemosiderin deposits and is suspected to be a mucosal reaction pattern due to variety of insults, most often local irritation or trauma.
CASE REPORT: A 50-year-old female with longstanding complaints of spotting, vulvar dryness, irritation, and dyspareunia presented with circumscribed, purpuric, erythematous vulvar patches. Past estrogen cream treatment evoked symptoms of discomfort. On biopsy, siderophages and extravasated red blood cells were found in conjunction with a lichenoid, lymphocyte and plasma cell infiltrate, and dilated dermal and intraepithelial vessels.
CONCLUSIONS: Reported herein is an unusual vulvar dermatosis that is best classified as a localized variant of persistent pigmented dermatosis (lichen aureus) but overlaps clinically and histologically with Zoon's vulvitis. This constellation of findings may represent a site-specific mucosal reaction to an erosive process that could either be inflammatory (hypersensitivity reaction) and/or traumatic in nature.
HISTOLOGICAL TYPES CHARACTERIZATION Classic
Variable infiltrate of lymphocytes and macrophages in upper dermis with lymphocytic vasculitis in papillary dermis in active cases
May have more lichenoid changes in lichen aureus
Variable extravasation of red blood cells in papillary dermis with hemosiderin, sometimes in macrophages
- Granulomatous pigmented purpura: an unusual histological variant.
Department of Dermatology, Wright State University, Dayton, OH 45408, USA.
- Am J Dermatopathol. 2009 Feb;31(1):77-80. Abstract quote
Pigmented purpuric dermatoses (PPDs) tend to vary clinically, yet share a similar histology. Granulomas are only rarely seen, with 4 prior reported cases, designated granulomatous pigmented purpura. Historically, granulomatous pigmented purpura has been seen in Asians.
In this article, we report the case of a 42-year-old, white female with granulomatous pigmented purpura, the fifth such reported case in the literature. Histopathological examination revealed a granulomatous dermatitis with eosinophils, extravasated erythrocytes, melanophages, and vascular proliferation. These findings were consistently reported on subsequent biopsy 6 months later. This case illustrates a rare histological finding in PPDs. Medications associated with PPD are reviewed and the patient's use of mesalamine and balsalazide for ulcerative colitis are deemed potential triggers, given their relative similarity to aspirin, a known trigger of PPD.
Although other granulomatous processes must be excluded in such patients, one must consider the possibility of granulomatous pigmented purpura in the appropriate setting.
CHARACTERIZATION CD4+ Majority of lymphocytes
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Mycosis fungoides Rarely, MF may clinically and histologically resemble this disease
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Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
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