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Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis DH Kim, et al Ann Dermatol Vol. 27, No. 4, 2015 http://dx.doi.org/10.5021/ad.2015.27.4.404 ORIGINAL ARTICLE Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis Dai Hyun Kim, Soo Hong Seo, Hyo Hyun Ahn, Young Chul Kye, Jae Eun Choi Department of Dermatology, Korea University College of Medicine, Seoul, Korea Background: Pigmented purpuric dermatoses (PPD) are a sults were grossly consistent with the existing literature, ex- spectrum of disorders characterized by a distinct purpuric cluding several findings. Although a possible relationship rash. Although PPD can be easily diagnosed, the disease en- between PPD and cardiovascular disease or cardiovascular tity remains an enigma and a therapeutic challenge. medication was proposed at the beginning of the study, no Objective: The purpose of this study was to investigate the statistically significant correlations were found according to characteristics and clinical manifestations of PPD and to elu- the specific clinical types and treatment responses (p＞ cidate the relationship between assumed etiologic factors 0.05). (Ann Dermatol 27(4) 404∼410, 2015) and the clinical manifestations of PPD and treatment responses. Methods: Retrograde analyses were performed to -Keywords- identify appropriate PPD patients who visited Korea Classification, Etiology, Pigmented purpuric dermatoses, University Medical Center Anam Hospital from 2002 to Therapy 2012. Results: Information on 113 patients with PPD was an- alyzed, and 38 subjects with skin biopsy were included for this study. Schamberg's disease was the most frequent clin- INTRODUCTION ical type (60.5%). Concomitant diseases included hyper- tension (15.8%), diabetes (10.5%), and others. Associated Pigmented purpuric dermatosis (PPD) is a general term medication histories included statins (13.2%), beta blockers used to describe a group of chronic and relapsing cuta- (10.5%), and others. Possibly associated etiologic factors neous lesions. Such lesions are characterized by common were recent upper respiratory infection (5.3%), high ortho- petechiae and pigmentary macules of the skin that are pri- static pressure due to prolonged standing (2.6%), and stren- marily localized to the lower limbs; the histomorphologic uous exercise (2.6%). A total of 36 patients (94.7%) were features include superficial lymphocytic infiltration and treated with one or more treatment methods, including oral marked hemosiderin deposition with erythrocyte extra- antihistamines, pentoxifylline, topical steroids, and/or vasation1,2. phototherapy. There was no significant difference in disease PPD is generally categorized into six groups: progressive progress according to underlying diseases, medications, or PPD (i.e., Schamberg’s disease), purpura annularis te- association factors (p＞0.05). Conclusion: Our overall re- langiectodes (i.e., Majocchi’s disease), lichen aureus, PPD of Gougerot-Blum, itching purpura, and eczematid-like purpura of Doucas-Kapetanakis. Although the clinical pat- Received April 23, 2014, Revised November 18, 2014, Accepted for publication November 23, 2014 terns of PPD may represent different features of the same Corresponding author: Jae Eun Choi, Department of Dermatology, disorder with a similar histopathology, this does not gen- 2-4 Korea University Anam Hospital, Korea University College of erally influence management or prognosis . The cause of Medicine, 73 Inchon-ro, Seongbuk-gu, Seoul 136-705, Korea. Tel: 82- PPD is unknown, but several underlying diseases and 2-920-5470, Fax: 82-2-928-7540, E-mail: [email protected] drugs have been suggested to be associated with PPD, This is an Open Access article distributed under the terms of the such as chronic venous hypertension, diabetes mellitus, Creative Commons Attribution Non-Commercial License (http:// 2,4 creativecommons.org/licenses/by-nc/4.0) which permits unrestricted bezafibrate, and others . There are several published non-commercial use, distribution, and reproduction in any medium, case reports of certain clinical types of PPD or possible provided the original work is properly cited. causes, but few systematic reviews or large-scale studies 404 Ann Dermatol Characteristics of Pigmented Purpuric Dermatoses on PPD have been performed. RESULTS Therefore, this study investigated the characteristics and Sex and age clinical manifestations of PPD and elucidated the relation- ships among the assumed etiologic factors, clinical mani- There were a total of 38 patients including 18 men festations of PPD, and treatment responses in the Korean (47.4%) and 20 women (52.6%); the male to female ratio population. was 1:1.11. The overall mean age at diagnosis was 42.6±19.3 years, ranging from 13∼74 years. The mean MATERIALS AND METHODS ages of male and female patients were 37.4±21.2 and 47.3±16.6 years, respectively. Study participants Area of involvement A total of 113 patients diagnosed with PPD were retro- spectively selected from among patients who visited the The most commonly involved area was the lower ex- Dermatology Clinic of Korea University College of Medicine tremities (78.9%), followed by the upper and lower ex- (KUMC), Anam Hospital from January 2002 to December tremities (13.2%). Upper and lower extremity with trunk 2012. Among the 113 patients, only 38 underwent skin involvement (5.3%) and upper extremity and trunk in- biopsy and were included. The exclusion criteria were volvement (2.6%) were less common. lack of clinical photos or related medical history and am- Clinical characteristics biguous information about clinical progress. The diagnosis of PPD was made by clinical observation with or without On the basis of clinical and histopathological features, the histopathological examination by dermatologists. 38 patients were categorized into five clinical groups: Schamberg’s disease (n=23, 60.5%) (Fig. 1A), Majocchi’s Study design disease (n=8, 21.1%) (Fig. 1B), lichen aureus (n=2, 5.3%) Patient data including sex, age, disease duration, under- (Fig. 1C), PPD of Gougerot-Blum (n=3, 7.9%) (Fig. 2A) lying illness and medication, area of involvement, clinical and eczematid-like purpura of Doucas-Kapetanakis (n=2, type, histologic findings, treatment methods, and clinical 5.3%) (Fig. 2B, C). The sex, age, and involved areas of progress were gathered retrospectively from the electronic each clinical group are shown in Table 1. There were five medical records of KUMC, the archive of clinical photo- adolescent patients (13.2%), and the youngest was 13 graphs, and pathologic slides. This study was initially de- years old. Most adolescent patients had Majocchi’s dis- signed to investigate the growing number of PPD patients ease and not Schamberg’s disease. who had a history of cardiovascular disease and/or re- Histopathology ceived medications from the Department of Cardiovascu- lar, KUMC. Therefore, we focused on underlying illnesses Histologically, PPD showed numerous findings including and medications used to treat cardiovascular diseases to perivascular lymphocytic infiltration, red blood cell ex- analyze possible associations with PPD. Archived histo- travasation, hemosiderin deposition, endothelial cell swel- pathologic slides of the patients were evaluated, and typi- ling, spongiosis, lymphocyte exocytosis, and lichenoid cal findings and atypical features were analyzed according lymphocytic infiltration (Fig. 3A∼D)2,5-7. Other findings to clinical type. Furthermore, determine whether there recorded for further evaluation included interface change were differences in outcome with respect to clinical char- and basal hyperpigmentation. The most common patho- acteristics or specific treatment methods. The overall prog- logical characteristic was perivascular lymphocytic infiltra- ress of patients with PPD was classified into the following tion (79.0%), followed by endothelial cell swelling (52.6%), four categories: (1) completely resolved, (2) substantially red blood cell extravasation (50.0%), interface changes improved, (3) partially improved, and (4) unimproved or (42.1%), lichenoid lymphocytic infiltration (39.5%), basal aggravated. For multivariate analysis, the favorable catego- cell hyperpigmentation (29.0%), hemosiderin deposition ries (1) and (2) were classified as “responsive,” and catego- (26.3%), lymphocyte exocytosis (18.4%), and spongiosis ries (3) and (4) were classified as “resistant.” Statistical (13.2%) (Table 2). analysis was performed using Fisher’s exact test, and the Combined underlying diseases, etiologic factors, and level of significance was set at p＜0.05. All analyses were medications performed using IBM SPSS Statistics ver. 20.0 (IBM Co., Armonk, NY, USA). The concomitant underlying diseases were hypertension (15.8%), diabetes mellitus (10.5%), venous stasis (7.9%), angina pectoris (2.6%), thyroid dysfunction (2.6%), and Vol. 27, No. 4, 2015 405 DH Kim, et al Fig. 1. (A) Clinical findings of Schamberg’s disease. Characteristic “cayenne pepper” pigmentation on the lower leg. (B) Clinical findings of Majocchi’s disease. Red to light- brown annular patches on the lower leg exhibiting peripheral extension. (C) Clinical findings of lichen aureus. Multiple lichenoid purpuric papules on the lower leg. Fig. 2. (A) Clinical findings of pig- mented purpuric dermatosis of Gou- gerot-Blum. Lichenoid papules that tend to fuse into plaques of red- brown-colored pigmentation. (B, C) Clinical findings of eczematid-like purpura of Doucas-Kapetanakis. Se- verely pruritic diffusely eczematous purpuric patches are visible on both lower extremities.
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