Ann Dermatol.  2015 Aug;27(4):404-410. 10.5021/ad.2015.27.4.404.

Characteristics and Clinical Manifestations of Pigmented Purpuric Dermatosis

• Kim DH
• Seo SH
• Ahn HH
• Kye YC
• Choi JE

• KMID: 2171497
• DOI: http://doi.org/10.5021/ad.2015.27.4.404

Abstract

BACKGROUND
Pigmented purpuric dermatoses (PPD) are a spectrum of disorders characterized by a distinct purpuric rash. Although PPD can be easily diagnosed, the disease entity remains an enigma and a therapeutic challenge.
OBJECTIVE
The purpose of this study was to investigate the characteristics and clinical manifestations of PPD and to elucidate the relationship between assumed etiologic factors and the clinical manifestations of PPD and treatment responses.
METHODS
Retrograde analyses were performed to identify appropriate PPD patients who visited Korea University Medical Center Anam Hospital from 2002 to 2012.
RESULTS
Information on 113 patients with PPD was analyzed, and 38 subjects with skin biopsy were included for this study. Schamberg's disease was the most frequent clinical type (60.5%). Concomitant diseases included hypertension (15.8%), diabetes (10.5%), and others. Associated medication histories included statins (13.2%), beta blockers (10.5%), and others. Possibly associated etiologic factors were recent upper respiratory infection (5.3%), high orthostatic pressure due to prolonged standing (2.6%), and strenuous exercise (2.6%). A total of 36 patients (94.7%) were treated with one or more treatment methods, including oral antihistamines, pentoxifylline, topical steroids, and/or phototherapy. There was no significant difference in disease progress according to underlying diseases, medications, or association factors (p>0.05).
CONCLUSION
Our overall results were grossly consistent with the existing literature, excluding several findings. Although a possible relationship between PPD and cardiovascular disease or cardiovascular medication was proposed at the beginning of the study, no statistically significant correlations were found according to the specific clinical types and treatment responses (p>0.05).

Keyword

Classification; Etiology; Pigmented purpuric dermatoses; Therapy

MeSH Terms

Academic Medical Centers
Biopsy
Cardiovascular Diseases
Classification
Exanthema
Histamine Antagonists
Humans
Hydroxymethylglutaryl-CoA Reductase Inhibitors
Hypertension
Korea
Pentoxifylline
Phototherapy
Pigmentation Disorders
Skin
Skin Diseases*
Steroids
Histamine Antagonists
Pentoxifylline
Steroids

Figure

• 

  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 lightbrown 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 pigmented purpuric dermatosis of Gougerot-Blum. Lichenoid papules that tend to fuse into plaques of redbrown-colored pigmentation. (B, C) Clinical findings of eczematid-like purpura of Doucas-Kapetanakis. Severely pruritic diffusely eczematous purpuric patches are visible on both lower extremities.

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  Fig. 3 (A, B) Histologic findings of Schamberg's disease and (C, D) Majocchi's disease. (A) Dense perivascular lymphocytic infiltrations from the upper to deep dermis (H&E, ×40). (B) Mild spongiosis and vacuolar degeneration, extravasated red blood cells without the presence of leukocytoclastic vasculitis (H&E, ×100). (C) Lichenoid and superficial perivascular lymphocytic infiltrations (H&E, ×40). (D) Marked vacuolar degenerations, extravasated red blood cells, and numerous hemosiderin-laden macrophages without the presence of leukocytoclastic vasculitis (H&E, ×100).

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