The blue (or purple) toe syndrome consists of the development of blue or violaceous discoloration of one or more toes in the absence of obvious trauma, serious cold-induced injury, or disorders producing generalized cyanosis. The major general categories are: (1) decreased arterial flow, (2) impaired venous outflow, and (3) abnormal circulating blood. Depending on its pathogenesis, the discoloration may be blanching or nonblanching. An accurate diagnosis is critical, because many of the causes threaten life and limb, but the patient's medical history, accompanying nondermatologic findings on physical examination, and a discriminating use of laboratory tests are usually more important than the nature of the cutaneous abnormalities in determining the cause. Learning objectives After completing this learning activity, participants should be able to define the blue (or purple) toe syndrome, categorize the causes, and recognize the important historical, clinical, and laboratory findings that differentiate the causes and lead to the correct diagnosis.

https://www.jaad.org/article/S0190-9622(08)01218-8/pdf

Blue (or purple) toe syndrome

Importance Infective endocarditis (IE) is a rare disease with poor prognosis. When IE is suspected, skin examination is mandatory to look for a portal of entry and classic skin lesions to help diagnose and manage the condition. Objectives To describe the prevalence of and factors associated with dermatological manifestations in patients with definite IE. Design Observational, prospective, population-based epidemiological study between January 1 and December 31, 2008. Subsequently, collected dermatological data were subjected to post hoc analysis. Setting and Participants Patients (n = 497) diagnosed in 7 French regions and hospitalized in France for definite IE satisfying modified Duke criteria. Main Outcomes and Measures Patient and disease epidemiological information was collected, focusing on the most classic dermatological manifestations of IE (Osler nodes, Janeway lesions, purpura, and conjunctival hemorrhages). Disease outcome was also recorded. Results Among 497 definite IE cases, 487 had known dermatological status. Of 487 cases, 58 (11.9%) had skin manifestations, including 39 (8.0%) with purpura, 13 (2.7%) with Osler nodes, 8 (1.6%) with Janeway lesions, and 3 (0.6%) with conjunctival hemorrhages (5 patients had 2 skin manifestations). Patients with skin manifestations had a higher rate of IE-related extracardiac complications than patients without skin manifestations, particularly cerebral emboli (32.8% vs 18.4%, P  = .01), without increased mortality. Patients with purpura had larger cardiac vegetations (18.1 vs 13.7 mm, P  = .01), and Janeway lesions were associated with more extracerebral emboli (75.0% vs 31.8%, P  = .02). Conclusions and Relevance Specific skin manifestations of IE are associated with a higher risk of complications and should alert physicians to examine for extracardiac complications, notably with cerebral imaging.

https://jamanetwork.com/journals/jamadermatology/articlepdf/1819586/doi130089.pdf

Prognostic Value of Skin Manifestations of Infective Endocarditis

We review the most characteristic clinical and histopathologic findings of the cutaneous manifestations of the occlusive nonvasculitic vasculopathic disorders. Clinically, most of these conditions are characterized by retiform purpura. Histopathologic findings consist of occlusion of the vessel lumina with no vasculitis. Different disorders may produce nonvasculitic occlusive vasculopathy in cutaneous blood and lymphatic vessels, including embolization due to cholesterol and oxalate emboli, cutaneous intravascular metastasis from visceral malignancies, atrial myxomas, intravascular angiosarcoma, intralymphatic histiocytosis, intravascular lymphomas, endocarditis, crystal globulin vasculopathy, hypereosinophilic syndrome, and foreign material. Other times, the occlusive disorder is due to platelet pugging, including heparin necrosis, thrombocytosis secondary to myeloproliferative disorders, paroxysmal nocturnal hemoglobinuria, and thrombotic thrombocytopenic purpura. Occlusive vasculopathy may also appear in cold-related gelling agglutination, like that occurring in cryofibrinogenemia, cryoglobulinemia, cold agglutinin syndrome, and crystalglobulinemia. Microorganisms may also occlude the vessels lumina and this is especially frequent in ecthyma gangrenosum, opportunistic fungi as aspergillosis or fusariosis, Lucio phenomenon of lepromatous leprosy and disseminated strongyloidiasis. Systemic coagulopathies due to defects of C and S proteins, coumarin/warfarin-induced skin necrosis, disseminated intravascular coagulation, and antiphospholipid antibody/lupus anticoagulant syndrome may also result in occlusive nonvasculitic vasculopathy. Finally, vascular coagulopathies such as Sneddon syndrome, livedoid vasculopathy, and atrophic papulosis may also cause occlusion of the vessels of the dermis and/or subcutis. Histopathologic study of occlusive vasculopathic lesions is the first step to achieve an accurate diagnosis, and they should be correlated with clinical history, physical examination, and laboratory findings to reach a final diagnosis.

Occlusive Nonvasculitic Vasculopathy.

https://www.cidjournal.com/article/S0738-081X(11)00045-9/pdf

Cutaneous signs of systemic disease

The vasculopathic reaction pattern

Organochlorine exposure is an important cause of cutaneous and systemic toxicity. Exposure has been associated with industrial accidents, intentional poisoning, and the use of defoliants, such as Agent Orange in the Vietnam War. Although long-term health effects are systematically reviewed by the Institute of Medicine, skin diseases are not comprehensively assessed. This represents an important practice gap as patients can present with cutaneous findings. This article provides a systematic review of the cutaneous manifestations of known mass organochlorine exposures in military and industrial settings with the goal of providing clinically useful recommendations for dermatologists seeing patients inquiring about organochlorine effects. Patients with a new diagnosis of chloracne, porphyria cutanea tarda, cutaneous lymphomas (non-Hodgkin lymphoma), and soft-tissue sarcomas including dermatofibrosarcoma protuberans and leiomyosarcomas should be screened for a history of Vietnam service or industrial exposure. Inconclusive evidence exists for an increased risk of other skin diseases in Vietnam veterans exposed to Agent Orange including benign fatty tumors, melanomas, nonmelanoma skin cancers, milia, eczema, dyschromias, disturbance of skin sensation, and rashes not otherwise specified. Affected veterans should be informed of the uncertain data in those cases. Referral to Department of Veterans Affairs for disability assessment is indicated for conditions with established associations.

https://www.jaad.org/article/S0190-9622(15)01644-8/pdf

Richard A Keller

Papers

Skin diseases associated with Agent Orange and other organochlorine exposures

Septic microemboli in a Janeway lesion of bacterial endocarditis

Use of QuikClot Combat Gauze during Mohs stages for intraoperative hemostasis.

Iconography : Skin diseases associated with Agent Orange and other organochlorine exposures