Seminars in Diagnostic Pathology 34 (2017) 285–300

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Seminars in Diagnostic Pathology

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Vasculopathic and vasculitic dermatoses

Alejandro A. Gru a,n, Andrea L. Salavaggione b a Department of Pathology & Dermatology, University of Virginia, United States b Department of Radiation Oncology, The Ohio State University Wexner Medical Center, United States article info abstract

The inflammatory and non-inflammatory disorders affecting the blood vessels are an important cause of fi Keywords: death among patients. Additionally, some of the ndings could be very subtle, while the consequences of Leukocytoclastic misdiagnosis tragic. There is a limited number of factors by which the cutaneous blood vessels can be Polyarteritis nodosa affected and the most common examples in the clinical practice will be discussed here: non-in- Vasculitis flammatory purpuras; vascular-occlusive disorders; urticarias; vasculitides; and neutrophilic dermatoses. & 2017 Elsevier Inc. All rights reserved.

Introduction manifestations of vaso-occlusive disorders (VOD) are purpura, li- vedo reticularis, erythromelalgia, ulceration and infarction 3–5.Li- The inflammatory and non-inflammatory disorders affecting vedo reticularis is characterized by macular, violaceous, connecting the blood vessels are an important cause of death among patients. lines that form a net-like pattern in the skin 6. The following list of Additionally, some of the findings could be very subtle, while the entities can very frequently be associated with VODs, and are ty- consequences of misdiagnosis tragic. There is a limited number of pically worked-up in the setting of a thrombotic coagulopathy. – factors by which the cutaneous blood vessels can be affected and Those include: protein C and S deficiency,7 9 prothrombin gene the most common examples in the clinical practice will be dis- mutations,10 warfarin necrosis,11,12 atrophie blanche (livedoid cussed here: non-inflammatory purpuras; vascular-occlusive dis- vasculopathy),13 disseminated intravascular coagulation,14 purpura orders; urticarias; vasculitides; and neutrophilic dermatoses. fulminans,15,16 thrombotic (TTP),17 es- sential thrombocytemia,18 cryoglobulinemia,19 cholesterol emboli,20 antiphospholipid syndrome,21 factor V Leiden mutation,22 and Solar (senile) purpura others. Angioinvasive fungi can be an important cause of death among patients who are immune-supressed. Such cases are char- This is a common form of non-inflammatory purpura that acterized by the presence of thrombi containing hyphae and yeasts presents on the extensor surfaces of the forearms and hands of within the vascular lumens (Fig. 2). elderly individuals. It presents as large areas of purpura (ecchy- moses). Histologically is characterized by extravasation of red blood cells in the dermis, more typically in the superficial portions, Calciphylaxis in association with solar elastosis, and sometimes epidermal Calciphylaxis is a vasculopathic process characterized by the atrophy (Fig. 1).1,2 presence of calcification of the media of muscular arteries, and smaller sized caliber vessels, that occurs in association with chronic renal disease. The vessels from the superficial and deep Thrombotic coagulopathies dermis, and particularly adipose tissue are involved. Some cases occur in the absence of renal insufficiency. Calciphylaxis can be The consequence of the occlusion of the blood vessels in the accompanied by intimal hyperplasia, vascular thrombosis, super- dermis leads to numerous clinical manifestations. The prompt re- ficial ulceration, and extensive necrosis. There is usually an ele- cognition of this disorders is capital to start adequate therapy and vated calcium-phosphate product and secondary hyperparathyr- ultimately avoid the infarction of numerous organs and systems oidism. The calcium deposits can be further demonstrated by a that can lead to the patient's demise. The most frequent clinical Von-Kossa stain. Many biopsies (including deep surgical ones) are often required to establish the diagnosis, and it's in the author n Corresponding author. experience that serial sectioning and multiple Von-Kossa stains 23 E-mail address: [email protected] (A.A. Gru). can help to establish this diagnosis (Fig. 3). http://dx.doi.org/10.1053/j.semdp.2017.04.001 0740-2570/& 2017 Elsevier Inc. All rights reserved. 286 A.A. Gru, A.L. Salavaggione / Seminars in Diagnostic Pathology 34 (2017) 285–300

Fig. 1. Solar purpura. The biopsy shows very mild epidermal atrophy and a subtle perivascular infiltrate (1a, 40x)), in a background of solar elastosis. Closer views show a superficial and perivascular mild lymphocytic infiltrate with red blood cell extravasation (1b-1d, 200x).

Fig. 2. Angioinvasive fungal infection. There is ischemia of the surface , manifested by the presence of pallor of the basal keratinocytes. Large vascular thrombi are noted. There is a negative image on the H&E stained sections, which corresponds to fungal hyphae by a GMS stain. Download English Version: https://daneshyari.com/en/article/5716695

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