Isolated Benign Primary Cutaneous Plasmacytosis in a Child

Isolated benign primary cutaneous plasmacytosis in a child is a very rare and benign disease. Herein we present a case of this condition occurring in a child who showed good response to topical corticosteroid.     

Increased number of circulating endothelial cells (CECs) in patients with psoriasis ‐ preliminary report

Background Numerous studies have demonstrated increased cardiovascular risk in psoriasis. Circulating endothelial cells (CECs) have been proposed as a new marker of endothelial dysfunction that plays an important role in pathogenesis of atherosclerosis. Objective The aim of this study was to compare the number of CECs in psoriatic patients to a control group and to analyze possible correlations between the numbers of CECs and the plasma levels of classical markers of endothelial dysfunction, such as: sICAM‐1, sE‐selectin and von Willebrand factor (vWF). Methods The number of CECs, identified as CD146 + / CD45‐ cells, were determined in peripheral blood with using flow cytometry in psoriatic patients (n = 63) and controls (n = 31). The plasma levels of: sICAM‐1, sE‐selectin, vWF were measured with ELISA. The severity of psoriasis was assessed with PASI. Results The number of CECs was significantly increased in psoriatic patients compared with controls (P < 0.00001) and positively correlated with disease severity (R = 0.360; P = 0.0037). The levels of sICAM‐1, sE‐selectin and vWF were significantly elevated in psoriatic patients (P < 0.00001; P < 0.00001; P = 0.00072, respectively). The number of CECs was significantly, positively correlated with the levels of sICAM‐1 (R = 0.393; P = 0.0014) and vWF (R = 0.314; P = 0.012) in psoriatic patients. The levels of sICAM‐1 and sE‐selectin were positively correlated with disease severity (R = 0.356; P = 0.0041 and R = 0.407; P = 0.0009, respectively). Conclusion The increased number of CECs that correlates with disease severity and plasma levels of sICAM‐1 and vWF may indicate endothelial dysfunction or injury in patients with psoriasis.     

Favre–Racouchot syndrome concurrent with chronic granulomatous reaction

Favre–Racouchot syndrome (FRS) is a disorder consisting of solar elastosis with the presence of multiple cysts and open comedones. Although the exact mechanism of the condition is not known, FRS has been specifically connected to sun exposure, smoking and radiation exposure. The histological changes present in FRS reveal an atrophic epidermis and large masses of keratinous material, causing follicular plugging, as well as solar elastotic changes and possible basophilic degeneration of connective tissue. Herein, we report an unusual case of FRS concurrent with chronic granulomatous reaction and review relevant published works.     

Cutaneous necrotizing vasculitis as a manifestation of familial Mediterranean fever

Familial Mediterranean Fever (FMF) is a hereditary autoinflammatory disease, which is characterized by recurrent and paroxysmal fever, peritonitis, arthritis, myalgia, and skin rashes. Although various skin lesions such as “erysipelas‐like erythema”, urticaria, nonspecific purpura, and subcutaneous nodules have been described, cutaneous vasculitis is rare. We report a Japanese case of sporadic FMF accompanied by cutaneous arteritis at the time of febrile attacks of FMF. Gene analysis revealed M694I mutation in a single allele of the MEFV gene, and oral colchicine successfully controlled both periodic fever and subcutaneous nodules of arteritis. Cutaneous necrotizing vasculitis repeatedly emerging with febrile attacks should be included among the skin manifestations of FMF.     

A Different Kind of Christmas Tree: Anomalous Origin of the Right Coronary Artery from the Pulmonary Artery (ARCAPA)

Anomalous right coronary artery from the pulmonary artery (ARCAPA) is a rare congenital coronary anomaly that has an incidence of 0.002%. We report a case of a previously healthy female who presented to our hospital with pneumonia and was incidentally discovered to have ARCAPA. This was initially diagnosed on echocardiography by the unusual echocardiographic finding of multiple color flow Doppler signals around the right ventricular free wall and apex which were subsequently confirmed by angiography to be due to extensive collateral circulation between the left and right coronary arteries. This represents an unusual echocardiographic manifestation of this very rare condition.