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Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm affecting infants or young children. KHE includes a spectrum of lesions, ranging from small and superficial tumors to large and invasive lesions with Kasabach-Merritt phenomenon (KMP). Currently, no published studies have reported a KHE presenting as thrombocytopenia and Raynaud phenomenon.
A 2-year-old boy with right hand swelling and thrombocytopenia was admitted to our hospital. His right hand turned swelling and red, even occasionally cyanotic. This condition became worse in response to cool environments and improved with warming, and platelet counts were between 50 ~ 80 × 10^9/L. Physical examination on admission revealed the swelling and frostbite-like rash of the right-hand fingers, and the skin temperature of the right hand was lower than the left. On day 3 of admission, chest CT results showed an irregular mass on the right side of the spine. The puncture biopsy demonstrated positive CD31, D2-40, and FLI1 immunohistochemical staining, but negative GLUT1 staining, confirming the diagnosis of KHE. Furthermore, endothelin-1 (ET1) expression levels significantly increased, and eNOS and A20 expression levels significantly decreased comparing with control patients. The patient received methylprednisolone and sirolimus treatments, and his condition gradually improved during the follow-up.
We reported the first case of KHE presenting with thrombocytopenia and Raynaud phenomenon. The development of Raynaud phenomenon could be associated with increased ET-1 and reduced eNOS and A20 expressions. Careful differential diagnosis of hidden KHE should be considered in children with thrombocytopenia and Raynaud phenomenon.

UNASSIGNED: In Raynaud\'s phenomenon of the nipple there is a change in color, accompanied by pain or discomfort during breastfeeding.
UNASSIGNED: A 29-years old female patient, breastfeeding, develops a severe bilateral nipple pain during and after breastfeeding and biphasic change in nipple color, with difficulties in the breastfeeding technique. She was medicated with nifedipine and recommended application of warm compresses to the nipples and use of electric breast pump, showing complete resolution after four weeks of treatment.
UNASSIGNED: Raynaud\'s phenomenon of the nipple should be considered in breastfeeding women who report nipple pain or discomfort. In clinical practice, nipple pain is a very frequent complaint, and responsible for many cases of early abandonment of breastfeeding. It is therefore essential to make an early diagnosis and implement a correct and immediate treatment.

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COVID-19 vaccine-related adverse events are mostly minor to moderate, and serious events are rare. Single cases of Raynaud\'s phenomenon (RP) in temporal proximity to COVID-19 vaccination have been reported. Demographic data, medical history, and detailed information regarding vaccination status and RP characteristics were obtained from patients with confirmed RP after COVID-19 vaccination. Fifteen participants reported the initial manifestation of RP, which occurred in 40% after the first, in 33% after the second, and in 27% after the third vaccination. RP development and occurrence of episodes were not linked to any specific vaccine type. New onset of disease was observed in 40% of the vaccinees after BNT162b2, in 33% after mRNA-1273, and in 27% after ChAdOx1 vaccination. Three out of four participants with preexisting RP prior to COVID-19 vaccination reported aggravation in frequency and intensity after immunization. Although COVID-19 vaccination is pivotal in controlling the pandemic, the observed temporal association between vaccine administration and RP occurrence warrants global activities to support pharmacovigilance for the detection of adverse reactions, one of which may include RP.

Systemic sclerosis (SSc) is a systemic autoimmune disease characterised by generalized microangiopathy and fibrosis of skin and internal organs. The 2013 American College of Rheumatology (ACR) / European League Against Rheumatism (EULAR) criteria have contributed considerably to classifying patients with SSc in earlier stages, but they still lack sensitivity for a very early stage of the disease. Criteria for a very early diagnosis of SSc (VEDOSS) have been proposed by EULAR Scleroderma Trial and Research group (EUSTAR) which include three red flags: Raynaud\'s phenomenon, puffy fingers and antinuclear antibody positivity, plus SSc specific antibodies positivity and/or abnormal nailfold capillaroscopy. We report a case of a 54-year-old female patient with 6-week history of puffy fingers, Raynaud phenomenon and positive antinuclear antibodies. Further workup revealed early pathologic capillary pattern by nailfold capillaroscopy and positive anticentromere antibodies. Screening for internal organ involvement detected no heart, lung, or upper gastrointestinal tract involvement. The patient was started on pentoxifylline with further follow-up. The aim of the implementation of VEDOSS criteria is to diagnose SSc at the earliest possible stage, so that subclinical internal organ involvement could be detected and appropriate treatment started at a potentially reversible stage.

We illustrate a notable case of a middle-aged male who presents to a community hospital with left third- and fourth-digit discoloration and pain for the past four days. On presentation to the emergency department, a urine drug screen was ordered which showed synthetic cannabinoids, cocaine, and amphetamines. Initial therapy of nitroglycerin paste, oral oxycodone, intravenous Dilaudid®, and aspirin was started, which resulted in decreased subjective pain. The pathophysiology and mechanism of cocaine-induced Raynaud\'s phenomenon (RP) are discussed. Our purpose in putting forward this case is to acknowledge cocaine use as a cause of secondary RP and to emphasize the importance of early recognition to reduce the occurrence of digital necrosis.

Raynaud\'s phenomenon (RP) is characterized by episodic vasospasm in peripheral vessels and ischemia of the fingers. Venous arterialization is thought to induce neovascularization and increased oxygen tension. In this report, we describe a patient with RP in the fingers of both hands in whom venous arterialization achieved an acceptable result in both hands. The patient was a 62-year-old woman with a 10-year history of worsening pain and cold sensation in the tips of the index, middle, ring, and little fingers on both sides. The venous arterialization procedure was performed on both hands simultaneously at the level of the anatomical snuff box between radial artery and cephalic vein in dorsal hand. There was no need for valvectomy in the level of hands. To prevent development of the steal phenomenon in the arterialized veins, the superficial basilic and median veins of the forearm were ligated via 1 cm skin incisions. The pain and cold sensation in the fingertips of both hands remained decreased, and the nonhealing ulcers on the fingertips healed without the need for amputation. The observation period was 14 months, and the surface temperature of the fingers was increased after venous arterialization, as was the temperature of the palm and forearm. There was no problem when administering intravenous infusion into the forearm on either side postoperatively. The case showed venous arterialization was effective for RP without increasing intravenous pressure in the affected limb, and further investigation is necessary.

BACKGROUND: Ethosuximide and other anti-epileptic drugs have been reported to cause idiosyncratic reactions such as lupus-like syndromes, with elevated antinuclear antibody (ANA) levels. Herein, we present a case of a girl who developed a very severe Raynaud\'s phenomenon reaction and anti-Scl-70 antibodies related to treatment with ethosuximide, due to juvenile absence epilepsy (JAE).
METHODS: A 12-year-old girl was diagnosed with JAE and treatment with ethosuximide was initiated. Two and a half months later her fingers, digits II-V bilaterally, began to ache and were discolored, alternatingly white, blue, or normal-colored. Two weeks later, her fingers were bluish-black, aching severely, almost continuously. The family sought medical advice. Ethosuximide was halted and due to the severe symptoms, treatment with both prednisolone and intravenous iloprost was commenced. Laboratory tests revealed high ANA levels with anti-Scl-70 pattern and confirmed anti-Scl-70 antibodies. After a few weeks, she started to improve and the symptoms slowly decreased over five months. Anti-Scl-70 was still detectable four months after onset of symptoms, though she was much improved. After eleven months, repeated ANA analyses were completely negative.
CONCLUSIONS: Although extremely rare, it is important to recognize that severe Raynaud\'s phenomenon, threatening peripheral digital circulation, may occur as an idiosyncratic reaction to ethosuximide, raising concern over possible onset of vasculitis.

A rare overlap syndrome between CREST (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia) syndrome and primary biliary cholangitis (PBC) is described as PACK syndrome, an acronym for primary biliary cholangitis, anticentromere antibodies, CREST syndrome, and keratoconjunctivitis sicca. In this retrospective cohort analysis and review, we present fourteen patients who meet diagnostic criteria for PACK syndrome in one of the largest case series of this group. All patients were female, 86% of whom were White with an average age of 66.7 years (range 39-78 years). The prevalence was 5.08% in our PBC cohort (n=256) similar to previous findings. CREST syndrome was diagnosed prior to PBC in 58% of our patients and limited pulmonary and renal involvement were observed. This syndrome is rare, but given its insidious development, clinicians should be aware of this potential overlap in CREST-only and PBC-only patients.