Rare skin

Temporary epicardial pacing wires (TEPWs) are widely used during open heart surgery to treat postoperative bradycardia or arrhythmia. They are usually removed, but the wire is cut at the skin entrance site if there is resistance upon removal. Residual TEPWs have risks of complications such as infection, but they rarely migrate to distant organs. We report a case of TEPW migration from the right ventricle to the pulmonary artery during the early stage after acute type A aortic dissection surgery. Residual TEPW migration was detected incidentally during follow-up imaging for aortic dissection, and no other complications, such as residual wire infection or thrombus, were noted...

Keratoacanthoma (KA) is a common, low-grade, rapidly growing cutaneous squamous cell carcinoma that presents as an enlarging crateriform nodule, which may spontaneously involute but rarely metastasizes. Immunosuppression, ultraviolet light, viral infection, surgical procedures, and trauma are associated with their development. Overall, tattoo-induced squamous cell neoplasms are infrequently described in the literature. Carcinogenesis is hypothesized to result from trauma caused by the tattooing procedure or a foreign body reaction to the pigment...

Inflammatory Breast Cancer (IBC) is a rare and aggressive form of locally advanced breast cancer, classified as stage T4d according to the tumor-node-metastasis staging criteria. This subtype of breast cancer is known for its rapid progression and significantly lower survival rates compared to other forms of breast cancer. Despite its distinctive clinical features outlined by the World Health Organization, the histopathological characteristics of IBC remain not fully elucidated, presenting challenges in its diagnosis and treatment...

Systemic sclerosis (SSc) is a rare autoimmune disease (AD), characterised by early diffuse vasculopathy, activation of the immune response and progressive skin and internal organ fibrosis. In severe progressive diffuse SSc (dSSc), autologous hematopoietic stem cell transplantation (aHSCT) improves survival, despite its own risk of complications and transplant related mortality (TRM). We present herein the case of a dSSc patient undergoing aHSCT with low dose cyclophosphamide conditioning and sudden acute myopericarditis and cardiogenic shock, four weeks after a second mRNA SARS-CoV-2 vaccine (Pfizer) injection...

INTRODUCTION: Sialadenoma papilliferum (SP), a rare minor salivary gland tumor, shares morphological and genetic similarities with syringocystadenoma papilliferum. Recent studies have identified BRAF V600E or HRAS mutations in SP, suggesting its neoplastic nature. Despite being uncommon, SP poses diagnostic challenges due to its resemblance to other lesions like squamous papilloma. The emergence of sialadenoma papilliferum-like intraductal papillary tumor (SP-IPT) further complicates its classification, emphasizing the need for thorough investigation...

Pregabalin is the first-line treatment for neuropathic pain. Cases of cutaneous hypersensitivity reactions caused by pregabalin generally occur within 2 weeks of initiating medication. We report a rare case of a delayed cutaneous hypersensitivity reaction caused by pregabalin, which was confirmed by a drug provocation test. A 72-year-old man with severe herpes zoster neuralgia developed maculopapular drug eruption covering 80% to 90% of his total body surface area after 40 days of combined multidrug analgesia...

Subperiosteal orbital hematoma secondary to sinusitis is rare. Thus far, 19 cases of this disease have been reported, of which none involved postoperative skin anesthesia in the region innervated by the supraorbital nerve. In this article, for the first time we report a case of subperiosteal orbital hematoma secondary to sinusitis with skin anesthesia in the area innervated by the supraorbital nerve after surgery.

KEY CLINICAL MESSAGE: High-dose intravenous immunoglobulin exhibits great potential in the treatment of Netherton syndrome. ABSTRACT: Netherton syndrome (NS) is a rare autosomal recessive genodermatosis (OMIM #256500) characterized by superficial scaling, atopic manifestations, and multisystemic complications. It is caused by loss-of-function mutations in the SPINK5 gene, which encode a key kallikrein protease inhibitor. There are two subtypes of the syndrome that differ in clinical presentation and immune profile: ichthyosiform erythroderma and ichthyosis linearis circumflexa...

Merkel cell carcinoma (MCC) is a rare type of skin cancer of the neuroendocrine Merkel mechanoreceptors. These cells are closely associated with nerve terminals and, given their proximity to cutaneous tissue, have the propensity to develop into deeply ulcerated, fungating malignancies. These friable wounds are easily irritated, and can cause significant pain for patients. We report a palliative case of severe, fungating MCC of the left scalp where the main contributor to the patient's illness burden is pain...

Among specific skin manifestations of systemic lupus erythematosus such as leukocytoclastic vasculitis, and vasculopathy, the development of leg ulcers is rare and frequently seen in patients with antiphospholipid antibody positivity. Here we report the rapid healing of a leg ulcer without antiphospholipid antibody positivity in a patient with lupus in response to anticoagulant therapy. As in our case, when immunosuppressive agents are inadequate in lupus patients who develop leg ulcers, it may be beneficial to support the treatment with anticoagulants...

BACKGROUND: Melanocytic nevi are frequently observed in the pediatric population. While newly acquired nevi can appear during childhood, congenital nevi can continue to grow and clinically change, making patient caregivers concerned. Reflectance confocal microscopy (RCM) in vivo is a noninvasive tool that might enhance the diagnostic accuracy of dermoscopy, reducing the rate of unnecessary surgical procedures. This study aimed to assess the utility of RCM in increasing the diagnostic accuracy of pediatric melanocytic nevi that show pigmentation changes or grow rapidly...

BACKGROUND: Generalized pustular psoriasis (GPP) is a rare, potentially life-threatening skin disease often requiring long-term therapy. We aimed to evaluate the use of Interleukin (IL)-17A inhibitors (secukinumab and ixekizumab) in GPP patients over 96 weeks. METHODS: We retrospectively analyzed a case series of 18 patients with GPP who received secukinumab (n = 13) and ixekizumab (n = 5) therapy with a 96-week follow-up period. The primary effectiveness analysis included determining the percentage of patients who achieved ≥90% or 100% improvement in the Generalized Pustular Psoriasis Area and Severity Index (GPPASI) score...

INTRODUCTION: Although multimodal intraoperative neuromonitoring (IONM), which has high sensitivity and specificity, is typically performed during spinal deformity surgery, neurological status may deteriorate with delay after surgical maneuvers. Here, we report a rare case of delayed postoperative neurological deficit (DPND) that was not detected by IONM during posterior spinal fusion (PSF) for congenital scoliosis. CASE PRESENTATION: A 14-year-old male presented with congenital scoliosis associated with T3 and T10 hemivertebrae...

Aplasia cutis congenita (ACC) is a group of rare heterogeneous disorders characterised by absent areas of skin at birth. The majority of cases involve the scalp region. ACC limited to one lower limb is extremely rare. We report an usual case of ACC limited to the left thigh of which healing occurred in utero. The case was managed conservatively and the disease course has been favourable with no limitations in limb function and an entirely normal development. Most cases of ACC are self-healing, justifying a conservative approach...

INTRODUCTION: Stevens Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious conditions that carry a high rate of morbidity and mortality. OBJECTIVE: This review highlights the pearls and pitfalls of SJS/TEN, including presentation, diagnosis, and management in the emergency department (ED) based on current evidence. DISCUSSION: SJS/TEN is a rare, delayed hypersensitivity reaction resulting in de-epithelialization of the skin and mucous membranes...

BACKGROUND: Vision loss secondary to aesthetic filler treatment is a rare but disastrous complication. OBJECTIVES: The aim was to update the published cases of blindness after filler injection that have occurred since our group published reviews of 98 cases in 2015 and an additional 48 cases in 2019. METHODS: A literature review was performed to identify all cases of visual complications caused by filler injection published between September 2018 and March 2023...

IMPORTANCE: Unlike medications, procedural interventions are rarely trialed against placebo prior to becoming accepted in clinical practice. When placebo-controlled trials are eventually conducted, procedural interventions may be less effective than previously believed. OBJECTIVE: To investigate the importance of including a placebo arm in trials of surgical and interventional procedures by comparing effect sizes from trials of the same procedure that do and do not include a placebo arm...

Primary squamous cell carcinoma of the parotid gland (pSCCP) has long been recognized as a separate entity and is included in the WHO classifications of salivary gland tumors. However, it is widely accepted among head and neck pathologists that pSCCP is exceptionally rare. Yet, there are many publications describing series of pSCCP and data from SEER and other cancer register databases indicate erroneously an increasing incidence of pSCCP. Importantly, pSCCP and metastatic (secondary) squamous cell carcinoma to the parotid gland (mSCCP) have nearly identical histological features, and the diagnosis of pSCCP should only be made after the exclusion of mSCCP...

Musculoskeletal coccidioidomycosis is a rare disseminated fungal infection caused by either Coccidioides immitis or Coccidioides posadasii endemic to the southwestern United States and northwestern Mexico, as well as Guatemala, Brazil, and other locations in Central and South America. Symptomatic primary infection of coccidioidomycosis can present as pneumonia with influenza-like symptoms, but the majority of cases remain asymptomatic. When dissemination occurs, the most common extrapulmonary sites include the skin, lymph nodes, musculoskeletal system, and meninges...

Elephantiasis nostras verrucosa (ENV) is a rare complication of chronic lymphedema, leading to progressive cutaneous hypertrophy and disfigured lower extremities. We present an unusual case of a 79-year-old man with ENV, who had an episode of erysipelas treated and cured with antibiotics. The patient presented with progressive painless lymphedema of the lower limb years ago, complicated with skin changes. The biology and radiology test does not objective any causes. The compression therapy was proposed to the patient...