KTWS (Klippel-Trenaunay-Weber syndrome): A systematic presentation of a rare disease.

BACKGROUND: Klippel-Trenaunay-Weber syndrome (KTWS) is a rare disease with a wide range of manifestations. KTWS is characterized by a clinical triad of varicosities of the extremities, cutaneous vascular malformations, and hypertrophy of soft tissues and long bones. The diagnosis is made clinically supplemented with magnetic resonance imaging and computed tomography. AIM: Hereby we aim to highlight the significance of the possible life-threatening first-time presentations associated with the GI system in previously undiagnosed KTWS patients. PATIENT: We report the case of a 47-year-old male with KTWS, who presented with various symptoms such as rectorrhagia since childhood, digestive problems and abnormal lateral vascular malformations of the left buttock which extended all the way to the leg, vascular malformations of the left fourth and fifth toes as well as soft tissue swelling of the left foot. There was no evidence of other clinical presentations. The patient was hospitalized with severe rectorrhagia and a hemoglobin level of 3/9. Physical examination revealed a blood pressure of 85/55 and pulse rate of 115. Ruptured aneurysm of the superior mesenteric artery was found on angiography and subsequently treated with embolization. Dermatologic evaluation showed pitting edema of the left leg and foot and multiple vascular lesions. Thus a diagnosis of KTWS was established. Pulsed dye laser therapy and compression bandage was performed for the patient. The patient's follow-up was done 3 months after discharge for which the patient was again consulted by a dermatologist and gastroenterologist. Lymphedema of the left leg had improved to a great extend so treatment with compression bandage was continued. Colonoscopy was repeated for the patient to evaluate and control possible active sources of bleeding, due to potential life-threating complications. RESULTS: According to previous findings, there have been few case reports of KTWS presenting with gastrointestinal manifestations, fewer of which have covered acute life-threatening bleedings associated with this system.

Guarding the heart: How SGLT-2 inhibitors protect against chemotherapy-induced cardiotoxicity: SGLT-2 inhibitors and chemotherapy-induced cardiotoxicity.

The introduction of chemotherapy agents has significantly transformed cancer treatment, with anthracyclines being one of the most commonly used drugs. While these agents have proven to be highly effective against various types of cancers, they come with complications, including neurotoxicity, nephrotoxicity, and cardiotoxicity. Among these side effects, cardiotoxicity is the leading cause of morbidity and mortality, with anthracyclines being the primary culprit. Chemotherapy medications have various mechanisms that can lead to cardiac injury. Hence, numerous studies have been conducted to decrease the cardiotoxicity of these treatments. Combination therapy with beta-blockers, Angiotensin-converting enzyme inhibitors, and angiotensin receptor blockers have effectively reduced such outcomes. However, a definitive preventive strategy is yet to be established. Meanwhile, sodium-glucose co-transporter-2 (SGLT-2) inhibitors lower blood glucose levels in type 2 diabetes by reducing its re-absorption in the kidneys. They are thus considered potent drugs for glycemic control and reduction of cardiovascular risks. Recent studies have shown that SGLT-2 inhibitors are crucial in preventing chemotherapy-induced cardiotoxicity. They enhance heart cell viability, prevent degenerative changes, stimulate autophagy, and reduce cell death. This drug class also reduces inflammation by inhibiting reactive oxygen species and inflammatory cytokine production. Moreover, it can not only reverse the harmful effects of anticancer agents on the heart structure but also enhance the effectiveness of chemotherapy by minimizing potential consequences on the heart. In conclusion, SGLT-2 inhibitors hold promise as a therapeutic strategy for protecting cancer patients from chemotherapy-induced heart damage and improving cardiovascular outcomes.

Lipoid proteinosis: A systematic presentation of an unusual disease.

BACKGROUND: Lipoid proteinosis (LP) or Hyalinosis Cutis et Mucosae or Urbach-Wiethe disease is a rare autosomal recessive genodermatosis characterized by an amorphous hyaline material deposition in the skin mucosa and viscera. The clinical symptoms of this disease often begin in childhood, which persist throughout life. Skin manifestations include inflammation, scaling, acne, and eventually ulceration, and hyaline amorphous deposits in these areas of the wound cause a waxy and thick appearance on the skin. In addition, wounds leave atrophic scars like chickenpox. AIM: Herein, we present the first case of LP in the north of Iran; although LP is a sporadic disease, it occurs all around the world, with about 400 cases worldwide having been reported thus far. PATIENT: We report the case of a 28-year-old female patient with a history of skin lesions on her face, scalp, extremities, and buttock, as well as hoarse cry, respiratory problems, dysphagia, and migraine since childhood. There was no evidence of other clinical presentations. A biopsy was taken from the lesions, and the patient was diagnosed with LP. A laryngeal laser was performed for the patient, and peeling creams were used for her skin lesions. RESULT: According to previous findings, there has been no case report of LP with systemic symptoms in the north of Iran.

First report of tamoxifen-induced baboon syndrome.

BACKGROUND: Baboon syndrome is a rare, type IV hypersensitivity reaction causing a maculopapular rash. Tamoxifen is an antineoplastic agent, working as an estrogen receptor antagonist, also called a selective estrogen receptor modulator. A variety of rashes were reported with Tamoxifen use to-date except baboon syndrome. The Tamoxifen-induced baboon syndrome seems to be reversible, as discontinuation of the drug improves clinical outcomes. AIM: Herein, we present the first case of Tamoxifen-induced baboon syndrome which occurred 8 years after initiation of Tamoxifen use. PATIENTS: A 44-year-old woman presented with papulovesicular eruption on her body and erythema on her face for a duration of 6 months. There was no evidence of ocular or mucosal involvement. She was diagnosed with breast cancer and treated with tamoxifen 10 mg twice daily over the past 8 years. She was not taking other medications or over-the-counter supplements at the time of presentation. The patient underwent urgent skin biopsies of two lesions on her buttock and thigh. No organisms were seen on Gram stain. The patient's skin biopsy revealed extensive hyperorthokeratosis, minimal parakeratosis, hypergranulosis, and lichenoid interface dermatitis in the irregularly acanthotic epidermis supporting diagnosis of fixed drug eruption. Following a multidisciplinary discussion, the patient was diagnosed with baboon syndrome or symmetrical drug-related intertriginous and flexural exanthema (SDRIFE) associated with Tamoxifen. RESULTS: Hence, Tamoxifen was immediately discontinued and treated with oral steroid along with topical agents. She showed improvement of clinical abnormalities within days after discontinuation of Tamoxifen. CONCLUSIONS: Given the widespread use of Tamoxifen in the management of patients with breast cancer, it is important that healthcare professionals monitor for rare, however clinically significant, and potentially life-threatening dermatological manifestations of Tamoxifen use, such as baboon syndrome.

A first case of fixed drug eruption due to Tamsulosin.

BACKGROUND: Fixed Drug Eruption (FDE) is a drug reaction involving the skin and less commonly the mucosal membranes. Tamsulosin is an alpha-1 adrenergic receptor blocker used to treat benign prostatic hyperplasia. Dizziness and headache are among its most common side effects (Singapore Med J, 2018;59:336). Although cutaneous drug eruption has been reported with other alpha-1 adrenergic receptor blockers. AIMS: Drug rash due to Tamsulosin is relatively uncommon (Singapore Med J, 2018;59:336). In this case we report an incidence of fixed drug eruption due to Tamsulosin. PATIENTS: A 54 year old male, with benign prostatic hyperplasia was referred to our office for evaluation of certain eruptions, having developed his hyper-pigmented patches after 2 weeks of using oral 0.4 mg Tamsulosin per day. Based on the clinical course and the skin biopsy we diagnosed the condition as Tamsulosin associated fixed drug eruption. The most important therapeutic measure was discontinuing Tamsulosin medication. Following this, his eruptions improved remarkably in a few days without complications. RESULTS: According to previous findings, there has been no case report on fixed drug eruption caused by Tamsulosin. CONCLUSION: Our case is a relatively mild form of fixed drug eruption. Therefore it should be kept in mind that a severe cutaneous drug eruption might occur after Tamsulosin administration.