Vancomycin has been documented to cause various adverse cutaneous reactions. We present a case report of a man, who developed a large localized erythematous plaque in his forearm following parenteral vancomycin therapy. We believe this to be the first reported case of such cutaneous reaction associated with parenteral vancomycin therapy.
Self-improving dystrophic epidermolysis bullosa is a rare subtype of dystrophic epidermolysis bullosa (DEB) characterized by significant improvement in skin fragility within the first few years of life. Genetic inheritance has previously been reported as autosomal dominant or recessive with both forms harboring mutations in COL7A1. To date, there have been no reports of this rare clinical entity from various Southeast Asian ethnicities. Here, we describe the clinical and molecular features of five patients from the Southeast Asia region who presented with predominantly acral-distributed blisters and erosions in the first few days of life. Blistering resolved over several months, without appearance of new blisters. By immunofluorescence, intraepidermal retention of Type VII collagen was observed in all patient skin biopsies when investigated with antibody staining. Genetic analysis of four patients revealed pathogenic variants in COL7A1 which have not been previously reported. The clinical diagnosis in these rare patients is confirmed with molecular histology and genetic characterization.
Goltz syndrome or Focal Dermal Hypoplasia (FDH) is an uncommon genetically inherited disorder characterized by distinctive skin abnormalities and a wide variety of multisystem defects which was first described by Goltz (an American dermatologist) in 1962. About 200- 300 cases have been reported worldwide. FDH can be inherited in an X-linked dominant manner with in-utero lethality in males. Majority of the cases are sporadic with new mutations arising in the embryo and not inherited from a parent. Approximately 10% of cases occur in males; postzygotic somatic mosaicism accounts for the findings in these affected males. FDH is caused by abnormalities or mutations at the PORCN gene in the X chromosome. We report a case of FDH with characteristic skin lesions as well as multiple digital anomalies - oligodactyly, syndactyly and ectrodactyly.(Copied from article)
Langerhans Cell Histiocytosis (LCH) is an uncommon neoplastic disorder characterized by accumulation of histiocytes in various tissues. The clinical manifestation is highly variable, ranging from an isolated skin rash or a single bony lesion, to fatal multi-organ failure. Due to its rarity and systemic involvement, the epidemiology of LCH is still not fully understood and most studies focus on the paediatric population. The extent of clinical involvement has important prognostic implications. Treatment options may be local or systemic depending on disease extent. Here we describe a rare case of adult onset LCH who presented with recurrent pneumothoraces, diabetes insipidus and papules and plaques over the scalp and forehead.
Lipomatous hypertrophy of the interatrial septum is a benign but less recognized pathology of the heart caused by benign fatty infiltration of the interatrial septum which most often spares the fossa ovalis. We share images of the incidentally detected fluorodeoxyglucose (FDG) uptake in the interatrial septum during the restaging of 18F-FDG positron emission tomography/computed tomography scan of cervical cancer.
Castleman disease (CD) is a rare lymphoproliferative disorder of unknown aetiology. It manifests in two distinct clinical presentations: unicentric and multicentric. Unicentric CD is rare and may present as an isolated neck mass. A 22-year-old man presented with a 6-month history of right neck swelling that occupied the posterior triangle of the right neck region. After surgical exploration, a solitary, well defined, and hyper vascular mass was excise. A histopathological examination confirmed the lesion as CD, hyaline-vascular variant. CD of the neck is a diagnosis that is usually not taken into consideration while evaluating neck masses due to its rarity and unassuming presentation. It should be keep in the differential diagnosis of neck masses as the clinical and radiological features evade a firm diagnosis. The treatment of unicentric CD is complete surgical excision, which cures the patient.