The New Sydenham Society's Atlas of Portraits of Diseases of the Skin - an Educational Tool.

In 1859, the New Sydenham Society undertook the publication of an Atlas of Portraits of Diseases of the Skin, having been inspired by Ferdinand Ritter von Hebra's Atlas der Hautkrankheiten. Utilizing information contained in Sir Jonathan Hutchinson's Descriptive Catalogue of the New Sydenham's Society Atlas of Portraits of Diseases of the Skin (published in 1869 and 1875), an exhibit of twenty-five illustrations of cutaneous disorders, along with teaching points, was held at the Harvey Cushing/John Jay Whitney Medical Library at Yale University. It served as an educational tool for dermatologists, dermatology residents, medical students, and even physicians from other specialties.

Small vessel disease in primary familial brain calcification with novel truncating PDGFB variants.

Introduction: Primary familial brain calcification (PFBC) is a neurodegenerative disease characterised by bilateral calcification in the brain, especially in the basal ganglia, leading to neurological and neuropsychiatric manifestations. White matter hyperintensities (WMH) have been described in patients with PFBC and pathogenic variants in the gene for platelet-derived growth factor beta polypeptide (PDGFB), suggesting a manifest cerebrovascular process. We present below the cases of two PFBC families with PDGFB variants and stroke or transient ischaemic attack (TIA) episodes.

Visual perception, cognition, and error in dermatologic diagnosis: Key cognitive principles.

Dermatologic diagnosis relies on vision primarily and auditory and verbal input secondarily. Accurate dermatologic diagnosis is predicated on seeing and perceiving a skin finding, categorizing and naming the finding correctly, and comparing the visual data and data obtained from the totality of the clinical encounter (ie, from other sensory modalities) with one's working mental database of dermatologic diagnoses. The baseline assumption-which is false-is that a dermatologist is an expert at each of the aforementioned steps and transitions sequentially between them seamlessly in an error-free fashion.

A 65-Year-Old Female from Connecticut with Orf Infection.

The virus, which causes orf and induces acute pustular skin lesions in sheep and goats, is transmissible to humans yet is rarely observed in North America. We present a case of a 65-year-old female farmer from Connecticut who contracted orf from her sheep. The clinical and histopathologic features, important to arrive at the correct diagnosis of this uncommon yet important infection, are described.

Skin manifestations of endocrine and neuroendocrine tumors.

The skin signs of benign and malignant endocrine and neuroendocrine tumors are manifold and early identification of these dermatologic features is crucial in initiating timely diagnosis and management. This article reviews the salient cutaneous features of these tumors that arise in the classic endocrine glands, lung and gastrointestinal tract either as individual neoplasms or as part of a syndrome.

Hypertrophic lupus erythematosus: the diagnostic utility of CD123 staining.

CD123-positive plasmacytoid dendrocytes are prominent in the infiltrate of discoid lupus erythematosus (LE). We hypothesized that these cells would also be present in hypertrophic LE and would aid in the histopathologic distinction from squamous cell carcinoma (SCC) and hypertrophic actinic keratosis (AK). Five cases of hypertrophic LE and 10 cases each of SCC and hypertrophic AK were stained with CD123.

Nephrogenic systemic fibrosis.

Nephrogenic systemic fibrosis, initially called nephrogenic fibrosing dermopathy, has been strongly linked to exposure to gadolinium-based contrast media used in magnetic resonance imaging in patients with renal insufficiency. This review discusses recent advances in our understanding of the pathophysiology and clinical approach to patients with chronic kidney disease who require diagnostic imaging with gadolinium-based contrast media.

Akhenaten and the strange physiques of Egypt's 18th dynasty.

Akhenaten was one of Egypt's most controversial pharaohs, in part because of his strange appearance in images produced after he had declared Aten, the Sun-disc, his one-and-only god. Whether these were symbolic representations or realistic ones that indicate a deforming genetic disorder is the subject of continuing debate. The authors present evidence that the bizarre physical features portrayed in these images are not only realistic but were shared by many members of Egypt's 18th Dynasty.

A randomized, double-blind, placebo-controlled trial of photopheresis in systemic sclerosis.

Background: Systemic sclerosis is a multisystemic connective tissue disease with marked involvement of the skin and joints for which few effective evidence based therapies are available. To further investigate the efficacy of extracorporeal photochemotherapy on early aggressive cutaneous disease, a randomized, double-blind, placebo-controlled trial was performed.

Objective: Our aim was to evaluate the efficacy of photopheresis in the treatment of patients with systemic sclerosis (scleroderma).

Epidermolysis bullosa acquisita and multiple myeloma.

The coexistence in the same patient of epidermolysis bullosa acquisita (a rare, autoimmune, acquired mucocutaneous blistering disorder) and multiple myeloma (a plasma cell neoplasm) is extremely uncommon. We describe a patient in whom both of these diseases occurred simultaneously. Intravenous immunoglobulins were used to treat both diseases.

Skin manifestations of internal malignancy.

This article concentrates on the major signs and syndromes that are associated with internal malignancies in the geriatric population. Included are cutaneous metastases, ectopic adrenocorticotropic hormone-producing syndromes, and disorders arising from APUD cell tumors. The major paraneoplastic disorders of dermatomyositis, generalized pruritus, Bazex's syndrome, and acanthosis nigricans also are discussed.