Related Experiment Video

Updated: Oct 1, 2026

A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts
07:50

A Metadata Extraction Approach for Clinical Case Reports to Enable Advanced Understanding of Biomedical Concepts

Published on: September 20, 2018

Childhood Köhlmeier-Degos disease with atypical skin lesions

A Sotrel, A G Lacson, K R Huff

    Neurology
    |September 1, 1983
    PubMed

    Insights

    This case study details a rare pediatric disease presenting with skin lesions, organomegaly, and neurological issues. Pathologic findings reveal widespread central nervous system (CNS) damage and arterial narrowing.

    Area of Science:

    • Pediatric Neurology
    • Vascular Pathology
    • Dermatology

    Background:

    • A rare multisystemic disease affecting a 16-year-old male since infancy.
    • Characterized by a complex array of clinical manifestations.

    Observation:

    • Initial symptoms included maculopapular skin lesions, cyclic nodular eruptions, hepatosplenomegaly, abdominal pain, and fever.
    • Progressed to significant ophthalmologic and neurologic deficits.

    Findings:

    • Central nervous system (CNS) pathology was severe, with leptomeningeal fibrosis, ventricular enlargement, and multiple brain infarcts.
    • Striking intimal thickening of medium and small extraparenchymal arteries was observed, leading to vessel narrowing or occlusion.

    Implications:

    • Highlights the critical role of vascular pathology in pediatric neurological disorders.
    • Suggests the need for early diagnosis and potential vascular-targeted therapies.
    • Underscores the importance of comprehensive evaluation in complex pediatric cases.

    More Related Videos

    Skin Biopsy for Diagnosing Discoid Lupus Erythematosus05:44

    Skin Biopsy for Diagnosing Discoid Lupus Erythematosus

    Published on: June 10, 2025

    Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus05:39

    Dermoscopy Aids in the Diagnosis of Discoid Lupus Erythematosus

    Published on: May 16, 2025

    Related Concept Videos

    Changes in Skin Color: Clinical Perspectives01:14

    Changes in Skin Color: Clinical Perspectives

    The first thing a clinician sees is the skin, so the examination of the skin should be part of any thorough physical examination. Most skin disorders are relatively benign, but a few, including melanomas, can be fatal if untreated. A couple of the more noticeable disorders, albinism and vitiligo, affect the appearance of the skin and its accessory organs.
    Albinism
    Albinism is a genetic disorder that affects (completely or partially) the coloring of skin, hair, and eyes. The defect is primarily...
    Skin Diseases and Disorders01:23

    Skin Diseases and Disorders

    Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
    Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...
    Skin Cancer01:30

    Skin Cancer

    Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
    Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
    Endocarditis II: Clinical Features of Infective Endocarditis01:25

    Endocarditis II: Clinical Features of Infective Endocarditis

    Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
    Acne Infection01:27

    Acne Infection

    Acne is a multifactorial skin condition primarily affecting adolescents and young adults, with a global prevalence estimated to exceed 75% in this demographic. The condition is characterized by the formation of comedones (blackheads and whiteheads), papules, pustules, nodules, and, in severe cases, cysts, particularly in areas rich in sebaceous glands such as the face, neck, chest, and back. The pathogenesis involves increased sebum production, follicular hyperkeratinization, colonization by...
    Cytomegalovirus Disease01:27

    Cytomegalovirus Disease

    Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...