Disease Information for Degos' acanthomatosis syndrome

Ads
Clinical Manifestations
Signs & Symptoms
Adolescent
Lesion precede intestinal complications/years
Lesions on trunk/upper extremities
Papular Rash
Porcelain red-ringed atrophic spots
Rash/Characteristic pattern/distribution
Skin lesions
Abdominal Pain
Abdominal Pain in Children
Apparent bowel obstruction syndrome
Diarrhea
Weight loss in Children
Pathognomonic/diagnostic lesions
Weight Loss
Clinical Presentation & Variations
Presentation/Young male porcelain spots acute abdomen
Disease Progression
Course/Chronic disorder
Course/Chronic only
Course/Progressive/slowly chronic illness
Demographics & Risk Factors
Established Disease Population
Patient/Degos cutaneointestinal/atrophic papulosis
Population Group
Child
Man
Population/Pediatrics population
Sex & Age Groups
Population/Adolescent
Population/Adult ('twenties')
Population/Child
Population/Child-Infant Only
Population/Children/all
Population/Male
Population/Man patient
Diagnostic Test Results
Pathology
BX/Occlusive arteriopathy/chronic
BX/Small arteries fibrosing occlusion/no inflammation
BX/Skin biopsy upper dermal mucin deposits
BX/Skin biopsy/Small vessel vasculitis
CT Scan
CT/Enterography Abnormal
X-RAY With contrast
SBS/Abnormal Small Bowel Series
Associated Diseases & Rule outs
Associated Disease & Complications
Acanthosis nigricans
Cerebral vascular accident
Degos' acanthomatosis syndrome
Epilepsy
Hemiparesis
Hemiplegia
Mesenteric vascular/artery insufficiency
Mesentery hemorrhage
Perforation GI tract
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
CLASS/Primary organ/system disorder (ex)
CLASS/Cardiovascular (category)
CLASS/Small artery disorder
CLASS/Vascular Small Vessel Disorder
CLASS/Dermatologic/Subcutaneous (category)
CLASS/Small intestine disorder (ex)
Pathophysiology
Pathophysiology/Gene locus Chromosome X.
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Pathophysiology/Occlusive low-grade vasculitis gut/skin
Pathophysiology/Small Vessel Vasculitis
Pathophysiology/Ischemic bowel process
Pathophysiology/Malabsorption
Process
PROCESS/Eponymic (category)
PROCESS/Hereditofamilial (category)
PROCESS/Hereditofamilial variant/pedigrees reported (ex)
PROCESS/Idiopathic/unclassified/unknown (category)
PROCESS/INCIDENCE/Esoteric disease (example)
PROCESS/INCIDENCE/Extremely rare disease
PROCESS/Neoplastic (category)
PROCESS/Sex-linked (X-linked) recessive inheritance (ex)
Synonyms
Synonym
Degos' acanthomatosis syndrome, Synonym/Cutaneointestinal syndrome, Synonym/Degos syndrome, Synonym/Degos-Kohlmeier disease, Synonym/Koehlmeier-Degos syndrome, Synonym/Malignant atrophic papulosis (Degos)
Treatment
Drug Therapy - Indication
RX/No effective/definitive treatment yet available
Other Treatments
TX/Expectant/supportive treatment.
Definition

Degos Disease; Degos Syndrome; Degos-Kohlmeier Disease;

Kohlmeier-Degos Disease; Malignant Atrophic Papulosis

Degos disease is a rare systemic disorder that affects small and medium sized arteries, causing them to become blocked (occlusive arteriopathy); Degos disease usually progresses through two stages: During the first stage, characteristic skin lesions appear that may last for a period of time ranging from weeks to years; The second stage of Degos disease is most frequently characterized by lesions in the small intestine, but other organs may also be affected; Major symptoms may include abdominal pain, diarrhea, and/or weight loss; Intestinal lesions may break through the wall of the bowel (perforation), a potentially life-threatening complication; The exact cause of Degos disease is unknown; ----------[NORD 2005]----------------------------------

Degos disease, also known as malignant atrophic papulosis is a rare disorder. It generally occurs in young Caucasian adults; Degos disease affects the lining of the small blood vessels resulting in occlusion (blockage); The blood vessels affected include those supplying the skin, gastrointestinal tract and central nervous system;

Skin lesions are the characteristic feature of Degos disease. The lesions are usually multiple and occur predominantly on the trunk and arms; They usually start as small red raised spots of 2-5mm in diameter; After a few days they enlarge and develop a central white spot that is depressed in comparison to the red skin around it; They heal leaving depressed porcelain-white scars;

Gastrointestinal complications result from lack of blood supply to the lining of the gut and may lead to serious complications like perforation of the bowel; The usual symptoms are sudden onset of abdominal pain or gastrointestinal bleeding (vomiting blood or passing blood with the bowel motion);Involvement of the nervous system can lead to strokes, headaches, epilepsy or non-specific neurological symptoms like memory loss or altered sensation;

Other organs can also be involved such as eyes, kidneys, heart and liver. Prognosis: Degos disease is generally regarded as serious because it leads to involvement of multiple organs resulting in death within 2-3 years; But in some cases it remains limited to skin involvement and appears benign (harmless);

[DermNet NZ 2005]---------------------------------------------

(Edit)

External Links Related to Degos' acanthomatosis syndrome
Google
Wikipedia
Merck
Images
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
Ads