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This page is mainly intended to help physicians inform their patients about Degos disease.

That being said, medical terms are explained in brackets so that patients who wish to learn these concepts on their own, can do so in order (for example) to understand doctors’ reports and scientific literature.

Overview

Degos disease, also known as Malignant Atrophic Papulosis (MAP), Atrophic Papulosis, or Kohlmeier-Degos disease, is an ultra-rare disease which can exist in a benign, cutaneous (skin only) or systemic, potentially malignant form. It was initially described by Köhlmeier in 1941 and documented as a distinct illness by Degos in the same year. 

Symptoms normally appear between ages 20-50, but can affect any age group. The illness predominantly occurs sporadically but on rare occasions has affected several members of the same family.

The systemic form of Degos disease affects one or more body organs, most commonly the gastrointestinal tract or brain. Complications can include bowel perforation and various neurologic and visual symptoms.

Diagnosing Degos disease

Skin lesions precede internal involvement and begin as small erythematous (red) macules, commonly affecting the trunk and upper extremities. Over days, the centers of the lesions become atrophic (sunken), with an older lesion therefore displaying the classic porcelain-white center with an erythematous rim. Characteristically, palmar (palms of hands), plantar (bottoms of feet), and facial regions remain unaffected.

The characteristic skin changes make clinical diagnosis possible, but a skin biopsy is required for conclusive pathologic diagnosis. Laboratory tests are not necessary for diagnosis, as coagulating disturbances may be present.

Our Goal: Promote early recognition/diagnosis for better patient outcomes.

The sooner skin lesions are noticed, the sooner next steps and treatments can begin, if necessary. If the disease remains confined to the skin, it is benign. If systemic disease develops, acute complications and even death may occur.

Degos disease cutaneous skin lesions.

Degos disease path to diagnosis logo

Early diagnosis can make a big difference.

Cutaneous Path to Diagnosis

  • STEP 1

    If a lesion appears on your patient’s skin, perform a skin biopsy.

  • STEP 2

    Send the biopsy slides to Doctor Cynthia Magro for analysis.

    Doctor Magro is the world expert on disease pathology.

    Contact her, especially if there is any uncertainty to diagnosis.

  • STEP 3

    If cutaneous Degos disease is confirmed by Doctor Magro, help enroll your patient in the research protocol at the NIH (National Institutes of Health).

    Help your patient pursue referrals to NIH:
    Review The Steps Here

    If the patient is too young to be enrolled, the work-up can be done by a specialist (dermatologist or pediatrician).  

  • STEP 4

    If your patient is experiencing abdominal pain, SEE PATH BELOW AND TAKE IMMEDIATE ACTION IN TREATMENT.

If patient presents with unexplained abdominal pain…

Gastrointestinal Path to Diagnosis

If the patient is presenting with cutaneous Degos disease and has neurological symptoms, please reach out to Dr. (name), as the treatment differs from that of gastrointestinal presentation.

Differential diagnosis of Degos disease: Why a skin biopsy is needed

Differential diagnosis of Degos disease underscores the necessity of a skin biopsy. This diagnostic step is crucial, particularly in cases where clinical context and morphological features bear a close resemblance.

One notable condition is Fibrosing Dermatomyositis, characterized by guttate-like Gottron’s sign or papules. There have been instances where early papular Degos disease was misdiagnosed as tumid lupus erythematosus, highlighting the importance of thorough and accurate diagnosis.

Evaluating for systemic disease is paramount, as Degos can rapidly evolve from being untreatable to treatable. Without timely intervention, Degos disease can progress swiftly. While treatments for Degos are not FDA-approved, the process of getting them approved can be arduous, often requiring detailed letters to insurance companies. Refer your patients to NIH.

Help your patient pursue referrals to NIH

Why should you preform a laparoscopy?

It is critical a laparoscopy is performed if experiencing abdominal pain. Refer to Gastrointestinal Kohlmeier–Degos disease: a narrative review for images of the lesions.

Laparoscopy Training Video

Patient prognosis

All of the Degos disease literature 15 years ago revealed no positive outcomes, but this has changed with recent treatment advances, and we have the opportunity to make incredible changes in patient outcomes. However, speed is off the essence in terms of diagnosing Degos. Receiving treatment in a timely manner is critical to halt disease progression and spare disease morbidity. If disease stabilization or control can be achieved early, the outcomes will be better. The challenge is getting access to needed medications. It is easier to get the medication if the patient is in the ICU as different approval from insurance companies is needed. Refer to our Treatments & Care page to learn more about drug access.

Support Network

Interested in reaching out to other physicians, check out our Physician Support Network.

Treatments & Care

We have the top experts dedicated to finding treatments for Degos Disease.

Resources

Looking for more information or this ultra-rare disease? Check out or resources section.

Reference

Vazquez- Doval F.J., F.R. De Erenchun, J.A. Paramo, E. Quintanilla (1993). Malignant atrophic papulosis. A report of two cases with altered fibrinolysis and platelet function. Clin. Exp. Dermatol. 18, 441-444