[MIM 602 248]

(malignant atrophying papulosis, Kohlmeier-Degos-Delort-Tricot syndrome)

Incidence < 1/106.  Chronic thrombo-obliterative vasculopathy.

It may present:

• either as a benign cutaneous form: small erythematous papules, localized mainly on the trunk and upper limbs. After several days, the lesions become depressed in their center and have a characteristic morphology: erythematous papules, 0.5-1 cm wide,  with a depressed white-porcelain atrophic central zone surrounded by a telangiectatic ring
• or as a systemic form, which can develop at the same time as several years after skin manifestations: the gastrointestinal form is the most common. Patients may have multiple infarctions limited to the intestine with abdominal pain, bleeding, diarrhea and perforations: at laparoscopy, serous lesions of the digestive tract are similar to the skin lesions. A CNS form with cerebrovascular accidents (sometimes spinal cord lesions). It can more rarely manifest as pericarditis or pleuritis. An ocular form affecting the eyelids, conjunctiva, retina, sclera and choroid plexus has also been described.

The cause is unknown: the proposed hypotheses are vasculitis, coagulopathy and primary endothelial cell dysfunction. Blood clotting defects have been reported in many patients. This pathology can be associated with dermatomyositis, systemic lupus erythematosus, antiphospholipid antibody syndrome or systemic sclerosis. Pediatric cases have been described.

Treatment of systemic forms: eculizumab (anti-C5 monoclonal antibody) and treprostinil (vasodilator, a prostacyclin analogue).

Anesthetic implications:

drug interactions with treatment; emergency digestive laparoscopy

References : 

• Sattler SS, Magro CM, Shapiro L, Merves JF, Levy R, Veenstra R, Patel P.
  Gastrointestinal Kohlmeier-Degos disease : a narrative review.
  Orphanet J Rare Diseases 2022; 17:10.1186/s13023-022-02322-9

Updated: May 2022