Malignant atrophic papulosis

DEGOS-KOHLMEIER disease is an extremely rare disorder (about 200 cases worldwide) named after the two authors who first described it in 1941-1942.

It affects the skin and sometimes the internal organs including the gastrointestinal and nervous system and eyes (table I), which is why it is such a serious disorder. There is still debate about its causes and no particular treatment has been recommended.

• The cutaneous signs are very distinctive and consist of a small, pink, raised lesion measuring a few millimetres (figure 1) which gradually heals leaving an atrophied, porcelain-white depression surrounded by a halo of small vessels (telangiectasias) (figure 2). Several lesions are often present on the trunk and limbs, while the face tends to be spared. They last several months.

The disease is diagnosed by taking a biopsy from one of the lesions. The skin will show the signs typical of thrombogenic vasculitis (figure 3).

• It is the internal organ and neurological symptoms (table II) that make thissuch a serious disorder and are the reason it was initially called malignant atrophic papulosis, referring to the gastrointestinal, neuro-ophthalmological, cardiac and renal damage it causes.

• This disease has an erratic course whose unpredictability is exacerbated by the absence of predictive factors. It can range from purely cutaneous and chronic forms seen mainly in women and in some familial cases, to the more serious types described above. Delayed recurrences are always possible. Conversely, the prognosis for the super-acute gastrointestinal and neurological forms is poor.

• Several causes and underlying pathophysiological mechanisms have been put forward for this disease. When it arises in patients with an autoimmune deficiency or is triggered by infectious or viral mechanisms, one of the possible causes suggested is a systemic thrombogenic vasculitis.

• No standard treatment has been officially established (table III): the treatment to be given is defined on the basis of the results of an exhaustive work up, the clinical symptoms and signs presented by the patient and the activity of the disease. (Table II)

The new monoclonal antibody eculizumab appears promising.

(1) Withdrawn from the French market .