Background/Purpose

Malignant atrophic papulosis (MAP) is a rare thrombo-occlusive vasculopathy that presents with cutaneous only lesions but can progress after months or years to rapidly fatal systemic involvement.   Until the very recent past, there were no reports of effective treatment of the systemic disease.  A recent publication described successful use of trepostinil, a prostacyclin analog, in treatment of systemic MAP and in MAP-scleroderma-SLE overlap. 

Methods

We performed a retrospective analysis of six patients using data collected from treating physicians worldwide through personal communications and our own experience. We also employed PUBMED for a literature search, which yielded 200 articles, using the keywords malignant atrophic papulosis and Degos disease. We evaluated outcomes of six MAP patients who were treated with treprostinil to identify discriminating factors between survivors and non-survivors.

Results

Among the six treprostinil MAP treated patients we know four are alive.  Our first treprostinil treated patient was a female with scleroderma/SLE overlap who developed MAP lesions without systemic MAP involvement. As a result of pulmonary hypertension she was started on treprostinil. Cutaneous lesions subsequently resolved. We then treated a primary MAP male who had progressive CNS disease despite ongoing therapy with eculizumab. Within months after treprostinil was started MRI showed resolution of the lesions. Another male on eculizumab with disease progression was started on treprostinil with resolution of symptoms. The fourth patient was a female with biopsy proven MAP who already had CNS involvement with loss of vision in the right eye that led to enucleation.  Treprostinil was started with temporary stabilization of symptoms. In the fifth patient, treprostinil was started after severe systemic involvement had taken place. She had GI perforations and CNS involvement which led to death. Eculizumab was not the first line therapy in patients four and five.  Lastly, through a literature search we found a sixth patient who was a female on treprostinil for pulmonary hypertension related to systemic sclerosis.  She then developed a restricted form of MAP while on treprostinil and is possibly alive, making a total of five patients alive today.

Conclusion

MAP is a rapidly fatal systemic disorder with a life expectancy of less than one year after development of visceral involvement.   After comprehensive review of treprostinil use for MAP, we found the majority of patients treated (4/5) are still alive up to 42 months after initiation of therapy.  All survivors with systemic disease are on dual therapy with eculizumab.  The first patient with cutaneous MAP and scleroderma/SLE overlap had resolution of skin lesions on treprostinil alone.  Patient six developed a restricted form of MAP while on treprostinil leading the authors to believe treprostinil limited the MAP vasculopathic process.  The efficacy of treprostinil in MAP may be related not only to antithrombotic and vasodilatory effects but also to its reported ability to increase the number of endothelial progenitor cells.

---

« Back to 2014 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/treprostinil-use-in-malignant-atrophic-papulosis-kohlmeier-degos-disease-review-of-worldwide-experience-to-date/