Background/Purpose

Malignant Atrophic Papulosis (MAP) is a rare vasculopathy of unknown etiology commonly presenting with cutaneous lesions, but can progress to multisystem disease with a fatal outcome. MAP has been reported in the setting of other CTDs. Currently, there is a limited understanding of the association between CTDs and overlapping MAP. We theorize that MAP can present independent or complicating a vasculopathic CTD.

Methods

We performed a retrospective review on approximately 200 MAP cases obtained through a PubMed literature search, personal experience and communications with treating physicians worldwide. Cases were then analyzed for overlapping CTDs and further stratified for factors associated with disease outcomes.

Results

Of the 200 MAP cases, we identified 33 with an overlapping CTD diagnosis.  These included: 11 SLE, 1 chronic cutaneous lupus erythematosus (CCLE), 3 SSc, 8 DM, 1 amyopathic DM, 6 APL syndrome (including patients positive for antibodies) 1 undifferentiated CTD, 1 granulomatous polyangiitis, and 1 RA. Patients with systemic MAP had fatal outcomes. Patients positive for APL antibodies with cutaneous MAP have survived but patients with APL syndrome and MAP have died. DM patients given steroids have died. In the setting of SSc there are two females who are alive and one male who died of fulminant SSc. Both females were on treprostinil for pulmonary hypertension.

Conclusion

MAP complicating CTD represents a challenging picture diagnostically and therapeutically. The presence of MAP may be a marker of more severe microvascular disease in those with SSc and DM and may be associated with worse prognosis because of concurrent steroid use. Steroids have been associated with increase risk of GI perforations and death. We believe MAP can present in the setting of a CTD in both cutaneous only and systemic forms. Histologically lupus and MAP can have similar findings but are not the same disease. MAP is not a systemic vasculitis but in its initial stages can present with an inflammatory infiltrate that later changes to the characteristic dermal necrosis and atrophic dermis with non-inflammatory endarterial thrombotic occlusion, leading to wedge-shaped skin necrosis, sclerosis and mucinosis. When MAP affects the CNS, the CNS pathology could have a perivascular lymphocytic infiltrate like a vasculitis, but MAP is not a vasculitis and seems to respond very poorly to steroids.  Early intervention of systemic MAP can be lifesaving.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/malignant-atrophic-papulosis-map-complicating-connective-tissue-diseases-ctds/