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Recurrent Pleural Effusions in Systemic Malignant Atrophic Papulosis (Köhlmeier-Degos’ disease)

Beth Israel Medical Center, New York, NY

INTRODUCTION: Malignant atrophic papulosis (MAP), also known as Köhlmeier-Degos’ disease, is a rare and clinically distinctive vasculopathy. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction in the involved organ systems. Its rarity and rapidly fatal course make the disease a difficult diagnostic and therapeutic challenge.

CASE PRESENTATION: A 38-year-old man was admitted to the hospital with a rash, vomiting and shortness of breath. Five years previously, he developed multiple papular, cutaneous lesions that were distributed mainly on his chest. The skin biopsy revealed morphea. Over the next two years, the skin lesions spread. Subsequent biopsies were interpreted as consistent with lupus, pyoderma gangrenosum and herpes zoster. In the current admission, evaluation for gastrointestinal, rheumatologic and hematologic disorders were unrevealing. A repeat skin biopsy was interpreted as consistent with MAP. Bilateral pleural effusions complicated the hospital course. These transudative effusions were massive and recurrent, requiring repeated thoracenteses. Echocardiography showed no abnormalities. Antiplatelet therapy, plasmapheresis and intravenous immunoglobulin (IVIG) were administered sequentially, as these modalities have been shown inconsistently to be of benefit in the treatment of MAP. He was discharged in stable condition but was readmitted one week later with large pleural effusions. He had repeated thoracenteses, bilateral tube thoracostomies and an attempt at talc pleurodesis. Additional plasmaphereses were ineffective in altering the progressive course of the disease. Eventually, he developed an ischemic cerebral infarction and acute respiratory distress syndrome, leading to his death.

DISCUSSIONS: MAP is typically limited to the skin, but progression to systemic disease occurs frequently. It is different from other vasculitides in that inflammation is not a prominent component of the disease. Systemic involvement is frequently characterized by distinctive infarctions of the skin, gastrointestinal tract and central nervous system (1). Involvement of the latter two sites is often the cause of death. Intrathoracic manifestations in MAP are unusual and are not a major cause of morbidity (2). Although there are reports of pleural adhesions in patients with MAP, massive recurrent pleural effusions, as in the present case, have not been reported.

CONCLUSION: This case highlights three major issues. First, intrathoracic manifestations (in this case, pleural effusions), are rare but potentially important complications. Second, we report a patient’s poor response to antiplatelet agents, plasmapheresis and IVIG. Finally, physicians should be mindful of MAP since prompt diagnosis and treatment may be of temporary benefit in this otherwise fatal disease.

DISCLOSURE: E. Fernández, None.

REFERENCES

1. Snow JL, Muller SA. Degos’ syndrome: malignant atrophic papulosis. Semin Dermatol.1995; 14 :99 –105.
2. Pierce RN, Smith GJ. Intrathoracic manifestations of Degos’ disease (malignant atrophic papulosis). Chest.1978; 73 :79 –84.