A fatal case of malignant atrophic papulosis

ARTICLE

Auteur(s) : Wen-Hua Zhao¹, Chun-Ying Wang¹, Wu-Yuan Zhou¹, Meng Guo¹, Yan Li¹, Li-Min Zhao¹, Fang-Lian Jiao¹, Ling Li², Sheng Li¹

¹Shandong Tumor Hospital, Jinan 250117, Shandong Province, China
²Shandong Academy of Medicine Sciences, Jinan 250086, Shandong Province, China

Malignant atrophic papulosis (MAPs, otherwise known as Degos disease) has a purely cutaneous variant and a systemic variant with cutaneous manifestations. We describe a 41-year-old woman who presented with a widespread skin eruption, consistent with malignant atrophic papulosis with gastrointestinal involvement.

The patient came to our hospital for abdominal distention and pain 20 days after an appendectomy. Ten days after the appendectomy, she developed pan-abdominal tenderness, which became progressively worse with apparent ascites and an enlarged spleen. 1400ml of purulent ascites fluid was removed during an abdominal puncture operation. Furthermore, the patient had experienced multiple skin papules for 2 years. The patient was in a poor nutritional state. A plethora of isolated wine papules 0.2 ~ 0.5 cm in size with clear demarcations were visible on the trunk and limbs. The papules featured a core of necrotic abscess and annular hyperemic bands at the borders. There was some fine scraping over the papule’s surface. Histopathological examination of skin biopsy tissue revealed lesions characteristic of malignant atrophic papulosis. The epidermis developed a cone-shaped necrosis, the subcutaneous accessory apparatus disappeared, visibility of the blood vessels was decreased and necrosis and thrombosis could be observed (figure 1A). Treatment included limited eating and drinking, gastrointestinal decompression, peritoneal cavity drainage, intravenous antibiotics and parenteral nutrition. Peritonitis was controlled and the skin macules decreased after a 20 day treatment period (figure 1B). But the patient left hospital for economic reasons and died of serious celiac infection two months later.

MAPs is a very rare condition characterized by atrophic papular skin lesions and a variable association of systemic involvement. Köhlmeier [1] reported the first case in 1941. It is characterized by narrowing and occlusion of the lumen by intimal proliferation and thrombosis, which leads to ischemia and infarction in the organ systems involved. Its rarity and rapidly fatal course make the disease a difficult diagnostic and therapeutic challenge [2]. Systemic MAPs can involve the nervous, ophthalmological, gastrointestinal, cardiothoracic and hepatorenal systems. No specific laboratory test can be used to aid diagnosis [3]. The laboratory investigations of Hohwy et al. [4] revealed a mutation of factor V Leiden and the presence of lupus anticoagulant, but no anti-cardiolipin antibodies. Individual patients can exhibit a variety of coagulation abnormalities. In one published series of 15 cases, 4 patients had raised serum fibrinogen levels, 3 had a prolonged euglobulin lysis time, and 1 had increased platelet adhesiveness [5]. Death usually occurs within 2-3 years from the onset of systemic involvement. In this case, the patient died 27 months after the occurrence of multiple skin lesions.

When she came to our hospital, the patient presented with intestinal perforation and acute diffuse peritonitis. We stopped oral intake (eating and drinking), decreased gastrointestinal tension and initiated anti-infection treatment and parenteral nutrition. Interestingly, the abdominal pain and skin lesions decreased simultaneously 20 days later. We consider fasting, gastrointestinal decompression and parenteral nutrition may play an important role in the 20 day treatment period. Anti-infection measures might alleviate peritonitis symptoms and decrease thrombosis of the skin and intestinal tract. Fasting and gastrointestinal decompression could lessen the stimulus of food on the intestinal mucosa and reduce the vascular inflammatory responses. Although no treatment has been shown to be effective in the treatment of MAPs so far, our observation showed that fasting and parenteral nutrition might be helpful measures in relieving the symptoms of MAPs.

In a patient with acute abdominal pain and atrophic papular skin lesions, clinicians should retain a suspicion of MAPs with gastrointestinal involvement. Fasting and parenteral nutrition might be helpful therapeutic measures, and more research is needed to find better treatment options.

Acknowledgements

References

2 Fernandez-Perez ER, Grabscheid E, Scheinfeld NS. A case of systemic malignant atrophic papulosis. J Natl Med Assoc 2005; 97: 421-5.

3 Scheinfeld N. Malignant atrophic papulosis. Clin Exp Dermatol 2007; 32: 483-7.

4 Hohwy T, Jensen MG, Tøttrup A, Steiniche T, Fogh K. A fatal case of malignant atrophic papulosis (Degos’ disease) in a man with factor V Leiden mutation and lupus anticoagulant. Acta Derm Venereol 2006; 86: 245-7.

5 Assier H, Chosidow O, Piette JC, Boffa MC, Youinou P, Thomas L, et al. Absence of antiphospholipid and anti-endothelial cell antibodies in malignant atrophic papulosis: a study of 15 cases. J Am Acad Dermatol 1995; 33: 831-3.

* Co-first authors.