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Degos disease - malignant atrophic papulosis or cutaneointestinal lethal
syndrome: rarity of the disease
#MMPMID25926751
Pirolla E
; Fregni F
; Miura IK
; Misiara AC
; Almeida F
; Zanoni E
Clin Exp Gastroenterol
2015[]; 8
(?): 141-7
PMID25926751
show ga
BACKGROUND: Degos disease is a very rare syndrome with a rare type of multisystem
vasculopathy of unknown cause that affects the skin, gastrointestinal tract, and
central nervous system. Other organs such as the kidneys, lungs, pleura, liver,
heart, and eyes, can also be involved. OBJECTIVE: To highlight the incidence of
Degos disease with regard to age and sex, discuss the necessity of its accurate
and early diagnosis, and demonstrate the most current techniques for its
diagnosis; to discuss whether early therapeutic intervention can impact patient
prognosis; and to present a literature review about this disease. DESIGN: With a
retrospective, observational, nonrandomized trial, we described the evolution of
the different forms of Degos disease and referenced the literature. DATA SOURCES:
Research on rare documented cases in the literature, including two cases of
potentially lethal form of the disease involving the skin and gastrointestinal
system and, possibly, the lungs, kidneys, and central nervous system. A case of
the benign form of the disease involving the skin was observed by the authors.
MAIN OUTCOME MEASURES: Differences between outcomes in patients with the
cutaneointestinal form and skin-only form of the disease. There was one fatal
outcome. We reviewed possible new approaches to diagnosis and treatment. RESULTS:
The study demonstrated the rapid evolution of the aggressive and malignant form
of the disease. It also described newly accessible Phase I diagnostic tools being
currently researched as well as new therapeutic approaches. LIMITATION: The
rarity of the disease, with only eleven cases throughout the literature.
CONCLUSION: The gastrointestinal form of Degos disease can be lethal. Its
vascular etiology has finally been confirmed; however, new and more accurate
early diagnostic modalities need to be developed. There are new therapeutic
possibilities, but the studies of them are still in the early stages and have not
yet shown the full effectiveness of these new therapies.
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