[Degos atrophic malignant papulosis. Not always malignant!].

Area of Science:

• Dermatology
• Internal Medicine
• Pathology

Background:

• Malignant atrophic papulosis (MAP) is a rare systemic disease characterized by thrombotic occlusion of small vessels.
• It typically affects multiple organs, including the skin, gastrointestinal tract, and central nervous system.
• The term 'malignant' implies a severe prognosis, often associated with significant morbidity and mortality.

Observation:

• A case of MAP is presented with exclusive cutaneous manifestations.
• Repeated colonoscopies were performed, revealing no evidence of malignant papules within the colon.
• This observation suggests a localized or potentially less severe form of the disease.

Findings:

• The patient's presentation deviates from the typical multi-organ involvement seen in malignant atrophic papulosis.
• The absence of gastrointestinal lesions, despite thorough investigation, indicates a potentially benign clinical course.
• Literature review supports the existence of variants of atrophic papulosis with a less aggressive clinical behavior.

Implications:

• The findings challenge the universal application of the term 'malignant' to all cases of atrophic papulosis.
• The case highlights the importance of considering localized cutaneous forms of MAP.
• The discussion of permanent anticoagulant therapy in this context warrants further investigation for efficacy in non-systemic variants.