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Degos syndrome: malignant atrophic papulosis
1Department of Dermatology, Mayo Clinic, Rochester, MN 55905, USA.
Seminars in Dermatology
|June 1, 1995
Summary
Malignant atrophic papulosis presents with skin lesions and can affect internal organs. A purely cutaneous form exists, questioning the "malignant" classification for all cases.
Area of Science:
- Dermatology
- Pathology
- Internal Medicine
Background:
- Malignant atrophic papulosis (MAP) is a rare disorder.
- Characterized by unique skin lesions and potential thrombotic events in organs like the GI tract and CNS.
- Systemic involvement can occur weeks to years after skin lesions or, rarely, precede them.
Purpose of the Study:
- To discuss the diagnostic challenges and classification of malignant atrophic papulosis.
- To highlight the increasing recognition of a prolonged, purely cutaneous variant.
- To question the universal application of the term "malignant" in MAP.
Main Methods:
- Review of existing literature on malignant atrophic papulosis.
- Analysis of clinical presentations and disease progression.
- Discussion of pathological and etiological hypotheses.
Main Results:
- Recognition of a prolonged, purely cutaneous form of MAP.
- Uncertainty regarding the precise cause, classifying it as either vasculopathy or coagulopathy.
- Lack of effective therapies for systemic MAP.
Conclusions:
- The existence of a purely cutaneous variant challenges the term "malignant" for all MAP cases.
- The etiology of MAP remains unknown, hindering classification and treatment.
- Effective treatments for systemic malignant atrophic papulosis are currently unavailable.