Rare form of systemic occlusive arteriopathy of small
and medium-size arteries with a typical two-stage evolution. Disease affects
vessels of the dermis with an aspect of erythematous, dome-shaped papules
that develop a central area of necrosis and leave a porcelain-like scar.
Gastrointestinal tract and CNS can be affected. Can be fatal.
Malignant Atrophic Papulosis; Papulosis, Malignant
Atrophic; Degos Disease; Degos-Kohlmeier Disease; Kohlmeier-Degos Disease.
Disease was first described in 1941 by W. Kohlmeier, a
German radiologist, and first individualized in 1942 by Robert Degos, a
Autosomal dominant inheritance is observed in
Unknown. Familial cases have been described.
Immunologic, infectious, or thrombotic factors have been evocated.
Clinically evocated onset usually in young adults
between 20 and 40 years old, probably with male predominance. Characteristic
skin lesions (pink, asymptomatic, diffuse papules with a telangiectatic
border followed by umbilication and a porcelain-white center) appear.
Histologic appearance is typically characterized by a wedge-shaped area of
necrosis from the epidermis through the dermis.
Skin lesions can be followed by systemic
manifestations, sometimes a few years later. Features can involve the eyes
(avascular patches), CNS (20% cerebrovascular or medullar accident), and
GI system (40-60%, particularly small bowel with abdominal pain,
bleeding, and diarrhea; intestinal perforations and peritonitis are the
leading causes of death). Pleural and pericardial effusion can be observed.
Obtain full history. Evaluate
neurologic lesions (clinical, CT/MRI). Pleural and pericardial effusion must
be searched and treated before anesthesia if necessary (clinical, ECG, chest
radiographs, echography). Full blood count is necessary.
Eye protection is needed. Spinal and
perimedullar blockades probably should be avoided because of the frequency
of medullar accidents. Should obtain a bleeding time and coagulation profile.
Aspirin is often used to treat
patients. Heparin substitution should be considered.
Degos R, Delort J, Triscot R: Dermatite papulo squameuse atrophiante.
Bull Soc Franc Derm Syph 49:148, 1942.
Ojeda Cuchillero RM, Sanchez Regana M, Umbert Millet P: Benign cutaneous
Degos' disease. Clin Exp Dermatol 28:145, 2003.
Torrelo A, Sevilla J, Mediero IG, et al: Malignant atrophic papulosis in an
infant. Br J Dermatol