Rash, Myalgia, and Weakness

Posted by Sara Fazio • June 13th, 2014

In the latest Case Record of the Massachusetts General Hospital, a 32-year-old man was admitted to this hospital because of a violaceous eruption that was followed by muscle pain and weakness, leading to respiratory failure. New papules with white centers developed. A diagnostic procedure was performed.

Malignant atrophic papulosis can occur in association with autoimmune diseases, most often SLE [systemic lupus erythematosus], but it has also been reported in patients with progressive systemic sclerosis, rheumatoid arthritis, discoid lupus, and dermatomyositis.

Clinical Pearls

What are dermatologic findings seen in patients with dermatomyositis?

Gottron’s papules are erythematous papules that overlie interphalangeal and metacarpophalangeal joints, elbows, and knees. They are pathognomonic for dermatomyositis. A heliotrope rash is seen in 30 to 60% of patients with dermatomyositis. Periungual erythema and edema are less specific signs that may be seen in dermatomyositis and other connective-tissue diseases, such as SLE.

What is malignant atrophic papulosis?

Malignant atrophic papulosis, or Degos’s disease, is a rare small-vessel vasculopathy, in which crops of erythematous cutaneous papules progress to depressed, porcelain-white scars with a varicelliform or varioliform appearance and a violaceous rim (consisting of fine telangiectasias) and then to inactive, atrophic white scars. Lesions are small, measuring between 0.5 and 1 cm in diameter. New papules typically occur in crops, and lesions at differing stages are usually seen. Tens to hundreds of lesions may be seen at any given time. Classic sites of involvement are the trunk and proximal extremities; however, cases involving the face, scalp, and genitalia have been reported.

Morning Report Questions

Q: What are associated multi-system features of malignant atrophic papulosis?

A: In the majority of reported cases, malignant atrophic papulosis is a multisystem disease in which skin findings precede gastrointestinal and central nervous system involvement or, on rare occasions, other organ involvement by weeks to years. Abdominal pain, nausea, diarrhea, and melena signal the onset of gastrointestinal tract infarcts occurring anywhere from the mouth to the anus. Bowel perforation resulting in peritonitis is a common cause of death in these patients. Involvement of the central nervous system is seen in approximately 20% of patients, heralded by hemiparesis, monoplegia, sensory abnormalities, or visual changes. A chronic, benign, skin-limited form of malignant atrophic papulosis has been recognized, and malignant atrophic papulosis also has been reported in families with an autosomal-dominant mode of inheritance, with variable expressivity.

Q: What is the pathogenesis of malignant atrophic papulosis?

A: The pathogenesis of malignant atrophic papulosis has yet to be fully elucidated. A study of four patients showed increased expression of MxA (a type I interferon-inducible protein), signifying dysregulated interferon-(alpha), and C5b-C9 (membrane-attack complex) in endothelial cells, vessel walls, perivascular interstitium, inflammatory cells, and keratinocytes in involved skin, suggesting that complement-mediated injury to endothelial cells may be involved in the pathogenesis of malignant atrophic papulosis.

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