Pediatric Malignant Atrophic Papulosis

Abstract

Malignant atrophic papulosis (MAP), also known as Degos disease, is an extremely rare disease that is characterized by its unique skin presentation (namely, central, porcelain-white atrophic lesions with a telangiectatic rim). MAP has the following 2 variants: cutaneous MAP is manifested in the skin alone, whereas systemic MAP affects the gastrointestinal tract, central nervous system, lungs, and other internal organs. Some patients who presented with only cutaneous symptoms at first may develop systemic symptoms several years later. Although the exact pathologic mechanisms are unclear, Magro et al suggested in a recent study that MAP is a vascular injury syndrome that involves complement component C5b-9 complex deposition and high expression of interferon-α. The prognosis of systemic MAP is poor and typically fatal within a few years. Nonetheless, because the C5b-9 complex is detected in MAP, some researchers have suggested combined treatment with eculizumab (a humanized monoclonal antibody against C5) and treprostinil (a prostacyclin analog). Here, we report on a girl with systemic MAP who had severe central nervous system involvement and responded to eculizumab.

Interferon-complement loop in transplant-associated thrombotic microangiopathy

“…High levels of interferons administered exogenously as a therapeutic agent can be directly damaging to the endothelium and are known to result in TMA. [43][44][45][46][47] BK virus has been shown to trigger high interferon-g production in renal transplant recipients and also to be associated with an increased risk of complement mediated TA-TMA in HSCT recipients. 48 In addition, BK virus can directly injure endothelial cells and promote release of interferon-g. 49 Inflammatory cytokines like IL-6, IL-8, and interferong are also released from circulating activated T cells, NK cells, monocytes, and tissue macrophages as a result of BK virus infection, GVHD and in disorders like hemophagocytic lymphohistiocytosis, with high interferon production resulting in thrombotic microangiopathy.…”

Long-Term Follow-Up of A Patient with Juvenile Dermatomyositis and Degos-Like Disease

“…6 The patient here described had Gottron's sign and heliotrope erythema as cutaneous ndings of JDM, but also presented with papules with hematic crusts and white atrophic scars, suggestive of Degos' disease. 1 Degos' disease can be classi ed as classic or Degos-like disease. 5 Classic Degos' disease features typical skin lesions and multiple limited infarcts in other organs, such as bowel and central nervous system; approximately 15% of the cases are a benign form often limited to the skin.…”

“…4,7−13 In both types, skin lesions are distinctively characterized by diffuse papules with porcelain-white centers and slightly raised erythematous telangiectatic rims. 1,5 On histopathology, the lesions demonstrate hyperkeratosis, epidermal atrophy, and an underlying wedge-shaped area of dermal ischemia and necrosis with a prominent lymphocytic in ltrate; vessels in the papillary dermis show brinoid necrosis and thrombosis. Mucin deposition is seen in all stages, and brin deposition may be also observed.…”

“…Degos' disease, Kölhmeier-Degos' disease, fatal intestinal-cutaneous syndrome or malignant atrophic papulosis is a rare vasculopathy of unknown cause, which primarily involves the skin, intestinal tract, central nervous system and occasionally other organs. 1,2 Kohlmeier, in 1941, reported a case described as Buerger disease or thromboangiitis obliterans of mesenteric vessels. Later, Degos described a similar patient with "atrophic papulosquamous dermatitis".…”

“…The cutaneous component was then emphasized with the name of "malignant atrophic pustulosis". 1 Degos-like disease has been associated with several rheumatologic conditions such as systemic lupus erythematosus (SLE), antiphospholipid syndrome, systemic sclerosis (SS) and rheumatoid arthritis (RA). [3][4][5] Herein we describe a patient with juvenile dermatomyositis (JDM) associated with Degos-like disease.…”