Degos disease mimicking primary vasculitis of the CNS

Malignant atrophic papulosis with motor aphasia and intestinal perforation: A case report and review of published works

“…In patients with extracutaneous involvement (n = 43, excluding six patients whose information was not available and three patients with cutaneous involvement after systemic manifestations). [6][7][8] Moreover, we found that in patients in the systemic category, the first systemic manifestation occurred at a median of 2.0 years (95% CI, 0-7.8) after disease onset. The gastrointestinal tract and CNS were involved in 43 of 52 (82.7%) and seven of 22 patients (42.3%), respectively, of whom 30 (69.8%) and 12 patients (54.5%) died during follow up.…”

“…The mortality in all patients with the systemic type was 65.3% (34/52), whereas no patient death was observed among the 28 patients with pure cutaneous involvement ( P < 0.05). In patients with extracutaneous involvement ( n = 43, excluding six patients whose information was not available and three patients with cutaneous involvement after systemic manifestations) . Moreover, we found that in patients in the systemic category, the first systemic manifestation occurred at a median of 2.0 years (95% CI, 0–7.8) after disease onset.…”

Congenital Degos Disease: Case Report and Dermoscopic Findings

“…Although DD most commonly affects middle-aged Caucasian adults, with a male preponderance, onset from birth to 70 years has been described (1,2). Pediatric cases are rare, and only a single case with presentation at birth has been reported (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(18)(19)(20)(21)(22)(23). Most cases appear to be sporadic, but some suggest the possibility of autosomal dominant inheritance (1,9,12).…”

“…Systemic involvement, the result of multiple infarctions, affects the gastrointestinal tract, central nervous system, and rarely the pericardium, lungs, and kidney (2,9). Cerebral thrombosis, massive hemorrhage, and bowel perforation, among other systemic manifestations, are the major causes of mortality (2,3,6,7,10,14,20,22). It has recently been proposed that DD be classified into malignant and benign forms based on the presence of extracutaneous involvement, because not all patients follow the same course (1).…”

“…Systemic involvement occurred on average 18 months after initial typical skin presentation, demonstrating the same course as adult DD. Two cases developed cutaneous and systemic disease simultaneously, while three cases showed skin manifestations after systemic involvement (2,7). Only five cases, all females, appeared to have the benign cutaneous form (2,9,11,12), suggesting that in children, as in adults, the benign form is more common in female patients (1,2), although in our review, 88.5% (n = 31) of patients had the malignant systemic variant, in contrast to the recently described predominance of the benign form in adults (1,2) ( Table 1).…”