Search citation statements
Paper Sections
Citation Types
Year Published
Publication Types
Relationship
Authors
Journals
A 20-year-old previously healthy man awoke seven days prior to admission with four asymptomatic crusted papules ringed by erythema located on his right posterior elbow (Fig 1). He denied any pain, burning, itching, or other sensation in the affected area and reported he "felt fine."He elected to treat the lesions with bacitracin ointment and did not seek medical attention.Thirty hours prior to admission the patient awoke with new lesions on the left flank and supraumbilical (Fig 2) regions. He became alarmed at this point and sought medical attention. A punch biopsy of the left flank lesion was performed and the patient was referred to the Bethesda (Md) Naval Hospital for evaluation.On initial evaluation, his history revealed no lesional symptomatology and the patient denied any abdominal pain, melena, hematochezia, nausea, vomiting, diarrhea, aphasia, seizures, sleep disturbances, loss of sensation, paresis or paralysis, visual disturbances, or hematuria. Several crusted papules with porcelain-white centers and erythematous rims were noted, as were a few linear purpuric macules. The abdominal and neurologic examinations revealed no abnormalities and the remainder of the physi¬ cal examination was unremarkable. A biopsy specimen of one of the supraumbilical papules revealed full-thickness epidermal necrosis peripherally, central ulcération with some dermal necrosis, and very few scattered lymphocytes (Fig 3).
A 20-year-old previously healthy man awoke seven days prior to admission with four asymptomatic crusted papules ringed by erythema located on his right posterior elbow (Fig 1). He denied any pain, burning, itching, or other sensation in the affected area and reported he "felt fine."He elected to treat the lesions with bacitracin ointment and did not seek medical attention.Thirty hours prior to admission the patient awoke with new lesions on the left flank and supraumbilical (Fig 2) regions. He became alarmed at this point and sought medical attention. A punch biopsy of the left flank lesion was performed and the patient was referred to the Bethesda (Md) Naval Hospital for evaluation.On initial evaluation, his history revealed no lesional symptomatology and the patient denied any abdominal pain, melena, hematochezia, nausea, vomiting, diarrhea, aphasia, seizures, sleep disturbances, loss of sensation, paresis or paralysis, visual disturbances, or hematuria. Several crusted papules with porcelain-white centers and erythematous rims were noted, as were a few linear purpuric macules. The abdominal and neurologic examinations revealed no abnormalities and the remainder of the physi¬ cal examination was unremarkable. A biopsy specimen of one of the supraumbilical papules revealed full-thickness epidermal necrosis peripherally, central ulcération with some dermal necrosis, and very few scattered lymphocytes (Fig 3).
Degos disease is a rare disorder, characterized by the development of typical atrophic porcelain-white macules on the trunk and extremities. It most commonly occurs in middle-aged Caucasian adults and eventuates in fatality in the majority of patients. A minority of reported patients have a more benign course and a favorable outcome. Degos disease is extremely rare in children. We report Degos disease in a teenager with an extended survival and the absence of internal involvement. We discuss the concept of benign cutaneous Degos and review the 34 patients (30 adults and four children) identified in the world literature with skin biopsy and at least 1 year of follow-up; the average age was 37.6 years; 26 were female and eight were male. As opposed to the malignant form of Degos disease, benign cutaneous Degos is more commonly reported in women (3:1). No laboratory abnormalities have been predictive of a benign versus malignant course. We also discuss the 24 reported instances of Degos disease (malignant and benign) in the pediatric population, of which 14 (58%) were fatal, with death occurring on average 3.6 years after diagnosis.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.