Degos disease: a case report and review of the literature
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(2020) 14:204
CASE REPORT
Open Access
Degos disease: a case report and review of the literature Santosh Tummidi1*, Prabhakaran Nagendran2, Swaroopa Gedela1, Jami Rupa Ramani2 and Arundhathi Shankaralingappa1
Abstract Background: Degos disease is a very rare syndrome with multisystem vasculopathy of unknown cause. It can affect the skin, gastrointestinal tract, and central nervous system. However, other organs such as the kidney, lungs, pleura, and liver can also be involved. Case presentation: A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of depigmented painful lesions. A skin punch biopsy taken from the porcelain white atrophic papules which revealed features of Degos disease. Conclusion: The diagnosis of Degos disease is usually based on the presence of the pathognomonic skin lesions and a tissue biopsy demonstrating a wedge-shaped area of necrosis with thrombotic occlusion of the small arterioles. No specific treatment is currently available for this disease. Keywords: Degos disease, Malignant atrophic papulosis, Skin biopsy, Vasculopathy
Background Degos disease (DD), also called “malignant atrophic papulosis” or “lethal cutaneous and gastrointestinal arteriolar thrombosis,” is a rare occlusive arteriopathy having unknown pathogenesis and involves predominantly smallcaliber vessels of the dermis, gastrointestinal tract, central nervous system, and occasionally other organs [1–3]. Not more than 200 cases are reported in the literature [1, 4]. Case presentation A 35-year-old Hindu woman presented to our dermatology outpatient department with complaints of painful depigmented lesions of 1½ years’ duration. On examination, the lesions were porcelain white atrophic papules with surrounding erythema present over the trunk, upper limbs, thighs, and chest (Figure 1a-d). The lesions were associated with mild tenderness. She had a * Correspondence: [email protected] 1 Department of Pathology, All India Institute of Medical Sciences, Mangalagiri, Andhra Pradesh, India Full list of author information is available at the end of the article
history of joint pain for the last 7–8 years and a history of headache, giddiness, and ocular pain for the last 3 months. She had no history of proximal muscle weakness or skin tightening. Her menstrual cycles were regular. She had a history of irregular bowel habits (constipation). She had no history of hypertension or diabetes/any other medication. Results of her laboratory tests for antinuclear antibody, lupus anticoagulant, cardiolipin antibody, and β2glycoprotein were all negative. Ultrasonography of the abdomen was unremarkable. Ophthalmological examination of bilateral eyes showed myopic astigmatism. The result of stool occult testing of the patient’s blood was negative. A skin punch biopsy was taken from the porcelain white lesion over the trunk. Histopathological examination of the sections showed atrophic epidermis with mild hyperkeratosis and flattened rete ridges focally. The underlying papillary was showing a band-like m
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