Answer to the last #DiagnosticDilemma
Correct Answer: Cutaneous collagenous vasculopathy

A 38-year-old woman presented with a 2-year history of asymptomatic, widespread rash. On examination, multiple telangiectasias, which blanched on the application of pressure, were noted on the trunk (Panels A and B) and the arms and legs. There was no involvement of the nails or mucosal tissue. Numerous investigations, including autoimmune screening, cervical and thoracoabdominal computed tomography, venous Doppler ultrasonography, and angiography of the legs, were normal.

Examination of a biopsy specimen obtained from the abdominal wall revealed dilated vascular spaces in the upper dermis, with perivascular hyalinization (highlighted with period acid–Schiff and collagen IV staining, Panel C), normal numbers of mast cells, and no indication of amyloid deposition.

On the basis of these findings, a diagnosis of cutaneous collagenous vasculopathy was made. This condition is a microangiopathy that is thought to be caused by a genetic defect that alters collagen production in skin microvasculature.
Given the absence of other symptoms and the extent of the disease, no treatment was proposed. The lesions were stable at a 5-month follow-up visit, and the patient was lost to follow-up after a visit at 6 months.

Clinical Pearls:-
- Clinically, cutaneous collagenous vasculopathy is indistinguishable from generalized essential telangiectasia, but histologic analysis reveals the characteristic thickening of capillary walls.

Given the absence of other symptoms and the extent of the disease, no treatment was proposed.

Source: https://www.nejm.org/doi/full/10.1056/NEJMicm1408473
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s●●●●d m●●●●●●d h●●●s General Medicine
UVवः ह6गव्हक़U
Oct 6, 2018Like