DP09: A case of cutaneous collagenous vasculopathy with concomitant gastric antral vascular ectasia

Stephanie Bowe, Anna Wolinska, Gregg Murray, Cliona Feighery and Jane Thorne

Our Lady of Lourdes Hospital, Drogheda, Drogheda, Ireland

A 65-year-old man was referred to dermatology with a diffuse telangiectatic rash. A diagnosis of cutaneous collagenous vasculopathy (CCV) was made following punch biopsy. Sheets of blanchable telangiectasias had appeared bilaterally at the age of 14 years and over weeks became widespread and progressed in no particular pattern. He was asymptomatic and so never pursued treatment. His sister developed an identical rash in her early teens that followed the same clinical course. No other family members, including offspring, have a telangiectatic rash. His past medical history included hypertension and symptomatic iron deficiency anaemia due to gastric antral vascular ectasia (GAVE), which was diagnosed on gastroscopy in 2014. He does not have coeliac disease and has no liver disease. His sister has not been diagnosed with GAVE but has not undergone endoscopy. A skin punch biopsy of a representative site showed characteristic mildly ectatic superficial dermal vessels with mural hyaline periodic acid–Schiff-positive material and sparse chronic inflammatory infiltrate. CCV was first described in 2000, with fewer than 50 case reports to date. It is a rare microangiopathy of dermal blood vessels with a unique histological appearance. It is considered a benign condition. It is clinically indistinguishable from generalized essential telangiectasia, but CCV has no history of an ‘upward march’ of lesions and no female predominance. The aetiology is currently unknown. While there is no definite association with systemic illness or medication, many of the currently reported cases are in patients diagnosed with hypertension and cardiovascular disease. There are also reports of cases in those with autoimmune conditions and those taking at least one regular medication. GAVE is an uncommon cause of upper gastrointestinal bleeding with a distinct endoscopic appearance. Histopathologically, it is also characterized by vascular ectasia. It is not generally related to systemic diseases, but it has been associated with cirrhosis and connective tissue diseases, including systemic sclerosis. CCV may be a symptom of an underlying systemic disease or possibly a genetic defect causing abnormal collagen deposition, but its cause remains unclear. We recommend a skin biopsy to exclude CCV in patients who present with an acquired diffuse cutaneous telangiectatic rash and detailed past medical, family and medication history should be done. Our case describes CCV that presented in adolescence in a man with concomitant diagnosis of GAVE, which has a similar histological appearance. There may be a genetic predisposition to CCV, but further study is needed.