British Journal of Dermatology | 2021
P01: A rare case of cutaneous collagenous vasculopathy in a young man
Abstract
P01 A rare case of cutaneous collagenous vasculopathy in a young man H. Kaur Bansal, V. Shpadaruk and M. El Naggar University Hospitals of Leicester, Leicester, UK; Kettering General Hospital, Kettering, UK; and Northampton General hospital, Northampton, UK Cutaneous collagenous vasculopathy (CCV) is an uncommon and underdiagnosed microangiopathy of dermal blood vessels usually seen in middle-aged to older adults, with a mean age of onset of 62 years. There is no sexual predilection (Burdick L, Losher S, Somach S, Billings S. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol 2012; 39: 741–6). There are < 40 cases reported in literature. It is clinically difficult to distinguish it from generalized essential telangiectasia or pigmented purpuric dermatosis. Therefore, histology is vital to making a confirmative diagnosis. Skin biopsy is distinctive and shows dilated capillaries and postcapillary venules, with marked eosinophilic hyaline material deposition in the vessel walls, which stain positive with periodic acid–Schiff (PAS) stain. We report a case of cutaneous collagenous vasculopathy in an 18-year-old man. He presented with a 3-month history of asymptomatic telangiectatic, blanching patches on his lower legs and ankles. Clinically, he was well and had no significant medical or drug history. The clinical differential diagnoses were mastocytosis, cutaneous collagenous vasculopathy, pigmented purpuric dermatosis and cutaneous small-vessel vasculitis. His blood tests and vasculitic screen were all within normal limits. The histology showed dilated vessels in the papillary dermis. There was no perivascular inflammation or evidence of vasculitis. There was telangiectasia with amorphous PAS-positive material within the blood vessels, in keeping with collagen deposition. Clinically, cutaneous collagenous vasculopathy presents as blanching macules and patches, telangiectasia or petechiae. It usually appears initially on the lower limbs and then spreads to the torso and upper limbs. It is usually symmetrical. The first reported case of CCV was reported by Salama and Rosenthal in 2000 (Burdick et al.). To date, only one paediatric case of CCV has been reported in a 16-year-old (Lloyd BM, Pruden SJ, II, Lind AC, Berk DR. Cutaneous collagenous vasculopathy: report of the first pediatric case. Pediatr Dermatol 2011; 28: 598–9). The cause and pathogenesis are still unknown. No underlying medical condition or medications have been associated with this condition. However, most adult cases in the literature have diabetes mellitus, hypertension and hyperlipidaemia (cardiovascular risk factors). CCV does not affect mucosal surfaces and has no systemic involvement. The disease duration ranges from a few months to several years, with only about three cases presenting with pruritus. This condition is mainly treated with laser, to improve its cosmetic appearance. P02 A case of porokeratosis of Mibelli extending into a graft site C. Drumm, C. Gulmann and S. N ı Raghallaigh St Vincent’s University Hospital and Beaumont Hospital, Dublin, Ireland Porokeratoses (PKs) are a group of heterogeneous and rare acquired or congenital skin diseases, identified by marginate scaling lesions. They are characterized by abnormal keratinization resulting from abnormal clonal expansion of keratinocytes, with numerous genetic mutations implicated. In addition to genetic factors, various triggers such as immunosuppression, infection, drugs, ultraviolet radiation and mechanical trauma have been documented. The histopathological hallmark of PK is the cornoid lamella, a column of parakeratotic cells that occupies the small epidermal invaginations (Weidner T, Illing T, Miguel D, Elsner P. Treatment of porokeratosis: a systematic review. Am J Clin Dermatol 2017; 18: 435–49). Classic PK, the most common variant, was first described by Vittorio Mibelli and is thus called PK of Mibelli (PM). We present the case of a 71-year-old man who was referred to our service with a squamous cell carcinoma (SCC) on his right ear; the SCC was excised. He was noted to have an annular hyperkeratotic plaque with central atrophy and raised borders on his right forearm, which had been present for 10 years and treated as a plane wart with cryotherapy and Salactol by his family physician. He was diagnosed with PK of the right forearm and treated with four 3-week courses of 5% fluorouracil cream with minimal improvement. Subsequent treatments included repeated cryotherapy, imiquimod cream 5%, Dermovate with 40% paraffin gel and salicylic acid 2%, retin A 0 5%, calmurid cream (10% urea, 5% lactic acid and 1% hydrocortisone) and regular filing. After 4 years of attending our service, he was lost to follow-up. Six years later he represented. The porokeratotic plaque had increased in size, measuring 18 cm 9 17 cm. In addition, two nodules suspicious for SCC had developed within the plaque. Biopsy of both these nodules revealed well-differentiated SCC. These were subsequently excised with two split-thickness skin grafts from the left lateral thigh. His residual PK was managed with topical fluorouracil 5% to the border of the plaque. A year following the excisions with split-thickness skin grafts it was noted that the PK was extending into the graft site. Acitretin 10 mg daily was started, and he applies Flexitol heel balm as his PK continues to slowly extend into the skin graft on his arm, as well as circumferentially onto the palmar aspect of his right hand. To our knowledge this is the first report of PK extending into a graft site. As surgical management, including excision and skin grafting has been described in the management of PK this finding could alter management decisions.