A purple plaque in a patient with systemic sclerosis.

We present the case of a 43-year old woman with anti-U3 ribonucleoprotein antibody-positive systemic sclerosis presenting with an enlarging purple plaque on the left upper arm. The skin was not sclerotic; however, there had been a cluster of long-standing telangiectases preceding the plaque. Histology and immunohistochemistry confirmed an angiosarcoma. There are five reported cases in the literature about angiosarcoma arising in the skin of patients with systemic sclerosis; however, to our knowledge, this is the first to have arisen from non-sclerotic skin. We would urge clinicians to adopt a high index of suspicion for atypical vascular tumours presenting in patients with systemic sclerosis.

The modified HoVert (mHoVert) method improves diagnostic certainty compared to the St John's protocol for alopecia biopsy specimens: A retrospective single center study.

BACKGROUND: Access to vertical and transverse sections of a punch biopsy specimen improves the diagnosis of alopecia. Both two biopsy specimen and single-punch biopsy specimen techniques to visualize both transverse and vertical sections have been described. Their comparative diagnostic certainty is not known. We aimed to assess the diagnostic certainty of a modified HoVert (mHoVert) method, without direct immunofluorescence (DIF), compared to the St John's protocol, a two-biopsy technique with DIF. METHODS: Fifty-seven cases of alopecia processed using the St John's protocol and 60 cases of alopecia processed using mHoVert were reviewed. Diagnoses made were rated as certain/probable, possible, or uncertain, depending on the language in the histopathology report. Cases processed by the St John's protocol had final diagnosis and DIF result recorded. RESULTS: In the mHoVert group, significantly more diagnoses were certain/probable (66%, 95% confidence interval [CI]: 57%-75%), compared to 46% (95% CI: 36%-56%) of diagnoses in the St John's protocol group (p = 0.005). DIF result did not affect the final diagnosis in any of the 57 cases reviewed. CONCLUSIONS: DIF is not required in the diagnosis of most cases of alopecia. The mHoVert method provides more certain/probable diagnoses than the St John's protocol and can reduce cost and patient morbidity.

Livedoid Vasculopathy: A French Observational Study Including Therapeutic Options.

Livedoid vasculopathy is a rare thrombotic cutaneous disease. This observational study aimed to assess the clinical and biological features of livedoid vasculopathy and the efficacy of treatments. Patients enrolled had typical livedoid vasculopathy both clinically and histologically. Investigation of thrombophilia was performed. Electromyography was undertaken in the presence of symptoms suggesting peripheral neuropathy. Eighteen women and 8 men were included, with a mean age of 35.5 years at onset. Twenty patients had at least one thrombophilia factor. Ten patients had a peripheral neuropathy with 2 of these patients demonstrating a specific thrombo-occlusive vasculopathy on muscle biopsy. Anticoagulation with low molecular weight heparin was the most prescribed therapy and was associated with the best outcome (effective in 14 patients). Eight patients had severe disease refractory to anticoagulation and required intravenous immunoglobulins, producing a good response in 6 patients.