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Diffuse dermal angiomatosis is a rare, benign, reactive cutaneous vascular proliferation that has been reported in the context of end-stage renal failure and can rarely be associated with arteriovenous fistulas. We report a striking clinical resolution following prompt diagnosis and subsequent arteriovenous fistula reversal. This case further demonstrates that accurate diagnosis is particularly rewarding since correct therapeutic approach can be curative.

SummaryBackground Intravascular large B-cell lymphoma (IVLBCL) is a rare type of extranodal LBCL. It is characterized by the proliferation of tumour cells exclusively intraluminally in small blood vessels of different organs. The clinical manifestation depends on the type of organ affected; additionally, a haemophagocytic syndrome can be observed in some patients. Objectives The aim was to further understand the nosology of this lymphoma as, due to its rarity and in spite of detailed immunohistochemical investigations, its exact nosology is only incompletely understood. Methods We used microarray-based analysis of gene expression of tumour cells isolated from a patient with primary manifestation of the lymphoma in the skin and compared it with various other diffuse LBCLs (DLBCLs) as well as a previously published DLBCL classifier. Results In unsupervised analyses, the tumour cells clustered together with non-germinal centre B-cell (non-GCB) DLBCL samples but were clearly distinct from GCB-DLBCL. Analogous to non-GCB DLBCL, molecular cell-of-origin classification revealed similarity to bone-marrow derived plasma cells. Conclusions The IVLBCL of this patient showed molecular similarity to non-GCB DLBCL. Due to the prognostic and increasingly also therapeutic relevance of molecular subtyping in DLBCL, this method, in addition to immunohistochemistry, should also be considered for the diagnosis of IVLBCL in the future.

Cutaneous polyarteritis nodosa (CPAN) is characterized by a necrotizing vasculitis of small and medium-sized arteries in the skin, which can be associated with fever, arthralgia, myalgia, and neuropathy, but, unlike polyarteritis nodosa (PAN), there is no visceral involvement. CPAN is rare in childhood. We report two siblings who developed CPAN during childhood. Interestingly, both had Mediterranean fever gene (MEFV) mutation, i.e. heterozygous E148Q. They also shared HLA-A24, -DR15 alleles. Simultaneous occurrence of MEFV mutation and HLA alleles with CPAN has never been reported in Japan. These cases could provide some hereditary clue for the development of CPAN.

Summary von Recklinghausen disease/neurofibromatosis (NF) is caused by an autosomal dominant mutation in NF1, resulting in a deficiency of neurofibromin 1, a protein with a tumour suppressor function in the Ras–extracellular regulated kinase pathway. The disease comprises a variety of clinical manifestations, including vascular abnormalities. Large vessel abnormalities are well known, while small vessels of the skin are very rarely involved. The latter can cause livedo, necrosis and painful ulcers. For such ulcers, all invasive therapies (e.g. surgery and radiotherapy) are harmful and should be avoided. Herein, we describe a patient with NF and cutaneous vasculopathy treated with imatinib, a tyrosine kinase inhibitor.

Summary We present the case of a 54-year-old patient with renal transplant who developed unusual vascular changes on the forearm distal to a functioning arteriovenous fistula, as well as a painful ulcerated lesion on her anterior abdominal wall. We believe that the diffuse dermal angioendotheliomatosis variant of reactive angioendotheliomatosis had a role in the pathogenesis of this patient's lesions.

Summary Vascular anomalies can be subdivided into vascular tumours and vascular malformations (VMs). While most VMs are present at birth and do not exhibit significant postnatal growth, vascular tumours are characterized by their dynamics of growth and (sometimes) spontaneous regression. This review focuses on benign vascular tumours other than infantile haemangiomas (IHs), namely pyogenic granuloma, eruptive pseudoangiomatosis, glomangioma, rapidly involuting and noninvoluting congenital haemangioma, verrucous haemangioma and spindle cell haemangioma. While some of them bear clinical resemblance to IH, they can be separated by age of appearance, growth characteristics and/or negative staining for glucose transporter 1. Separation of these tumours from IH is necessary because their outcome and therapeutic options are different. Semimalignant and malignant vascular tumours will be addressed in a separate review.

Objective. We report the use of nicotine-patch therapy on active mucocutaneous lesions of Behcet's disease (BD). Methods. Five BD ex-smoker patients with refractory active mucocutaneous manifestations were treated with nicotine patches for 6 months. Results. Four out of five patients quickly responded to nicotine-patch therapy and experienced a complete regression of mucocutaneous lesions. Other manifestations of BD did not respond and new manifestations appeared during this treatment. One patient had no benefit from therapy but on restarting smoking it was promptly effective. Conclusions. Mucocutaneous lesions associated with BD may be modulated by smoking. Both smoking and nicotine-replacement therapy may be efficacious not only on oral aphthae, but also on other mucocutaneous manifestations, whereas the efficacy in the treatment and prevention of other systemic manifestations of BD is not proven. At least in ex-smokers, nicotine in its pure form is well tolerated and its use could be justified in selected cases of BD with predominant and recurrent refractory mucocutaneous manifestations.

Objective FMF is an autosomal recessive hereditary disease, associated with a single gene named MEFV. This gene is considered to be responsible only for FMF. In the present study, we tried to find out whether the MEFV gene is associated with or responsible for clinical conditions other than FMF. Methods We looked for patients who presented with signs and symptoms not typical for FMF but carried MEFV mutations. We also searched for reports about similar conditions in the English medical literature, and we surveyed the website 'Infevers' for MEFV mutations defined as associated with 'atypical FMF'. Results We encountered three patients carrying MEFV mutations who presented with distinct clinical presentations not typical of FMF. We identified additional reports about MEFV-related non-FMF disease entities such as palindromic rheumatism. By screening the 'Infevers' website, we further disclosed 13 cases with MEFV mutations that were defined as 'atypical FMF' and 4 cases categorized as 'recurrent arthritis'. Conclusions These findings suggest that the MEFV gene is associated with clinical conditions other than FMF. Changing our concept regarding the MEFV gene and its link to such clinical phenotypes may call for a higher awareness of the existence of additional autoinflammatory diseases. Furthermore, a correct diagnosis of these MEFV gene mutation-associated syndromes will justify a therapeutic trial with colchicine, thereby relieving suffering of many patients who up to now have been misdiagnosed.

Objectives We examined the prevalence of LAC, aCL antibodies (Abs), anti-beta(2)-glycoprotein I (anti-beta(2)GPI) Abs and anti-phosphatidylserine-prothrombin complex (anti-PS/PT) Abs in patients with regular livedo reticularis or with livedo racemosa to determine whether those Abs correlate with the clinical or serological features. Assuming that a correlation exists, early recognition of the serological features of the cutaneous manifestations may aid in the treatment and prediction of complications. Methods We examined the prevalence of LAC, aCL Abs, anti-beta(2)GPI Abs and anti-PS/PT Abs in 143 Japanese patients who presented at our department with regular livedo reticularis or livedo racemosa between 2003 and 2008. LAC was determined according to the guidelines recommended by the Subcommittee on Lupus Anticoagulant/Phospholipid-Dependent Antibodies. Levels of anti-PS/PT, aCL and anti-beta(2)GPI Abs in serum samples taken from patients were measured by specific ELISAs. Results Anti-PS/PT Abs were detected in 94 (65.7%) of the livedo patients. Further, IgM anti-PS/PT Abs were detected in 90 (62.9%) of the livedo patients. Serum IgM anti-PS/PT Ab levels were significantly higher in livedo racemosa patients compared with regular livedo reticularis (19.2 +/- 17.0 vs 8.93 +/- 8.48 U/ml, P = 0.0013). Cutaneous vasculitis was significantly more prevalent among patients with livedo racemosa compared with regular livedo reticularis (P = 0.0014). Livedo racemosa patients had significantly higher CRP serum levels than regular livedo reticularis patients. Livedo racemosa has a stronger association with skin ulceration and arthralgia compared with regular livedo reticularis. Overall, we found a statistically significant association between cutaneous vasculitis and ischaemic cerebrovascular events in our livedo patients. Conclusions We speculate that IgM anti-PS/PT Abs could be implicated in disease susceptibility for livedo racemosa. We further suspect that cutaneous vasculitis could be closely related to pathogenic factors that trigger the development of livedo racemosa. Early detection of cutaneous vasculitis in skin biopsies of livedo patients should be useful for prognostic evaluation, including ischaemic cerebrovascular events.

Summary Background The cutaneous manifestations of microscopic polyangiitis (MPA) and polyarteritis nodosa (PAN) have not been compared since their distinction. Objectives To compare the clinical and pathological cutaneous manifestations in a series of patients with systemic MPA and PAN. Methods Patients with MPA (n = 162) and PAN (n = 248) from the database of the French Vasculitis Study Group were diagnosed according to the American College of Rheumatology and/or the Chapel Hill Consensus criteria. Purpura, livedo, nodules, urticaria, skin necrosis, oral and genital ulcers were recorded when present. Fifty-five skin biopsies were analysed. Clinical and histological skin data were compared in the following groups: MPA, PAN and two PAN subsets: PAN with and PAN without hepatitis B infection. The prevalence of systemic and biological manifestations were analysed in relation to the presence or absence of skin lesions. The χ2 test was used for statistical studies. Results Cutaneous manifestations were present in 44% of MPA and PAN. Purpura was the most frequent manifestation (26% cases of MPA vs. 19% cases of PAN, P = 0·026). Urticaria was more frequent during PAN (6% vs. 1·2%, P = 0·015). Skin lesions were more frequent during PAN in the absence of HBV infection (54% vs. 30%, P

A patient with elevated levels of serum IgA developed purpuric lesions histologically resembling Henoch-Schönlein purpura brought on by consuming alcohol. Alcohol challenge with 5 units of alcohol reproduced the lesions, with a rapid rise of circulating CD4+ and CD8+ T cells followed by a fall of serum IgA and C3 concentration. The skin lesions and serum abnormalities resolved spontaneously within 6 weeks of the alcohol challenge.

Two patients with an unusual association of extensive cutis marmorata telangiectatica congenita and aberrant mongolian spots are reported. We believe that such association is best explained as a phenomenon of non-allelic twin spotting. We found only one previous similar case in a textbook, and we support the classification of this condition as phacomatosis pigmentovascularis type V.

Acute haemorrhagic oedema (AHO) of infancy is a cutaneous leukocytoclastic vasculitis, clinically characterized by the acute development of peripheral oedema and targetoid purpuric lesions on the face and extremities. It usually affects children younger than 2 years of age. The disorder follows a benign course usually without recurrence or long-term complication. In most cases the origin is not clear, but underlying infections are assumed to play an aetiological role. We describe a 7-month-old boy whose clinical and histopathological features are typical of AHO. Serological tests clearly demonstrated a primary infection for cytomegalovirus (CMV). To our knowledge, this is the first reported case of AHO associated with CMV infection.

Background Antineutrophil cytoplasmic antibodies (ANCA) of the IgA isotype have, for the most part, been detected in patients with Henoch-Schonlein purpura (HSP) or inflammatory bowel disease. Objectives We have evaluated the prevalence of IgA ANCA in a series of patients with different causes of cutaneous vasculitis. Methods Forty consecutive patients with histologically proven leucocytoclastic vasculitis were included in the study: 18 had systemic vasculitis as well as cutaneous lesions, 10 of whom were diagnosed as having HSP, and 22 had only cutaneous vasculitis (with no identified cause in 10 cases). IgA ANCA were sought by indirect immunofluorescence using ethanol-fixed human neutrophil preparations as the substrate. Results IgA ANCA were detected in six of 40 patients (15%) (one each with HSP, ulcerative colitis, Sjogren's syndrome, hypergammaglobulinaemia associated with Castelman's disease, erythema elevatum diutinum and bacterial endocarditis). Three of these patients also had IgG ANCA whose target antigen remained unidentified. Conclusions IgA ANCA are rarely observed in HSP (10%) and can be detected in a wide variety of other cutaneous vasculitides.

Leucocytoclastic vasculitis (LV) is characterized by necrotizing inflammation around small blood vessels, composed mainly of neutrophils and their debris. The skin lesions of LV are polymorphous, but an annular variant is rare. Four previous reports have named this variant 'annular LV' (ALV) and in this paper, we report two additional cases. Patient 1 was a 80-year-old man, who presented with pruritic, erythematous target lesions on his legs, soles, abdomen and axillae; histological examination showed typical LV throughout the entire dermis. Oral prednisolone 15 mg daily rapidly resolved the patient's symptoms. Patient 2 was a 64-year-old man, who was referred to our hospital because of multiple purpuric target lesions on the buttocks, right thigh, lower legs, upper arms and forearms. Histopathology revealed LV throughout the entire dermis. Oral prednisolone 20 mg daily rapidly resolved the lesions. We categorized these cases morphologically as ALV and clinically as small-vessel vasculitis. ALV is not a distinct condition, but includes a broad range of small-vessel vasculitides.

Livedoid vasculitis, otherwise known as segmental hyalinizing vasculitis or livedo reticularis with summer ulceration, is a chronic disease with lesions affecting the feet and lower legs. Early lesions show petechiae, but characteristic features are recurrent, bizarrely shaped ulcers that heal to leave hyperpigmentation and atrophie blanche. The aetiology of the disorder is unknown, but the histology shows fibrin deposition within both the wall and lumen of affected vessels. The absence of a sufficient perivascular infiltrate or leucocytoclasia argues against a vasculitis, being more in keeping with a thrombo-occlusive process. Four patients with livedoid vasculitis with ulceration are described, all of whom had associated raised anticardiolipin antibodies but no other evidence of systemic disease. We suggest that livedoid vasculitis may be a manifestation of the antiphospholipid syndrome and recommend that all patients are screened for this. We also discuss treatment options for this often resistant condition.

The development of satellite lesions after removal of a pyogenic granuloma is well known. However, it is unusual to see this complication after the removal of a non-angiomatous lesion. We describe a patient who developed multiple small vascular lesions after excision of a dermal melanocytic naevus on her back; new lesions continued to appear over the next 20 years. Little is known about the pathogenesis of eruptive lesions developing after excision of a primary tumour. Several hypotheses, such as the influence of mechanical forces, abnormalities in the vascular system and the existence of a circulating stimulatory factor, have been suggested.

Pustular vasculitis of the hands is a distinctive clinical entity. It has recently been proposed by some authors to rename pustular vasculitis of the hands as 'neutrophilic dermatosis of the hands' to reflect its histological resemblance to the neutrophilic dermatoses. We report the case of a 66-year-old woman who presented with clinical appearances resembling pustular vasculitis of the hands associated with palmo-plantar pustulosis due to Streptococcus-induced 'pustular vasculitis'.

Summary Amiodarone is a valuable agent, used in the management of intractable cardiac arrbythmias. Its widespread use persists despite a list of well recognized side-effects. Skin reactions are common, usually presenting as photosensitivity or less frequently as a blue/grey pigmentation on light-exposed sites. Leucocytoclastic vasculitis following treatment with amiodarone bas been reported rarely. We describe a further case and briefly review the unwanted cutaneous manifestations of amiodarone therapy.

A 76-year-old woman presented with an indurated and painful, ecchymotic plaque on her right buttock 48 h after an intramuscular injection of ketorolac tromethamine for coxarthrosis (Figure 1). She stated that injection had been extremely painful, with initial blanching at the injection site. At presentation, blood results, including creatinine kinase, were unremarkable. Superficial ultrasonography showed diffuse oedema, with sparing of the muscle and no fluid collections. Bacteriological …

A 30-year-old woman with antiphospholipid syndrome (APS) was admitted for arthralgia, skin ulceration (Figure 1) and auricular chondritis, 1 week after an intrauterine fetal death at 25th week of gestation. Physical examination showed …

Answers on p 210. A 73 year old man was admitted with progressive dyspnoea. His past medical history included peripheral artery disease (intermittent claudication of lower extremities) and ischaemic heart disease (angina pectoris). Two months before …