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Pyoderma gangrenosum (PG) is a rare inflammatory condition with an immense disease burden that remains understudied. With limited approved treatments and low-quality clinical evidence, PG continues to have poor patient outcomes. Unfortunately, improvement in PG treatments and patient care is based on additional research endeavors that can only be developed from existing high-quality data. The following protocol outlines the development of the Minimum Data Set for Treatment Effectiveness in Pyoderma gangrenosum (MIDSTEP), a core set of domains and domain items for the Pyoderma Gangrenosum Treatment Effectiveness (PyGaTE) international registry. The outcomes and benefits are focused on providing real-world data for physicians to improve their clinical decisions on PG treatment and inform clinical trial design, promoting clinical research among the international scientific community. MIDSTEP is a multi-phase project. The first phase will produce a domain item list from a literature review to take into the second phase which would finalize the core data set by an e-Delphi exercise. There will be a single stakeholder group participating together in the e-Delphi consisting of PG experts (healthcare providers, researchers, methodologists, industry representatives, and regulators), ulcerative PG patients, and PG patient advocates. The methodology outlined in the protocol is a systematic method based on several guidelines through COMET and established dermatologic registries and outcome sets with systematic methodologies of their own. The third phase will identify the instruments for the items, the 'when to measure' the items, and the platform for the registry. The last phase is the implementation and continued maintenance of the international registry PyGaTE. By solidifying a consensus on standardized outcomes and collecting information on PG treatment effectiveness in a centralized database, existing treatments can be compared more systematically and analyzed with increased evidence. MIDSTEP and the PyGaTE international registry will have the ambitious goal to generate and disseminate real-world data that can be used by all stakeholders to improve health outcomes for PG patients. Future potential for the outcome of this project includes the development of a gold-standard PG treatment. [ABSTRACT FROM AUTHOR]

Background: Intravenous immunoglobulins (IVIG) have been increasingly used for various inflammatory dermatoses with success. Small case series and case reports suggest a role for IVIG in the management of refractory pyoderma gangrenosum (PG). Objective: The objective was to study the characteristics of PG patients treated with IVIG and the efficacy and safety of IVIG for patients with refractory PG. Methods: An analysis was performed of all patients with PG treated with IVIG from 2012 to 2022 at an Australian tertiary hospital seeing a high volume of PG patients. Results: We identified 12 patients, 9 females and 3 males, with median age of 61 years (29–77) at IVIG commencement. All patients were taking systemic corticosteroid therapy prior to IVIG treatment, and all had been treated with a steroid‐sparing agent—including ten patients who had been treated with a biologic agent. IVIG was used with corticosteroids in one patient, concurrently with a steroid‐sparing agent in nine patients and with a biologic agent in eight patients. Eleven patients demonstrated treatment response to IVIG—six with excellent response and five with good response. Three patients had complete healing of their most active ulcer. One patient did not respond to IVIG. Nine patients were able to wean their prednisolone dose and one patient was able to cease prednisolone. Four adverse events were recorded, and only one patient had to cease treatment due to aseptic meningitis and headaches. Conclusion: Our experience suggests that IVIG may be an efficacious treatment for patients with refractory PG due to its pleiotropic and immunomodulatory effects, particularly for patients with malignancy or other systemic conditions where high‐dose immunosuppressive agents are contraindicated. [ABSTRACT FROM AUTHOR]

Lymphocytic thrombophilic arteritis and Sneddon syndrome can have very similar clinical presentations with chronic persistent widespread blanchable livedo racemosa. Lymphocytic thrombophilic arteritis has only recently been described and generally is associated with a benign prognosis. Sneddon syndrome is associated with the development of multiple cerebrovascular accidents and progressive neurological impairment. We present three cases of Sneddon syndrome and compare them with lymphocytic thrombophilic arteritis to identify patients at risk of neurological events. [ABSTRACT FROM AUTHOR]

Oral corticosteroid therapy was used as the initial therapy in all but a few patients where topical or intralesional therapy was employed. Dear Editor, We present one of the largest case series of pyoderma gangrenosum (PG) consisting of 118 patients managed at a dedicated tertiary hospital dermatology ulcer clinic (Tables 1-3). [Extracted from the article]

Thus, no cases of true cribriform or wrinkled paper scarring were seen in any of the 62 scars with 59 of them ultimately healing with smooth and even scars. Reported examples of cribriform scarring and wrinkled paper scarring appear to be examples of incomplete epithelialisation or residual scaliness rather than true scarring.2,5 Our cases suggest that these changes resolve with continued healing or maturation of the scar. [Extracted from the article]

Abstract: We describe three patients who presented with a striking erythematous non‐blanching annular eruption and features of lymphocytic thrombophilic arteritis (LTA), with a prominent lymphocytic vasculitis involving deep dermal vessels. Lymphocytic inflammation was also evident in the superficial vessels and one patient had small superficial ulcers over the ankle area resembling livedoid vasculopathy (LV). Multiple biopsies demonstrated a persistent absence of neutrophils in the infiltrate consistent with a lymphocytic process. In addition to highlighting the annular morphology as a novel presentation of LTA, these cases suggest a possible relationship between LV and LTA and support the notion that they are distinct from neutrophilic vasculitides such as cutaneous polyarteritis nodosa. [ABSTRACT FROM AUTHOR]

Nodular vasculitis (NV) refers to a chronic relapsing lobular panniculitis that is thought to be a hypersensitivity reaction to antigenic triggers. While it is commonly associated with tuberculosis, in many cases no underlying cause is found and the condition is difficult to manage. Here, we report three patients with refractory idiopathic NV effectively treated with colchicine, leading to a significant improvement or complete resolution of their symptoms. Colchicine was successfully used as a steroid-sparing agent, and in one patient its cessation was associated with a flare of disease. [ABSTRACT FROM AUTHOR]

The article reports that Pyoderma gangrenosum (PG) is an inflammatory neutrophilic dermatosis usually characterised by tender papules/pustules that evolve into painful and rapidly expanding ulcers. Rarely, PG can have extracutaneous involvement in virtually any organ with case reports describing PG in the lungs, eyes, bones, spleen, kidney, brain, bowel, muscle and even heart.

Abstract: Lymphocytic thrombophilic arteritis (LTA) is a recently described entity defined by primary lymphocytic vasculitis; it typically has a chronic indolent course. We describe a patient who presented with clinical and histological findings consistent with LTA and later developed bilateral focal testicular infarcts as well as an acute median nerve neuropathy. [ABSTRACT FROM AUTHOR]

(b) Pathergy: Further attempts at surgical repair were complicated by wound breakdown resulting in extensive scarring and a larger deeper ulcer almost extending to the anus. gl Prednisolone 25 mg daily was not tolerated and was ceased after 4 weeks. Dear Editor An otherwise well 32-year-old woman presented with a 7-year history of a painless ulcer in the natal cleft measuring 4.5 cm in length and 1cm in depth (Fig. Factors that favour the diagnosis include a clear demonstration of pathergy and definitive response to immunosuppressive therapy after failure of persistent attempts at wound management and surgical repair. [Extracted from the article]

ABSTRACT A 35-year-old Lebanese woman presented with a 3-year history of persistent, localized livedo racemosa over her feet, distal legs and forearms that was associated with the development of lower limb sensorimotor neuropathy. Investigations revealed the patient was heterozygous for prothrombin gene mutation and was also found to have a T-cell receptor gamma chain gene rearrangement. Histological examination revealed a mid-lower dermal medium vessel lymphocytic vasculitis with prominent fibrinoid ring within its wall. These findings are consistent with a recently described condition known as lymphocytic thrombophilic arteritis. This has so far been considered to be a benign clinical condition not associated with extra cutaneous manifestations. The novel findings in the present case are the associated sensorimotor neuropathy and the characteristic fibrin ring appears to be intramural rather intraluminal in location. The findings of a T cell gene rearrangement and a prothombin gene mutation suggest that both immunological and thrombophilic factors might contribute to the development of this condition. [ABSTRACT FROM AUTHOR]

Human skin is a visible indicator of age. Structural and physiological changes that occur intrinsically with ageing skin combined with the effects of cumulative extrinsic damage can result in a marked susceptibility to cutaneous disorders in older patients. The progressive degeneration of ageing skin results in atrophy of the vasculature, deterioration of supporting dermis and erosion of normal immune function. Changes from the intrinsic and extrinsic effects of ageing skin have a wide clinical expression, e.g. xerosis, eczema, seborrheic dermatitis and contact dermatitis. Although rarely fatal, these cutaneous disorders can cause morbidity and reduce quality of life. Primary skin malignancies resulting from cumulative solar damage also carry considerable mortality. There have been exciting advances in the diagnosis and treatment of skin diseases in older people. However, comorbidities, polypharmacy and altered pharmacokinetics seen in older people pose significant challenges to effective treatment. Managing skin disorders in older people is often less than optimal because their special needs and limitations are not adequately addressed. This review provides an overview of treatments available for managing skin conditions commonly encountered in older people. [ABSTRACT FROM AUTHOR]

A retrospective analysis was conducted on 93 adult patients with cutaneous leukocytoclastic vasculitis from St. Vincent's Hospital Melbourne to determine the classification, aetiology, severity and prognosis of this population of patients. We developed a new classification system for the purposes of our study based on modifications to the Chapel Hill Consensus Conference definitions for vasculitic syndromes. The results of our study indicate that an obvious cause was not found in 44.1% of patients. Of the patients with secondary vasculitis, the commonest causes were drugs and infections, accounting for a total of 40.9% of patients. Extracutaneous involvement was found in 39.8% of patients. Patients with symptoms resolving in less than 3 months accounted for 59.1% of the population, whereas 24.8% of patients had either symptoms lasting three or more months or evidence of recurrent symptomatology. There were 6 deaths (6.91%) and the rest were lost to follow up. The majority of patients in this retrospective series were classified as having hypersensitivity vasculitis, which is a relatively benign disorder limited mostly to skin with a low incidence of extracutaneous involvement (15.8%). Nevertheless, evidence of systemic involvement or sepsis need to be excluded as this may have important implications for patient treatment and outcome. [ABSTRACT FROM AUTHOR]

A number of patients presented with an erythematous, purpuric rash occurring on the legs in association with playing golf and also after prolonged walks or hikes. Many patients believed that it was an allergic reaction to grasses or insecticides and had sometimes undergone extensive allergy testing. We collected reports of 17 such cases from dermatologists in the state of Victoria, Australia. Patients were interviewed by phone and asked to submit photographs of the rash if possible. Of these, the eruption developed in 15 after playing 18 holes of golf and in three following prolonged hikes. The rash would usually develop over the summer months under hot conditions. Most patients were over 50 years of age when the tendency to develop the eruption began. Biopsies of the rash in the active phase showed leukocytoclastic vasculitis. Patch testing and investigations for potential underlying causes for vasculitis were negative or unremarkable. It would seem that this is a common but poorly documented condition. The clinical presentation and histology would support the conclusion that it represents a leukocytoclastic vasculitis induced by prolonged exercise under hot conditions. The findings would suggest that it occurs in healthy people and extensive investigation with blood tests or allergy testing is inappropriate. We believe the condition should be termed‘golfer's vasculitis', as golf appears to be the most common precipitating event and such a term would enable the condition to become more widely recognized. [ABSTRACT FROM AUTHOR]

Cutaneous involvement may occur with virtually all syndromes of vasculitis. This can occur primarily as a dermatologic disorder or as a manifestation of a potentially life threatening systemic vasculitis. In this review article, classification, clinical manifestations, pathogenesis and therapy of cutaneous vasculitis will be discussed. Disorders which are primarily vascular in origin but lack a well defined inflammatory phase, referred to as 'vasculopathies' wilt also be discussed. [ABSTRACT FROM AUTHOR]

Skin changes associated with long term hydroxyurea therapy have only rarely been reported and are not widely known. This report concerns a patient in whom extensive and debilitating skin changes developed secondary to hydroxyurea therapy. In addition to previously reported manifestations such as ichthyosis, a dermatomyositis-like eruption on the dorsal hands, facial erythema and accelerated development of premalignant and malignant skin lesions, the patient developed an erosive eruption on the glans penis. He also developed a rapidly enlarging viral wart which clinically resembled a squamous cell carcinoma and histologically showed marked cytological atypia. It is possible that hydroxyurea caused accelerated photoageing and also contributed to the rapid growth of the viral wart and the atypical histological features. The time course of the clinical changes suggests that they result from direct toxicity of hydroxyurea on the basal layer of the epidermis and mucosal surfaces. [ABSTRACT FROM AUTHOR]

Political deviance is a broad concept, less frequently invoked than the concept of political crime. It can be perpetrated by those in power, in the name of the state or individually, or by those struggling to effect social change. The focus on the latter brings to the fore two types of movements, terrorism and civil disobedience, with their partial similarities in goals and their apparent dissimilarities in the means employed to achieve these goals. Both groups tend to assume responsibility for their acts, although for different reasons. The assumption of responsibility itself is a clouded and sometimes ambiguous notion, both linguistically and in its ramifications in the political arena. [ABSTRACT FROM AUTHOR]

A 33-year-old man presented with a 3-month history of a widespread pigmented purpuric eruption over his trunk and limbs. The clinical presentation and histology were consistent with a diagnosis of Schamberg's disease. The rash initially cleared following a short course of oral prednisolone at 25 mg daily for 3 weeks, which was weaned over the subsequent 4 weeks. Topical mometasone furoate ointment 0.1% daily was also applied to active areas. The rash recurred when prednisolone was reduced to below 5 mg per day. To prevent a further recurrence with weaning prednisolone, narrowband UVB therapy was commenced three times per week. The patient was continued on UV therapy over the next 5 months. The rash would flare after 2 to 3 weeks without treatment. The frequency of UV therapy was weaned and the patient remained clear on as little as one treatment every 2 weeks. Any further reduction, however, was associated with a recurrence. Narrowband UVB therapy should be considered for difficult or persistent cases of pigmented purpuric eruption. [ABSTRACT FROM AUTHOR]