A case of late-stage lymphogranuloma venereum in a woman in Europe.

Lymphogranuloma venereum is caused by Chlamydia trachomatis serovars L1-L3. The third stage of the disease leads to chronic progressive lymphangitis. The first European case of elephantiasic enlargement of the female genitalia caused by C. trachomatis L2 serovar in decades is reported.

Conidiobolomycosis in a young Malaysian woman showing chronic localized fibrosing leukocytoclastic vasculitis: a case report and meta-analysis focusing on clinicopathologic and therapeutic correlations with outcome.

BACKGROUND: Conidiobolomycosis (also known as rhinoentomophthoramycosis) is a rare cutaneous/mucosal fungal infection seen mainly in the tropical rain forest regions of the world that can be associated with disfiguring facial elephantiasis, and rarely, death. OBJECTIVE: To present an exemplary case report and perform a systematic review of the world's literature to more accurately describe the natural history and the effect of therapy on outcome in conidiobolomycosis. METHODS: Case report and meta-analysis of published case reports and series of conidiobolomycosis to determine which clinical, pathologic, mycologic, and treatment factors impact on prognosis. RESULTS: We document delay in diagnosis of conidiobolomycosis in a young Malaysian woman, whose biopsy showed pathognomonic features-massive tissue eosinophilia and Splendore-Hoeppli phenomenon surrounding broad hyphae. These findings coexisted with granuloma faciale-like changes (fibrosing leukocytoclastic vasculitis) and lymphedema. Treatment with multiple antifungals was followed by complete resolution. For the meta-analysis, pooled data from 199 cases (162 with full outcome data) from 120 reports revealed a similar course for most cases: a disease affecting healthy young adults who present with progressive nasal symptoms (eg, nasal obstruction) and central facial swelling and show improvement or cure after surgical excision and/or treatment with one or more antifungal agents in 83%. Persistent-progressive facial disease occurred in 11%, and 6% died rapidly of fungal infection. Presentation with facial elephantiasis correlated with persistent-progressive rhinoentomophthoramycosis and a longer duration of disease before diagnosis (P = 0.02). Lethal infections were significantly associated with nonstereotypical presentation (eg, orbital cellulitis), visceral infection, absence of the Splendore-Hoeppli phenomenon, presence of comorbidities (eg, immunosuppression, hematolymphoid malignancy), infection with Conidiobolus incongruus or Conidiobolus lamprauges (not Conidiobolus coronatus), lack of response to amphotericin B, and female sex (all P ≤ 0.002). The few sensitivity studies performed demonstrated in vitro multidrug resistance of Conidiobolus species to most available antifungal agents. LIMITATIONS: Publication bias, reporting heterogeneity, and data deficits may affect results. CONCLUSIONS: Conidiobolomycosis should be included in the differential diagnosis of patients who present with nasal symptoms and painless centrofacial swelling. Massive tissue eosinophilia and Splendore-Hoeppli material coating thin-walled hyphae confirms the clinical diagnosis. The granuloma faciale-like histology found in this case can explain the onset of facial lymphedema by fibroinflammatory destruction of lymphatic vessels; the duration of disease and severity of inflammation likely predicts whether the lymphedema is reversible or not. Although rhinoentomophthoramycosis ostensibly responds in vivo to most available antifungal agents, routine culture and susceptibility testing is recommended to better define the efficacy of these therapeutic agents.

A case of elephantiasis nostras verrucosa treated by acitretin.

Elephantiasis nostras verrucosa is a rare disorder characterized by dermal fibrosis, hyperkeratotic, verrucous, and papillomatous lesions that result from both chronic filarial and nonfilarial lymphedema. Various treatment options have been reported for this disease. We present a 64-year-old man with erythrodermic psoriasis and elephantiasis nostras verrucosa in whom the lesions were resolved almost completely after acitretin treatment.

Surgical debulking of podoconiosis nodules and its impact on quality of life in Ethiopia.

BACKGROUND: In Ethiopia, severe lymphedema and acute dermato-lymphangio-adenitis (ADLA) of the legs as a consequence of podoconiosis affects approximately 1.5 million people. In some this condition may lead to woody-hard fibrotic nodules, which are resistant to conventional treatment. We present a series of patients who underwent surgical nodulectomy in a resource-limited setting and their outcome. METHODS: In two teaching hospitals, we offered surgical nodulectomies under local anaesthesia to patients with persisting significant fibrotic nodules due to podoconiosis. Excisions after nodulectomy were left to heal by secondary intention with compression bandaging. As outcome, we recorded time to re-epithelialization after surgery, change in number of ADLA episodes, change in quality of life measured with the Dermatology Quality of Live Index (DQLI) questionnaire, and recurrence rate one year after surgery. RESULTS: 37nodulectomy operations were performed on 21 patients. All wounds re-reepithelialised within 21 days (range 17-42). 4 patients developed clinically relevant wound infections. The DLQI values were significantly better six months after surgery than before surgery (P<0.0001). Also the number of ADLA episodes per three months was significantly lower six months after surgery than before surgery (P<0.0001). CONCLUSION: Nodulectomy in podoconiosis patients leads to a significant improvement in the quality of life with no serious complications, and we recommend this to be a standard procedure in resource-poor settings.

Elephantiasis nostras verrucosa on the abdomen of a Turkish female patient caused by morbid obesity.

Elephantiasis Nostras Verrucosa is a rare disorder of an extremity or a body region, which is associated with chronic lymphedema. There are 7 reported cases of abdominal elephantiasis in the medical literature. Here we report a morbidly obese female patient with elephantiasis nostras verrucosa on the abdominal wall.

The design and development of a multicentric protocol to investigate the impact of adjunctive doxycycline on the management of peripheral lymphoedema caused by lymphatic filariasis and podoconiosis.

BACKGROUND: As new lymphatic filariasis infections are eliminated through mass chemotherapy, previously affected individuals are left with the sequellae, especially chronic progressive lymphoedema. Currently this is managed by careful attention to limb hygiene to prevent infection. Studies over the past 15 years have suggested that the incorporation of doxycycline treatment may arrest or even reverse progression of lymphoedema. Most of this work has been observational or based on small studies, and if this intervention is effective, studies need to be conducted on a larger scale and under diverse geographical and social conditions before it can be incorporated into treatment policy. METHODS/DESIGN: The double-blind, placebo-controlled study was designed to investigate the impact of six weeks treatment with doxycycline added to standard limb hygiene on early stage filarial lymphoedema in five sites in Africa and the Indian subcontinent. One site in Cameroon is selected for studying lymphoedema in podoconiosis. Each site was individually powered with the potential to undertake a meta-analysis on completion. Evaluation methods followed those used in Ghana in 2012 with additions resulting from advances in technology. The details of the core protocol and how it was varied to take account of differing situations at each of the sites are provided. The study will enrol up to 1800 patients and will complete in mid-2021. CONCLUSIONS: This paper provides details of what challenges were faced during its development and discusses the issues and how they were resolved. In particular, the reasons for inclusion of new technology and the problems encountered with the supply of drugs for the studies are described in detail. By making these details available, it is hoped that the study protocol will help others interested in improving treatment for filarial lymphoedema in the design of future studies. Trial registration India: Clintrials.gov. NCT02929121 registered 10 Oct 2016: https://clinicaltrials.gov/ct2/show/NCT02929121 Mali: Clintrials.gov. NCT02927496 registered 7 Oct 2016: https://clinicaltrials.gov/ct2/show/NCT0292749 Sri Lanka: Clintrials.gov. NCT02929134 registered 10 Oct 2016: https://clinicaltrials.gov/ct2/show/NCT02929134 Ghana: ISRCTN. 14042737 registered 10 July 2017: https://doi.org/10.1186/ISRCTN14042737 Tanzania: ISRCTN. 65756724 registered 21 July 2017: https://doi.org/10.1186/ISRCTN65756724 Cameroon: ISRCTN. 1181662 registered 25 July 2017: https://doi.org/10.1186/ISRCTN11881662.

Bilateral nonpitting edema and xerotic skin.

When treatment for cellulitis and a suspected exacerbation of congestive heart failure failed, we ordered a biopsy. The pathology report made the diagnosis clear.

[Elephantiasic pretibial myxoedema: study of five cases]. / Myxoedème prétibial eléphantiasique: une etude de cinq cas.

INTRODUCTION: Elephantiasic myxoedema is very rare. We report 5 pretibial myxoedema cases observed in the Dermatological department of Le Dantec hospital in Dakar. PATIENTS AND METHODS: Our 5 cases were 3 women (age: 52, 45 and 18 years) and 2 men (age: 54 and 32 years). RESULTS: All the cases were enormous, multinodular pachydermic, bilateral, elephantiasis in the low leg and associated to Graves 'disease. The duration was 4 month to 12 year. In 3 cases there was a Diamond syndrome which is a severe form. The cutaneous lesions were not influenced by medical treatment of thyroid disease. Systemic steroids achieved improvement of the elephantiasis in 1 case. CONCLUSION: Elephantiasis is a very unusual presentation of pretibial myxoedema and its occurrence doesn't depend to the intensity of thyrotoxicosis and its evolution. However in our cases, it was a severity indicator factor, like Diamond syndrome. Local and systemic steroids give very rarely improvement.

Preparing for and Executing a Randomised Controlled Trial of Podoconiosis Treatment in Northern Ethiopia: The Utility of Rapid Ethical Assessment.

BACKGROUND: Community-based randomized controlled trials are often complex pieces of research with significant challenges around the approach to the community, information provision, and decision-making, all of which are fundamental to the informed consent process. We conducted a rapid ethical assessment to guide the preparation for and conduct of a randomized controlled trial of podoconiosis treatment in northern Ethiopia. METHODS: A qualitative study was carried out in Aneded woreda, East Gojjam Zone, Amhara Regional State from August to September, 2013. A total of 14 In-depth Interviews (IDIs) with researchers, experts, and leaders, and 8 Focus Group Discussions (FGDs) involving 80 participants (people of both gender, with and without podoconiosis), were conducted. Interviews were carried out in Amharic. Data analysis was started alongside collection. Final data analysis used a thematic approach based on themes identified a priori and those that emerged during the analysis. RESULTS: Respondents made a range of specific suggestions, including that sensitisation meetings were called by woreda or kebele leaders or the police; that Health Extension Workers were asked to accompany the research team to patients' houses; that detailed trial information was explained by someone with deep local knowledge; that analogies from agriculture and local social organisations be used to explain randomisation; that participants in the 'delayed' intervention arm be given small incentives to continue in the trial; and that key community members be asked to quell rumours arising in the course of the trial. CONCLUSION: Many of these recommendations were incorporated into the preparatory phases of the trial, or were used during the course of the trial itself. This demonstrates the utility of rapid ethical assessment preceding a complex piece of research in a relatively research-naive setting.

Long-term follow-up and epidemiological trends in patients with pretibial myxedema: an 11-year study from a tertiary care center in northern India.

INTRODUCTION: Pretibial myxedema (PTM) is a rare manifestation of Graves' disease. There is paucity of data regarding long-term follow-up and response to treatment in PTM. MATERIALS AND METHODS: Retrospective study wherein 30 patients of PTM presenting during 2001-2011 attending dermatology and endocrinology outpatient departments were analyzed. RESULTS: Among 30 patients with PTM, 12 were males and 18 females with a ratio of 1 : 1.5 males/females. Four morphological forms were identified: plaques (18 patients), diffuse non-pitting edema of both lower legs (five), nodules (five), and elephantiasis lesions (two). Eighty percent were diagnosed with hyperthyroidism before the development of dermopathy. Twenty-six patients presented with ophthalmopathy. Fourteen patients with plaque had an excellent response to topical clobetasol propionate ointment and attained complete resolution by 3.6 years. Out of 16 patients treated with combination therapy, which included nine treated with topical corticosteroids/intralesional triamcinolone and seven treated with oral, intralesional, and topical corticosteroids, nine attained complete resolution in the lesions by 3.4 years, and none relapsed anytime during four years of post-treatment follow-up. However, the remaining patients (elephantiasis and diffuse forms) failed to achieve complete resolution. CONCLUSIONS: Plaques and nodules are common variants with a favorable clinical response to topical and intralesional corticosteroid; elephantine and diffuse forms responded poorly to therapy. Studies analyzing larger cohorts of patients with PTM and their long-term follow-up are limited, hence more such studies are required.

Towards a filariasis-free community: evaluation of filariasis control over an eleven year period in Flores, Indonesia.

A population of 202 residents in an area endemic for Brugia timori lymphatic filariasis was treated in a diethylcarbamazine control programme commencing in 1977. All individuals were treated twice with diethylcarbamazine on a mass basis with additional selected treatment for cases with manifestations of infection. Clinical features of lymphatic filariasis were recorded annually until 1982, and the population re-assessed in 1988, six years after the completion of chemotherapy. Microfilarial counts were made on each occasion, and circulating filarial antigen levels measured for 1982 and 1988. The results showed a dramatic and sustained reduction in the rate of elephantiasis and adenolymphangitic disease, and of circulating antigenaemia, and the prevalence of microfilaraemia was reduced to zero by the end of the study.

The long term effects of repeated diethylcarbamazine administration with special reference to microfilaraemia and elephantiasis.

The result of mass treatment with 50 mg diethylcarbamazine (DEC) per kg body weight followed by two annual selective retreatments in an area highly endemic for Brugia timori infections are described. The criteria for selective re-treatment are simple and practical for use in rural areas. An education programme was developed which focused on describing the relationship between adenolymphangitis and filarial infections, the danger of repeated attacks and the efficiency of DEC in eliminating these attacks. Motivated persons in the community were charged with the responsibility of promptly treating all cases with acute clinical manifestations. With this programme the microfilaria rate by finger prick decreased from 24% to 0%, and by Nuclepore filtration from 30% to 5%. The adenolymphangitis rate decreased from 46% to 11% and the "elephantiasis' rate from 17% to 4%.

Elephantiasis nostrum verrucosa of the abdomen: clinical results with tazarotene.

Elephantiasis nostras verrucosa represents an uncommon yet distinct clinical entity resulting from chronic lymphedema of an extremity or body region. Characterized by profound non-pitting edema with cobblestone-like papules, plaques, and nodules, it typically occurs secondary to infections, surgeries, tumor obstruction, radiation, congestive heart failure, and obesity. This progressively deformative disorder has been treated with various medical and surgical measures. In the following case report, the history, clinical, and pathologic appearance of elephantiasis nostras verrucosa are discussed, as well as newer treatment options.