Tuberculosis verrucosa cutis presenting with unilateral elephantiasis nostras verrucosa of the left lower limb: A rare entity.

Elephantiasis nostras verrucosa is used to describe lymphedema of the lower limbs caused by a nonfilarial etiology. Infective etiologies include bacterial and fungal infections along with chronic venous stasis, postirradiation, congestive heart failure, obesity, and malignancies, all causes leading to lymphatic obstruction and edema. We herein describe a case of a 70-year-old male who presented with diffuse nonpitting edema of the entire left lower limb with cobblestoning of the surface with a positive Kaposi-Stemmer sign and presence of a verrucous plaque over the left ankle. Histopathology of the verrucous plaque revealed it to be tuberculosis verrucosa cutis (TVC). We report this rare presentation of TVC as a cause of unilateral lymphedema.

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.

Elephantiasis Nostras Verrucosa (ENV): a complication of congestive heart failure and obesity.

Congestive heart failure (CHF) and obesity are common medical conditions that have many complications and an increasing incidence in the United States. Presented here is a case of a disfiguring skin condition that visually highlights the dermatologic consequences of poorly controlled CHF and obesity. This condition will probably become more common as CHF and obesity increase in the US.

[A text-book case of tropical facial elephantiasis]. / Un cas historique d'éléphantiasis facial tropical.

INTRODUCTION: Tropical facial elephantiasis is a nosological entity which can arise from various underlying causes: von Recklinghausen neurofibromatosis, lymphatic and cutaneodermal filarioses, deep mycosis. CASE REPORT: We report an exceptional case of tropical facial elephantiasis caused by onchocercosis and entomophtoromycosis (rhinophycomycosis). The patient's facial morphology was noted "hippopotamus-face" or "dog-face". DISCUSSION: Onchocercosis and entomophtoromycosis are two diseases known to cause facial elephantiasis. We have not however been able to find any case report in the literature of co-morbidity nor any information on factors predictive of concomitant occurrence.

Genital elephantiasis and sexually transmitted infections - revisited.

Genital elephantiasis is an important medical problem in the tropics. It usually affects young and productive age group, and is associated with physical disability and extreme mental anguish. The majority of cases are due to filariasis; however, a small but significant proportion of patients develop genital elephantiasis due to bacterial sexually transmitted infections (STIs), mainly lymphogranuloma venereum (LGV) and donovanosis. STI-related genital elephantiasis should be differentiated from elephantiasis due to other causes, including filariasis, tuberculosis, haematological malignancies, iatrogenic, or dermatological diseases. Laboratory investigations like microscopy of tissue smear and nucleic acid amplification test for donovanosis, and serology and polymerase chain reaction for LGV may help in the diagnosis, but in endemic areas, in the absence of laboratory facilities, diagnosis largely depends on clinical characteristics. The causative agent of LGV, Chlamydia trachomatis serovar L1-L3, is a lymphotropic organism which leads to the development of thrombolymphangitis and perilymphangitis, and lymphadenitis. Long-standing oedema, fibrosis and lymphogranulomatous infiltration result in the final picture of elephantiasis. Elephantiasis in donovanosis is mainly due to constriction of the lymphatics which are trapped in the chronic granulomatous inflammatory response generated by the causative agent, Calymmatobacterium (Klebsiella) granulomatis. The LGV-associated genital elephantiasis should be treated with a prolonged course of doxycycline given orally, while donovanosis should be treated with azithromycin or trimethoprim-sulphamethoxazole combination given for a minimum of three weeks. Genital elephantiasis is not completely reversible with medical therapy alone and often needs to be reduced surgically.

Genital elephantiasis.

Genital elephantiasis (esthiomene), which is the dramatic end-result of lymphatic obstruction, is rather rare. Although mainly associated with filariasis and sexually transmitted diseases, such as lymphogranuloma venereum and donovanosis, it could also be an uncommon complication of tubercular lymphadenitis, a common infection in tropical countries. We report a rare case of a 32-year-old Indian female in whom genital elephantiasis occurred as a complication of tubercular lymphadenitis.