Primary cutaneous amyloidosis includes several forms of localized amyloidosis characterized by superficial amyloid deposits occurring at or near the dermal-epidermal junction in the absence of systemic involvement. Primary cutaneous amyloidosis of the auricular concha and external ear represents a rarely described variant. There have been 27 cases reported in the ...

We report a case of a 9-year-old boy who presented with 2 lesions that were compatible clinically with cutaneous leishmaniasis of the New World. A skin biopsy showed tuberculoid granulomas with rounded calcified bodies. The diagnosis was supported by a positive leishmanin test and a positive polymerase chain reaction. The ...

Leishmaniasis is an important tropical vector-borne disease. This infection can be seen in tropical area and it is considered to be one of the most important vector-borne infections at present. The general situation of the leishmaniasis in Thailand is hereby reviewed. Although Thailand is a tropical country, the leishmaniasis is ...

Cutaneous mercury (Hg) granuloma is a rare disorder caused by the traumatic introduction of elemental Hg into skin or soft tissue. Typically, cutaneous elemental Hg deposits cause limited systemic effects. Prominent systemic toxicity may, however, occasionally occur. Herein we report a case of cutaneous Hg granuloma resulting in chronic painful ...

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We present a case of a child with Leishmania infantum cutaneous leishmaniasis unresponsive to 2 courses of intravenous liposomal amphotericin B, a treatment failure that has not been reported in this Leishmania species. The patient responded to topical imiquimod and had no relapse. We review the literature on the treatment ...

Nevoid hypertrichosis is a rare entity characterized by circumscribed patches of terminal hair. It is associated with many cutaneous and extra-cutaneous abnormalities. In our case, a single circumscribed patch of terminal hair growth was present without any underlying or associated cutaneous or systemic abnormalities.

Since the early 1980s, visceral leishmaniasis (VL) which is, in general, a rural zoonotic disease, has spread to the urban centers of the north, and now the south and west of Brazil. The principal drivers differ between cities, though human migration, large urban canid populations (animal reservoir), and a decidedly ...

Penile reconstruction still represents a formidable challenge for the urologist. In this review, the most recent advances in penile reconstruction after trauma, excision of benign and malignant disease and in patients with micropenis, aphallia or female to male gender dysphoria are reported.

An epithelioid hemangioendothelioma arising from the right common femoral vein in a 50-year-old woman was treated by wide local excision and venous reconstruction with the ePTFE graft. This clinicopathologic entity is a rare cause of intravascular mass and is characterized by an intermediate malignant potential. To our knowledge, very few ...

OBJECTIVE: Scrotal lymphedema (SL) is a rare clinical pathology with multiple etiologies. We report a case of idiopathic giant scrotal lymphedema and review the existing medical literature in Medline from the last ten years. METHODS: We report the case of a male patient with a giant scrotal lymphedema (43×40 cm) ...

BACKGROUND: Auricular surgery is a challenging subject in plastic surgery due to the complicated surface topography of the external ear. Although various techniques for ear reduction and helical rim reconstruction have been reported in the literature, an ideal method is yet to be defined. Double helical rim advancement flaps with ...

Total extrusion of the talus with interruption of all ligaments (missing talus) is a rare injury. We describe the case of a 27-year-old man who reported total extrusion of the talus after a motorbike accident with interruption of all talar ligaments. In the first repair effort, the articular void left ...

Verruciform xanthoma (VX) is an uncommon benign inflammatory mucocutaneous condition that chiefly occurs in the oral cavity. It is often associated with pre-existing epithelial and/or inflammatory disorder and is characterized histopathologically by papillary epithelial hyperplasia and the presence of foamy macrophages in connective tissue papillae. We report of a series ...

A variety of the flaps used to reconstruct defects of the head and neck region following surgery for malignant disease contain hair follicles that may result in unwanted hair growth. This can cause significant distress to the patients in a variety of ways. We report 5 cases of significant intraoral ...

Adult-onset Still's disease is a multisystem inflammatory disorder of unknown etiology characterized by typical spiking fever, evanescent rash, arthralgia, and leucocytosis. Neurologic manifestations are infrequent, seen in 7 to 12% of cases. We present the case of a young male admitted with aseptic meningitis that satisfied the diagnostic criteria of ...

Using the microsurgical technique for reconstruction in trauma cases represents a challenge for the reconstructive surgeon. Several methods of salvaging of a compromised free flap have been reported, among them: intravenous heparin washing, thrombolitic therapy, thrombectomy, use of grafts and others. Here, we present our experience from nine cases and ...

Complications of pedal wounds closed primarily after release of neglected cases of club foot are well known in literature. We treated a 12-year-old boy presenting with widespread necrosis involving medial aspect of foot after release of neglected talipes equinovarus. Such widespread necrosis is even known to end up in amputations ...

The aim of this critical review is to determine what constitutes an ideal disc replacement material and whether any of the existing materials reported in the literature satisfy the requirements of an effective disc substitute following temporomandibular joint (TMJ) discectomy. Over the last half century a myriad of interpositional materials ...

Although basal cell carcinoma (BCC) is one of the most common forms of cancer worldwide, it rarely occurs in the axilla. Only 31 cases have been reported in the literature. The incidence of metastatic BCC, particularly in areas not exposed to the sun, is very low. We present a new ...

Kidney disease is a rare complication of Campylobacter jejuni (C. jejuni) enteritis. We here present the case of an 18-year-old male patient with crampy abdominal pain, vomiting, diarrhea, and fever. Three weeks later urinalysis revealed mild proteinuria and hematuria and a marked raise in serum creatinine was observed. Renal biopsy ...

Transfusion-associated graft-versus-host disease (TA-GVHD) is a rare complication of transfusion of nonirradiated blood components. It usually affects children in high-risk groups, including those who have primary immunodeficiencies (PIDs). It usually presents with skin, hepatic, digestive, and hematologic involvement and is normally fatal. We report the case of a nonlethal, attenuated, ...

Hirschsprung's disease is characterised by the congenital absence of ganglion cells beginning in the distal rectum and extending proximally for varying distances. 'Zonal aganglionosis' is a phenomenon involving a zone of aganglionosis occurring within normally innervated intestine. 'Skip segment' Hirschsprung's disease (SSHD) involves a 'skip area' of normally ganglionated intestine, ...

Von Hippel-Lindau disease (VHL) comprises a series of complicated clinical manifestations. We hereby report one unique case of VHL with a natural history that mimics acute myelitis. MRI and biopsy in this patient showed multiple solid hemangioblastomas of the central nervous system and kidney. This study further confirmed that VHL ...

Extramammary Paget's disease that occurs in non-apocrine-bearing regions is referred to as ectopic and has been rarely reported. A 62-year-old man presented with a slowly progressive, asymptomatic light-brown plaque on his back. Histopathological examination revealed the presence of large pale cells with prominent nuclei, which proliferated diffusely and focally in ...

Several studies have reported an association between idiopathic intracranial hypertension (IIH) and deficits of the hypothalamic-pituitary-adrenal (HPA) axis. CASE ILLUSTRATION: A 33-yr-old woman with Cushing's disease underwent successful surgical resection of a pituitary adenoma and developed IIH 11 months later after inadvertent withdrawal of oral glucocorticoids. A review of the ...

Adult-onset Still's disease (AOSD) is an uncommon systemic inflammatory disease of unknown aetiology. Up to 80% of AOSD cases can be controlled with corticosteroids; however, reports on those unresponsive to corticosteroids, conventional disease modifying drugs and biological agents, including anti-IL1 inhibitors, are emerging. We present a case of AOSD with ...

BACKGROUND: Lipoatrophic panniculitis (LP) is a rare disease of childhood characterized by eruption of tender erythematous nodules and plaques followed by circumferential bands of lipoatrophy often seen on the arms and legs. This condition has also been known as lipophagic panniculitis of childhood, annular atrophy of the ankles, and partial ...

The strong genetic component in the etiology of Paget's disease of bone (PDB), together with marked geographic variation in its prevalence, with high frequencies in British populations, has led some to suggest that the disease originated in Britain and spread around the world in recent times by the migration and ...

Invasive dematiaceous fungal sinusitis is an uncommon and aggressive disease in immunocompromised individuals. We report a unique case of invasive Exserohilum sinusitis in a pregnant, immunocompromised woman. After treating the woman with pregnancy-induced aplastic anemia and invasive Exserohilum sinusitis and pulmonary disease, we performed a Medline/PubMed review of invasive dematiaceous ...

Rhinofacial entomophthoramycosis is an uncommon chronic mycotic disease caused by exposure to the organism Conidiobolus coronatus. The authors report a case series of 5 patients with rhinofacial entomophthoramycosis and review the literature. All patients had typical involvement of the rhinofacial area with formation of subcutaneous lesions causing a chronic granulomatous ...

Temozolomide chemotherapy has become part of the therapy used to treat glioblastoma multiforme and refractory anaplastic astrocytoma. Temozolomide frequently produces profound lymphopenia. We report 2 cases of cytomegalovirus disease that occurred in patients receiving temozolomide therapy and review 4 additional cases reported in the literature. Narrow monitoring with cytomegalovirus antigenemia ...

Calcium pyrophosphate dihydrate deposition disease (CPPD), also known as pseudogout or chondrocalcinosis, is a variety of metabolic arthropathy caused by the deposition of calcium pyrophosphate dihydrate crystals in and around joints. Despite many case reports, extra-articular CPPD often goes unrecognized. Here, we report a unique case of pancreatic tail pseudocyst ...

Hepatic encephalopathy is a syndrome of neuropsychiatric dysfunction caused by portosystemic venous shunting with or without the presence of intrinsic liver disease. Clinical presentations are variable ranging from an abnormal sleep pattern to somnolence and deep coma. Decerebrate and decorticate posturing, have been rarely reported with hepatic encephalopathy. We report ...

Abstract The aim of this study was to call the attention to the often disregarded message that hypertransaminasemia may be a marker of both liver and muscle diseases by presenting personal case reports and a systematic literature review. Three male children (mean age 5.7 years) were inappropriately addressed, during the ...

The complication of Menkes disease (MD) and gastroesophageal reflux disease (GERD) is extremely rare. This report describes the very rare case of a one-year-old boy with MD complicated with GERD, and the successful surgical treatment of GERD. We review the literature on this relationship between MD and GERD, and discuss ...

A case of adult-onset Still's disease complicated by hemophagocytic lymphohistiocytosis is reported. Its management is being discussed on the background of the latest literature with special regard on the use of high-dose corticosteroids and immunosuppressive agents.

BACKGROUND: Gouty panniculitis is a very rare disease. To date, only eight patients have been reported. In each case, time to diagnosis was lengthy. We describe a 68-year-old man with a 6-month history of tender, erythematous plaques and nodules involving the anterior aspects of the legs. METHODS AND RESULTS: Histologic ...

The coexistence of Behçet's disease (BD) and ankylosing spondylitis (AS) is a rare combination and described only in case reports in the literature. Although the number of reports is increasing, vascular thrombosis, which is characteristic feature of BD, was not reported in this combination until now. We present a case ...

We report the clinicopathologic features of 4 cases of pure pleomorphic rhabdomyosarcoma of the uterine corpus with an emphasis on their frequent expression of CD10 and CD56, review the relevant literature, and discuss differential diagnostic considerations. The patients ranged from 51 to 79 years (mean 68 y). All were FIGO ...

We report a case of multicentric reticulohistiocytosis (MRH) demonstrating classic clinical and histologic findings. This rare idiopathic disease is manifested by severe destructive polyarthritis and papulonodular mucocutaneous lesions. The characteristic clinical and histologic findings are discussed as well as the workup and treatment of the disease.

A 74 year old male patient with weight loss, diarrohea, loss of appetite, anemia, thrombocytopenia and culture negative endocarditis was diagnosed to have Whipple's disease. We are reporting this case, as it is a rare disease with fewer than 1000 validated cases reported in literature.

Aseptic metacarpal head necrosis or 'Dieterich disease' is a rare condition, especially in children or adolescents, not routinely described in hand surgery literature reviews. This case report presents course and treatment of a teenage boy with the exceptional fifth ray involved.

A case of Gorham's disease in the maxilla of a 56-year-old male patient is described. The clinical presentation, radiographic and histopathological features and treatment are presented. A discussion of the current understanding of this rare disease, based on review of the literature, is offered.

The management of metastatic spinal disease is generally considered palliative, as the progression of systemic disease is likely to hinder survival. Although the occurrence of C1-C2 instability due to metastatic disease is not uncommon and thus treatment options have been well-defined, craniocervical instability due to lesions occurring at the junction ...

A case of alopecia areata followed by relapsing polychondritis is presented. Similar cases from the literature are reviewed and speculation about the relationship of these diseases is offered. Although the occurrence of these diseases together could be coincidental, an association seems immunologically plausible. Thus, relapsing polychondritis might be an unusual ...

OPINION STATEMENT: Melanoma-associated retinopathy is a rare paraneoplastic disorder that is challenging to diagnose and even more difficult to treat. Because of the rarity of the disease, therapy is based on analysis of case series and case reports. Based on evidence from these reports, first-line therapy is cytoreduction of metastatic ...

Numbness of the lower lip, the skin of the chin, or the gingiva of the lower anterior teeth secondary to various dental diseases is a common manifestation that is largely underappreciated. The association of numb chin syndrome (NCS) with serious disease like metastatic malignancy and systemic conditions is frequent enough ...

Cutaneous leishmaniasis is a disease endemic to Central and South America, Mexico and the Caribbean, and affects millions of people. As travel to these regions becomes more common, cutaneous leishmaniasis is becoming a disease of increasing importance in the developed world. However, disease recognition and access to appropriate therapy for ...

Disseminated Superficial Actinic Porokeratosis (DSAP) is usually triggered by sun exposure. In some cases sun exposure is not essential and this skin disease is related to immunosuppression. Many associated diseases are described in the literature. We report a clinical case of a patient affected by pyoderma gangrenosum, who developed DSAP.