Management of pemphigoid vegetans with mycophenolate mofetil: A case report.

Pemphigoid vegetans is a rare variant of bullous pemphigoid. Only 13 cases have been documented in the medical literature. The lesions clinically resemble pemphigus vegetans, but the histology is consistent with bullous pemphigoid. We present the case of a 63-year-old woman with painful vesicular and vegetative lesions progressing for 4 months. Histopathology showed a dermal-epidermal cleavage, epidermal acanthosis and an eosinophilic infiltrate. Direct immunofluorescence showed linear deposition of IgG and IgA at the junctional level. The diagnosis of pemphigoid vegetans was retained and successfully treated with oral corticosteroid, dapsone and mycophenolate mofetil.

Disseminated juvenile xanthogranulomas with ocular involvement: A case report and literature review.

Cutaneous juvenile xanthogranuloma is an uncommon disorder usually arising during infancy. Systemic involvement of juvenile xanthogranuloma remains rare, and there are no published guidelines to date on screening extracutaneous manifestations in these patients. Ocular involvement is the most common extracutaneous manifestation of juvenile xanthogranuloma. We present the case of an infant with disseminated juvenile xanthogranulomas and associated ocular involvement and present a review of literature, focusing on identifying risk factors for ocular and systemic involvement in disseminated cases.

Localized ulcerative nodular amyloidosis presenting as ulcerative panniculitis, an unusual clinical manifestation: A case report.

INTRODUCTION AND OBJECTIVES: The main objective of this article is to present and discuss a case of localized ulcerative nodular amyloidosis with deep involvement clinically manifesting as ulcerative panniculitis and discuss its impact on the choice of treatment. METHODS AND RESULTS: We present a 73-year-old woman with a history of painful ulcerated nodules on the inferior limbs. Microscopy confirmed amyloid deposits deep in the dermis and subcutaneous fat. No systemic involvement was found. Considering that skin-directed treatments often are not able to reach subcutaneous fat or were contraindicated because of the ulcers, she was successfully treated with cyclophosphamide and prednisone. CONCLUSION: Localized ulcerative nodular amyloidosis with deep involvement is a rare clinical presentation that can present as ulcerative panniculitis. Such a clinical manifestation might be misleading. Systemic treatment might be necessary to control symptoms when conventional skin-directed therapies are contraindicated.

Calciphylaxis in a Patient With Non-Hodgkin Lymphoma: Case Report and Literature Review.

BACKGROUND: Calciphylaxis is a well-known entity in end-stage kidney disease and after renal transplant. Cases of nonuremic calciphylaxis (NUC) have also been reported, but data on this rare condition are mainly empirical. OBJECTIVE: We discuss a case of NUC secondary to spontaneous tumour lysis syndrome in a patient who had chemotherapy for non-Hodgkin lymphoma and present a review of the literature to better characterize malignancy-associated NUC. METHODS: We identified 12 published cases of malignancy-associated NUC. RESULTS: This systematic review of malignancy-associated NUC did not show a relationship between the type of malignancy, the distribution of skin lesions, or mortality. However, distal more than proximal involvement seems to be more frequently associated with calcium phosphate imbalance. CONCLUSION: Clinicians must maintain a high index of suspicion for calciphylaxis when evaluating patients with cutaneous lesions developed in the setting of malignancy.

A challenging case of squamous metaplasia in a pleomorphic adenoma: diagnostic and clinical pitfalls.

Squamous metaplasia in a pleomorphic adenoma (PA) has been reported, but rarely has it been documented as being extensive enough to cause significant misdiagnosis. We present a case of a 37-year-old man presenting with a 1.7-cm minor salivary gland PA of the palate. It exhibited extensive squamous metaplasia mimicking a squamous cell carcinoma (SCC) on multiple preoperative biopsies. The final diagnosis was only made after a complete oncological excision with margins and free flap reconstruction. Florid squamous metaplasia in a PA, although rare, should be recognized and distinguished from SCC. Failure to differentiate these entities can lead to patient overtreatment.

Trichodysplasia spinulosa in a renal transplant patient.

BACKGROUND: Trichodysplasia spinulosa (TS) is a rare skin affection seen in immunocompromised patients, mainly those with solid organ tranplants. OBJECTIVE: To report a case of a patient with classic clinical and pathologic findings for the disease so that physicians caring for this population are aware of the clinical presentation. METHOD: We report the case of a female patient we saw at our clinic with a diagnosis of TS. RESULTS: The diagnosis of TS was confirmed by pathologic findings. CONCLUSION: TS should be considered in any immunocompromised patient with a papular facial eruption reminiscent of acne vulgaris and with keratotic spiny papules as a distinctive feature.

A classic clinical case: neutrophilic eccrine hidradenitis.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is a rare condition described mostly in adult patients receiving chemotherapy for acute myelogenous leukemia. When it affects the facial region, it can mimic cellulitis and delay the diagnostic, thus proper recognition is essential. OBJECTIVE: This article describes a classic case of NEH. We will review the diagnostic, the differential diagnostic (mostly cellulitis) and the management of this condition. METHODS: After a literature review, the patient's file was properly studied in order to portray a clear picture of this condition. Medical photographs and appropriate physical examination upon presentation are also included. RESULTS: The diagnostic for NEH was suggested by the clinical presentation and confirmed histopathologically (skin biopsy). CONCLUSION: The diagnostic of NEH is essential in order to prevent multiple unnecessary antibiotics.

Unusual presentation of a severe autosomal recessive anhydrotic ectodermal dysplasia with a novel mutation in the EDAR gene.

We report on an 18-year-old woman, born to first-cousin parents, presenting with a severe form of anhydrotic ectodermal dysplasia (EDA/HED). She had sparse hair, absent limb hair, absent sweating, episodes of hyperpyrexia, important hypodontia, and hyperconvex nails. She also showed unusual clinical manifestations such as an absence of breasts, a rudimentary extranumerary areola and nipple on the left side, and marked palmo-plantar hyperkeratosis. Light microscopy of skin biopsies showed orthokeratotic hyperkeratosis and absence of sweat glands. A novel homozygous mutation (IVS9 + 1G > A) in the EDAR gene was identified. This mutation results in a total absence of EDAR transcripts and consequently of the EDAR protein, which likely results in abolition of all ectodysplasin-mediated NF-kappaB signaling. This is the first complete loss-of-function mutation in the EDAR gene reported to date, which may explain the unusual presentation of HED in this patient, enlarging the clinical spectrum linked to the dysfunction of the ectodysplasin mediated NF-kappaB signaling.

Toxic effects of methyl methacrylate monomer on male genital tissues. In vitro study in rats.

INTRODUCTION: Methyl methacrylate (MMA), a widely used monomer in dentistry and medicine has been reported to cause abnormalities or lesions in several organs. Experimental and clinical studies have documented that monomers may cause a wide range of adverse health effects such as irritation to skin, eyes, and mucous membranes, allergic dermatitis, stomatitis, asthma, neuropathy, disturbances of the central nervous system, liver toxicity, and fertility disturbances. OBJECTIVES: The purpose of this study was to determine whether MMA mixed with water at four different concentrations is able to affect the histological structure of testicular tissues and seminal vesicle on male rats. METHODS: The target population consisted of 60 male Sprague-Dawley rats. They were housed in colony cages and divided into five groups: The first group (n=15) designated as the control group and four experimental groups (n=45). Experiments were conducted by exposing the four experimental groups to MMA administered per os mixed with water at different concentrations (4% per hundred, 8% per hundred, 16% per hundred, 32% per hundred). The exposure duration was eight months. The testicles and the seminal vesicles were then extracted, dissected, fixed in Bouin liquid fixative and were submitted to the pathology laboratory (National Institute of Pathology) for histopathological examination. RESULTS: Seven out of 10 rats to which the MMA was administrated at a concentration of 32% per hundred showed partial seminal vesicle atrophy. The seminal vesicles in the remaining rats showed normal histology in all specimens. Testis, epididymis and vas deferens showed normal histology in all rats. CONCLUSION: The data in this study showed that MMA administered at high concentration is associated to seminal vesicle atrophy. These findings let us suggest that this effect could be the result of either a direct effect of MMA on testosterone levels (as shown in our first study), or through its possible action on other organs involved in testosterone metabolism and seminal vesicle trophicity such as the hypophysis.