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A 35-year-old woman receiving immunosuppressionfor renal transplantation presented with a onemonthhistory of tender skin nodules on herbilateral upper extremities. A skin biopsy revealedgranulomatous inflammation in the deep dermisand the subcutaneous fat with foci of necrosis.Within the foci of necrosis were large histiocytoidstructures with prominent nuclei. Periodic acid-Schiffstain revealed a round organism with a thick capsule,consistent with amoebal trophozoites. Testing withthe Center for Disease Control revealed the organismto be Acanthamoeba. Despite antimicrobial therapy,the patient continued to develop subcutaneousnodules that extended to the lower extremities andtrunk and ultimately extended to the bone, causingacanthamoebal osteomyelitis. Throughout thehospital course, the patient remained neurologicallyintact without evidence of central nervousinvolvement. A diagnosis of isolated disseminatedcutaneous acanthamoebiasis secondary to iatrogenicimmunosuppression was made. Historically, mostcases of granulomatous amoebic encephalitisand cutaneous acanthamoebiasis have occurredin patients with HIV/AIDS. However, with the useof newer and more effective immunosuppressiveregimens, both are occurring more frequently inthe setting of iatrogenic immunosuppression. Therare and isolated cutaneous nature of this patient'spresentation makes this case unique.

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BACKGROUND: Nodular scleroderma, also known as keloidal scleroderma, is a rare variant of systemic sclerosis. PURPOSE: The clinical features, pathologic findings and postulated pathogenesis of nodular scleroderma are discussed. METHODS: A woman with previously undiagnosed systemic sclerosis who presented with nodular scleroderma is described. Using the PubMed database, a literature search was performed on keloidal scleroderma, nodular scleroderma, and systemic sclerosis. RESULTS: Nodular scleroderma is characterized by firm plaques or nodules, which can mimic a keloid, that are typically located on the anterior orposterior upper trunk and the arms; they show pathologic changes of scleroderma, keloid, or hypertrophic scar. Akeloidal response of inflamed skin that is involved in an active fibrotic process inherent to systemic sclerosis, in individuals who are genetically predisposed to keloid formation, is the hypothesized pathogenesis. CONCLUSION: Nodular scleroderma is rare. The authors' patient presented with diarrhea, dysphagia, fatigue, Raynaud's phenomenon, shortness of breath, and annular keloidal plaques of morphea whose biopsy showed features of hypertrophic scar; additional studies confirmed the diagnosis of the nodular scleroderma variant of systemic sclerosis. The possibility of systemic sclerosis should be entertained in patients who present with nodularor keloidal plaques that morphologically resemble morphea and have histologic findings of a scar or a keloid-especially if there are associated symptoms suggestive for systemic sclerosis.

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Neuropathy as extramuscular manifestation of dermatomyositis (DM) is controversial due to uncommon occurrence, heterogeneity of associated nerve pathology, and lack of unifying pathogenetic mechanism(s). We describe a patient with classic manifestations of DM and extramuscular manifestation of neuropathy. Nerve pathology showed deposits of terminal complement complex (C5b-9). Her examination showed mild proximal weakness, rash, and sensory impairment in fingertips, toes, and nose. EMG/NCS revealed irritable myopathy and mild sensory neuropathy. Muscle biopsy showed features suggestive of DM, including deposition of C5b-9. CK was elevated to 214 and ANA was positive at 1:160. Etiological work up for neuropathy, including diabetes, was negative. Sural nerve biopsy at light level revealed very mild large fiber sensory neuropathy. EM showed moderately severe involvement of small sensory fibers. Neuropathy may be an underrecognized manifestation of DM. Nerve pathology demonstrating complement-mediated damage could be a unifying mechanism of muscle and nerve injury.

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To properly evaluate therapies for cutaneous dermatomyositis (DM), it is essential to administer an outcome instrument that is reliable, valid, and responsive to clinical change, particularly when measuring disease activity. The purpose of this study was to compare two skin severity DM outcome measures, the Cutaneous Disease and Activity Severity Index (CDASI) and the Cutaneous Assessment Tool-Binary Method (CAT-BM), with the Physician Global Assessment (PGA) as the "gold standard". Ten dermatologists evaluated 14 patients with DM using the CDASI, CAT-BM, and PGA scales. Inter- and intra-rater reliability, validity, responsiveness, and completion time were compared for each outcome instrument. Responsiveness was assessed from a different study population, where one physician evaluated 35 patients with 110 visits. The CDASI was found to have a higher inter- and intra-rater reliability. Regarding construct validity, both the CDASI and the CAT-BM were significant predictors of the PGA scales. The CDASI had the best responsiveness among the three outcome instruments examined. The CDASI had a statistically longer completion time than the CAT-BM by about 1.5 minutes. The small patient population may limit the external validity of the findings observed. The CDASI is a better clinical tool to assess skin severity in DM.

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Herpes zoster, also known as shingles, occurs upon reactivation of a primary infection with varicella zoster virus (VZV). Risk factors for reactivation include stress, older age, and immunosuppression, all of which are associated with a decrease in host immunity. Common complications of herpes zoster include scarring and post-herpetic neuralgia (PHN). Cutaneous lesions such as granuloma annulare, lymphomas, and sarcoid granulomas have also been reported to potentially arise at the site of herpes zoster. Here, we report a case that to our knowledge is the first presentation of diffuse large B-cell lymphoma with its only cutaneous manifestation arising in a herpes zoster scar. Punch biopsy was performed on a nodule appearing in a dermatomal distribution within the herpes zoster scar. Histopathology revealed an atypical lymphoid infiltrate in the dermis that was determined to be CD20 positive B-cells. Immunostains for CD20, CD79a, and PAX-5 showed strong positive staining of the atypical cells, confirming B-cell origin and resulting in the diagnosis of lymphoma, large B-cell type. This case highlights the importance of raising clinical suspicion for a malignant process in patients who present with a changing or unresolving skin manifestation after infection with varicella zoster virus.

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Methotrexate is a folic acid analog pioneered for use in inflammatory diseases by dermatologists, and used successfully for over 40 years for a wide variety of cutaneous diseases. In addition to its antiproliferative properties, methotrexate has other more recently recognized anti-inflammatory properties related to its effects on adenosine. Further research concerning its mechanism of action and genetic enzymatic variations suggest future possibilities for maximizing therapy and predicting adverse events. In this review the present authors will explore methotrexate's pharmacokinetics, mode of administration, dosing guidelines, side effect profile, and medication interactions. In addition, the present authors hope to offer practical guidelines for dose initiation and adjustment, and to summarize new research on its mechanism of action and implications for future therapy.

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