Porphyria cutanea tarda with multiple nodular foci in the liver

Álvarez, Ramón; López, R.; Sotorrío, N.G.; Rodrigo, Luı́s

doi:10.1016/j.gastrohep.2011.08.005

Cited by 4 publications

(8 citation statements)

References 9 publications

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“…Of these histological changes, hepatic steatosis has important significance regarding US findings. In CHP, hepatic steatosis is diffuse in most cases, but less frequently occurs in multinodular form, as in our three cases. Hepatic steatosis has been generally reported to be triggered by alcohol ingestion or hepatitis viral infection in CHP patients .…”

Section: Discussionsupporting

confidence: 72%

“…A review of US findings in previous reports showed that the echogenic mass lesions were described as homogeneous echogenic masses, as seen in Figure1 but also as isoechoic areas surrounded by an echogenic rim, as seen in Figure . Such a difference in US appearance has not been reported until now, and is noteworthy especially when US and CT findings are compared: in our first case 1 the echogenic masses were detected by CT, whereas in our second case (isoechoic areas surrounded by an echogenic rim), the lesions were not detected by CT. We speculate that in the second case, fatty changes were less prominent than in the first case but analysis of more cases of CHP is required.…”

Section: Discussionmentioning

confidence: 95%

“…US is the first diagnostic imaging tool for focal liver lesions, and on grayscale US, MNFC is very likely to be misdiagnosed as liver tumors, especially multinodular HCC on alcoholic liver injury, raising a diagnostic dilemma …”

Section: Discussionmentioning

confidence: 99%

“…Chronic hepatic porphyria (CHP) is a relatively rare pathologic condition of the liver characterized by periportal inflammation, hemosiderin deposits, and fatty changes in hepatic parenchyma, induced mainly by acquired reduced activity of heme biosynthetic enzyme . It has been known for a long time through previous reports that some CHP cases present with multinodular fatty change (MNFC) in the liver, which mimics liver tumors on grayscale ultrasound (US) and CT . To discriminate MNFC from true liver tumors, US‐guided biopsy is usually performed to yield a definitive.…”

Section: Introductionmentioning

confidence: 99%

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Multinodular fatty change in the liver in three patients with chronic hepatic porphyria: Contribution of sonography to the diagnosis

Naganuma

Ishida

Yoshida

et al. 2018

J of Clinical Ultrasound

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We present three cases of chronic hepatic porphyria (CHP) in alcoholic patients, in which grayscale ultrasound (US) revealed multiple echogenic masses in the liver, mimicking multinodular hepatocellular carcinoma on alcoholic liver injury. In all cases, contrast-enhanced US (CEUS) showed iso-enhancement of the mass lesions throughout all vascular phases. Additionally, twodimensional shear wave elastography (2DSWE) (performed in two cases) revealed the mass to have almost the same SWE value as the surrounding parenchyma. When encountering alcoholic patients with multiple echogenic masses in the liver, CHP must be included in the differential diagnosis. CEUS and 2DSWE allow us to increase our diagnostic confidence of CHP. K E Y W O R D S chronic hepatic porphyria, contrast-enhanced ultrasound, elastography, liver

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Section: Discussionsupporting

confidence: 72%

Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Multinodular fatty change in the liver in three patients with chronic hepatic porphyria: Contribution of sonography to the diagnosis

Naganuma

Ishida

Yoshida

et al. 2018

J of Clinical Ultrasound

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“…PCT was suspected because of the blistering skin lesions on the back of his hands and other sun-exposed areas of his skin. Laboratory testing for porphyrin levels yielded the following results: total urine porphyrins, 1.838 mg/24 h (NV, <0.150 mg/24 h);4 total serum porphyrins, 0.069 mg/L (NV, <0.004 mg/L);5 plasmatic fluorimetric peak, 620 nm; and erythrocyte porphyrins, within the normal range. A skin biopsy procedure was performed on the patient's thigh, and a final diagnosis of PCT was confirmed by a dermatologist.…”

mentioning

confidence: 99%

Sporadic Porphyria Cutanea Tarda in a Patient with Multiple Sclerosis Treated with Interferon Beta 1-a Therapy: A Case Report

et al. 2013

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Dear Editor, Multiple sclerosis (MS) is the most common inflammatory disease of the central nervous system, and interferon beta-1a (IFN-β1a) is a valuable medication in the treatment of the relapsing-remitting form of MS (RR MS).Porphyria cutanea tarda (PCT), which is the most frequent type of porphyria, is the result of a catalytic deficiency of uroporphyrinogen decarboxylase. In sporadic PCT (sPCT), which represents 75% of cases, the deficiency is limited to the liver. 1 Accumulated uroporphyrin and other highly carboxylated porphyrins, which are soluble, diffuse from the plasma into the upper dermis. The clinical picture reflects the phototoxic reaction that subsequently occurs in the upper dermis, in that lesions occur almost exclusively in light-exposed areas.2 The cutaneous findings include increased photosensitivity, skin fragility, blistering, erosions, and crusts. 1In this letter we describe new onset of sPCT in a patient with RR MS who was treated with IFN-β1a. In brief, a 33-year-old man without a family history of PCT was admitted to our department complaining of dizziness and diplopia of 3 weeks duration. Neuroimaging revealed several demyelinating lesions in the subcortical white matter and the periventricular regions. Within 4 months he presented with paresthesia and hyposthenia of the left arm, and repeat MRI revealed additional demyelinating lesions (Fig. 1A). The patient was diagnosed with MS according to McDonald criteria 3 and was started on treatment with IFN-β1a, which was administered subcutaneously three times weekly.In the course of his treatment, the patient exhibited no relevant neurological alterations; however, after 5 years he observed the development of skin lesions that were localized to the back of his hands, appearing as bullae up to 1 cm wide, and sometimes larger. There were also crusts, atrophic scars, and areas of hypopigmentation (Fig. 1B). He was diagnosed with dermatitis bullosa, for which he was prescribed prednisone at 25 mg/day, which had no effect. His levels of transaminases were elevated as follows: glutamic-oxaloacetic transaminase, 47 U/L [normal value (NV), <38 U/L]; and glutamic-pyruvate transaminase, 138 U/L (NV, <41 U/L). However, testing for hepatitis, including viral markers, antinuclear antibodies, smooth muscle antibodies, and antibodies to liver and kidney microsomes, alcoholic liver disease, and hemochromatosis yielded negative findings. PCT was suspected because of the blistering skin lesions on the back of his hands and other sun-exposed areas of his skin. Laboratory testing for porphyrin levels yielded the following results: total urine porphyrins, 1.838 mg/24 h (NV, <0.150 mg/24 h); 4 total serum porphyrins, 0.069 mg/L (NV, <0.004 mg/L); 5 plasmatic fluorimetric peak, 620 nm; and erythrocyte porphyrins, within the normal range. A skin biopsy procedure was performed on the patient's thigh, and a final diagnosis of PCT was confirmed by a dermatologist.Repeated phlebotomy resulted in the almost complete disappearance of the skin lesions. The patient sus...

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Multiple focal fatty changes in the liver in patients with porphyria cutanea tarda: A case series and review of the literature

Takata

Fukunaga

Nishizawa

et al. 2021

J of Clinical Ultrasound

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Porphyria cutanea tarda (PCT) is commonly diagnosed in cases where multiple hyperechoic nodules are observed in the liver. Pathologically, these nodules associated with PCT are focal fatty deposits. We report here, seven cases of PCT with fatty changes over multiple foci in the liver. Furthermore, the characteristics of ultrasonography (US) findings of 32 previously reported cases are summarized. The US features of these nodules showed a homogenous hyperechoic or hyperechoic rim pattern, partial confluence, and no mass effect in the vascular structures. Because multiple hyperechoic liver nodules occasionally mimic malignancies, and because their diagnosis can be challenging, clinicians should consider checking urine porphyrin levels to rule out PCT when such nodules are observed on US.

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Porphyria cutanea tarda with multiple nodular foci in the liver

Cited by 4 publications

References 9 publications

Multinodular fatty change in the liver in three patients with chronic hepatic porphyria: Contribution of sonography to the diagnosis

Multinodular fatty change in the liver in three patients with chronic hepatic porphyria: Contribution of sonography to the diagnosis

Sporadic Porphyria Cutanea Tarda in a Patient with Multiple Sclerosis Treated with Interferon Beta 1-a Therapy: A Case Report

Multiple focal fatty changes in the liver in patients with porphyria cutanea tarda: A case series and review of the literature

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