Pseudoporphyria Research Articles

Progressive ascending telangiectasias

Progressive ascending telangiectasias

POS-706 PSEUDOPORPHYRIA IN A PERITONEAL DIALYSIS PATIENT

Dermatological manifestations in patients with end-stage renal disease (ESRD) are very common, such as pruritus, xerosis and pigmentation disorders, other skin disorders are quite rare such as pseudoporphyria cutanea tarda (PCT). It is a photodistributed vesicobullous disorder with clinical and histologic features similar to those of porphyria cutanea tarda but without accompanying biochemical porphyrin abnormalities. Although PCT is seen in 13% of hemodialysis patients, it is rare in peritoneal dialysis (PD) patients.

POS-581 PSEUDOPORPHYRIA CUTANEA TARDA IN HEMODIALYSIS

Although dermatological manifestations in patients with end-stage renal disease (ESRD) are very common, such as pruritus, xerosis and pigmentation disorders, other skin disorders are quite rare such as pseudoporphyria cutanea tarda (PCT). It is a bullous photodermatosis with the same clinical and histological characteristics of porphyria cutanea tarda but in the absence of abnormalities in the metabolism of porphyrin. We report in this work 2 cases of ESRD patients who developed a PCT. We report in this work 2 cases of ESRD patients who developed a PCT in our hemodialysis (HD) unit. Case report 1: A 49-year-old man with a history of Marfan syndrome complicated by bilateral blindness and hypertension and ESRD secodary to an indeterminate nephropathy. He started HD in 2007. His renal failure was complicated by secondary hyperparathyroidism requiring 7/8 parathyroidectomy associated with total thyroidectomy because of the presence of a Tirads V classified thyroid nodule. After 10 years of HD, he developed a bullous dermatosis, a fragile skin with multiple erosions covered with brownish scabs. The lesions were localized in the photo-exposed areas : the face and the hands. He had no family history of porphyria, photosensitivity, or previous episodes of this dermatosis. The diagnosis retained was PCT of the dialysis patient. Ferritin was within the standards at this time. The treatment was based on N-acetylcysteine and photoprotective creams. The outcome was favorable with the disappearance of the bullous lesions leaving multiple atrophic and dyschromic scars without subsequent recurrence. Case report 2: A 48 year old man with a history of hypertension, diabetes, ESRD on indeterminate nephropathy. He started peritoneal dialysis in 2010. After 9 years, faced with recurrent peritonitis, he was switched to HD. Encapsulating peritonitis was diagnosed after starting HD, treated with tamoxifen and corticosteroids. After one year of HD, he developed bullous skin lesions with the presence of folliculitis on the back of his hand, arm and abdomen. Biologically, there was no biological inflammatory syndrome and the ferritin was at 212ng / ml. He was initially treated by local and systemic antibiotic therapy, but without improvement. The diagnosis subsequently retained was PCT of the dialysis patient and he was treated with N acetylcysteine, the prescription of photoprotection and antibiotic therapy for his surinfected lesions. The evolution was marked by the progressive regression of the lesions giving way to atrophic scars. There are multiple factors that can cause a push of PCT in dialysis patients, such as erythropoietin, iron overload, diuretics, but the cause may remain unknown. The main thing is to always think about this diagnosis in face of any bullous dermatosis and knowing how to stop the incriminating agent, prescribe photoprotection, in addition to N acetylcysteine having encouraging results in these patients.

Neuropsychiatric Manifestations of Acute Intermittent Porphyria – A Diagnostic Dilemma

Porphyrias are a group of inherited or acquired disorders of certain enzymes in the heme bio-synthetic pathway (also called porphyrin pathway). They are broadly classified as acute (hepatic) porphyrias and cutaneous (erythropoietic) porphyrias, based on the site of the overproduction and accumulation of the porphyrins or their chemical precursors. They manifest with either neuropsychiatric complications or skin problems or occasionally both. A clinically induced and histologically identical condition is called pseudo porphyria which is characterized by normal serum and urine porphyrin levels.

A Case of Voriconazole-Induced Pseudoporphyria

A Case of Voriconazole-Induced Pseudoporphyria

Investigation of consciousness disorders caused by mushroom poisoning Zhang Yizhe, Sun Chengye, Li Haijiao, Sun Jian, Zhang Hongshun, Ma Peibin, Yao Qunmei, Yu Chengmin

Objective To analyze a case of mushroom poisoning presenting coma occurred two hours after ingestion of mushroom from Nanhua County, Yunnan Province. Methods With the epidemiological investigation, clinical treatment data and suspicious mushroom samples, the case was discussed. Results The mushroom samples were identified as amanita pseudoporphyria and amanita parvipantherina. Conclusions According to epidemiology, clinical feature, morphological and molecular biological identification, this case can be confirmed to be a mushroom poisoning incident caused by more than one species. Key words: Amanita pseudoporphyria; Amanita parvipantherina; Poisonous mushroom; Consciousness disorders; Molecular identification

Pseudoporphyria Associated with Nonhemodialyzed Renal Insufficiency, Successfully Treated with Oral N-Acetylcysteine

Pseudoporphyria (PP) is a relatively rare, photodistributed bullous dermatosis that resembles porphyria cutanea tarda (PCT), but it is not accompanied by porphyrin abnormalities in the serum, urine, or stool. It was initially described in renal failure patients on dialysis. Thereafter, it has been associated with several aetiological factors. We report a case of PP in a 67-year-old woman with mild renal failure, successfully treated with N-acetylcysteine. This is the second reported case of PP developing in nondialyzed chronic renal failure. Such cases support the view that renal impairment itself may play a more important aetiological role in developing PP than it was originally considered.

Cutaneous changes in patients with chronic renal failure on hemodialysis

Context: A number of skin diseases can be observed in chronic renal failure (CRF). Their incidence have changed in different series.Objective: To compare the prevalence of cutaneous changes in CRF undergoing hemodialysis (HD) with healthy persons and to study the potential relationship with various parameters in the patients.Materials and methods: The study comprised one hundred patients with CRF under regular HD as well as one hundred healthy control subjects of matched age and sex. Both groups were subjected to full history and thorough skin examination.Results: Numerous skin lesions (at least one in each patient) were detected in all patients examined. Of these patients, nail abnormalities were observed in 92%, xerosis in 87%, fungal infection in 68%, hyperpigmentation in 62%, pruritus in 51%, and paleness in 51%. The nail changes included absence of lunula (55%), half and half nail (51%), splinter hemorrhages (36%), subungual hyperkeratosis (34%), onychomycosis (31%), koilonychia (19%), and onycholysis (9%). Oral changes were oral candidiasis (50%), xerostomia (40%), angular cheilitis (27%), and ulcerative stomatitis (18%). Specific cutaneous findings such as acquired perforating dermatosis, pseudoporphyria cutanea tarda, calciphylaxis, calcinosis cutis, and nephrogenic fibrosing dermopathy were not detected in any of the patients. In our study, when the patients were evaluated on the relationship between xerosis and pruritus, pruritus was found to be significantly increased parallel to the increase in the severity of xerosis.Conclusions: Xerosis and pruritus are common problems in HD-dependent patients. The early recognition of cutaneous signs can relieve suffering and decrease morbidity.

Finasteride-Induced Pseudoporphyria

Finasteride-Induced Pseudoporphyria

Melanoma Associated With Long-term Voriconazole Therapy

Voriconazole is a triazole antifungal agent approved by the US Food and Drug Administration for serious fungal infections, including with Aspergillus, Fusarium, Pseudallescheria, and Scedosporium species. In initial clinical trials, approximately 2% of patients developed cutaneous reactions, including photosensitivity, cheilitis, and xerosis. Subsequent reports have implicated voriconazole as a cause of severe photosensitivity and accelerated photoaging, pseudoporphyria cutanea tarda, and aggressive squamous cell carcinoma. We report 5 melanoma in situ lesions in the setting of extreme photosensitivity associated with long-term voriconazole therapy. We recommend surveillance for skin cancer formation in all patients who require long-term voriconazole treatment, particularly those who manifest signs or symptoms of photosensitivity or chronic photodamage. Further study of the mechanism underlying voriconazole photosensitivity and oncogenesis is warranted.

Torsemide-Induced Pseudoporphyria

Torsemide-Induced Pseudoporphyria

Early onset pauciarticular arthritis is the major risk factor for naproxen-induced pseudoporphyria in juvenile idiopathic arthritis

Pseudoporphyria (PP) is characterized by skin fragility, blistering and scarring in sun-exposed skin areas without abnormalities in porphyrin metabolism. The phenylpropionic acid derivative group of nonsteroidal anti-inflammatory drugs, especially naproxen, is known to cause PP. Naproxen is currently one of the most prescribed drugs in the therapy of juvenile idiopathic arthritis (JIA). The prevalence of PP was determined in a 9-year retrospective study of children with JIA and associated diseases. In addition, we prospectively studied the incidence of PP in 196 patients (127 girls and 69 boys) with JIA and associated diseases treated with naproxen from July 2001 to March 2002. We compared these data with those from a matched control group with JIA and associated diseases not treated with naproxen in order to identify risk factors for development of PP. The incidence of PP in the group of children taking naproxen was 11.4%. PP was particularly frequent in children with the early-onset pauciarticular subtype of JIA (mean age 4.5 years). PP was associated with signs of disease activity, such as reduced haemoglobin (<11.75 g/dl), and increased leucocyte counts (>10,400/μl) and erythocyte sedimentation rate (>26 mm/hour). Comedications, especially chloroquine intake, appeared to be additional risk factors. The mean duration of naproxen therapy before the onset of PP was 18.1 months, and most children with PP developed their lesions within the first 2 years of naproxen treatment. JIA disease activity seems to be a confounding factor for PP. In particular, patients with early-onset pauciarticular JIA patients who have significant inflammation appear to be prone to developing PP upon treatment with naproxen.

Leflunomide-induced pseudoporphyria

Leflunomide-induced pseudoporphyria

Rofecoxib-induced pseudoporphyria

Rofecoxib-induced pseudoporphyria

Pseudoporphyria associated with hemodialysis treated with N-acetylcysteine*

Pseudoporphyria associated with hemodialysis treated with N-acetylcysteine*

SUBACUTE CUTANEOUS LUPUS ERYTHEMATOSUS WITH PSEUDO-PORPHYRIA AND VITILIGO

Sub acute Cutaneous Lupus Erythematosus (SCLE) is a distinct subset of LupusErythematosus. It can present as non scarring psoriasiform or annular polycycliclesions. About half the patients fulfil the criteria of the American RheumatismAssociation for systemic lupus erythematosus. In this report, we present the case of a45 years old male who came with psoriasiform depigmented plaques and papulo-vesicular lesions developed after taking some medication. Clinically diagnosed as subacute cutaneous lupus erythematosus with acral vitiligo. A histopathological studyand urine analysis however revealed presence of pseudo porphyria along with theSCLE. Although SCLE is not a rare disease, the association of it with pseudo porphyriaand vitiligo is of significance as the treatment for SCLE and pseudo porphyria isphoto protection and for the vitiligo it is photo chemotherapy.ABSTRACTKey Words: Sub acute cutaneous lupus erythematosus, pseudo porphyria,vitiligo, psoriasiform eruptions.

NSAID-Induced Pseudoporphyria Is Common in Children with Arthritis

Pseudoporphyria simulates porphyria: In adults it mimics porphyria cutanea tarda, and in children, the erythema, skin fragility, blistering, and

New Insights into Pseudoporphyria

The term pseudoporphyria describes a cutaneous condition that mimics porphyria cutanea tarda (PCT) in adults and erythropoietic protoporphyria in children. Pseudoporphyria is frequently linked to the ingestion of drugs, primarily nonsteroidal anti-inflammatory drugs (NSAIDs). The course is prolonged in some patients, but most reports describe symptoms that disappear several weeks to several months after the offending agent is withdrawn. These authors retrospectively studied patients with pseudoporphyria who were treated at the Mayo Clinic between 1992 and 1996. Normal porphyrin levels were required for a …

Celecoxib, a New Cause of Pseudoporphyria

Pseudoporphyria is a condition associated with blisters, erosions, scars, and milia on sun-exposed surfaces. This condition has been strongly linked to nonsteroidal antiinflammatory drugs (NSAIDs) -- in particular, to naproxen. These authors present the first report of a patient in whom pseudoporphyria is linked to celecoxib …

A clinical case of pseudo-porphyria cutanea tarda by hemodialysis

A clinical case of pseudo-porphyria cutanea tarda by hemodialysis