Pseudoporphyria: An Atypical Variant Resembling Toxic Epidermal Necrolysis

Papadopoulos, Anthony J.; Schwartz, Robert A.; Fekete, Zoltán; Kihiczak, George; Samady, Joseph A.; Atkin, Suzanne H.; Lambert, W. Clark

doi:10.1007/s10227-001-0013-1

Cited by 9 publications

(11 citation statements)

References 37 publications

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“…In comparison to the most common cutaneous conditions seen in kidney disease, chronic renal failure, and dialysis, uremic frost is relatively rare, yet the incidence of uremic frost is as high as 3% in recent reports . Similarly rare signs of renal failure not shown in Table included calcinosis cutis, perforating disorders (such as Kyrle disease), and porphyria/pseudoporphyria . Outpatient access to dialysis may have played a role in the reported incidences of uremic frost.…”

Section: Introductionmentioning

confidence: 93%

Uremic frost: a harbinger of impending renal failure

Saardi

Schwartz

2015

Int J Dermatology

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Uremic frost is a striking cutaneous finding seen in patients with severe kidney disease.Familiarity with this condition can be a life-saving signal to initiate urgent dialysis. Uremic frost generally occurs at blood urea nitrogen levels of approximately 200 mg/dl, although it may arise with less severe uremia. Recently confirmed urea transporters in the skin may play a role in the development of uremic frost. Alternatively, damage to the cutaneous microvasculature and pilosebaceous units, as seen in chronic kidney disease, could account for the high levels of urea deposited outside the skin. The treatment of uremic frost is largely aimed at correcting the underlying cause of uremia and the other lifethreatening conditions associated with renal failure.

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Section: Introductionmentioning

confidence: 93%

Uremic frost: a harbinger of impending renal failure

Saardi

Schwartz

2015

Int J Dermatology

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“…Pseudoporphyria was deemed unlikely given the absence of an offending medication and characteristic histopathological findings. 3 GFDE likewise became less likely due to the lack of dermal infiltration of eosinophils and melanophages and no pertinent medication history. 4 LABD was excluded due to the absence of characteristic findings on immunofluorescence.…”

Section: A Case Of Toxic Epidermal Necrosis-like Cutaneous Eruption Amentioning

confidence: 99%

A case of toxic epidermal necrosis–like cutaneous eruption as the first manifestation and clue to the diagnosis of systemic lupus erythematosus: A case report

Zargham

Ghazal

Watters

et al. 2020

SAGE Open Medical Case Reports

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We present a rare case of a 61-year-old woman presenting with a widespread erosive eruption on her torso and extremities. Although the lesions were histologically compatible with toxic epidermal necrolysis, clinically the patient was hemodynamically stable, had no mucosal involvement and had no relevant medical history or potentially incriminating medications. Further investigations uncovered a new diagnosis of systemic lupus erythematosus, with this toxic epidermal necrolysis–like eruption being the first presentation of the disease. This case highlights the importance of broadening the differential diagnosis in patients presenting with acute widespread cleavage of the epidermis, using the spectrum of acute syndrome of apoptotic pan-epidermolysis as a reference.

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“…Clinical features, similar to those seen in patients with PCT, are present in patients with pseudoporphyria, also referred to as drug‐induced pseudoporphyria cutanea tarda. As the disease name suggests, pseudoporphyria is not a true porphyria and lacks any genetic defects of UROD . Patients with pseudoporphyria are susceptible to development of skin fragility, blisters, erosions, and scarring on the back of the hands, face, and extensor surface of the legs .…”

Section: Categories Of Pctmentioning

confidence: 99%

“…As the disease name suggests, pseudoporphyria is not a true porphyria and lacks any genetic defects of UROD . Patients with pseudoporphyria are susceptible to development of skin fragility, blisters, erosions, and scarring on the back of the hands, face, and extensor surface of the legs . Unlike patients with PCT or HEP, patients with pseudoporphyria rarely present with hypertrichosis, hyperpigmentation, or sclerodermoid changes .…”

Section: Categories Of Pctmentioning

confidence: 99%

“…Pseudoporphyria describes a disease with symptoms that mimic cutaneous porphyria symptoms, yet unlike PCT, urinary porphyrin levels are in the normal range . Pseudoporphyria is associated with nonsteroidal anti‐inflammatory drugs (NSAIDs), UV light, chronic renal failure and dialysis, antibiotics, diuretics, retinoids, and additional miscellaneous drugs (Table ) .…”

Section: The Pct Mimicker: Pseudoporphyriamentioning

confidence: 99%

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Porphyria cutanea tarda: an intriguing genetic disease and marker

Handler

Stephany

et al. 2017

Int J Dermatology

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Porphyrias are a group of intriguing genetic diseases of the heme pathway, of which porphyria cutanea tarda (PCT) is the most common. Resulting from a defect in enzymes in the porphyria pathway, PCT has been linked to several conditions. Recent studies have demonstrated a change in thinking regarding the human immunodeficiency virus (HIV) and development of PCT. The exacerbation of PCT with contraction of HIV is now believed to result from coinfection from the hepatitis C virus (HCV). Blistering of sun-exposed skin, a classic presenting sign of PCT, is not exclusive to the condition. Cutaneous findings must also trigger physicians to consider additional types of porphyrias, such as variegate porphyria. The diagnosis of pseudoporphyria, which does not result from enzymatic absence, must be considered in patients with photosensitivity and cutaneous bullae. Recent health food trends, such as chlorophyll, have been linked to pseudoporphyria. PCT is a serious condition in which accurate diagnosis is necessary for appropriate management.

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Pseudoporphyria: An Atypical Variant Resembling Toxic Epidermal Necrolysis

Abstract: We describe a previously unreported, generalized variant of pseudoporphyria that resembles toxic epidermal necrolysis. We provide a review of pseudoporphyria and compare our variant to toxic epidermal necrolysis and mimicking disorders.

Cited by 9 publications

References 37 publications

Uremic frost: a harbinger of impending renal failure

Uremic frost: a harbinger of impending renal failure

A case of toxic epidermal necrosis–like cutaneous eruption as the first manifestation and clue to the diagnosis of systemic lupus erythematosus: A case report

Porphyria cutanea tarda: an intriguing genetic disease and marker

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