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Case Report Clinical Research in Dermatology: Open Access Open Access Pseudoporyphyria Associated with Lemon Water and Nwanneka Okwundu1*, John Moesch2, Sarah Belden3 1Department of Dermatology, Huntsman Cancer Center, University of Utah, Salt-lake City, Utah 2Department of Dermatology, Largo Medical Center, Largo, Florida 3Department of Dermatology,Case Western Reserve University, Cleveland, Ohio

Received: March 05, 2020; Accepted: May 05, 2020; Published: June 18, 2020

*Corresponding author: Nwanneka Okwundu,University of Utah Department of Dermatology, Salt-lake City, Utah, USA. E-mail: Nwanneka. [email protected]

Abstract Background: Pseudoporyphyria is an uncommon bullous dermatosis. vitiligo. Medications included alendronate, cyclobenzaprine, It shares clinical and histological features with cutanea beforefluticasone, the initial tramadol blister and outbreak. naproxen, Patient celecoxib. reported Of note,that biopsies patient tarda, but it occurs in the absence of porphyrin metabolic dysfunction. reported being on the medication regimen for several years It is characterized by skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. results. Patient stated that the blisters appeared shortly after he of the lesions were performed in the past with inconclusive Case: We present a patient on naproxen with recurrent pseudoporyphyria of the dorsal hands associated with the began ingesting lemon water; he would cut up lemons and leave consumption of lemon water. them in his water overnight to consume the following day. Patient Findings: reported that whenever he did not consume the lemon water, the with caterpillar bodies and laboratory studies lacked any urine or handsblisters werewould scattered resolve.Dermatologic depigmented examination patches consistent revealed tensewith serum porphyrin Biopsy abnormalities.showed a pauci-inflammatory The cutaneous sublesions epidermal were noted vesicle to bullae and vesicles isolated to the dorsal hands. Also, on the dorsal instructed to discontinue naproxen and to follow up in 6 weeks. resolveKeywords: with Pseudoporyphyria; discontinuation of Porphyrin;the consumption Porphyria of lemon Cutanea water. Tarda; the patients known history of vitiligo(Figures 1,2). Patient was Chlorophyll; Vesicle and Depigmented were within normal limits, such as hepatitis panel, complete At follow-up, tense vesicles were present. Labs were drawn and

Introduction blood count with differential, complete metabolic profile Pseudoporyphyria is a rare photodermatosis with clinical porphyrin(including theanalysis blood ureaand nitrogen, serum creatinine,porphyrin sodium, were iron,normal. and magnesium, and calcium levels). A 24-hour urine quantitative and histological features similar to those of porphyria cutanea tarda, but lacks the abnormalities seen in porphyrin metabolism Punch biopsy showed a pauci-inflammatory sub some known etiological agents. The two most common causes epidermal vesicle with caterpillar bodies (Figure 3). Skin [1]. This skin photosensitivity disorder has been associated with organisms.immunofluorescence Differential showeddiagnosis focalfor pseudoporyphyria positivity for IgGinclude in superficial vessel walls and skin. PAS stain was negative for fungal include ultraviolet (UV) radiation and medications [2]. Patients the dorsum of the hands, forearms, face, lower legs, and feet coproporphyria, congenital erythropoietic porphyria, and other may develop bullae and vesicles on sun exposed areas such as [3]. They may also present with skin fragility and easy bruising bullouserythropoietic disorders protoporphyria, presenting with variegate similar clinical porphyria, manifestations, hereditary after minor trauma. Bullae heal with scarring and milia in most for photoprotection and suspension of possible medications allincluding of these epidermolysis are histopathologically bullosa acquisita, and biochemically bullous pemphigoid, different patients. Pseudoporyphyria is often difficult to treat and except bullous lupus erythematosus, and hydroa vacciniforme. However, involved, there is currently no specific treatment regimen [4]. laboratoryfrom pseudoporyphyria results make them and unlikely the unremarkable as well. 24-hour urine Case report quantitative porphyrin analysis, serum porphyrin, and other A 60-year-old Caucasian male presented with intermittently denied any family history of blisters. He worked as a landscaper forrecurrent a golf course.vesicles His on the past dorsal medical hands history for the included past 15 years.arthritis Patient and

Symbiosis Group *Corresponding author email: [email protected] Copyright: © Pseudoporyphyria Associated with Lemon Water and Naproxen 2020 Nwanneka Okwundu, et al.

Figure 1 & 2: Depigmented Patches on Dorsal Hands

hyperpigmentation, and sclerodermoid changes, all of which are rarelytarda mayseen in have pseudoporyphyria associated symptoms [7]. such as hypertrichosis,

studyThe was thickness done on of biopsy the blood samples vessel of wallpatients could with also PCT be andhelpful PP. in differentiating PP from PCT [8]. A comparative histological

wallsIt was infound PP patientsthat the blooddisplayed vessel these walls features. were thickened Other innotable 11 of 13 patients with PCT while only 1 out of 9 of the blood vessel

epidermalfeatures of junctionPP are the of presencePP patients of [9].IgG, IgM, C3, and fibrinogen on direct immunofloresence on the vessel walls and at the dermal-

common reported triggers include the potentially phototoxic medicationsDiagnosis such of PP requiresas naproxen, an underlying , trigger. , The most Figure 3: bodies Pauci-inflammatory sub epidermal vesicle with caterpillar Discussion drug-inducedciprofloxacin, phototoxic acitretin, reactions voriconazole usually appear [10,11]. as exaggerated However, sunburns.pseudoporphyria The reason manifests for this with is vesicles unknown. and bullae,Other reported whereas disorder that commonly occurs after exposure to phototoxic associations include history of chronic renal failure and dialysis. or Pseudoporyphyriaphotosensitizing chemicals. (PP) is a rareCutaneous bullous and dermatologic histologic The suspected photosensitizing agent in dialysis solution is manifestations of pseudoporyphyria are the same as poryphyria tubing [12,13]. aluminum hydroxide or chemicals in polyvinyl chloride dialysis cutanea tarda (PCT) with the appearance of skin fragility features. PP has also been reported with exposure to chlorophyll Clinical manifestation of these conditions include bullae, vesicles, and scarring on superficial sun exposed skin, and histology in various forms [14]. Chlorophyll is a natural constituent of characteristically shows non-inflammatory, subepidermal bulla such as spinach, and is usually metabolized without causing pseudoporphyria can be differentiated from poryphyria cutanea the human diet, occurring naturally in green leafy vegetables with pauci perivascular lymphocytic infiltrate [5]. However, any symptoms. However, some chlorophyll metabolites are tarda by the presence of normal porphyrin levels in plasma, known to be active photosensitizers [15]. Chlorophyll-derived erythrocytes, urine, and stool [6]. Furthermore, porphyria cutanea Citation: Nwanneka Okwundu, John Moesch, Sarah Belden (2020) Pseudoporyphyria Associated with Lemon Water and Naproxen. Page 2 of Clin Res Dermatol Open Access 7(3): 1-4. DOI: 10.15226/2378-1726/7/3/001115 4 Copyright: © Pseudoporyphyria Associated with Lemon Water and Naproxen 2020 Nwanneka Okwundu, et al.

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Citation: Nwanneka Okwundu, John Moesch, Sarah Belden (2020) Pseudoporyphyria Associated with Lemon Water and Naproxen. Page of Clin Res Dermatol Open Access 7(3): 1-4. DOI: 10.15226/2378-1726/7/3/001115 4 4