acquired reactive perforating dermatosis: a rare skin manifestation in end stage renal disease
TRANSCRIPT
sequelae during the follow-up period and the diagnosis ofposterior reversible encephalopathy syndrome (PRES) wasconfirmed.
Posterior reversible encephalopathy syndrome is prevalentin children who had nephrotic syndrome, hypertension orwho received immunosuppressive therapy;1 however, it israrely reported in adults with nephrotic syndrome. Cerebralhyperperfusion and vasogenic oedema were considered themajor pathognomonic change of PRES.2 Both autoregulatoryfailure and vasoconstriction of the cerebral vasculature wereoccurrence concomitantly or alternatively. Patients withhypertension and especially those with nephrotic syndromeor associated calcineurin inhibitor therapy were at increasedrisk of PRES.3 Cranial MRI was the first-line study for detect-ing PRES in patients presenting with altered consciousness,seizure, or visual disturbance combined with predisposingfactors, and was necessary to rule out cerebral infarction andvenous thrombosis. A reversible course had been revealedafter proper management including adequate control ofblood pressure, treatment of seizures, and removal or reduc-tion of causative factors. This diagnosis should be kept inmind in adults with nephrotic syndrome complicated withcertain neurological symptoms for a better comprehensivecare.
Accepted for publication 2 May 2014.
doi:10.1111/nep.12275
REFERENCES
1. Ishikura K, Ikeda M, Hamasaki Y et al. Posterior reversible
encephalopathy syndrome in children: Its high prevalence and more
extensive imaging findings. Am. J. Kidney Dis. 2006; 48: 231–8.
2. Hinchey J, Chaves C, Appignani B et al. A reversible posterior
leukoencephalopathy syndrome. N. Engl. J. Med. 1996; 334:
494–500.
3. Ishikura K, Hamasaki Y, Sakai T, Hataya H, Mak RH, Honda M.
Posterior reversible encephalopathy syndrome in children with
kidney diseases. Pediatr. Nephrol. 2012; 27: 375–84.
ACQUIRED REACTIVE PERFORATINGDERMATOSIS: A RARE SKINMANIFESTATION IN END STAGERENAL DISEASE
Hayas Haseer Koya, Dinesh Ananthan,Dona Varghese, Musa Njeru, Christopher Curtiss andApurv Khanna, Upstate Medical University, Syracuse,New York, USA
A wide range of cutaneous manifestations occur inpatients with end stage renal disease (ESRD). Acquired reac-tive perforating dermatosis (ARPD) is a specific and rare skin
disorder seen in ESRD, characterized by transepidermalelimination of altered collagen through the epidermis.
A 55-year-old lady presented with diffuse pruritic skineruptions of one year duration which started on the flanksand then spread to the abdomen, legs, and arms. Her pastmedical history included ESRD due to diabeticnephropathy-on peritoneal dialysis and type 2 diabetesmellitus. Physical examination revealed multiple, slightlyerythematous, dome shaped umbilicated papules withcentral adherent keratotic plugs (Fig. 1). The patient did nothave any associated systemic symptoms such as fever, chillsetc. A punch biopsy of the skin lesions was performed whichshowed follicular plugging with central hyper eosinophilicmaterial, perifollicular fibrosis and chronic inflammatoryinfiltrate (Fig. 2), consistent with perforating dermatosis. Adiagnosis of ARPD was made and the patient was startedon tretinoin and fluocinonide. Three month follow-up
Fig. 1 Dome shaped umbilicated papules with central keratotic plugs.
Fig. 2 Skin biopsy showing follicular plugging with central hyper eosinophilic
material, perifollicular fibrosis and chronic inflammatory infiltrates.
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Correspondence
© 2014 Asian Pacific Society of Nephrology 515
examination revealed complete resolution of pruritus andimprovement of the skin rash.
ARPD is a subtype of primary perforating dermatosisusually associated with diabetes mellitus, renal failure orhyperuricaemia. ARPD usually occurs after initiationof dialysis treatment (prevalence is 2–11%).1 Exactaetiopathogenesis is unclear, but the suspected causesinclude inflammatory skin reaction secondary to uremic skintoxins, uric acid deposits or scratching induced skin trauma.2
Investigations include skin biopsy and a search for associatedsystemic diseases. Goals of treatment include management ofunderlying disease and treatment of pruritus. Commonlyused treatment strategies include systemic or topicalcorticosteroids, retinoids and UVB phototherapy.3 ARPDshould be considered as a differential diagnosis for pruriticskin lesions, especially in patients with conditions like renalfailure, because early diagnosis and treatment can prevent
the progression of the disease, symptomatic resolution andimprovement of quality of life.
Accepted for publication 4 June 2014.
doi:10.1111/nep.12289
REFERENCES
1. Morton CA, Henderson IS, Jones MC, Lowe JG. Acquired
perforating dermatosis in a British dialysis population. Br. J.
Dermatol. 1996; 135: 671–7.
2. Karpouzis A, Giatromanolaki A, Sivridis E, Kouskoukis C. Acquired
reactive perforating collagenosis: Current status. J. Dermatol. 2010;
37: 585–92.
3. Shakery K, Zimmermann J, Bahmer FA. Erworbene reaktive
perforierende Dermatose im Patientengut der Dermatologischen
Klinik Bremen 2003–2006. Akt. Dermatol. 2008; 34: 409–13.
Correspondence
© 2014 Asian Pacific Society of Nephrology516