CASE REPORT Bali Medical Journal (Bali Med J) 2020, Volume 9, Number 1: 163-166 P-ISSN.2089-1180, E-ISSN.2302-2914 Penile necrosis due to calciphylaxis in peritoneal Case Report dyalisis patient: A case report Published by DiscoverSys CrossMark Doi: http://dx.doi.org/10.15562/bmj.v9i1.1712 Dahril,1* Fitro Manurun,2 Rovy Pratama3 ABSTRACT Volume No.: 9 Introduction: Calciphylaxis (or calcific uremic arteriolopathy) is a pressure of 141/80 mmHg, pulse rate of 88 bpm, respiration rate of life-threatening condition that affects 1–4% of patients with end- 20 times per minute, and temperature of 36.8ºC. A yellow purulent stage renal disease (ESRD). Penile necrosis is one of the complications tissue was noticed over the glans penis, foul-smelling discharge was Issue: 1 of calciphylaxis that occur in ESRD patients undergone haemodialysis. observed from urethra and undersurface of penile lesion. Laboratory Although rarely happen, this condition is potentially life-threatening examination showed elevated calcium level of 7.3 mg/dL. Ultrasound and usually has a poor prognosis. It is associated with diabetes revealed multiple calcifications at the base of penis, hypoechoic lesion, mellitus (DM), cholesterol embolism. The treatment varies, from nearly circular, irregular border, likely sac with thin wall, attached to First page No.: 163 surgical to conservative, and is usually depends on patient’s general the gland penis, and filled with exudate. Doppler confirmation showed condition. avascular area. The patient was treated with partial penectomy, Case presentation: A 41-years-old male, known to have ESRD and debridement, and antibiotic. P-ISSN.2089-1180 underwent hemodialysis for 1 year in 2014, but has been switched Conclusion: Calciphylaxis in peritoneal dialysis patients as reported in to peritoneal dialysis ever since, presented with complaints of severe this case was rarely found. Although the treatment is still debatable, pain, purulent and progressive necrosis of glans penis within the however, we decided to impose surgical approach in this patient along last two months, and history of long-term DM and hypertension. with antibiotics. Despite its poor prognosis, the patient in this case E-ISSN.2302-2914 Physical examination showed that the patient was alert, with blood showed improvement after the procedure. Keywords: calciphylaxis, end-stage renal disease, penile necrosis. Cite This Article: Dahril, Manurun, F., Pratama, R. 2020. Penile necrosis due to calciphylaxis in peritoneal dyalisis patient: A case report. Bali Medical Journal 9(1): 163-166. DOI:10.15562/bmj.v9i1.1712 1Urology Department, Faculty of INTRODUCTION commonly present in the thigh, areas of increased Medicine Universitas Syiah Kuala, adiposity, and the penis. Treatment consists of dial- Dr. Zainoel Abidin General Hospital, Penile necrosis as a consequence of a vasculopa- ysis, sodium thiosulfate, wound care, correction of Banda Aceh, Indonesia thy is seen in patients with diabetes with chronic 2 calcium–phosphate abnormalities, cinacalcet, para- Urology Department, Faculty of renal failure on long term hemodialysis. It was first Medicine Padjadjaran University, thyroidectomy (if the parathyroid hormone (PTH) Hasan Sadikin Hospital, Bandung, described in uremic patients by Bryant and White in is elevated) and supportive care.4 Indonesia 1898, though the term calciphylaxis was coined by This case is about a very rare disease that has 3Medical Research Unit, Faculty of Hans Seyle in 1962. It has been termed Calciphylaxis been described in the literature. What makes our Medicine Universitas Syiah Kuala, or Calcemic uremic arteriolopathy.1 Calciphylaxis Banda Aceh, Indonesia case unique is that it involves the penis and was (or calcific uremic arteriolopathy) is a life-threat- seen in a patient on peritoneal dialysis (CAPD). ening condition that affects 1–4% of patients with Treatment is supportive. This patient was taken end-stage renal disease on haemodialysis or those to the operating room for partial penectomy, who received renal transplant. It is characterised by penile debridement, and antibiotic therapy, then medial calcification and intimal fibrosis of medium converted to haemodialysis. and small arteries. Macroscopically, cutaneous In this study, we report a rare clinical case of necrosis is a characteristic clinical presentation that penile and generalised calciphylaxis in a patient on affects the distal extremities, buttocks, thighs and, * peritoneal dialysis. Correspondence to: Dahril, Urology less frequently, the penis. It is a clinical condition Department, Faculty of Medicine 1,2 Universitas Syiah Kuala, Dr. Zainoel with a high mortality rate >60% within 6 months. CASE DESCRIPTION Abidin General Hospital, Banda Risk factors include female, obesity, diabe- Aceh, Indonesia tes, high phosphate concentration, medications A 41-years-old Asian man was admitted to Zainoel [email protected] (warfarin, systemic steroids, calcium binders, Abidin hospital on May 4th 2018, known to have vitamin D analogues), a hypercoagulable state and ESRD and was on hemodialysis for 1 year in 2014, 3 Received: 2019-12-19 hypoalbuminaemia. Diagnosis is clinical: painful, but has been on maintenance peritoneal dialy- Accepted: 2020-03-13 non-ulcerating subcutaneous nodules or plaques, sis until now. The patient presented with chief Published: 2020-04-01 non-healing ulcers and/or necrosis, which are most complaints of severe pain, purulent and progressive Open access: www.balimedicaljournal.org and ojs.unud.ac.id/index.php/bmj 163 CASE REPORT was 2 months prior to admission. The initial lesion was like mosquitos bite with reddish wound at first. The patient had history of long-standing diabetes mellitus and hypertension. On physical examination, the patient was alert mental status, blood pressure from right brachial artery in supine position was 141/80 mm/Hg, normal pulse rate (88 beat per minute), normal respiration rate (20 times per minute), and normal temperature (36.8ºC). Localized examination at region Penis showed a yellow purulent tissue was noticed over the glans penis, foul-smelling discharge was coming out of lesion and necrotic of penile Figure 1 Gangrenous glans penis (Figure 1). His Laboratory examination showed Anemia (9.3g/dl), leukocytosis (16.800/mm3), low calcium level (7.3mg/dl), elevated uremic (222mg/dl), elevated creatinine (13.89mg/dl), low sodium (129mg/dl), and elevated potassium (5.5mg/dl). No other significant electrolyte abnor- malities were noted. Ultrasonography at regio gland penis revealed multiple calcifications at base of penis, hypoechoic lesion, nearly circular, irregu- lar border, likely sac with thin wall, attached to the Figure 2 Ultrasonography examination showed gland penis, and filled with exudate. Doppler confir- Calcification at Base of Penis mation showed avascular area. Echocardiography examination revealed intracardiac thrombus and vegetation with ejection fraction 64%, left ventricle dilatation, and left ventricle hypertrophy. He was diagnosed with calciphylaxis based on a clinical presentation combined with a medical history of diabetes and end-stage renal disease, and calcification on USG Figure( 3). The patient was treated with partial penectomy, debridement, and antibiotic therapy. The patient underwent blood Figure 3 Ultrasonography examination showed culture before discharged from hospital, and the exudate with irregular border attached result showed none growth organism in the blood, to gland penis suspected due to given antibiotics treatment or caused by organism instead of bacteria (Figure 4). DISCUSSION Calciphylaxis (calcific uremic arteriolopathy) is an obliterative calcific vasculopathy in which calcifica- tion occurs in the media of small arteries of up to 600 μm in diameter, arterioles, and capillaries, with intimal fibrosis and thrombosis leading to tissue necrosis. Most commonly it occurs in haemodial- ysis dependent patients who have ESRD or renal transplant recipients. It affected up to 5% of dialy- sis-dependent patients. Risk factors include female gender, diabetes, and obesity. Other associations are raised calcium/phosphate product (CPP), high serum alkaline phosphatase level, malnutrition, and Figure 4 Partial penectomy low serum albumin level. Calciphylaxis also occurs necrosis of glans penis of two-months duration. On in the absence of renal failure in the context of review of medical history, the onset of skin lesions hyperparathyroidism, malignancy, chemotherapy, 164 Published by DiscoverSys | Bali Med J 2020; 9(1): 163-166 | doi: 10.15562/bmj.v9i1.1712 CASE REPORT corticosteroid and warfarin therapy, alcoholic liver of the gangrenous penile lesions is also debated. disease, autoimmune disease, and inflammatory Wood et al. recommended penectomy, while others bowel disease.5 CUA typically presents with isch- believe that local wound care and debridement of emic necrosis predominantly involving areas of penile lesions are sufficient. In the presence of infec- adiposity in the body such as the trunk, buttocks, or tion, aggressive surgical debridement or penectomy proximal extremity. However, non-adipose regions is often required for any chance of survival.6 Prompt can be involved and patients can present with digi- treatment of calciphylaxis is important to reduce tal ischemia and more rarely penile gangrene as is the risk of progressive necrosis and sepsis. Surgical the case with this patient.6 treatment of penile calciphylaxis includes circum- Isolated gangrene of the penis represents a local-