Rare case of Calciphylaxis of penis and distal digits: a report

Written By : Dr Supreeth D R |Medically Reviewed By : Dr. Kamal Kant Kohli Published On 2022-02-28T09:00:01+05:30 | Updated On 28 Feb 2022 9:00 AM IST

Calciphylaxis is a rare, often fatal disease resulting from calcification of dermal arterioles and capillaries. Usually diagnosed in patients with end-stage renal disease, this disorder typically presents as necrotic, non-healing ulcers in acral or adipose areas. Smilnak et al. reported the case of an elderly man who was found to have calciphylaxis of the distal digits and penis, the latter of which is an uncommon site of disease that carries a particularly poor prognosis.

Case presentation: A 73-year-old African American man with multiple medical comorbidities including dialysis dependent end-stage renal disease presented with worsening painful, necrotic lesions on his glans penis and several distal digits over the last 2 months. The patient denied any new exposures or trauma to the areas of ulceration and had no history of tobacco use. The lesion on his glans penis was recently treated with a 1-week course of oral amoxicillin–clavulanic acid without improvement.

Physical examination revealed several small, exquisitely tender, necrotic ulcers with overlying black eschars on multiple finger pads; a gangrenous distal left third toe; and a foul-smelling lesion on the glans penis with overlying purulence. The digital lesions were cool to the touch and without surrounding edema or erythema.

Discovery of diffuse intravascular calcification on computed tomography, in addition to a markedly elevated calcium–phosphate product immediately prior to the onset of his ulcers, led to the diagnosis of calciphylaxis. His serum calcium and phosphorus were found to be 9.2 mg/ dL and 9.0 mg/dL (calcium phosphate product = 82.8 mg/dL). Product more than >70 mg/dL is consistent with calciphylaxis.

There is currently no consensus on the optimal treatment for calciphylaxis; management is guided by observational studies that advocate for a multimodal approach involving wound care, proper analgesia, infection prevention, and correction of serum calcium and phosphate levels. A trial of sodium thiosulfate (STS) is also recommended for all patients with calciphylaxis owing to its reported success in several case series. Therefore treatment with thrice weekly sodium thiosulfate (STS) (25 g) during dialysis was initiated.

Although the patient tolerated treatment without appreciable side effects, he experienced no improvement in the digital or penile lesions; in fact, he developed several more painful, necrotic ulcers corresponding with finger-stick blood glucose sample points. He was discharged home with plans to continue regular STS during dialysis. On follow-up, his wounds remained painful but showed no further progression; unfortunately, he died 3 months later after another prolonged hospitalization.

Even with appropriate management, calciphylaxis is a lethal disease: 6-month mortality rate is estimated to be 50%, with most patients dying from overwhelming infection. Involvement of the penis, as in this patient, is a particularly poor prognostic indicator, with 6-month mortality rates as high as 70%.

Overall, this case highlights the particularly poor prognosis associated with penile calciphylaxis. It also underscores the need for more effective management of the disease, especially in patients with multiple comorbidities.

MBBS, Dip. Ortho, DNB ortho, MNAMS

Dr Supreeth D R (MBBS, Dip. Ortho, DNB ortho, MNAMS) is a practicing orthopedician with interest in medical research and publishing articles. He completed MBBS from mysore medical college, dip ortho from Trivandrum medical college and sec. DNB from Manipal Hospital, Bengaluru. He has expirence of 7years in the field of orthopedics. He has presented scientific papers & posters in various state, national and international conferences. His interest in writing articles lead the way to join medical dialogues. He can be contacted at editorial@medicaldialogues.in.

Dr. Kamal Kant Kohli

Dr Kamal Kant Kohli-MBBS, DTCD- a chest specialist with more than 30 years of practice and a flair for writing clinical articles, Dr Kamal Kant Kohli joined Medical Dialogues as a Chief Editor of Medical News. Besides writing articles, as an editor, he proofreads and verifies all the medical content published on Medical Dialogues including those coming from journals, studies,medical conferences,guidelines etc. Email: drkohli@medicaldialogues.in. Contact no. 011-43720751