The Eurasian

Case Report

Journal of Medicine

_______

Eurasian J Med 20M ; 46: 131-4

Diffuse Idiopathic Calcinosis Cutis in an Adult: A Rare Case Bir Yetişkinde Diffüz Idiopatik Kalsinosis Kutis: Nadir Bir Vaka Raghunath Prabhu, Yashdeep Sinha Sarma, Karan Phillip, Sakshi Sadhu Department o f Surgery, Manipal University Kasturba Medical College, Karnataka, India

Abstract

Özet

Idiopathic calcinosis cutis is a condition involving the deposition of calcium salts in the skin and subcutaneous tissue. The disease is a pathological condition of unknown origin and hence is idiopathic. The salt deposition is confined to areas such as the breast and vulva in females and scrotum and penis in males. Diffuse calcification with multiple complications in an adult is a rare entity. Only one such case has been reported in literature. A 59-year-old female presented to us with swelling o f the right elbow, multiple calcific nodular lesions all over her fingers approximately 0.5x0.5 cm in size, and ulcers on her left great toe and right thum b with pain for the past tw o months. The ul­ cers were 2x2 cm and were observed to be healing w ithout active dis­ charge or signs o f inflammation. The elbow was diffusely swollen and tender. Flexion deformity was present at the elbow. X-ray of hand and feet revealed calcinosis o f the elbow and interphalangeal joints of the foot and hand. Blood tests revealed elevated C-reactive protein levels of 24 mg/dL, elevated Erythrocyte Sedimentation Rate (ESR) o f 52 mm/ hr., serum calcium o f 9.7 mg/dL and a serum phosphorous o f 5 mg/ dL. Cultures from the foot ulcer were positive for methicillin-resistant staphylococcus aureus (MRSA). Workup for collagen vascular disease was negative. Histopathology confirmed calcinosis cutis. Treatment involved a conservative approach, including physiotherapy for the flexion deformity, antibiotics for MRSA, analgesics for pain relief and daily dressings. This case demonstrates that if a patient presents with multiple chalky nodular lesions with or w ithout ulceration, pain and discharge involving areas o f the upper limb or lower limb, diagnosis of idiopathic calcinosis cutis could be considered as a differential, despite its common confinement to the scrotum, breast, vulva and penis.

idiyopatik Calcinosis Cutis, deri ve deri altı dokuda kalsiyum tuzla­ rının çökelmesini içeren bir durumdur. Hastalık sebebi bilinmeyen ve bu nedenle idiyopatik bir patolojidir. Tuz birikimi, kadınlarda ve erkeklerde skrotum ve penis, meme ve vulva gibi alanlarda sınırlıdır. Bir yetişkinde birden fazla komplikasyon ile diffüz kalsifikasyonun bulunması nadir bir durumdur. Literatürde bu şekilde sadece bir vaka rapor edilmiştir. Sol ayak başparmağında ağrı, sağ dirsek şişmesi ve sağ başparmak üzerinde 0.5x0.5 cm boyutunda ülserler ile iki ay önce bize başvuran 59 yaşındaki kadında, tüm parmaklarının üzerinde kalsifik nodüler lezyonları bulunmaktaydı. Ülserler 2x2 cm idi ve aktif akıntılı veya enflamasyon belirtileri olmadan iyileşme gözlemlendi. Dirsek diffüz olarak şişmiş ve hassastı. Dirsekte fleksiyon deformitesi mevcuttu. El ve ayakların röntgeninde, ayak ve el dirsek ve interfalengeal eklemlerin kalsinozisi saptandı. Kan testleri 24 mg/dL'lik artmış C-reaktif protein seviyeleri, 52 mm/saat'lik yüksek bir ESR, 9,7 m g/ dL serum kalsiyum, 5 mg/dL'lik bir serum fosfor ortaya çıkardı. Ayak ülseri kültürlerinde metisilin dirençli Staphylococcus aureus (MRSA) pozitif bulunmuştur. Kolajen vasküler hastalık için yapılan çalışma negatifti. Histopatoloji kalsinozis kutisi doğruladı. Tedavi fleksiyon deformitesi için fizyoterapi, MRSA için antibiyotik, ağrı kesici ve gün­ lük pansuman için analjezikler de dahil olmak üzere, konservatif yak­ laşım içeriyordu. Bu durum bir hastanın ülser, ağrı ve üst uzuv veya alt ekstremitenin alanlarını içeren akıntılı olduğu ya da olmadığı, birden fazla kireçli nodüler lezyon mevcut olması durumunda, idiyopatik calsinosis cutis'in tanısının skrotum, meme, vulva ve penisde de yay­ gın olmasına rağmen, bir diferansiyel tanı olarak kabul edilebileceği­ ni göstermektedir.

Keywords: Calcinosis cutis, idiopathic, diffuse, adult

Anahtar Kelimeler: Kalsinozis kütis, idiopatik, diffüz, yetişkin

Introduction Calcinosis cutis is a pathology characterized by the depo­ sition of calcium salts in the skin and subcutaneous tissue. Etiologically, calcinosis cutis is divided into 4 major types: dystrophic calcification (most common, 95-98% cases), meta­ static calcification, idiopathic calcification and iatrogenic

calcification [1]. Idiopathic calcinosis cutis is rare and hence is usually a diagnosis of exclusion [2]. The disease is often restricted to the vulva, scrotum, penis, and breast [3], Our patient was an elderly woman who presented with calcinosis cutis in the hands, feet and elbow with ulceration and flexion deformity. Only one such case of diffuse Idiopathic calcinosis cutis (ICC) in an adult has been reported in the literature to the best of the author's knowledge [4],

Received: April 06, 2013 / Accepted: July 31,2013 Correspondence to: Raghunath Prabhu, Department o f Surgery, Manipal University Kasturba Medical College, Karnataka, India Pin code 576104, India Phone: 9448903817 e-mail: [email protected] ©Copyright 2014 by the Atatürk University School o f Medicine - Available online at www.eajm.org doi:10.5152/eajm.2014.29

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Figure J. a, b. PlainX-rayofthe foot demonstrating calcification at the heel as well as the great toe (a). Clinical photograph revealing a superficial ulcer at the sole of the foot (b).

Figure 2, a, b. Clinical photograph demonstrating nodularity in the right thumb (a). Plain x-ray of the hand

revealing calcifications around the V distal phalynx (b).

Figure 3. a, b. Clinical photograph demonstrating flexion deformity at the right elbow (a). Plain x-ray reveal­

ing calcification around the elbow (b).

Case Report

hypertensive medications, respectively, for the past 2 months. There was no significant family history.

A 59-year-old female agriculturist presented with an ulcer on the ball of left great toe and of the right thumb of 2 months duration with associated pain and purulent discharge for the past 2 weeks. She also complained of pain and swelling of the right elbow and multiple nodular lesions over the hand and the sole (Figure 1). No history of similar lesions over the breast or genitalia was present. There was no history of trauma or pathologic lesions at the site of the nodular lesions. No history of loss of weight or appetite or fever was present. The patient was postmenopausal and had been diagnosed as diabetic and hypertensive and was on oral hypoglycemic drugs and anti­

On examination, the ulcer at the ball of the toe was approximately 2x2x2 cm (Figure 1) with signs of healing, and the ulcer of the right thum b was 1x1x1 cm (Figure 2). There was no active discharge or signs o f inflammation, including redness, temperature increases or tenderness. The multiple nodular lesions of the upper limb digits were approximately 0.5x0.5 cm and calcific in nature. The elbow was diffusely swollen and tender. Fixed flexion deformity was present at the elbow (Figure 3). Blood Tests revealed C-reactive protein (CRP) levels o f 24.0 mg/dl_ (0-6 mg/dL); ESR of 52 mm/hr. (0-20 mm/hr.); serum

Eurasian J Med 2014; 46:131-4

Prabhu et al. Idiopathic Calcinosis Cutis

133

scrotal calcinosis and m ilia-like calcinosis [8]. The diagnosis o f ICC was made in our patient in the absence o f previous trauma, pathologic lesions at site o f calcinosis, history o f parenteral therapy and presence o f calcium in the dermis on histopathology. The calcium and phosphate levels were w ith in norm al limits. Collagen vascular screening was normal. Calcifications in ICC are usually localized to one area but can be seen all over the genitals and the breast [1, 2]. Our patient presented w ith calcinosis in the hands and feet. She also exhibited deposition o f calcium in the subcutaneous tissue around the elbow leading to a perm anent position Figure 4. Histopathology demonstrating the deposition o f calcium salts in the dermis.

o f flexion at the elbow jo in t. Calcinosis at the ball o f fo o t resulted in ulceration over a period o f time.

calcium o f 9.7 m g/dL (8.9-10.3) and serum phosphorus o f

There is no single treatm ent option for ICC. A variety of drugs, mainly bisphosphonates, intralesional corticosteroids,

5 m g/dL (2.4-5.0). Ultrasonography o f the elbow revealed

aluminum hydroxide, warfarin and diltiazem, have been tried

calcinosis. X-ray o f hand and feet dem onstrated calcinosis

w ith limited success [9,10], Local recurrence rates are high w ith

o f the heel and toes and around the interphalangeal jo in t o f

surgical excision and are reserved for painful ulcerated nodules.

the fo o t and hand. In view o f the calcific cutaneous lesions,

A better understanding o f the mechanism o f calcium

a derm atological consult and biopsy was advised to rule out

deposition in ICC is necessary, especially in cases where it is

Hansen's disease. The biopsy revealed the accum ulation o f

diffuse, as in our case. Newer treatm ent options, both m edi­

calcium salts in the dermis (Figure 4).

cal and surgical, have to be form ulated fo r this rare disease so th a t com plications like flexion deform ities, recurrent ulcer­

Treatm ent involved a conservative approach th a t includ­ ed physiotherapy fo r the flexion deform ity, oral clindamycin

ation and m o rb id ity can be avoided.

300 mg 1-1 -1 x10 days fo r M ethicillin-resistant staphylococcus aureus (MRSA), non-steroidal anti-inflam m atory drugs 1-01x10 days for pain relief and daily dressing. The patient was

Informed Consent: W ritten inform ed consent was obtained from the patient w ho participated in this case.

advised to follow -up and undergo excision o f the nodules if

Peer-review: Externally peer-reviewed.

ulcer persists.

Author Contributions: Concept - R.R, Y.S.; Design - R.P.,

Discussion

K.P.; Supervision - R.P., Y.S.; Funding - S.S., Y.S.; Materials - Y.S.,

Calcinosis cutis is divided into the follow ing subtypes:

and/or Interpretation - S.S.,Y.S.; Literature Review - S.S., R e ­

K.P.; Data Collection and/or Processing - S.S., K.P.; Analysis dystrophic, metastatic, iatrogenic and idiopathic. Dystrophic

w ritin g - K.P., R.P.; Critical Review - R.P., Y.S.; O ther - R.P.

calcinosis is the most com m only observed form and usually fol­ lows trauma associated w ith damaged, inflamed and necrotic skin and is also associated w ith connective tissue disorders such as the lim iting form o f systemic scleroderma (CREST syndrome) and dermatomyositis [5], Metastatic calcification usually occurs

Conflict of Interest: No conflict o f interest was declared by the authors.

Financial Disclosure: The authors declared th a t this study has received no financial support.

in situation o f hypercalcemia and/or hyperphosphatemia when

References

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Diffuse idiopathic calcinosis cutis in an adult: a rare case.

İdiyopatik Calcinosis Cutis, deri ve deri altı dokuda kalsiyum tuzlarının çökelmesini içeren bir durumdur. Hastalık sebebi bilinmeyen ve bu nedenle id...
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