19 year old male resident of Nalgonda and currently studying intermediate ,came to opd with complaints of :
-Itchy Ring leisons over arms ,abdomen ,thigh and groin since 1 and half year .
-Purple stretch marks all over abdomen ,lower back ,upper limbs ,thighs since 1 year .
-Abdominal distension and facial puffiness since 6 months.
- Pedal edema since 3 months.
- Low back ache since 3 months .
- Feeling low , not feeling to talk to anyone.
- Weight gain and decreased libido since 3months.
- Loss of libido and erectile dysfunction since 2 months .
Pt was apparently alright one and half year ago , when he slowly developed erythematous round leisons which are annular shaped and itchy all over abdomen , upper limb ,groin and inner thigh region .
No history of fever back then. No other complaints apart from skin lesions.
Pt visited local RMP where he prescribed auyurvedic medications and other creams ( unknown composition as pt don't have them currently ). He also prescribed tablets (unknown composition) . Patient started using all these medications for 1-2 months .
Leisons reduced a bit after using medications .
Later after 2 months he developed multiple hyper pigmented plaques over lower limbs ,abdomen , for which he again visited same place and used ayurvedic oils over the leisons.
He also used clobetasol ointment over the leisons.(for approximately 1 year all over the body)
He started noticing pink striae over his abdomen first 1 year ago and later on back and over arms,which were gradually increasing in size .
Later he visited a hospital and used miconazole and luliconazole ointments also.
He used clobetasol ointment all over the leisons for long time .
He started noticing abdominal distension and facial puffiness ,weight gain, but never visited any hospital.
Later he developed pedal edema and low back ache since 3 months .
His consulted a dermatologist at this point of time who advised to consult physician and prescribed monteleukast , itraconazole tablets ,luliconazole ointment for tenia corporis.
He stopped all medications one month ago and visited our opd with complaints of pink striae and easy fatigue ,weakness and low back ache .
His brother also gave history of pt being moody and feeling of low self esteem due to multiple leisons.
He even complaints pt wouldn't step out of house and always stays indoor and wouldn't interact with others .
No complaints of chest pain ,sob , palpitations .
No complaints of decreased or frothy urine.
No other negative history.
No h/o DM,HTN,TB,ASTHMA,CAD.
ALLERGIC HISTORY - pt gives h/o allergy to eggs ,brinjal .
O/E : Pt was c/c/c
BP - 160/100 mmHg
Pr - 96 BPM ,regular ,normovolemic .
Rr - 18/min
Spo2- 98% on ra.
Weight - 63 kg.
Height - 175 cm.
GENERAL EXAMINATION :
NO pallor ,icterus ,cyanosis , clubbing, lymphadenopathy.
Pedal edema present - pitting type extending upto knee.
Abdominal distension present.
Moon face present
Pink striae noted over anterior abdominal wall and on low back and on upper arms and thighs.
Thin skin present .
Poor healing noticed over leg ulcers and easy bruising noted .
Acne present over face .
Acanthosis nigrans noted over neck.
GYNECOMASTIA PRESENT .
neck pad of fat present .
Sparse scalp hair .
.
Skin examination - Multiple itchy erythematous annular leisons noted all over abdomen , upper limb ,groin and inner thigh region .
Multiple hyperpigmented plaques noted over bilateral lower limbs .
SYSTEMIC EXAMINATION :
CVS - S1S2 heard .No murmurs
RS - BAE present .
No adventitious sounds .
P/A - Soft , distended .
No organomegaly .
Bowel sounds present .
CNS - HMF - INTACT R. L
MOTOR SYSTEM - POWER - UL 5/5 5/5
LL 5/5 5/5
Proximal muscles lower limb - power is 4/5 .
TONE - NORMAL.
REFLEXES - B. T. S. K. A. P
R. +2 +2. +1. +2. +1. FELXOR
L. +2. +2. +2. +2. +1. FLEXOR.
CRANIAL NERVES - NORMAL .
Difficulty in getting up from chair was noticed.
PROVISIONAL DIAGNOSIS -
? IATROGENIC CUSHINGS SYNDROME .
TINEA CORPORIS .
DENOVO HTN .
INVESTIGATIONS :
CBP - HB - 13.4 g/dl
TLC - 6,800
PLT - 1.5 lakhs.
RBS - 139 mg/dl
CUE - ALBUMIN - +1
SUGARS - NIL .
PUS CELLS - 3-4
RBC - NIL .
LFT - TB -1.03
DB-0.21
ALBUMIN - 3.9
RFT - UREA - 22
SERUM CREATININE -0.6
ELECTROLYTES - NA - 136
K- 4
CL-98
USG ABDOMEN - NORMAL.
ECG - SINUS TACHYCARDIA
LVH PRESENT.
This was picture of striae one year ago when it gradually started :
On presentation to opd pictures : 28/05/21
We took dermatologist opinion for tenia corporis where they advised
Ointment AMLORFINE
FUSIDIC ACID CREAM.
SALINE COMPRESS OVER LEISONS.
Plan to start anti fungals on next visit once dose of steroids is reduced .
OPTHAL opinion Was taken to look for visual acuity and cataract .
No features of lens opacities noted .
BUT IOP was high ,where they advised to follow up .
We advised pt to get fasting 8am serum cortisol levels and was planned to start on low dose steroids to avoid adrenal crisis.
8AM S CORTISOL LEVELS (30/5/21)
- 0.46 mcg/dl ( very low) .
( normal range - 4.3-22.4 mcg/dl).
In view of lvh pt was started on tab telma 20 mg od .
On 3/6/21 - ACTH STIMULATION TEST WAS DONE .
BY INJECTING 0.4 ML OF ACTOM PROLONGATUM INJECTION (ACTH) INTRA MUSCULAR @ 7am
1 HR LATER FASTING SERUM CORTISOL SAMPLE WAS SENT .
VALUE - 0.73 mcg/dl
Indicating there was HPA AXIS suppression and pt was started on TAB HIZONE 15 mg per day in three divided doses @ 8am ,12 pm and 4 pm.
Pt was asked to follow up after one month .
FINAL DIAGNOSIS :
IATROGENIC CUSHINGS SYNDROME SECONDARY TO TOPICAL CLOBETASOL APPLICATION ALL OVER BODY FOR APPROXIMATELY ONE YEAR.
TINEA CORPORIS
DENOVO HTN .
FOLLOW UP -
ON NEXT VISIT : ( 25/6/21).
Pt was symptomatically better , pedal edema subsided.
Striae were pale in color and we're subsiding.
Weight - 67kg
Ht -175 cm.
Bp- 160/100 mmHg.
Pr -88bpm.
Dose of Tab hizone was reduced to 10 mg per day in divided doses for one month.
In view of low back ache Xray LS spine was done which was normal and pt was advised.:
Tab Shelcal 500 OD and Tab Vit D 3 Od.
Tab ULTRACET /PO/SOS.
Psychiatry opinion was taken and he was diagnosed with moderate depression .
ON JULY 5 - Patient came for follow up in dermatology opd , IMPROVEMENT SEEN . PT COMPLAINING OF NEW RING LEISONS OVER LEGS . PUS DISCHARGE NOTED OVER ABDOMINAL STRIAE WITH ERYTHEMATIZATION .
THEY ADVISED ,
- TRETIN GEL 0.05 % L/A OD X 2 weeks
- TAB ATARAX 10MG OD
-AMOROLFINE CREAM L/A BD X 5 DAYS
-CAP AMOXICLAV 625 MG BD X 5 DAYS
FUSIDIC CREAM L/A BD X 5 DAYS.
PLAN - TO START ANTIFUNGALS ONCE HIS TOTAL DOSAGE OF HISONE IS 5 MG/DAY.
In July 2021 pt was complaining of fever ,sore throat and dry cough since 3 days and he was tested positive for COVID 19 , we advised him home isolation and PCM 650 Mg /po /tid x 5 days .
He was advised to continue tab hizone tablets as it was advised. ( 10mg/day in divided doses.)
-INJ HYDROCORTISONE 100mg sos if pt is in adrenal shock.
He recovered from COVID within one week .
Next visit : ( 6/8/21).
BP- 170/100 - TELMA DOSE WAS INCREASED TO 40 MG OD.
PR - 88bpm.regular , normovolemic.
Wt- 69 kg
Height -
Abdominal girth - 96cm
Pt complaints of excoriation over striae and appearance of erythematous macules over abdomen whenever he takes food he is allergic to.
Took dermatologist opinion for it . They started him on Tab Itraconazole 100 mg bd for 2 weeks. And lulifin cream and tab levocitrixine 5mg od.
His brother complaints of depressed mood , pt not going out due to social stigma. Psychiatric counselling was given .
He still complaints of low back ache..othropedics opinion was taken and advised to continue Ultracet and tab Shelcal .
Cbp , cue and electoltes were repeated which were all in normal range .
USG ABDOMEN was done - Normal kidney size bilateral and CMD maintained. No other sonological abnormality noted.
As his lesions dint subside we reduced dose of hisone to 7.5 mg per day ,to see response.
Review psychiatry and opthal opinion was taken , where his lens was clear - no e/o cataract .
no retinopathy changes and no raised IOP and his visual acuity was 6/6.
0N 20/8/21 - IN view of constant low back ache , MRI LS SPINE WAS done with whole soine screening .
which showed lumbar epidural lipomatosis .
Mild depression of superior endplates of D12 and L4 - Likely subacute /chronic compression .
L3-L4 disc degeneration with mild diffuse disc bulge causing no significant neural foramen stenosis .
ON 24/8/21 - Pt developed painful swelling and redness of right lower limb along with intermittent fever - DIAGNOSED TO HAVE RIGHT LOWE LIMB CELLULITIS .
Pt was admitted and was given IV AUGMENTIN for 5 days and MGSO4 dressings were done .
TAB chymerol forte was given for 5 days.
Tab hisone 7.5 mg was continued in divided doses .
-INJ HYDROCORTISONE 100mg sos if pt is in adrenal shock.
PT again came for follow up in september , complaints of weight gain , but striae reduced and patient attender was giving history of patient having low mood and constantly being at home and not doing any exercise .
He was having social inhibition and was avoiding contact with other people.
We sent him for psychiatry where brief counselling was given .
TAB HISONE dose was reduced to 5 mg /day.
Next follow up was in october (22/10/11) ,pt improved and dose was further reduced to 2.5 mg/day .
PT again visited to our OPD on 23/ 11/21 , again with
c/o increasing striae and abdominal distension.since one month .( Striae were same as before ,but pt was feeling that way )
Abdominal distension could be due to fat accumulation,as pt was hardly exercising and is only staying at home and eating food.
weight gain present .( 76kg ).
c/o diminished vision
BP-160/100 mmhg
PR - 110 bpm
CVS- S1 S2 PRESENT
RS - BAE present and clear .
Pt was giving history of good compliance to medications and was using tab hisone 2.5mg only since past one month .No history of other medications ( ayurvedic or homeopathic ) usuage .
He is still having social inhibiton and is not going out of house or doing regular exercise .
P is having lot of anxiety and psychiatric issues.
As his blood pressure is uncontrolled and ECG having LVH - we increased the dose of anti-htn to TAB TELMA AM 40/5 mg OD.
HIS ROUTINE investigations were repeated , cbp,s. electrolytes ,s.creat was normal .
HIS RBS was 178 mg/dl
HBA1C was 6.5 %
Review Dermat opinion was taken ,
PT dint use his cream and itraconazole tablets regularly last month .
Review psychiatry opinion taken - Adjustment issues due to underlying illness.
Brief psychotherapy given .
REVIEW ENDOCRINE OPINION TAKEN (26/11/21) : Pt apprehensive .
Stopped hisone 2.5 mg and observe patient.
Only stress dose - Inj hydrocortisone 100 mg IV IF PT IS IN Adrenal shock .
Adv -8am serum cortsiol
Inspite of using the minimal dose of tapering steroids , pt has all these complaints , so a diagnostic uncertainity was prevailing .
1) COULD THIS PT HAVE UNDERLYING ENDOGENOUS CUSHINGS ?
A) In this patient ,it is not possible as his basal cortisol and post ACTH stimulation ,his serum cortisol levels were very low .(so ruling out possibility of endogenous cushings).
-( It is very rare to have this co-occurence of endogenous and exogenous cushings syndrome , although one such case was reported from malasiya .
But in this patient ,her basacl cortisol levels were not available/done .So hence they missed diagnosis .
We present a case of a 66-year-old lady who was noted to have typical features of Cushing's syndrome. As she gave a very clear history of ingesting exogenous GC for a year, no further work up was undertaken. Despite cessation of GC for a year, she continued to have thin skin and easy bruising. Upon admission for hypertensive emergency, her clinician took note of her changes and investigated her for endogenous Cushing's syndrome. Her cortisol post overnight dexamethasone suppression test was 707 nmol/l. Post low dose dexamethasone suppression test yielded a cortisol of 1133.2 nmol/l. 24 hours urine cortisol was 432.2 nmol/l. Plasma ACTH was 1.1 pmol/l, indicating an ACTH independent Cushing's syndrome. We proceeded with Computed tomography scan (CT scan) of adrenals which revealed a right adrenal adenoma measuring 4.4 × 3.4 × 4.0 cm. Right retroperiteneoscopic adrenalectomy was done. Histopathology examination was consistent with adrenal cortical adenoma with foci of myelolipoma. Post adrenalectomy she developed hypocortisolism secondary to contralateral adrenal suppression which lasted up to the present date. Her cutaneous and musculoskeletal manifestations improved substantially. Co-occurrence of endogenous and exogenous Cushing's syndromes is uncommon but should be considered in patients whose Cushingnoid features do not resolve after cessation of exogenous GC. )
2) OR is patient using other steroids/ ayurvedic/homeo medications ,which he is concealing the history.
is he doing it for medical attention ?
is he using the medications as prescribed or using double doses ?? although pt and his attender denies any such history .
all these questions remain un-answerwed .
Current plan - to admit the patient and stop tab hisone 2.5 mg and monitor him for any adrenal crisis.
fasting 8 am serum cortisol to be sent again .
Hisone 2.5 mg tablet was stopped for 2 days. No symptoms as of now.
26/11/21 - Repeat 8am fasting serum cortisol is 0.54 mcg/dl
ACTH stimulation test was done . Report awaited .
1/12/21 -Pt was observed for one week and had no episodes of hypotension .
So after taking patient and his attenders consent ,and explaining them about condition and course and prognosis of disease , tapering dose of steroids was stopped .
Pt was explained to take stress dose of steroid INJ HYDROCORTISONE 100mg IV STAT ,in case of fever,acute illnesses, syncope and hypotension .
Pt again followed up with us in December . Had no complaints and was still having low mood and was not having any physical activity .
His vitals were stable . Good appetite .
On 21/1/22 -
Pt visited our hospital again with complaints of giddiness and vomiitings since 3 days and loss of appetite since 1 week .
His sbp on presentation was 40 mmHg and ot was rushed to casualty and immediately IV HYDROCORTISONE 100 mg was given and fluid resuscitation was done . Following which his blood pressure improved .
He was admitted into ICU and intense monitoring was done along with IV HYDROCORTISONE 100 MG 6 th hourly for 2 days. Plan to shift to oral hydrocortisone 15 mg later and send 8 am Serum cortisol levels and ACTH stimulation test to be done .
He started of with Exogenous cushings syndrome, but a more pressing problem right now is HPA -axis suppression and secondary adrenal insufficiency .
Inspite of using the minimal dose of tapering steroids , his HPA- Axis wasn’t improving .
Possible reasons could be :
1)Obesity
2)Concomitant usage of Anti-fungal drugs ,which can reduce metabolism of exogenous steroid (Tab.Hisone) by inhibiting CYP 3A 4 . So this causes prolonged HPA-Axis suppression and thereby reduced serum cortisol levels.
3)Is Patient using other steroids/ ayurvedic/homeo medications ,without our knowledge and is concealing the history. Is he doing it for medical attention ?
4) Is he using the medications as prescribed or using double doses ?? although pt and his attender denies any such history .
All these questions remain un-answered .
DISCUSSION :
Sequence of images of our current patient :
MAY -2022 :
NO complaints. Weight reduced .
CUSHING'S SYNDROME :
CLASSIFICATION OF CUSHINGS SYNDROME :
APPROACH TO A CASE OF CUSHINGS SYNDROME :
NIEMAN LK,BILLER BM et al. THE DIAGNOSIS OF CUSHINGS SYNDROME,J CLIN ENDOCRINE METABOLISM.2008;93:1526-1540.
POST –LDDST SERUM CORTISOL VALUE >1.8 mcg/dl – SUGGESTS CUSHINGS SYNDROME.
Source : WILLIAM TEXTBOOK OF ENDOCRINOLOGY 14TH EDITION .
MANAGEMENT OF EXOGENOUS CUSHINGS SYNDROME :
Source : Hopkins, Rachel L.; Leinung, Matthew C. (2005). Exogenous Cushing's Syndrome and Glucocorticoid Withdrawal. , 34(2), 371–384.
MANAGEMENT OF ENDOGENOUS CUSHING'S SYNDROME:
Medical management:
R.PIVONELLO et al-Treatment of cushings disease. Endocrine Reviews, Volume 36, Issue 4, 10 August 2015
Supporting evidences :
1)
CASE REPORT : Janiel Pimentel; Chirag Kapadia et al-ADRENAL SUPPRESSION SECONDARY TO INTERACTION OF COMBINED INHALED CORTICOSTEROID AND ANTIFUNGAL AGENT .AACE CLINICAL CASE REPORTS Vol 4 No. 4 July/August 2018 .
Inhaled corticosteroids (ICS) are exogenous glucocorticoids that typically have minimal systemic effects at standard doses. They are metabolized by the cytochrome P450 (CYP450) enzyme pathway in the liver.
Posaconazole, is a known CYP450 inhibitor.
P- 9-year-old Caucasian female with history of hyper IgE syndrome. On chronic fluticasone and posaconazole for Aspergillus infection, she presented with fatigue and “facial puffiness” (cushingoid features).
8AM cortisol was (0.3 μg/dL) and Post (ACTH)- stimulation test cortisol level < 1 μg/dl.
I- Started on physiologic dose of hydrocortisone (10 mg/day) and fluticasone and antifungals discontinued. Hydrocortisone supplementation was slowly weaned over a 7-month period.
O- After weaning hydrocortisone, repeat low-dose ACTH-stimulation test with peak cortisol of 21.8 μg/dL, consistent with resolution of AS.
2
) Skov, K.M main et al -Iatrogenic adrenal insufficiency as a side-effect of combined treatment of Itraconazole and Budesonide. European Respiratory Journal 2002 20: 127-133;
P-
CASES -37 cystic fibrosis patients with ABPA , out of which 25 CF patients treated with both itraconazole and budesonide, and in 12 patients treated with itraconazole alone .
Controls -30 cystic fibrosis patients.
I – ACTH –stimulation test was done in both cases and controls and cortisol levels were measured .
C- Eleven of the 25 patients treated with both itraconazole and budesonide had adrenal insufficiency. None of the patients on itraconazole therapy alone nor the control CF patients had a pathological ACTH test. Mineralocorticoid and gonadal insufficiency was not observed in any of the patients.
These patients improved but had not achieved normalized adrenal function 2–10 months after itraconazole treatment had been discontinued.
3
)Tempark T, Wananukul S et al - Exogenous Cushing's syndrome due to topical corticosteroid application: case report and review literature. Endocrine. 2010 Dec;38(3):328-34
Prolonged use of topical corticosteroids causes systemic adverse effects including Cushing's syndrome and hypothalamic-pituitary-adrenal (HPA) axis suppression, which is less common than that of the oral or parenteral route.
P- 43 cases with iatrogenic Cushing syndrome from very potent topical steroid usage (Clobetasol) in children and adult have been studied.
In children group (n = 22), most are infants with diaper dermatitis. For the adult group(n = 21), the most common purpose of steroid use was for treatment of Psoriasis.
The recovery period of HPA axis suppression was 3.49 ± 2.92 and 3.84 ± 2.51 months in children and adult, respectively.
4) Varshney I, Amin SS, Adil M, Mohtashim M, Bansal R, Khan HQ. Topical Coticosteroid Abuse- Risk factors and Consequences. JDA Indian Journal of Clinical Dermtology. 2019;2:72-77.
Topical corticosteroids are one of the most widely prescribed class of topical drugs. They have been abused in developing countries as they provide rapid symptomatic relief in inflammatory dermatoses.
Aim: This study was done to find the risk factors related to topical corticosteroids abuse and consequences thereof.
Methods : A hospital-based cross-sectional study was conducted in the dermatology OPD from July 2017 to June 2018.
P - 2032 patients abusing topical steroids, 1365 (67.2%) patients were recorded with adverse effects of topical corticosteroids.
The majority (60.78%) were from urban areas. 20.9% were abusing topical steroids for more than 12 months
O- The most common abused steroid formulation was mixed combination consisting of steroids, anti-fungals and anti-bacterials (47.9%).
Fungal infection (59.5%) and acne (15.3%) were the most common indications of steroid abuse. Quacks (31.9%) and pharmacists (26.8%) were the most common prescribers of these topicals .
Most common adverse effects of topical corticosteroid abuse were tinea incognito (41.1%), steroid-induced acne/ aggravation of acne vulgaris (18.2%) and telangiectasia (14.1%).
Most common source of abuse was non-prescriptional and it was mainly due to easy availability of mixed combinations of steroid containing creams and lack of public education regarding its adverse effects.
So final take home message is STOP steroid abuse and public health education and awareness regarding the lethal effects of usuage of non -prescriptional medications and long term usuage of topical applications .
Only awarness would save one's life .
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