CASE REPORT |
https://doi.org/10.5005/jp-journals-10028-1666 |
An Unusual Case of Extensive Dry Gangrene with Mummification of Face, Lower Limbs, and Autoamputation of Nose in Sepsis: A Case Report
1Department of Medicine, Moti Lal Nehru Medical College, Allahabad, Uttar Pradesh, India
2Department of Medicine, Moti Lal Nehru Medical College and Swaroop Rani Nehru Hospital, Allahabad, Uttar Pradesh, India
3Department of Endocrinology, Sawai Man Singh Medical College and Hospital, Jaipur, Rajasthan, India
Corresponding Author: Poonam Gupta, Department of Medicine, Moti Lal Nehru Medical College and Swaroop Rani Nehru Hospital, Allahabad, Uttar Pradesh, India, Phone: +91 2147483647, e-mail: poonam_gupta16@yahoo.com
Received: 09 January 2024; Accepted: 08 April 2024; Published on: 05 July 2024
ABSTRACT
Aim and background: The case is exceptional in view of its rarity, lack of medical literature, unfamiliar clinical presentation, and devastating disease course.
Case description: An 80-year-old male with extensive dry gangrene resulting from disseminated intravascular coagulation (DIC) in urosepsis due to Escherichia coli. The disease course was devastating, and the patient pleaded for euthanasia due to his debilitating illness, which was denied.
Diagnosis: Symmetrical peripheral gangrene (SPG) in sepsis with involvement of face and lower limbs and sparing of upper limbs.
Interventions: Strict medical management in the intensive care unit, and thrombolysis was attempted.
Outcomes: The patient succumbed to the disease within 2 months of the onset of illness.
Clinical significance: Gangrene in sepsis-associated DIC can also involve the face, and the definition of SPG may need an expansion to accommodate that. It is a rapidly progressive condition with a high mortality rate, and a formal consensus needs to be established for appropriate diagnosis and management.
How to cite this article: Chaurasia AK, Gupta P, Purwar N. An Unusual Case of Extensive Dry Gangrene with Mummification of Face, Lower Limbs, and Autoamputation of Nose in Sepsis: A Case Report. J Postgrad Med Edu Res 2024;58(2):84–87.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient for publication of the case report details and related images.
Keywords: Autoamputation, Case report, Dry gangrene, Mummification, Sepsis, Symmetrical peripheral gangrene
INTRODUCTION
Symmetrical peripheral gangrene (SPG) is a rare disorder identified by sudden onset peripheral gangrene, mostly symmetric, involving two or more limbs. Characteristically, it occurs in the absence of any major occlusive vessel disease. It may represent a severe complication of disseminated intravascular coagulation (DIC), usually co-occurring with sepsis.1-3 As evident from available literature, it starts symmetrically from peripheries, first involving the lower limbs and progressing to the upper limbs if not reversed in time.4 There are several known aggravating factors such as diabetes mellitus (DM), enhanced sympathetic tone, immunosuppression, cold injury, and use of vasopressor drugs.5-7 Gangrene of face with extension up to ears leading to mummification and autoamputation has not been included in the definition of SPG, and no such cases have been described in the literature so far.
Symmetrical peripheral gangrene has high mortality (up to 40%)3 and morbidity rates, and no formal consensus has yet been established on definition, diagnostic criteria, and management of SPG. Early diagnosis of SPG and the predisposing underlying factors can have significant impact on the treatment and final outcome. In this article, we attempt to share an intriguing case of an 80-year-old male with SPG, involving face and lower limbs (upper limbs spared), resulting from Escherichia coli urosepsis. Our intention is to expand the definition and clinical presentation patterns of SPG associated with sepsis and DIC and discuss challenges in management and areas of research.
CASE DESCRIPTION
An 80-year-old male presented with progressive blue–black discoloration of skin over face, ears, lower limbs, and buttocks since 2 months; inability to talk and eat since 1 month, and inability to stand or walk since 2 days. On interrogation, the patient revealed ingestion of some drug for an episode of upper respiratory tract infection 15 days before the onset of skin discoloration. Any prior history of hypertension, diabetes, Raynaud’s phenomenon, or autoimmune illness was denied. However, he was a tobacco chewer and had a history of allergy to sulfa drugs. The extensive gangrene started from face, involving nose and philtrum, gradually progressing to cheeks, chin, supraorbital areas, forehead, and ears; simultaneously also started from feet to progressed to legs and later appeared on buttocks. Interestingly, the upper limbs were spared. Gangrene was dry, symmetrical, painful, and resulted in mummification and autoamputation of nose and philtrum, exposing the oral cavity. On examination, vitals were stable, and all peripheral pulses were palpable except bilateral anterior tibial, posterior tibial, and dorsalis pedis arteries, which were feeble. There was no evidence of any suspicious lumps or mass lesions. Systemic examination was unremarkable with no signs of internal malignancy. The patient was thoroughly investigated (Tables 1 and 2).
| S. No. | Investigation | Day 1 | Day 2 | Day 4 | Day 7 |
|---|---|---|---|---|---|
| 1 | Hemoglobin (gm%) | 11.3 | 12.7 | ||
| 2 | TLC (cells/mm3) | 20,100 | 21,000 | 36,000 | 24,000 |
| 3 | DLC | N86.7 L3.5 M9.8 | N86 L10 | N90 L6 | |
| 4 | Peripheral blood film | Platelets reduced | Platelets reduced | Toxic granulations, schistocytes (fragmented RBCs), and platelet count reduced | |
| 5 | Platelets (cells/mm3) | 30,000 | 60,000 | ||
| 6 | Na+/K+ | 148.2/5.05 | 144.1/4.36 | ||
| 7 | Serum urea/creatinine | 144.93/1.82 | 75.87/1.09 | 113.03/1.28 | |
| 8 | Liver functions | WNL | WNL | ||
| 9 | D-dimer (μg/mL) (0.06–0.14) | 4.2 | 1.6 |
DLC: differential leukocyte count; TLC: total leukocyte count; WNL: within normal limits
| S. No. | Investigation | Report |
|---|---|---|
| 1 | Serum fibrinogen (180–380 mg/dL) | 176 mg/dL |
| 2 | Lipid parameters (mg/dL) | TG: 165 |
| TC: 204.65 | ||
| HDL: 36.15 | ||
| LDL: 135 | ||
| VLDL: 33 | ||
| 3 | BT (minutes) | 3.15 |
| CT (minutes) | 4.45 | |
| 4 | PT (seconds) | 21 |
| INR | 2.4 | |
| APTT (seconds) | 28 | |
| 5 | FBS | 176 mg/dL |
| 6 | HbA1c | 6.1% |
| 7 | ESR | 14 mm at 1 hour |
| 8 | RA factor | Negative |
| 9 | CRP | Negative |
| 10 | Urine routine/microscopy | Glucose 3+, Protein 1+, Pus cells 8–10/hpf |
| 11 | Urine microalbumin | 63.60 mg/dL |
| 12 | ANA | WNL |
| 13 | ANCA | WNL |
| 14 | APLA | IgM and IgG WNL |
| 15 | Blood culture/sensitivity | Sterile |
| 16 | Urine culture/sensitivity | E. coli |
| 17 | USG abdomen | Normal scan |
| 18 | Chest X-ray PA | No abnormality detected |
| 19 | Color Doppler of B/L lower limbs | Bilateral isoechoic intraluminal thrombus, arterial narrowing and occlusion of distal ant tibial, post tibial, dorsalis pedis arteries with distal flow insufficiency, diffuse atherosclerosis present |
| 20 | USG carotid doppler | Right carotid: PSU 30 cm/sec; Left carotid: PSU 60.3 cm/sec. B/L normal flow pattern and spectrum with ECA and ICA normal till bifurcation |
| 21 | 2D Echo | LVEF—46%, EPSS raised, dilated cardiomyopathy with no evidence of vegetations |
| 22 | CECT thorax | Multiple small scattered fibrotic parenchymal bands in middle lobe of right lung |
| 23 | Skin biopsy | Normal |
| 24 | Ocular and fundus examination | Immature cataract (LE > RE), fundus WNL B/L |
APTT: activated partial thromboplastin time; B/L: bilateral; BT: bleeding time; CECT: contrast-enhanced computed tomography; CRP: C-reactive protein; CT: clotting time; EPSS: elevated pulmonary systolic pressure; ESR: erythrocyte sedimentation rate; FBS: fasting blood sugar; HDL: high-density lipoprotein; IgM: immunoglobulin M; IgG: immunoglobulin G; LE: left eye; LDL: low-density lipoprotein; LVEF: left ventricular ejection fraction; PA: posteroanterior; PT: prothrombin time; RA: rheumatoid arthritis; RE: right eye; TC: total cholesterol; TG: triglycerides; VLDL: very low-density lipoprotein
Within hours of presentation, thrombolysis was attempted, and the patient was shifted to intensive care unit for further management. Investigations showed persistently rising leukocytes and toxic granulations favoring septicemia. Lower respiratory tract infection and urosepsis were possible foci. Urine culture revealed growth of E. coli, while blood culture was sterile. Raised D-dimer and international normalized ratio (INR) with thrombocytopenia and low fibrinogen levels suggested DIC. Ultrasonography (USG) Doppler revealed isoechoic intraluminal thrombus, arterial narrowing, and occlusion of distal anterior tibial, posterior tibial, and dorsalis pedis arteries with distal flow insufficiency and diffuse atherosclerosis. Deranged renal functions and cardiomyopathy suggested multiorgan dysfunction. Persistently raised fasting plasma glucose and urinary glucose favored stress-induced hyperglycemia. Also, glycated hemoglobin (HbA1c) was only mildly raised. Vasculitis was ruled out on grounds of negative antinuclear antibody (ANA), antineutrophil cytoplasmic antibody (ANCA), antiphospholipid antibody (APLA) profile, and skin histopathology. The patient was managed as per standard treatment guidelines of septicemia, urinary tract infection, and DIC with intravenous broad-spectrum antibiotics, streptokinase, unfractionated heparin, warfarin, cilostazol, pentoxifylline, nifedipine, and intravenous (IV) fluids with strict glycemic control, but still, the patient deteriorated. Due to his debilitating condition, the patient pleaded euthanasia, which was denied, and the patient was discharged against medical advice, on persistent request. Sadly, the patient died at home 5 days after discharge from the hospital (Figs 1 to 4).
Fig. 1: Frontal view showing gangrenous face involving cheek, eyelids, supraorbital areas, forehead, ears with autoamputated tip of nose and philtrum
Fig. 2: Dry gangrene of buttocks with clear line of demarcation
Fig. 3: Bilateral lower limb gangrene with definite line of demarcation
Fig. 4: Upper limb sparing with no gangrenous involvement
DISCUSSION AND CONCLUSION
Symmetrical peripheral gangrene was first described by Hutchison in 1891.8 Despite this being a century ago, literature has descriptions of SPG solely as individual case reports.9-11
On the basis of above clinical findings and investigations, following differentials were kept: DIC in septicemia, SPG, drug-induced vasculitis, atherosclerotic arterial disease, thromboembolic gangrene, and thromboangiitis obliterans.
Purpura fulminans, though looks similar but does not appropriately depict this clinical scenario. Purpura fulminans is characterized by acute onset, rapidly progressive purpuric skin lesions leading to necrosis and gangrene of limbs or digits along with multi-organ dysfunction.12 It is a fast-progressing syndrome of skin microvascular thrombosis and accompanying hemorrhagic necrosis.13
Initially, peripheral symmetric gangrene was kept as probable diagnosis but predominant involvement of face and philtrum has yet not been described in SPG, and also the patient was elderly with abnormal color Doppler study.1,2 Atherosclerosis and thromboembolic disease were scored out in view of marked facial gangrene despite normal two-dimensional (2D) echocardiography and carotid doppler. There was a history of unknown drug intake for upper respiratory tract infection, and the patient had a history of sulfa drug allergy. We could not attribute it to drug-induced vasculitis as skin biopsy was normal and even after stoppage of inculcating drug, the gangrene kept on progressing instead of expected improvement.14 Vasculitis was ruled out in view of negative ANA, ANCA, APLA profile, and cutaneous histopathology.
In our case, the infective organism E. coli was the culprit, a well-known cause predisposing to SPG.10 The etiopathogenesis of SPG may involve multiple mechanisms namely bacterial endotoxin release, platelet plugging, Shwartzman reaction, and DIC.15 The areas of predilection in SPG are upper limbs, lower limbs, tip of nose, border of ears, genitalia, and scalp.3,16 Unfortunately, SPG survivors suffer from high risk of limb amputation (autoamputation or surgical amputation).10 Thus development of SPG, especially on the background of DIC proves to be an ominous prognostic sign.17
CLINICAL SIGNIFICANCE
Till date, very few cases have been reported with the clinical presentation of gangrene in DIC, as most cases usually present with bleeding manifestations, due to consumption of platelets and coagulation factors and further aggravation by secondary fibrinolysis. Here DIC was suggested due to markedly raised D-dimer levels, deranged INR values, thrombocytopenia, and schistocytes [fragmented red blood cells (RBCs)].18,19 Leukocytosis, which was persistently rising, and toxic granulations on peripheral blood film favoring sepsis. Chronic DIC characterized by slow evolution over weeks or months, long lasting and usually does not lead to bleeding, can be kept as a possibility. Though cancers are the most frequent cause of chronic DIC, we could not trace any evidence of malignancy in our patient.20 The case is exceptional in view of its rarity, lack of medical literature, unfamiliar clinical presentation, and devastating disease course without an apparent etiology.
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