| Abstract|| |
Mucormycosis is a relatively rare fungal infection seen in immunocompromised patients. Very few cases of invasive cutaneous mucormycosis occurring in neonates have been reported in literature. It is an aggressive disease with a mortality rate of around 64% in neonates, so a high index of suspicion is essential for rapid diagnosis and definitive treatment with broad-spectrum antifungals such as Amphotericin B. We present a case of a premature infant born at 25 weeks of gestation who developed vesicobullous lesions all over the body on day 5 of life. Biopsy from the vesicles confirmed the presence of angioinvasive fungal hyphae of mucormycosis which were highlighted on Periodic acid-Schiff and Grocott stain.
Keywords: Mucormycosis, neonate, prematurity
|How to cite this article:|
Mishra S, Shelly D, Gupta D, Bharadwaj R. Invasive cutaneous mucormycosis in a preterm neonate presenting as a vesicobullous lesion. Indian J Pathol Microbiol 2018;61:103-5
|How to cite this URL:|
Mishra S, Shelly D, Gupta D, Bharadwaj R. Invasive cutaneous mucormycosis in a preterm neonate presenting as a vesicobullous lesion. Indian J Pathol Microbiol [serial online] 2018 [cited 2020 Jul 9];61:103-5. Available from: http://www.ijpmonline.org/text.asp?2018/61/1/103/228196
| Introduction|| |
Mucormycosis is an uncommon, opportunistic, and frequently fatal mycosis caused by fungi of the class Zygomycetes. Cutaneous mucormycosis is the third most common clinical form of the disease, after pulmonary and rhino-cerebral. Very few cases of invasive cutaneous mucormycosis occurring in neonates have been reported in the literature. In neonates, predisposing factors are prematurity, low birth weight, and use of corticosteroids for respiratory distress. Damage to the skin from adhesive tapes and invasive catheters are additional risk factors., We hereby report a case of cutaneous mucormycosis in a premature neonate, who presented with vesicobullous lesions, a rare presentation.
| Case Report|| |
Our patient, a case of twin pregnancy was born to a HIV negative and nondiabetic mother at 25 weeks of gestation by cesarean section. The second twin was stillborn. The neonate weighed 500 g at birth (extremely low birth weight) and had complaints of respiratory distress for which he was intubated and mechanical ventilation was started. Surfactant and dexamethasone were administered to treat respiratory distress. A central line was established for venous access to provide fluid and nutritional support; which was covered with an adhesive tape. On the 3rd day of life, the child developed fever for which injectable cefotaxime and gentamicin were started empirically for suspected neonatal sepsis. On the 5th day of life, the baby developed multiple variable-sized vesicobullous and pustular lesions all over the body with some showing ulceration and crusting. At this stage, the common causes of infectious vesicobullous disorders with a differential of congenital herpes, cytomegalovirus, candida and bacterial dermatosis (Streptococcus and Staphylococcus) as well as drug-induced vesicobullous disorders were considered. Skin scrapings of the blisters for Tzanck smear were done which did not show any giant cells or features suggestive of herpes. Bacterial cultures of blister fluid were negative.
Consequently, a skin biopsy was taken from the vesicle and adjoining ulcerated skin. On Hematoxylin and Eosin (H and E) examination, the epidermis was sloughed off and the dermis and subcutaneous tissue showed moderate mixed inflammatory infiltrate. Broad, pauci-septate fungal hyphae with right angle branching were seen involving the whole of the dermis and underlying subcutaneous fat. Characteristic angioinvasion of dermal blood vessels by these fungal hyphae was also present [Figure 1]. Periodic acid-Schiff (PAS) and Grocott stains were done which highlighted the presence of these fungal hyphae [Figure 2]. Based on the morphology of fungal hyphae and angioinvasion, diagnosis of invasive cutaneous mucormycosis was given. The patient was started on systemic Amphotericin B and debridement of necrotic areas was done. However, the condition of patient continued to worsen, and despite antifungal therapy, he succumbed to his illness.
|Figure 1: Section showing sloughed epidermis with the presence of fungal hyphae involving dermis and subcutaneous tissue. These fungal hyphae are highlighted on Periodic acid-Schiff and Grocott stains (×40)|
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|Figure 2: Section showing characteristic broad, pauci-septate hyphae of mucormycosis which are better appreciated on Periodic acid-Schiff and Grocott stain. The presence of angioinvasion is also seen (Right lower figure)|
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| Discussion|| |
Mucormycosis is an acute, fulminant and fatal fungal infection, occurring primarily in immunocompromised individuals. Diabetes mellitus, thermal burns, corticosteroid therapy, neutropenia, malignancy, malnutrition, deferoxamine therapy and metabolic acidosis are the common predisposing factors in adults. Risk factors and presentation in infants are slightly different from adults, and the most important predisposing factor has been identified as prematurity and low birth weight apart from corticosteroid use for respiratory distress, intravenous catheterization and broad-spectrum antibiotic use for presumed sepsis. Inoculation from wooden tongue depressors and adhesive cutaneous dressing has also been reported., Many manufacturers now treat these dressings with cobalt radiation to prevent inoculation by mucor.
Cutaneous mucormycosis in children is very rare. Oh and Notrica, in their review of cutaneous mucormycosis, had reported only 18 cases of cutaneous mucormycosis in infants. The common risk factors in these cases were prematurity, low birth weight, broad-spectrum antibiotics, corticosteroid therapy, and local trauma to the skin site. In our case, mucormycosis emerged in a very similar clinical setting. The baby was born prematurely at 25 weeks of gestation and had an extremely low birth weight of 500 g. He had respiratory distress at birth and corticosteroid therapy was administered which again is a cause of immunosuppression. Broad spectrum empirical antibiotics were given for presumed sepsis. Disruption of skin integrity by local trauma due to central line insertion and use of adhesive tape might have served as the route of entry of pathogen inside the skin.
Primary cutaneous mucormycosis presents in two different forms. The superficial type appears as vesicles and pustules which may ulcerate and lead to eschar formation. Patients with low immunity have a more rapidly progressive and disseminated disease with extensive ulceration and gangrene; known as the gangrenous type, which is often fatal.
Our patient presented with vesicobullous lesions. Common differentials considered are congenital herpes, varicella, CMV, candida and bacterial infections caused by Group A Streptococcus and Staphylococcus. Mucormycosis is an uncommon cause and index of suspicion is very low. Congenital herpes simplex infection is identified by a history of herpes infection in the mother especially during the third trimester and demonstration of multinucleated giant cells on Tzanck smear. Neonatal candidiasis is a perinatally acquired candida infection acquired during passage through birth canal and manifesting on the 6th day of life. It often presents as oral thrush and pustular eruptions on flexural aspect. Diagnosis can be made by identification of spores and pseudohyphae of Candida albicans in skin scraping and culture of the organism. Bacterial infections can be diagnosed by culture of Streptococcus and Staphylococcus from blister content. Diagnosis of mucormycosis in an appropriate setting can be confirmed by identifying the characteristic broad pauci-septate fungal hyphae having right angle branching on H and E staining. Vascular invasion with thrombosis of blood vessels and necrosis of tissue is hallmark of mucormycosis. Rapid identification of the fungus can be done rapidly by 10% KOH wet mount. The use of special stains such as Grocott and PAS improve visualization of fungus. Early recognition, systemic Amphotericin B (1–1.5 mg/kg/day), and surgical debridement is the choice of treatment in this highly fatal condition. Amputation may also be required in severe cases.
We have presented this case to emphasize that vesicobullous lesion in a premature neonate who has associated risk factors of very low birth weight, corticosteroid use, disruption of skin integrity due to use of intravenous catheters and use of adhesive dressing or wooden tongue depressors should be viewed with extreme caution. Invasive cutaneous mucormycosis presenting as a vesicobullous lesion is very rare, but is associated with high mortality. A high index of suspicion and early diagnosis by biopsy followed by prompt treatment with debridement and Amphotericin B cannot be overemphasized.
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Department of Pathology, Armed Forces Medical College, Sholapur Road, Pune - 411 040, Maharashtra
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2]