Sclerema Neonatorum Research Articles

Subcutaneous Nodules in a 28-week-old Girl.

Subcutaneous Nodules in a 28-week-old Girl.

Subcutaneous fat necrosis of newborn - A rare case report of lobular panniculitis in a neonate

Subcutaneous fat necrosis of newborn is a rare cutaneous disorder affecting neonates. It usually presents as subcutaneous nodules or plaques, within the first few weeks of life, following an eventful delivery. It is characterized by hypercalcemia, which may present with lethargy, irritability, hypotonia and dehydration, mimicking sepsis. Histopathology is proven to be the gold standard in diagnosis with characteristic lobular panniculitis, mixed inflammatory cell infiltrate and radially arranged crystals. This needs to be differentiated from other causes of lobular panniculitis, as early diagnosis and treatment to prevent long‑term complications are advocated. Education of parents regarding the disease and danger signs of hypercalcemia and weekly monitoring of serum calcium is recommended. Treatment based on rehydration, dietary vitamin D and calcium restriction, Furosemide and prednisolone are considered. We have discussed a case of subcutaneous fat necrosis, in an 8-week-old male baby.Key Messages: Subcutaneous fat necrosis is an important differential in neonates presenting with palpable subcutaneous nodules, along with sclerema neonatorum. Severe complications like hypercalcemia should be detected early and managed aggressively to prevent morbidities and mortalities associated with it. Symptomatic management, use of calcium lowering drugs and regular monitoring of calcium levels are recommended.

Risk Factors for Mortality in Neonatal Gastric Perforation: A Retrospective Cohort Study.

Background: Neonatal gastric perforation is a rare but life-threatening issue. The aim of this study was to describe the clinical characteristics and prognosis of patients with neonatal gastric perforation and identify predictive factors for poor prognosis.Methods: This was a retrospective cohort study of patients with neonatal gastric perforation treated in a tertiary pediatric public hospital between April 2009 and October 2020. The enrolled patients were divided into survival and non-survival groups. Demographic information, clinical characteristics, laboratory and imaging features, and outcomes were collected from the electronic medical record. Univariate and multivariate logistic regression analyses were performed to obtain the independent factors associated with death risk. Additionally, we separated this population into two groups (pre-term and term groups) and explored the mortality predictors of these two groups, respectively.Results: A total of 101 patients with neonatal gastric perforation were included in this study. The overall survival rate was 70.3%. Seventy-one (70.3%) were pre-term neonates, and sixty-two (61.4%) were low-birth-weight neonates. The median age of onset was 3 days (range: 1–11 days). Abdominal distension [98 (97.0%) patients] was the most common symptom, followed by lethargy [78 (77.2%) patients], shortness of breath [60 (59.4%) patients] and vomiting [34 (33.7%) patients]. Three independent mortality risk factors were identified: shock (OR, 3.749; 95% CI, 1.247–11.269; p = 0.019), serum lactic acid > 2.5 mmol/L (5.346; 1.727–16.547; p = 0.004) and platelet count <150 × 109/L (3.510; 1.115–11.053; p = 0.032). There was a borderline significant association between sclerema neonatorum and total mortality (4.827; 0.889–26.220; p = 0.068). In pre-term infants, serum lactic acid > 2.5 mmol/L and platelet count <150 × 109/L remained independent risk factors for death. In term infants, the incidence of shock, coagulopathy, pH < 7.3, serum lactic acid > 2.5 mmol/L, and hyponatremia were statistically different between non-survival and survival groups.Conclusion: Shock, hyperlactatemia, and thrombocytopenia are independently associated with an increased risk of death in patients with neonatal gastric perforation. Identification of modifiable risk factors during the critical periods of life will contribute to the development of effective prevention and intervention strategies of neonatal gastric perforation.

Sclerema neonatorum associated with early onset neonatal sepsis

Sclerema neonatorum associated with early onset neonatal sepsis

Neonatal Skin Emergencies.

Although the majority of neonatal skin rashes can be safely monitored without intervention, there are a significant few that are dermatologic emergencies. When called to assess a neonate, it is important to distinguish what requires immediate diagnosis and treatment from those that represent benign etiologies. The skin may be the first clue to certain infections such as herpes simplex virus, syphilis, varicella, cytomegalovirus, fungal infections, and staphylococcal scalded skin syndrome, all of which require immediate testing and some of which may lead to severe sequelae. Cutaneous findings in neonates may also indicate the need for further evaluation. Purpura fulminans, sclerema neonatorum, neonatal lupus, and blueberry muffin rash can be indications of other underlying disorders and are reviewed as well. This article outlines these potential neonatal dermatologic emergencies and highlights the important clinical clues to each. [Pediatr Ann. 2019;48(1):e36-e42.].

Neonatal Cellulitis

Introduction: Newborn cellulitis is rare and often atypical disease. It is a diagnostic and therapeutic emergency and its progress leads to instant death. We report three cases of neonatal cellulitis from the Neonatal ICU Department of Mohammed VI university hospital in Marrakesh, highlighting this rare, serious and unknown disease. Case 1: 17-day-old female newborn admitted for sepsis and breast refusal since 5 days. Clinical examination revealed a hypotonic newborn, hypothermic at 34.2 °C with sclerema neonatorum. Skin examination found Erythema and edema on the left hemi-face with necrosis of the ipsilateral nostril. The blood culture had isolated coagulase-negative staphylococci. The newborn was put under triple antimicrobial therapy of 3rd Generation Cephalosporin (3GCs), gentamicin and vancomycin with good progress. Case 2: 20-day-old male newborn, was circumcised ten days before admission, had sepsis with a fever at 40 °C evolving since 24h. The clinical examination found hard, hot and very painful inflammatory lesion starting at the scrotum and extending to suprapubic region and the start of the lower limbs. Blood tests revealed strongly positive infectious status. The progress was positive when put under triple antimicrobial therapy. Case 3: 25-day-old female newborn, visited a traditional healer who gave her a mixture of unknown nature to apply on her face, hospitalized in our department for cellulitis of the right hemi-face extending to the periorbital and cervical region evolving since two days together with a fever. Infectious blood assessment was positive. The progress was favorable when put under triple antimicrobial therapy. Conclusion: Cellulitis is a rare disease in newborn and it must be diagnosed early because it can be complicated into sepsis. The search for other localizations, mainly meningeal, is essential.

Panniculitis: A summary.

The diagnosis of panniculitis is felt to be a confusing topic by some pathologists. This summarical article presents inflammatory diseases of the subcutis in a systematic fashion, based on whether they are centered on fibrovascular septa or the adipose lobules, and whether morphologic vasculitis is present or not. Septocentric, non-vasculitis disorders include erythema nodosum, panniculitis that follows the use of "biological" therapeutic agents, lipodermatosclerosis, post-irradiation panniculitis, morphea profunda, and necrobiosis lipodica profunda. Polyarteritis nodosa and Behçet's disease are the conditions that are based in the subcutaneous septa with vasculitis. Predominantly-lobular panniculitides with no vasculitis include pancreatogenic panniculitis, the panniculitis of alpha-1-antitrypsin deficiency, panniculitis associated with lupus erythematosus and dermatomyositis, subcutaneous Sweet syndrome, eosinophilic panniculitis, factitial panniculitis, cold panniculitis, panniculitis following injections of corticosteroids, lipomembranous (ischemic) panniculitis; sclerema neonatorum and subcutaneous fat necrosis of the newborn, and Rosai-Dorfman disease of the subcutis. Erythema induratum and infectious panniculitis are vasculitic and lobulocentric conditions. This article reviews the histological features of these diseases.

Panniculitis

The subcutaneous fat is a metabolic depot and also provides insulation from trauma. The subcutis is divided into lobules by fibrous septa. Within the fibrous septae course the small arteries, arterioles, veins and small nerve fibres. The nutrient artery supplies the centre of the lobule and drains to venules within the septum. Inflammatory disorders of the subcutaneous fat can be grouped into three main categories: 1) Septal panniculitis. 2) Lobular panniculitis. 3) Panniculitis associated with large vessel vasculitis. Most panniculitis are strictly speaking mixed but the classification is based on the predominant area of involvement. In septal panniculitis the inflammation is centred upon the connective tissue septa. There is often some spill over of the inflammatory cells into the periphery of the lobule. Erythema nodosum (EN) is the prototype of septal panniculitis. Other disorders associated with a septal panniculitis include necrobiosis lipoidica, factitial panniculitis, nephrogenic systemic fibrosis, cellulitis and microscopic polyangiitis. In the lobular panniculitides the inflammation is centered mainly within the lobule. Examples of lobular panniculitis include nodular vasculitis, lupus panniculitis, subcutaneous fat necrosis of the newborn, sclerema neonatorum, cold panniculitis, a1 anti-trypsin deficiency and pancreatic panniculitis.

Gemcitabine‐associated livedoid thrombotic microangiopathy with associated sclerema neonatorum‐like microscopic changes

Gemcitabine is a deoxycytidine analog antimetabolite that is now accepted as first-line treatment for advanced and metastatic pancreatic carcinoma. Gemcitabine-related thrombotic microangiopathy associated with systemic hemolytic-uremic syndrome or thrombotic thrombocytopenia purpura has rarely been described. Herein, we report a patient who developed a livedoid thrombotic microangiopathy with no signs of associated hemolytic-uremic syndrome. Cutaneous thrombotic microangiopathy occurred after the administration of his 17th cycle and a cumulative dose of 53.65 g/m(2) of gemcitabine. Some authors have suggested that this toxicity may be dose-related, and a 10th cycle or a cumulative dose of 9-56 g/m(2) have been proposed as the prothrombotic threshold. Interestingly, dermatopathologic findings were limited to the subcutis and they consisted of small-vessel occlusion by intravascular fibrin and leukocytes, vessel wall thickening and endothelial cell swelling. Surprisingly, we observed some structures arranged radially with needle-shaped clefts resembling those of sclerema neonatorum. Awareness of this potential cutaneous toxicity by dermatologists and dermatopathologists is extremely important.

Congenital nephrotic syndrome with acute renal failure: questions

We report a case of congenital nephrotic syndrome with acute renal failure associated with multiple organ injury requiring peritoneal dialysis. Maternal serology, including hepatitis B, HIV, and Treponema pallidum hemagglutination (TPHA) and the Venereal Disease Research Laboratory (VDRL) tests were all negative at 10 weeks of gestational age (GA). At 34 weeks of gestation, the ultrasound examination showed hydrops fetalis. The umbilical cord puncture found anemia, with hemoglobin at 8.4 g/dl, thrombocytopenia at 43,000/mm, cytomegalovirus (CMV)-polymerase chain reaction (PCR) and parvovirus-PCR were negative. The child was born at 35 weeks of gestational age and immediately transferred to an intensive care unit because of septic shock. The clinical findings were sclerema neonatorum with blisters, hepatomegaly, splenomegaly, and macroscopic hematuria. The initial blood analysis showed hemoglobin 11 g/dl, thrombocytopenia 22,000/mm (despite intrauterine blood and platelets transfusion), reticulocytes >150,000/mm, C-reactive protein (CRP) 233 mg/L, urea 4 mmol/L, serum creatinine 72 μmol/L, proteins 56 g/L, serum glutamic oxaloacetic transaminase (SGOT) 482 UI/L, serum glutamic pyruvic transaminase (SGPT) 224 UI/L, haptoglobin 600 μg/dl. Symptomatic treatment was initiated by albumin, anticoagulation, and recombinant erythropoietin. At the 9th day of life, CRP was 51 ng/ml, and hemoculture showed Staphylococcus epidermidis, motivating vancomycin treatment. Serum creatinine increased concomitantly to 262 μmol/L.

Panniculitis in childhood

Panniculitis refers to disorders with inflammation of the subcutaneous fat. Such inflammation can be primary or can be a reaction pattern induced by a systemic process. Some types of panniculitis are seen more commonly or exclusively in children. These include erythema nodosum, subcutaneous fat necrosis of the newborn, sclerema neonatorum, poststeroid panniculitis, and cold panniculitis. The most typical clinical finding is tender, erythematous subcutaneous nodules. Clinical clues can aid in the diagnosis of the panniculitides, but pathology is often necessary to confirm the diagnosis. In general, the pediatric panniculitides are treated with supportive care and management of any underlying disorders, but certain types such as infectious panniculitis and malignancy-related panniculitis require more specific therapies.

RISK FACTORS FOR SCLEREMA NEONATORUM IN PRETERM NEONATES IN BANGLADESH

This study presents a retrospective analysis of risk factors for sclerema neonatorum in preterm neonates in Bangladesh. Preterm neonates admitted to Dhaka Shishu Hospital in Bangladesh were enrolled in a clinical trial to evaluate the effects of topical treatment with skin barrier-enhancing emollients on prevention of sepsis and mortality. Four hundred ninety-seven neonates were enrolled in the study and 51 (10.3%) developed sclerema neonatorum. We explored risk factors for sclerema neonatorum by comparing patients with and without sclerema neonatorum. Diagnosis of sclerema neonatorum was based on the presence of uniform hardening of skin and subcutaneous tissues to the extent that the skin could not be pitted nor picked up and pinched into a fold. Cultures of blood and cerebrospinal fluid were obtained in all neonates with clinical suspicion of sepsis. In multivariate analysis, lower maternal education (OR: 1.94; 95% CI: 1.02-3.69; P = 0.043), and signs of jaundice (OR: 2.82; 95% CI: 1.19-6.69; P = 0.018) and poor feeding (OR: 4.71; 95% CI: 1.02-21.74; P = 0.047) on admission were risk factors for developing sclerema neonatorum. The incidence rate ratio of sepsis in neonates who developed sclerema neonatorum was 1.81 (95% CI: 1.16-2.73; P = 0.004), primarily due to Gram-negative pathogens, and risk of death in infants with sclerema neonatorum was 46.5-fold higher (P < 0.001, 95% CI: 6.37-339.81) than for those without sclerema neonatorum. Sclerema neonatorum was a relatively common, grave condition in this setting, heralded by poor feeding, jaundice, and bacteremia, and signaling the need for prompt antibiotic treatment.

Cold Panniculitis

Cold panniculitis has been described in children and young women following cold exposure. Histopathologically, cold panniculitis shows a mostly lobular panniculitis, which consists of an infiltrate of lymphocytes and histiocytes in the fat lobules. Usually, the dermis shows a superficial and deep perivascular infiltrate mostly composed of lymphocytes, with no vasculitis. Inflammation is most intense at the dermal-subcutaneous junction. Differential diagnosis of cold panniculitis should be established with subcutaneous fat necrosis of the newborn, sclerema neonatorum, poststeroid panniculitis, chilblains, and frostbites.

Panniculitis in Children

The panniculitides include a group of disorders of varied etiology that manifest as inflamed nodules in the subcutaneous tissue. They are rarely seen in infants and children. The panniculitides of the newborn represent a unique response of the infant's fat to different injuries, and are a specific type of panniculitis that is only seen in neonates and very young infants. These specific panniculitides of children include subcutaneous fat of the newborn, poststeroid panniculitis, sclerema neonatorum, and cold panniculitis. This article reviews in detail the specific types of panniculitis of the newborn and discusses the pediatric aspects of the panniculitis that is often seen in adults.

Sclerema neonatorum: a review of nomenclature, clinical presentation, histological features, differential diagnoses and management

To review published literature on sclerema neonatorum (SN) in order to clarify its clinical presentation, histological features and management compared with two other diseases: subcutaneous fat necrosis of the newborn (SCFN) and scleredema. PubMed database was searched using the key words Sclerema neonatorum. A total of 55 articles from peer-reviewed journals were reviewed and summarized. SN, SCFN and scleredema are diseases of the subcutaneous adipose tissue. SN is characterized by hardening of the skin that gets bound down to the underlying muscle and bone, hindering respiration and feeding and is associated with congenital anomalies, cyanosis, respiratory illnesses and sepsis. Histology of the skin biopsy shows thickening of the trabeculae supporting the subcutaneous adipose tissue and a sparse inflammatory infiltrate of lymphocytes, histiocytes and multinucleate giant cells. SCFN has circumscribed hardening of skin on bony prominences with necrosis of adipocytes in subcutaneous tissue and a dense granulomatous infiltrate on histology. Scleredema is characterized by hardening of the skin along with edema; histology shows inflammatory infiltrate and edema in skin and subcutaneous tissues. SN has a high case fatality rate whereas SCFN and scleredema are self-limiting and lesions resolve within a few weeks to months. Exchange transfusion may improve survival in SN. The histological features of skin biopsy should be used to establish diagnosis of SN, SCFN and scleredema as disease-specific treatment is imperative in SN due to high fatality.

Normal Subcutaneous Fat, Necrosis of Adipocytes and Classification of the Panniculitides

The panniculitides represent a group of heterogeneous inflammatory diseases that involve the subcutaneous fat. The specific diagnosis of these diseases requires histopathologic study because different panniculitides usually show the same clinical appearance, which consists of erythematous nodules on the lower extremities. However, the histopathologic study of panniculitis is difficult because of an inadequate clinicopathologic correlation and the changing evolutive nature of the lesions. In addition, large scalpel incisional biopsies are required. From histopathologic point of view, all panniculitides are somewhat mixed because the inflammatory infiltrate involves both the septa and lobules. However, nearly always the differential diagnosis between a mostly septal and a mostly lobular panniculitis is straightforward at scanning magnification on the basis of the structures more intensely involved by the inflammatory infiltrate. Mostly septal panniculitides with vasculitis are actually more vasculitis than panniculitis and include superficial thrombophlebitis and cutaneous polyarteritis nodosa. Mostly septal panniculitides with no vasculitis include erythema nodosum, necrobiosis lipoidica, deep morphea, subcutaneous granuloma annulare, rheumatoid nodule, and necrobiotic xanthogranuloma. Mostly lobular panniculitis with vasculitis is only represented by erythema induratum of Bazin. In contrast, mostly lobular panniculitides without vasculitis comprise a large series of disparate disorders, including sclerosing panniculitis, calciphylaxis, sclerema neonatorum, subcutaneous fat necrosis of the newborn, poststeroid panniculitis, lupus erythematosus profundus, pancreatic panniculitis, alpha(1)-antitrypsin deficiency panniculitis, subcutaneous Sweet syndrome, infective panniculitis, factitial panniculitis, lipodystrophy, traumatic panniculitis, subcutaneous sarcoidosis, and sclerosing postirradiation panniculitis. Finally, some cutaneous lymphomas may simulate panniculitis, both from clinical and histopathologic points of view and, for that reason, they will be included in this review, although they are not inflammatory processes, but authentic lymphocytic neoplasms involving subcutaneous tissue.

Panniculitis. Part II. Mostly lobular panniculitis

The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and α1-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) Learning objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.

Subcutaneous fat necrosis of the newborn: a review of 11 cases.

Subcutaneous fat necrosis (SFN) of the newborn is uncommon and usually occurs in the first weeks of life following a complicated delivery. The frequency with which hypercalcemia develops as a complication is uncertain. We report the clinical features of SFN in 11 patients seen between 1991 and 1998. Ten were born by cesarean section and fetal distress was present in the majority. It was not possible to distinguish SFN from sclerema neonatorum by time of onset or related infant diseases. Hypercalcemia developed in four infants up to 7 weeks after the onset of SFN. Infants with this condition should be carefully monitored for hypercalcemia.

Chronic colitis associated to diverticulosis with perianal skin lesion

The second part of our review of panniculitis summarizes the clinicopathologic features of the mostly lobular panniculitides. Erythema induratum of Bazin (nodular vasculitis) represents the most common variant of lobular panniculitis with vasculitis, although controversy persists about the nature of the involved vessels. Mostly lobular panniculitides without vasculitis comprise a series of disparate disorders. These include sclerosing panniculitis that results from chronic venous insufficiency of the lower extremities; panniculitis with calcification of the vessel walls such as calciphylaxis and oxalosis; and inflammatory diseases with crystals within the adipocytes such as sclerema neonatorum, subcutaneous fat necrosis of the newborn, and poststeroid panniculitis. Connective tissue diseases, such as systemic lupus erythematosus and dermatomyositis, pancreatic diseases, and α1-antitrypsin deficiency may also show a mostly lobular panniculitis with characteristic histopathologic features. Lobular panniculitis may also be an expression of infections, trauma, or factitial causes involving the subcutaneous fat. Lipoatrophy refers to a loss of subcutaneous fat due to a previous inflammatory process involving the subcutis, and it may be the late-stage lesion of several types of panniculitis. In contrast, lipodystrophy means an absence of subcutaneous fat with no evidence of inflammation and often the process is associated with endocrinologic, metabolic, or autoimmune diseases. Finally, cytophagic histiocytic panniculitis is the term that has been used to describe two different processes: one is inflammatory, a lobular panniculitis, and the other one is neoplastic, a subcutaneous T-cell lymphoma. The only common feature of these two different processes is the presence of cytophagocytosis in the lesions. (J Am Acad Dermatol 2001;45:325-61.) Learning objective: At the completion of this learning activity, participants should be familiar with the pathogenesis, clinical manifestations, histopathologic findings, and treatment options for the most frequent variants of the lobular panniculitides.

Restrictive dermopathy. Report of two affected siblings and a review of the literature

Restrictive dermopathy is a lethal genetic disorder consisting of abnormally tight skin, generalized joint contractures, distinctive facies, and pulmonary hypoplasia. Autosomal recessive inheritance has been suggested based on multiply affected siblings and some reports of parental consanguinity. This article describes two siblings with the restrictive dermopathy syndrome and reviews previously reported cases. Eight other cases have been reported in the literature as restrictive dermopathy. These cases have shared striking similarities in their clinical histories and phenotypes. The skin in these infants has been described as rigid and tense, with skin biopsy specimens showing a thick epidermis, thin dermis, abnormally arranged collagen bundles, and poorly developed appendages. Other prominent features are flexion contractures and craniofacial and pulmonary abnormalities. The genetic and/or developmental defects leading to the restrictive dermopathy syndrome are presently not known. The restrictive dermopathy syndrome is distinct and is easily differentiated from other congenital diseases such as the icthyoses and also from the clinical conditions of sclerema neonatorum and subcutaneous fat necrosis of the newborn. Recognition of this syndrome is important for determining the prognosis of affected infants and for recommending genetic counseling to affected families.