This layer is called the panniculus, or subcutaneous fat layer. Erysipelas or lymphangitis is red, tender, and more localized than sclerema. This infant also had transient thrombocytopenia. Seizures are variable and may develop later in childhood. Within each MDC are MS-DRGs (Medicare Severity Diagnosis Related Groups).Each of the Medicare Severity Diagnosis Related Groups is defined by a particular set of patient attributes which include principal diagnosis, specific secondary diagnoses, procedures, sex and discharge status. Subcutaneous adipose tissue diseases involving adipose tissue and its fascia, also known as adipofascial disorders, represent variations in the spectrum of obesity. Late histologic changes may include fibrosis, small fat cysts, and dystrophic calcification.48 However, a biopsy is usually not necessary. There may be other signs of systemic infection or sepsis. Similar findings have been observed in extra-abdominal desmoid tumors, juvenile hyaline fibromatosis, scleroderma, and the tight skin mouse model which is transmitted in an autosomal dominant pattern and is located on chromosome 2.36 Cutaneous fibrosis in the Tsk mouse appears to be caused by a mutation in Fibrillin-1. Soft tissue calcification may occur in the absence of hypercalcemia and can be detected radiographically. When the child was 22 months old, ice applied for 15 min did not trigger panniculitis. Nephrocalcinosis, vomiting, failure to thrive, poor weight gain, irritability, and seizures can complicate high calcium levels or chronic moderate elevations. and Gram-negative (Pseudomonas sp., Klebsiella sp., Fusobacterium, Fusarium) bacteria, fungi (Candida sp., Nocardia sp. The skin feels cold, smooth, hard, and bound down. Causes … Disease Ontology - OBO (Open Biomedical Ontologies) DISEASES AND INJURIES; Disease Ontology V2.1 2005. Treatment should be directed against the specific organism. Rarely, large plaques may cover extensive areas of the trunk or extremities. A red plaque developed 12–18 h later and resolved after 13 days. The history of cold exposure in an otherwise healthy infant will help to distinguish cold panniculitis from other causes of subcutaneous nodules. Chapter XII: Diseases and disorders of the skin and subcutaneous tissue: From chapter XVII: Congenital malformations, deformations and chromosomal abnormalities Q80-Q82. Although most cases have been sporadic, disease affecting two siblings, a mother and two siblings, and another family with affected family members in multiple generations, support a hereditary transmission. Varying histologic changes from patient to patient and in the same patient over time, may reflect different triggers which result in similar clinical findings.32–34,36 In some cases, thickening of the collagen in the fascia was noted, whereas in others the fascia was normal and increased mucopolysaccharide deposition was found in the dermis. The risk of hypercalcemia increases with the severity of the perinatal insult and extent of fat necrosis. However, there are several reports of NLCS associated with pigment anomalies, including café-au-lait spots and hypopigmented macules. As a consequence, at-risk infants should be monitored for the first 6 months of life and should not receive vitamin D supplementation for rickets prophylaxis during this period. Variable amounts of calcification develop, which can be appreciated radiographically.10. Similar cases have been reported following the use of ice to induce hypothermia before cardiac surgery, and the application of ice bags to the face for management of supraventricular tachycardia. Although lipomas represent the most common tumor of the mesenchyme in adults, they are rare in infants and account for <5% of tumors of childhood. Although the clinical appearance of NLCS varies, the presence of typical nodules and plaques in the pelvic girdle region should suggest the diagnosis. and Gram-negative ( Pseudomonas sp., Klebsiella sp., Fusobacterium , Fusarium ) bacteria, fungi ( Candida sp., Nocardia sp. On the scalp, these lesions are sometimes referred to as nevus psiloliparus (see Chapter 31 ). The aims of … Nodules heal in 1–3 weeks without scarring. Congenital diseases and disorders of skin From Chapter XVIII: Symptoms, signs and abnormal clinical and laboratory findings R20–R23. Affected infants typically present with one or several indurated, variably circumscribed, violaceous or red plaques or subcutaneous nodules from one to several centimeters in diameter on the buttocks, thighs, trunk, face, and/or arms (Figs 27.1–27.4). Applying ice to the skin for 50 seconds results in nodules in all newborns, but only in 40% of 6-month-old and only occasionally in 9-month-old infants.43 In 1966, Duncan and colleagues45 described a child in whom nodules followed the application of ice for several minutes at 6 months of age, and 8 min at 18 months of age. Ultrasound is a reliable noninvasive screening tool for infants during the first 6 months of life. Other laboratory findings in neonates with sclerema are nonspecific and usually reflect the underlying systemic medical problems. Tumors of fat include a number of neoplasms and hamartomatous malformations. In healthy infants who develop widespread slowly progressive scleroderma-like plaques on the trunk and proximal extremities, the diagnosis of stiff skin syndrome should be considered (see below). In some cases, the nodules may be subtle, not associated with overlying color change, and only appreciated by careful palpation of the underlying fat. A mutation in Fibrillin-1 has also been demonstrated in 4 families with autosomal dominant stiff skin syndrome.31, In infants and young children with progressive bound-down plaques beginning on the trunk, limited joint mobility, and no evidence of systemic disease, stiff skin syndrome should be considered. In a study by Rotman the application of an icecube to the volar aspect of the forearm of an 8-month-old girl resulted in mild transient erythema for 15 min. Although infectious panniculitis is more common in immunocompromised individuals,55 it has rarely been reported in immunocompetent children.52. Although not fully functional at birth, a well-developed fatty layer is present in the neonate, even when premature. A recent report suggests that, although noninflammatory fibrosis of the fat and fascia is typical but not specific for incisional biopsies of stiff skin syndrome, the presence of a lattice-like array of thickened, horizontally oriented collagen bundles may be a clue to diagnosis.37. Disorders of the fat can interfere with normal function and may have systemic implications. Disorders of the fat can interfere with normal function and may have systemic implications. The clinical features of scleroderma overlap with some cases of stiff skin syndrome. In 1966, Duncan and colleagues described a child in whom nodules followed the application of ice for several minutes at 6 months of age, and 8 min at 18 months of age. Although most infants with sclerema succumb to sepsis and shock, reversal of the underlying systemic disease can result in recovery. Occasionally, liponecrosis leads to ulceration and/or lipoatrophy, with persistent dimpling of the skin. When hypercalcemia and/or soft tissue calcification is present, primary hyperparathyroidism, osteoma cutis, and calcification associated with Albright osteodystrophy should be excluded. These disorders often involve the joints, muscles, and skin, but they can also involve other organs and organ systems, including the eyes, heart, lungs, kidneys, gastrointestinal tract, and blood vessels. UNSTAGEABLE PRESSURE ULCERS (L89.--0) NON-PRESSURE CHRONIC ULCERS No code is assigned if the non-pressure ulcer is completely at the time of admission. This collection focuses on conditions related to skin and subcutaneous tissue. Since then, a number of cases of this rare hamartomatous disorder have been reported, demonstrating the variability of clinical and histologic findings. In erythema nodosum, the panniculitis occurs primarily in the fat septa, and the infecting organisms are not found in the skin nodules. Post-steroid panniculitis can be clinically indistinguishable from cold panniculitis. Disorder of skin and/or subcutaneous tissue (80659006); Skin and subcutaneous tissue disease (80659006); Disorder of the dermis and subcutaneous tissue (80659006); Disorder of skin and subcutaneous tissue (80659006) Recent clinical studies. Noninflammatory sclerosis in the deep reticular dermis extending into the subcutaneous fat has also been noted. The history of cold exposure in an otherwise healthy infant will help to distinguish cold panniculitis from other causes of subcutaneous nodules. However, lesions are usually freely movable over subjacent muscles and fascia. Connective tissue disease refers to a group of disorders involving the protein-rich tissue that supports organs and other parts of the body. In some cases, thickening of the collagen in the fascia was noted, whereas in others the fascia was normal and increased mucopolysaccharide deposition was found in the dermis. Occasionally, fluctuance and abscess-like changes occur, resulting in spontaneous drainage and scar formation. Etidronate therapy has also been reported to be successful in controlling severe hypercalcemia in SCFN.13 Ulcerated lesions, which rarely occur in otherwise healthy infants, usually respond to topical antibiotics and bio-occlusive dressings. Atrophic hairless patches may also be present on the scalp and face. Several investigators have reported a favorable outcome when exchange transfusion was combined with conventional therapy.21,26, In 1971, Esterly and McKusick28 described a disorder in infants and young children characterized by diffuse skin induration and thickening, with limitation of joint mobility, flexion contractures, and hypertrichosis. Deep soft tissue infections in neonates are usually associated with fever and other signs of sepsis. Blood cultures and cultures of other body fluids may also be positive. Over a century later, the term subcutaneous fat necrosis was first applied to this clinically benign condition with histologic characteristics of fat necrosis.5. However, in lymphedema, the infant is otherwise healthy, and a skin biopsy reveals normal fat and dilated lymphatics. These disorders can be distinguished from stiff skin syndrome by their characteristic clinical, histologic, biochemical, and genetic findings. Thrombocytopenia, neutropenia, active bleeding, and worsening acidosis carry a poor prognosis. In milder cases, the condition may mimic a connective tissue nevus, smooth muscle hamartoma, or myofibroma. The clinician must distinguish disorders that are innocent and self-limiting from those that are associated with significant morbidity or underlying systemic disease. There's more to see -- the rest of this entry is available only to subscribers. Learn about its purpose and medical conditions that affect it. However, a number of entities have been recognized because of their distinctive clinical patterns, histopathology, biochemical and genetic markers, inheritance, and course. It occasionally occurs in older infants up to 4 months of age with severe underlying disease. Tests of parathyroid function, vitamin D metabolites, and urinary prostaglandins may be useful in the evaluation of infants with hypercalcemia. Post-steroid panniculitis can be clinically indistinguishable from cold panniculitis.46 Subcutaneous nodules or plaques appear on the cheeks of infants within 2 weeks of rapidly discontinuing high-dose systemic steroids after a prolonged course. Skin And Subcutaneous Tissue Disorders … Recently, low-grade gliomas and intracranial vascular malformations have also been described. Although lesions can develop in infants with a normal delivery and neonatal course, SCFN has been associated with perinatal complications, including asphyxia, hypothermia, seizures, pre-eclampsia, meconium aspiration, and intrapartum medication.2–5 Extensive subcutaneous fat necrosis has also been reported following therapeutic hypothermia used in newborns with severe perinatal asphyxia and surgical procedures.6–8, Although the first reports of SCFN appeared during the early nineteenth century, many investigators continued to use the terms scleroderma or scleredema to describe SCFN, as well as a number of diverse disorders of the subcutaneous tissue associated with the development of distinct nodules or widespread induration. Although not fully functional at birth, a well-developed fatty layer is present in the neonate, even when premature.1 Disorders of the fat can interfere with normal function and may have systemic implications. Unfortunately, many patients present in later childhood and adolescence with neurologic defects in the lower extremities, including weakness and foot deformities. However, in a child with a typical history, a biopsy is unnecessary, and nodules resolve over a period of months without treatment. This can occur with certain medications and intravenous fluid extravasation.51 Munchhausen syndrome by proxy should be considered when recurrent panniculitis with associated cellulitis and/or ulceration occurs in an otherwise healthy infant without a clear diagnosis. Heterogeneous central system structural anomalies have also been described. Diseases of the heart and blood vessels (cardiology) Mental health (psychiatry) Injuries and poisonings; Diseases of the skin and subcutaneous tissue (dermatology) Diseases of the lungs, bronchi and pleura (pulmonology) Diseases of the ear, throat and nose (otolaryngology) Diseases of the endocrine system and metabolic disorders (endocrinology) Skin biopsy for pathology and cultures, blood cultures, and other appropriate cultures will hopefully identify a specific organism and direct antibiotic and/or antifungal therapy. Cellulitis should also be considered in any child with tender red facial nodules. Applying ice to the skin for 50 seconds results in nodules in all newborns, but only in 40% of 6-month-old and only occasionally in 9-month-old infants. However, this is a primary disorder of fascia and, unlike sclerema, is not associated with systemic symptoms. Ocular and cutaneous lesions appear to be static and amenable to surgical repair. Histologic findings are typical of lipomas in other sites and show mature adipocytes within a thin connective tissue capsule. Although infectious panniculitis is more common in immunocompromised individuals, it has rarely been reported in immunocompetent children. The earliest changes 24 h after cold injury include an infiltrate of macrophages and lymphocytes at the dermoepidermal junction extending into the dermis and fat. A recent report suggests that, although noninflammatory fibrosis of the fat and fascia is typical but not specific for incisional biopsies of stiff skin syndrome, the presence of a lattice-like array of thickened, horizontally oriented collagen bundles may be a clue to diagnosis. Late histologic changes may include fibrosis, small fat cysts, and dystrophic calcification. Soft, spongy, hairless, pink-yellowish tumors characteristically involve the scalp, often in a linear configuration, but may extend to the legs and paravertebral area. Cases reported with bony, dental, and other anomalies probably represent focal dermal hypoplasia (Goltz syndrome), which can be confused clinically and histologically with NLCS. However, histology demonstrates characteristic hypertrophy and sclerosis of collagen, which eventually replaces the fat in scleroderma. The diagnosis is apparent when one or several nodules develop in a vaccination injection site. Although restrictive pulmonary changes and growth retardation have occasionally been reported, immunologic, visceral, bony, muscular, and vascular involvement is characteristically absent.29,32–34, Although the cause is unclear, investigators have proposed a primary fibroblastic defect resulting in increased mucopolysaccharide deposition in the dermis, a primary fascial dystrophy resulting from increased collagen, and an inflammatory process.29,32–34 Some patients with stiff skin syndrome have been noted to have increased myofibroblastic activity in fascia, with overproduction of type VI collagen. However, nodules can also develop in infants and over other bony prominences after accidental or deliberate injury. However, in a child with a typical history, a biopsy is unnecessary, and nodules resolve over a period of months without treatment. However, they may extend into the papillary dermis, and the distinction between the dermis and subcutaneous fat may be poorly defined. These comprehensive and concise factsheets are physician-reviewed and reflect the most current, evidence-based information. Hypocalcemia with pseudohypoparathyroidism requiring therapy, as well as transient hypoglycemia, hypertriglyceridemia, and thrombocytopenia, have also been reported in several children. This condition, which has been called ‘congenital fascial dystrophy’ or the ‘stiff skin syndrome’ was further defined by Jablonska and colleagues29 as a generalized, noninflammatory disease of fascia without evidence of visceral or muscle involvement, immunologic abnormalities, or vascular hyperreactivity. Skin and Subcutaneous Tissue Disorders: SNOMED CT: Disorder of skin and/or subcutaneous tissue (80659006); Skin and subcutaneous tissue disease (80659006); Disorder of the dermis and subcutaneous tissue (80659006); Disorder of skin and subcutaneous tissue (80659006) Other laboratory studies, including blood counts, cold agglutinins, cryoglobulins, and general chemistry studies, are usually normal. Hypocalcemia with pseudohypoparathyroidism requiring therapy. Rarely, large plaques may cover extensive areas of the trunk or extremities. In ICD-9-CM, there are 2 codes available for lipomas of the skin and subcutaneous tissue, differentiated between face and other site. Children with PTEN-associated disorders are also at risk for the development of malignant tumors in adulthood and should be screened accordingly. 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