Scleredema in an infant

Physiopathogenic Investigations in a Case of Familial Stiff-Skin Syndrome

“…SSS differs from other disorders with skin stiffness, when considering the neonatal period: scleredema neonatorum [18](no prematurity, no associated diseases, no adipose cell lesions), subcutaneous fat necrosis [19](no nodules, no fat necrosis with granulomatous inflammation), restrictive dermopathy [19](characterized by a distinctive facies with craniofacial abnormalities, pulmonary hypoplasia, multiple congenital contractures, rigid tense skin) and infantile systemic hyalinosis [20]. SSS also differs, when considering infancy, from systemic scleroderma (no Raynaud’s phenomenon, no visceral involvement, no immunological abnormalities), from scleredema (no increased deposition of acid mucopolysaccharides in the dermis, no preceding febrile illness or diabetes) and from sclerodermatomyositis (no myogenic lesions).…”

A case of congenital scleredema

“…In addition, sclerema neonatorum has a fatal course, 5 while our patient demonstrated a benign outcome. In addition, our case showed a gneralized hardening of the skin present from birth, while in scleroderma, the process usually occurs first on the acral area, before progressing to the trunk and face 3 . The limited movements achieved by the patient were thought to be due to the skin stiffness, and thus resolved partially as the stiffness improved.…”

“…There are three forms of the condition, one with an abrupt onset during a respiratory infection, one with associated diabetes and one with an insidious onset and prolonged course 4 . Scleredema under the age of 10 years is known to occur in 29% of patients; 2 however, to our knowledge only one case has so far been reported in an infant, and in that case, which was associated with cytomegalovirus and was fatal, the skin lesions were not present until about 6 weeks after birth 3 . In our case, however, there was no cytomegalovirus infection, and there was a fairly early spontaneous softening of the skin beginning on the upper part of the trunk, further, because the skin lesions were present from birth, in contrast with the scleredema seen in adults and children, we have used the term congenital scleredema to describe this variant.…”

“…S ir , Scleredema is characterized by a thickening of the dermis of the neck, head, and upper trunk. It may occur in adults or children, 1 , 2 but very rarely in infants, 3 and must be differentiated from scleroderma, sclerema neonatorum, and subcutaneous fat necrosis of the newborn.…”

Síndrome stiff skin: relato de caso

“…Neonatal scleredema is more related to prematurity, and the histology shows lesions to the adipocytes, which were absent on the actual patient. 16 Scleredema usually starts abruptly and results from infiltration of mucin into the dermis, causing the clinical aspect of non-pitting edema and more uniform cutaneous hardening, not as well defined as in SSS, predominantly on the upper trunk, neck and face (unusual locations for the syndrome), and develops mostly in adults. Sclerodermatomyositis has characteristics of both scleroderma and dermatomyositis, with the elevation of muscular enzymes, auto antibodies and vascular hyper reactivity.…”