Scleredema in an infant

Two Cases for Diagnosis: Asymmetric Childhood Scleredema or Stiff Skin Syndrome?

“…Both our patients have experienced difficulty walking and participating in recreational activities secondary to restricted joint range of motion. Other reports of pediatric patients have described similar joint restrictions (11,13). Periocular involvement can decrease eye opening (20) and closure (15).…”

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.…”

Scleredema adultorum due to streptococcal infection

“…Although there was no limitation of our patient's daily activities, there can be a number of clinical complications secondary to the tightened skin itself, such as difficulties opening or closing the eyes or fully opening the mouth, 10 and restrictive lung disease secondary to the thickened skin of the chest 11 …”

“…There are relatively few laboratory abnormalities found in scleredema. The most consistent finding was an elevated ASO titer 3,10,11 . Hematologically, some patients have been noted to be mildly to moderately anemic.…”