Sclerema neonatorum

Sclerema neonatorum
Major points
• Characteristic widespread thickening and
induration of the skin in critically ill newborns
(e.g. sepsis, hypoglycemia and metabolic acidosis)
• May occur up to 4 months of age
• Presents with sudden onset of rapid hardening of
the skin that starts distally and progresses to
involve the skin diffusely but with sparing of the
palms, soles and genitalia
• No pitting of the skin is noted with pressure
• Skin becomes bound-down with mask-like facies
and immobile joints
Pathogenesis
• Etiology unknown; hypothesis of cold injury and
secondary solidification of the tissue due to
enzymatic dysfunction
Diagnosis
• Clinical findings in typical setting
• Histology: identical to subcutaneous fat necrosis
Differential diagnosis
• Subcutaneous fat necrosis of newborn
• Restrictive dermopathy
• Systemic sclerosis
• Stiff skin syndrome
Treatment
• Supportive measures to reverse the underlying
disorder: temperature control, hydration, electrolyte
balance and aggressive antibiotic therapy
• Systemic corticosteroid use is controversial
• Exchange transfusions
Prognosis
• High infant mortality related to the underlying
condition
• Skin changes can resolve with treatment of the
underlying disorder
• Worse prognosis with thrombocytopenia,
neutropenia, and progressive acidosis
References
Battin M, Harding J, Gunn A. Sclerema neonatorum following
hypothermia. J Paediatr Child Health. 2002; 38: 533–4
Burden AD, Krafchik BR. Subcutaneous fat necrosis of the
newborn: a review of 11 cases. Pediatr Dermatol 1999; 16:
384–7
Requena L, Sanchez Yus E, Panniculitis. Part II. Mostly
lobular panniculitis. J Am Acad Dermatol 2001; 45: 325–61,
quiz 362–4