Peeling skin syndrome in Infant/Neonate
Alerts and Notices
Synopsis
PSS often presents in infancy but can present in late childhood and occasionally adulthood. Skin loss in PSS is generally asymptomatic but is occasionally pruritic. Peeling is worsened by trauma, friction, humidity, heat, and water exposure.
PSS is divided into generalized and acral subtypes. Generalized PSS causes diffuse skin shedding that typically spares mucosal surfaces, palms, and soles. It may be noninflammatory (type A) or inflammatory (type B).
- Noninflammatory PSS is asymptomatic and has been associated with hyperpigmentation. Mutations in the CHST8 gene, in the SERPINB8 gene, and most recently, the FLG2 gene that encodes filaggrin, have been found.
- Inflammatory PSS can present with erythema, pruritus, and occasional flaccid vesicles. It can worsen during summer months. An association with atopy and high IgE levels may be seen. Loss of function mutations in the CDSN gene is responsible.
- Acral PSS (APSS) is a localized disease involving the dorsal and volar surfaces of the hands and feet. Mutations in the transglutaminase 5 (TGM5) gene or the cystatin A (CSTA) gene are responsible.
Codes
ICD10CM:R23.4 – Changes in skin texture
SNOMEDCT:
238643009 – Skin peeling disorder
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
The differential for PSS includes other causes of superficial desquamation of the skin. The presence of systemic symptoms should point away from the diagnosis of PSS. Lab findings are within normal limits in patients with isolated PSS.Other causes of peeling skin include:
- Netherton syndrome – differentiate from the inflammatory generalized subtype. Ichthyosis linearis circumflexa, trichorrhexis invaginata, and immunological dysfunction accompany Netherton syndrome.
- Epidermolysis bullosa simplex (EBS) – differentiate from the acral subtype. EBS has larger, more persistent bullae than APSS.
- Staphylococcal scalded skin syndrome
- Superficial epidermolytic ichthyosis
- Sunburn
- Pemphigus vulgaris – can involve mucous membranes, unlike PSS
- Severe dermatitis, multiple allergies, and metabolic wasting (SAM) syndrome
- Atopic dermatitis
- Dyshidrotic eczema
- Epidermolysis bullosa simplex, localized – autosomal dominant
- Erythrokeratolysis hiemalis (keratolytic winter erythema)
- Exfoliative ichthyosis
- Keratolysis exfoliativa
Best Tests
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Management Pearls
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Therapy
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References
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Last Reviewed:08/14/2018
Last Updated:01/20/2022
Last Updated:01/20/2022
Peeling skin syndrome in Infant/Neonate