‘Peeling paint’ dermatitis as a presenting sign of cystic fibrosis

O’Regan, Gráinne M.; Canny, Gerard J.; Irvine, Alan D.

doi:10.1016/j.jcf.2006.05.003

Cited by 12 publications

(10 citation statements)

References 18 publications

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“…3 If the desquamation is excessive and dominates the clinical picture, CFNDD is named "peeling paint" rash or "peeling paint" dermatitis ( Figure 1), and protein, zinc, and essential fatty acids deficiencies are significant due to malabsorption and malnutrition. 4,5 Desquamating erythema could be the first cutaneous sign, with periorificial, perineal, and acral distribution, associated with edema (induced by low albumin levels) and alopecia. 3 Histopathologic examination of a skin punch biopsy can reveal non-specific features such as epidermal hyperplasia, parakeratosis, intradermic spongiosis, thin granular layer, and perivascular inflammatory infiltrate with mononuclear cells in the dermis.…”

Section: Nutrient Deficiency Dermatitis Of Cystic Fibrosis (Cfndd)mentioning

confidence: 99%

Cutaneous Manifestations of Cystic Fibrosis

Chiriac

Trandafir

Podoleanu

et al. 2018

Journal of Interdisciplinary Medicine

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Cystic fibrosis (CF) is an autosomal recessive affliction triggered by genetic mutations in the cystic fibrosis transmembrane conductance regulator. The lung and pancreas are the most frequently affected organs in cystic fibrosis, cutaneous involvement is undervalued and underdiagnosed. Skin lesions observed in patients diagnosed with cystic fibrosis are not well known and can create confusions with other dermatological diseases. The diagnosis of cutaneous lesions as signs of cystic fibrosis by pediatricians or dermatologists, despite their overlapping with different nutritional deficiencies, would allow earlier diagnosis and proper treatment and could improve quality of life and outcomes.

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Section: Nutrient Deficiency Dermatitis Of Cystic Fibrosis (Cfndd)mentioning

confidence: 99%

Cutaneous Manifestations of Cystic Fibrosis

Chiriac

Trandafir

Podoleanu

et al. 2018

Journal of Interdisciplinary Medicine

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“…2,[4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] The dermatitis typically presents as erythematous, scaling papules that later progress to extensive coalescing, desquamating plaques. The eruption is usually first noted in the perineum anywhere from several days to 15 months after birth, with subsequent spread to the perioral region, extremities, and trunk.…”

Section: Commentmentioning

confidence: 99%

Cystic Fibrosis Presenting With Dermatitis

Wenk¹,

Higgins²,

Greer³

2010

Arch Dermatol

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Background Patients with cystic fibrosis classically present with evidence of pulmonary disease, exocrine pancreatic insufficiency, and high sweat chloride concentrations. Dermatitis as an initial manifestation of the disease is uncommon and has been attributed to multiple nutritional deficiencies. Observation We describe the case of a 3-month-old female infant with cystic fibrosis presenting with dermatitis in the setting of protein-energy malnutrition. A review of the laboratory study results in this case and others showed that a deficiency in zinc, essential fatty acids, and protein likely contributes to the development of the rash seen in cystic fibrosis. Conclusions Given the frequent delay in diagnosis, as well as the increased morbidity and mortality associated with protein-energy malnutrition in these patients, it is important to consider cystic fibrosis as a possible diagnosis in any infant presenting with a rash and other signs of malnutrition. The relative contribution of specific nutritional deficiencies and the degree to which they influence and interact with each other in producing the dermatitis remain unclear, although they may all affect a common underlying metabolic pathway

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“…In further support of this theory, a condition resembling oedematous malnutrition with dermatosis has been reported several times in children with cystic fibrosis . Compared with children with primary oedematous malnutrition, their symptoms appeared earlier, in infancy.…”

Section: Suggested Aetiologiesmentioning

confidence: 75%

Dermatosis in children with oedematous malnutrition (Kwashiorkor): a review of the literature

Heilskov

Rytter

Vestergaard

et al. 2014

Acad Dermatol Venereol

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Children with oedematous malnutrition, known as kwashiorkor, may develop a characteristic skin lesion, named 'Dermatosis of Kwashiorkor' (DoK). Only a few studies have been concerned with this condition, and the reason for the development of DoK remains unexplained. This study review the existing studies concerning DoK, including its clinical manifestations, histopathology, suggested pathophysiology, current treatment and prognosis for children of the age of 6 months to 5 years. Standardized clinical studies are needed to further understand the implications of DoK. Such studies would suffer from the lack of consistency concerning the terminology and scoring of the lesions in DoK. We therefore stress the need for a standardized scoring of the degree of DoK. This would facilitate valid and comparable studies and the development of better treatment for this vulnerable group of patients.

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‘Peeling paint’ dermatitis as a presenting sign of cystic fibrosis

Cited by 12 publications

References 18 publications

Cutaneous Manifestations of Cystic Fibrosis

Cutaneous Manifestations of Cystic Fibrosis

Cystic Fibrosis Presenting With Dermatitis

Dermatosis in children with oedematous malnutrition (Kwashiorkor): a review of the literature

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