Pityriasis rubra pilaris (PRP) is a rare papulosquamous dermatosis of uncertain etiology. Of the six subtypes described, the most frequent form is adult type I, which typically involves erythroderma and islets of respected skin, follicular hyperkeratosis and orange palmoplantar keratoderma. This entity continues to be a therapeutic challenge, as F I G U R E 1 Pityriasis rubra pilaris before and after treatment with ixekizumab. A-C, Exfoliative erythroderma with intense desquamation and orange palmoplantar keratoderma. D-F, After 6 months of treatment with ixekizumab
DEAR EDITOR, A 57-year-old man with diabetes presented with a 30-year period of pruritic skin lesions concurrent with a 12-kg loss of weight. The lesions presented as generalized arciform, erythematous and eroded plaques with overlying haemorrhagic crust (a). There was associated angular cheilitis and atrophic glossitis (b). The skin biopsy showed three structures within the epidermis: a layer with basaloid keratinocytes, a spiny zone with cleared cytoplasm cells and another part with thickened eosinophilic stratum corneum, suggestive of necrolytic migratory erythema (c). An abdominal computed tomography scan found a pancreatic mass and liver metastases whose biopsy revealed infiltration by a neuroendocrine tumour with glucagon immunoexpression. Plasma glucagon was increased. The skin lesions resolved following treatment with somatostatin analogues.R.
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