Generalized pustular psoriasis (GPP) is a rare type of psoriasis and is considered the most severe variant. Infantile pustular psoriasis (IPP) is a very rare form of GPP. Severe IPP may be difficult to control and is complicated by bacterial superinfection, dehydration, and sepsis. Herein, a 2.5-month-old infant with IPP is presented, who was successfully treated with systemic retinoid therapy, which appears to be a safe and effective option for this challenging pustular eruption of childhood.

Lipoid proteinosis (LP) is a rare autosomal recessive genodermatosis associated with deposition of periodic acid-Shiff (PAS)-positive hyaline material in skin, mucosa, and other tissues. LP is caused by loss-of-function mutations in the extracellular matrix protein 1 gene (ECM1). No curative therapy is available. In this report, we describe the clinicopathological and genetic features of a Turkish LP family with four cases, and evaluate the response of acitretin therapy. Patients were presented with hoarseness and beaded eyelid papules, thickened frenulum, hyperkeratotic plaques and infiltrated warty papules and nodules. Skin biopsies revealed deposition of PAS-positive hyaline material in dermis. A homozygous nonsense mutation in exon 3 of the ECM1 gene, R53X, was detected in the family. Acitretin therapy was administered in two patients, in whom some regression and softening of skin lesions were achieved. However, no histopathological change in PAS-positive deposition could be detected. Although there is no current effective treatment for LP, acitretin may be helpful for patients, especially those who complain about hyperkeratosis.

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