The early erosive vesicular stage of lipoid proteinosis: clinical and histopathological features

Sir, Lipoid proteinosis (LP) is a rare autosomal recessively inherited disorder, which is characterized by the deposition of hyaline‐like material in the skin, mucous membranes and other tissues.1–3 The presenting manifestation of LP is hoarseness due to involvement of the larynx, which remains throughout life.4 The skin lesions usually appear during the first 2 years of life and occur in two overlapping stages. In the first stage, vesicles, bullae and haemorrhagic crusts appear spontaneously, and resolve with scarring. In the second stage, the skin becomes thickened and yellowish due to the deposits, and waxy papules and plaques develop predominantly on the face, scrotum and axillae.1–3, 5, 6 As detail about the evolution and histopathology of early lesions of LP is lacking, we describe the early erosive vesicular lesions in detail both clinically and histologically. A 4‐year‐old girl presented with eroded lesions. She had been followed up for 3 years following the diagnosis of LP. In this period of time the disease course showed fluctuations and she had some severe activations, especially in summer. A life‐threatening exacerbation of LP in this patient was reported previously.7 Several small haemorrhagic crusts, measuring 3–5 mm in diameter, had been observed at almost every visit.