In this cohort of patients with PCL from southern Brazil, CTCL/NKCL was more prevalent than CBCL, and the MF subtype was predominant. Survival curves were similar to those reported in the literature, except for Sézary syndrome, which had a better prognosis in this sample.

Abstract:A 32-year-old male patient presented for 8 months an asymptomatic therapy-resistant acneiform eruption on his back and buttocks. Skin examination showed several inflammatory papules, which evolved to hyperpigmentation. At the same distribution non inflammatory papules, which resembled rice grains, were also observed. Light microscopy showed small keratin-filled cysts, with an epithelial multilayered wall, without granular layer. Keratin and some vellus hairs were identified inside the cyst, confirming the diagnosis of vellus hair cysts. Diagnosis of vellus hair cysts should be suspected in cases of multiple papules or therapy-resistant cases of acneiform eruptions. Keywords: Acne vulgaris; Acneiform eruptions; Follicular cyst; Isotretinoin; Young adult; Keratins Resumo: Um paciente de 32 anos apresentou há 8 meses uma erupção acneiforme resistente à terapêu-tica, localizada no dorso e nádegas. Ao exame apresentava inúmeras pápulas inflamatórias, que evoluiam para hiperpigmentação. Na mesma distribuição havia lesões não inflamatórias, lembrando grão de arroz. A microscopia óptica demonstrou cisto dérmico cuja parede era de epitélio estratificado, sem camada granular, preenchido de queratina e com pelos no interior, confirmando o diagnóstico de cisto de pelo veloso. Essa entidade deve ser suspeitada em casos de múltiplas pápulas ou erupções acneiformes resistentes à terapia.

Pasini's albopapuloid epidermolysis bullosa is a very rare subtype of generalized dystrophic dominant epidermolyis bullosa. A 30 year-old white female patient presented since her childhood disseminated small blisters and papules. Light microscopy of a blister showed dermal-epidermal cleavage; moreover, focal areas of dermal-epidermal splitting were also observed. Transmission electron microscopy also identified focal areas of cleavage, which were seen below the lamina densa. It is important to recognize this condition as a variant of epidermolysis bullosa, since the most important cutaneous findings are generalized papules and not blisters and erosions as in other forms of epidermolysis bullosa. Keywords: Epidermolysis bullosa dystrophica; Epidermolysis bullosa, junctional; Epidermolysis bullosa simplex; Histology; Microscopy, electron, transmissionResumo: A epidermólise bolhosa albo-papulóide de Pasini é uma variante rara da forma generalizada de epidermólise bolhosa distrófica dominante. Uma paciente de 30 anos apresenta desde a infância pápulas e bolhas disseminadas. A microscopia óptica de uma bolha demonstrou clivagem dermo-epidérmica; além disso áreas focais de desprendimento dermo-epidérmico foram encontradas. A microscopia eletrônica de transmissão identificou a clivagem abaixo da lâmina densa. É importante que se reconheça essa variante de epidermólise bolhosa, já que o aspecto clínico predominante são pápulas disseminadas e não bolhas como nas outras formas de epidermólise bolhosa.

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