The authors describe a case of a female patient with Acral Pseudolymphomatous Angiokeratoma of Children, known as APACHE. It is a rare benign cutaneous disease, of unknown etiology, characterized by multiple, asymptomatic erythematous-violaceous papules and nodules, usually located unilaterally with acral distribution. Today, this denomination is questionable, since there are published reports of this disease in adults and in different locations. Clinically, it is similar to an angiokeratoma, whereas hystologically, it corresponds to a distinct type of pseudolymphoma. The immunohistochemical study is required to distinguish APACHE from cutaneous lymphoma.

We report the case of a patient diagnosed with genitogluteal porokeratosis, a disorder of epidermal keratinization. The location described is extremely rare and very often late diagnosed or even misdiagnosed. Histopathology showed a typical cornoid lamella of great value to support this diagnosis. The importance of awareness of this entity by the specialist is emphasized as a differential diagnosis among genital diseases of chronic evolution and difficult treatment.

Síndrome de Graham-Little-Piccardi-Lassueur (SGLPL) é uma dermatose rara, de etiologia desconhecida, que acomete principalmente mulheres entre 30 a 60 anos de idade. Caracteriza-se pela tríade de alopecia cicatricial multifocal do couro cabeludo, erupção folicular liquenoide e hipotricose das regiões axilar e pubiana. O exame anatomopatológico revela diminuição do numero dos folículos pilosos, infiltrado perifolicular e fibrose. A terapêutica é um desafio, com recidivas frequentes após tratamentos locais e sistêmicos. Relatamos um caso típico desta síndrome rara, em um paciente sexo masculino de 46 anos.