Dermatoscopy of pigmented melanocytic nevi in patients with oculocutaneous albinism

Oculocutaneous albinism is a group of rare inherited disorders of pigmentation. Some albinos may have a certain degree of melanin production and, consequently, may develop pigmented lesions during their lives. Albinos are also at great risk for developing cutaneous malignancy, including melanomas. We report two patients with oculocutaneous albinism and pigmented melanocytic nevi and describe, as far as we known, for the first time the dermatoscopic findings in albinos' nevi. The predominant dermatoscopic pattern observed in pigmented melanocytic nevi in albino patients seems to have a similar pattern to that observed in nevi of patients with skin type I. It corresponds to light-brown coloration, reticular pattern, and central hypopigmentation. (J Am Acad Dennatol 2009;60:487-9.

Mastocitose sistêmica na infância: relato de 3 casos Systemic mastocytosis in childhood: report of 3 cases

Objetivo: mastocitose é o termo usado para um grupo de moléstias caracterizadas pelo acúmulo de mastócitos na pele, com ou sem comprometimento de outros órgãos. Os sintomas podem ser apenas cutâneos, associados aos decorrentes dos órgãos envolvidos ou também sistêmicos, pela liberação de mediadores químicos. São descritos três casos de mastocitose sistêmica, cada um com diferentes manifestações próprias da doença.Descrição: no primeiro caso é relatado um paciente com urticária pigmentosa que evoluiu com persistência das lesões até a puberdade, quando iniciaram as manifestações sistêmicas com o comprometimento linforreticular, esplenomegalia e infiltração da medula óssea. No segundo caso, a paciente apresentava mastocitose bolhosa acompanhada de sintomas gastrintestinais, cuja investigação mostrou infiltração de mastócitos na parede intestinal. A terceira paciente ilustra a manifestação cutânea precoce e extensa da mastocitose, com uma evolução dramática para choque e posterior óbito. Comentários: são discutidos aspectos clínicos, laboratoriais, tratamento e diagnóstico.<br>Objective: mastocytosis comprises a group of diseases characterized by accumulation of mast cells on the skin, with the possibility of affecting other systems. Symptoms can be exclusively cutaneous, associated with symptoms of the organs involved or even systemic, due to the release of chemical mediators. Three cases of systemic mastocytosis are described, each case presenting different manifestations of the disorder. Description: the first report is about a patient with urticaria pigmentosa that presented persistent lesions until puberty when systemic manifestations initiated with lymphoreticular involvement, splenomegaly and bone marrow infiltration. In the second case, the child had bullous mastocytosis associated with gastrointestinal symptoms, whose investigation showed mast cell infiltration in the intestinal wall. The third patient presented an early and extensive cutaneous manifestation of mastocytosis, with a dramatic evolution to shock and posterior death.Discussion: clinical aspects, treatment and prognosis of such forms of the disease are discussed

Escalpe lipedematoso com início precoce Lipedematous scalp with early onset

Escalpe lipedematoso é uma rara doença de origem desconhecida, caracterizada pelo aumento amolecido do couro cabeludo. Apresentamos um caso em paciente feminina de 13 anos de idade, com espessamento amolecido do vértex, prurido e crescimento lento dos fios na região afetada. O anatomopatológico revelou edema, infiltrado mononuclear esparso e perifolicular discretos, vasos linfáticos ectásicos e aumento do tecido subcutâneo, o qual foi ratificado pela ultrassonografia. Este é o segundo relato antes dos 18 anos de idade.<br>Lipedematous scalp is a rare disease of unknown cause characterized by increased scalp thickness with a soft consistence. We report the case of a 13-year-old female with pruriginous soft thickening of the scalp vertex and slow growth of hair in the affected area. Anatomopathological examination showed edema, sparse mononuclear inflammatory perifollicular infiltrate, and ectatic lymphatic vessels. Ultrasonography confirmed thickening of the subcutaneous tissue. This is the second report of the disorder in a patient younger than 18 years old