Immunohistochemistry and electron microscopy in Langerhans cell histiocytosis confined to the skin

Late manifestation of diabetes insipidus in “pure” cutaneous Langerhans cell histiocytosis

“…Following the initial report by Hashimoto and Pritzker [9], about 100 additional cases of CSHLCH have been described [10]. This entity appears to be characterised by four features [2,14]: (1) congenital or perinatal occurrence of papules, nodules or papulovesicules; (2) in an otherwise healthy child lacking visceral involvement; (3) histopathology demonstrating an LC in®ltrate; and (4) spontaneous regression of skin lesions without recurrence.…”

“…First, CSHLCH can manifest itself beyond the neonatal period, i.e. during infancy [5,6,13,15] or later [6,10,18,23]. Second, skin lesions can persist beyond infancy [10,15], recur within the 1st year of life [13,14] or later [5], and may not be``self-healing'' but require speci®c therapy (excision, steroids, chemotherapy) [6,10,20] or even be unresponsive to therapy [10].…”

“…Hashimoto et al [10] separated three categories: (1) mild cases with early onset (birth to 4 months) and spontaneous resolution (1 month±1.5 years); (2) severe cases with onset from birth to 64 years, responsive to therapy and not recurring; and (3) cases with persistent or recurrent lesions, unresponsive to therapy, with manifestation from 8 months to 76 years (mostly >30 years). However, the individual outcome cannot be predicted at the time of onset [6].…”

Langerhans Cell Histiocytosis With Involvement of Nails and Lungs in an Adolescent

“…2,11,12 Incidence of skin and pulmonary involvement was reported as 50% and 20% to 50%, respectively, in patients with multisystem disease. [7][8][9][10][13][14][15][16] In a study from our institution, Karadeniz et al 2 reported that 66/99 children with LCH had multiple sites of involvement, of which 27 (40.9%) showed skin lesions and 7/29 patients with organ dysfunction showed pulmonary involvement. LCH is a slowly progressive and self-limiting disease with possible spontaneous remissions.…”

Caso para diagnóstico

“…O estudo imunoistoquímico demonstra marcadores CD1a e S100 positivos enquanto a microscopia eletrônica evidencia a presença de grânulos de Birbeck, que são patognomônicos. 3 O prognóstico depende da idade do paciente, da extensão do acometimento e da presença de falência em órgão vital. O tratamento depende da extensão e severidade da doença.…”