Os histiócitos e as histiocitoses não Langerhans em dermatologia

Abstract

Atualmente, os histiócitos não são mais compreendidos como células únicas, mas como um grupo heterogêneo de células com o mesmo aspecto histológico, mas com características e funções distintas entre si. Várias doenças proliferativas de histiócitos, conhecidas como histiocitoses, são descritas. Tais doenças são raras, e seu estudo costuma ser difícil. Este artigo objetiva simplificar o entendimento desse grupo de doenças, adequando-o a esse novo paradigma da heterogeneidade dos histiócitos.

Rosai Dorfman disease- A rare entity: Case report in North Indian male

“…7 This disease is characterized by extensive lymphadenopathy, fever, weight loss, night sweats polyclonal gammopathy, auto immune hemolytic anaemia and raised erythrocyte sedimentation rate. 2 Forty-three percent of cases also shows extranodal manifestations with skin being the most common site. 8 Other sites are soft tissue, nasal cavity, eye, retro orbital tissue, GI tract, liver, pancreas, breast, urogenital tract, parotid gland and CNS.…”

“…1 Patients with this disease presents with massive lymphadenopathy with systemic symptoms like fever, neutrophilia, leucocytosis, raised ESR and polyclonal gammopathy. 2,3 The Etiology of this disease entity is still unclear. Less than 1000 cases have been reported in literature worldwide with young adult males being most affected.…”

Doença de Rosai-Dorfman cutânea: relato de caso

“…Around 10% of patients have skin lesions and in 3% the disease is limited exclusively to the skin. 1,9 The etiology of the disease remains unknown despite some reports that the systemic form of the disease coexists with herpesvirus hominis-6 and 8 (HHV-6 and 8) and Epstein-Barr virus (EBV) infection. Investigators have also attempted to demonstrate this association in small series of cases in the cutaneous forms of the disease; however, results have been varied, with the majority testing negative.…”

“…2 Contrary to the systemic form, which affects principally children and young adults and which shows no preference for gender or ethnic group, the purely cutaneous form of the disease is slightly more common in older age-groups, in women and in nonblack ethnic groups. 1,3,4,6,11,12 Clinically, it presents with papules, nodules, plaques, masses or tumors of a brownish-or yellowish-erythematous color, varying in size from less than 1 cm to 30 cm, either localized or disseminated. The most common sites affected are, in order of decreasing frequency, the trunk, head, neck, lower and upper extremities.…”

“…[1][2][3][4][5][6][7][8] It is characterized clinically by a massive, bilateral, painless cervical lymphadenopathy, usually in association with fever, weight loss, night sweats, leukocytosis with neutrophilia, increased erythrocyte sedimentation rate and polyclonal gammopathy. 1,2,[8][9][10] Although less common, other lymph node chains such as the axillary, inguinal, paraaortic or mediastinal chains may be affected. 8,11 Extranodal involvement occurs in 43% of cases, either in association with lymph node involvement or otherwise.…”