Dermatose Neutrofílica Do Dorso Das Mãos – A Propósito De Dois Casos Clínicos

Abstract

O conceito de dermatoses neutrofílicas engloba diversas entidades que partilham aspetos clínicos e histológicos. Recentemente descrita, a dermatose neutrofílica do dorso das mãos é uma patologia com etiologia desconhecida que se assemelha, clinicamente, a uma variante localizada da Síndrome de Sweet com lesões predo- minantemente localizadas no dorso das mãos e com presença variável de sintomas gerais acompanhantes. Do ponto de vista histológico é caracterizada por um denso infiltrado inflamatório neutrofílico, sendo variável a existência de achados de vasculite. Os autores apresentam dois doentes com dermatose neutrofílica do dorso das mãos e discutem os aspetos clínicos, laboratoriais, histopatológicos e a as opções terapêuticas desta entidade. A dermatose neutrofílica do dorso das mãos é um conceito em evolução, que partilha diversos aspetos comuns com outras dermatoses neutrofílicas, sugerindo um espetro continuo deste grupo de doenças. PALAVRAS-CHAVE – Dermatose da mão; Síndrome de Sweet.

Neutrophilic dermatosis of the dorsal hands: response to dapsone monotherapy

“…Currently, NDDH is a rare entity, with about 60 cases described in the literature. 1 It preferentially affects female patients with an average age of 62, commonly manifesting as papules, plaques, pustules, blisters and ulcerations, and usually limited to the dorsum of one or both hands 1 -a similar situation was observed in this case. Signs of vasculitis can be observed, as previously indicated.…”

“…The etiology of NDDH is unknown, though there are reports of its association with infections of the respiratory tract, hepatitis C infection, inflammatory bowel disease, neoplasms (particularly hematological), autoimmune diseases (seropositive arthritis), medications (lenalidomide, thalidomide), vaccines and exposure to fertilizers. 1 , 3 , 5 None of these associations have been observed in the patient thus far.…”

“…Similar to what occurs in other neutrophilic dermatoses, the cutaneous lesions of NDDH may be accompanied by systemic manifestations-most frequently fever, general unwellness, arthralgia, or peripheral adenopathy-none of which were observed in this patient. 1 …”

“…Neutrophilic dermatoses represent a set of entities whose characteristic is the presence of an inflammatory infiltrate rich in neutrophils, without evidence of infectious agents, that can appear in localized cutaneous, generalized cutaneous and systemic forms. 1 - 3 Neutrophilic dermatosis of the dorsal hands (NDDH) presents morphological and histopathological characteristics resembling those of Sweet's syndrome (SS) and is considered a rare and localized variant of this syndrome. 1 , 3 , 4 …”

“… 1 - 3 Neutrophilic dermatosis of the dorsal hands (NDDH) presents morphological and histopathological characteristics resembling those of Sweet's syndrome (SS) and is considered a rare and localized variant of this syndrome. 1 , 3 , 4 …”