Progressive nodular histiocytosis | |
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Specialty | Dermatology |
Progressive nodular histiocytosis is a cutaneous condition clinically characterized by the development of two types of skin lesions: superficial papules and deeper larger subcutaneous nodules. [1]: 718 Progressive nodular histiocytosis was first reported in 1978 by Taunton et al. [2] It is a subclass of non-Langerhans cell histiocytosis and a subgroup of xanthogranuloma. [3]
Progressive nodular histiocytosis most frequently affects young to middle-aged adults who show up with widely distributed, randomly distributed, reddish-brown, cutaneous papules and nodules that are not painful or pruritic. [4] Progressive nodular histiocytosis's clinical course is characterized by an unwavering lack of spontaneous remission. [5] Over time, lesions grow larger and more numerous, and they can cause noticeable disfigurement. [6] [3] Although mucous involvement is possible, [7] internal organs are typically unaffected. [8] Mechanical interference caused by lesions in critical locations, such as the eyelids or the soles of the feet, can result in functional impairment. [8] [9] There is a rare possibility that the cutaneous lesions could cause systemic effects directly; microcytic anemia due to significant intralesional iron sequestration has been documented. [8] Obstructive lesions in the upper airway have been associated with death, despite the fact that they are usually not life-threatening. [9]
Histologically, it is typified by a diffuse infiltrate of Touton giant cells and xanthomatized histiocytes mixed in with spindle-shaped histiocytes with a whorl-like growth pattern. [3]
The primary method of treating progressive nodular histiocytosis is surgical excision. [7] [8] However, a few cases have been reported to have improved following methotrexate administration. [10] Other treatments such as carbon dioxide laser, intralesional as well as systemic steroids, [8] and antineoplastic agents such as imatinib have mostly shown no effect on progressive nodular histiocytosis. [4] Unfortunately, for those who are affected, recurrence is possible even after treatment. [7]
Progressive nodular histiocytosis | |
---|---|
Specialty | Dermatology |
Progressive nodular histiocytosis is a cutaneous condition clinically characterized by the development of two types of skin lesions: superficial papules and deeper larger subcutaneous nodules. [1]: 718 Progressive nodular histiocytosis was first reported in 1978 by Taunton et al. [2] It is a subclass of non-Langerhans cell histiocytosis and a subgroup of xanthogranuloma. [3]
Progressive nodular histiocytosis most frequently affects young to middle-aged adults who show up with widely distributed, randomly distributed, reddish-brown, cutaneous papules and nodules that are not painful or pruritic. [4] Progressive nodular histiocytosis's clinical course is characterized by an unwavering lack of spontaneous remission. [5] Over time, lesions grow larger and more numerous, and they can cause noticeable disfigurement. [6] [3] Although mucous involvement is possible, [7] internal organs are typically unaffected. [8] Mechanical interference caused by lesions in critical locations, such as the eyelids or the soles of the feet, can result in functional impairment. [8] [9] There is a rare possibility that the cutaneous lesions could cause systemic effects directly; microcytic anemia due to significant intralesional iron sequestration has been documented. [8] Obstructive lesions in the upper airway have been associated with death, despite the fact that they are usually not life-threatening. [9]
Histologically, it is typified by a diffuse infiltrate of Touton giant cells and xanthomatized histiocytes mixed in with spindle-shaped histiocytes with a whorl-like growth pattern. [3]
The primary method of treating progressive nodular histiocytosis is surgical excision. [7] [8] However, a few cases have been reported to have improved following methotrexate administration. [10] Other treatments such as carbon dioxide laser, intralesional as well as systemic steroids, [8] and antineoplastic agents such as imatinib have mostly shown no effect on progressive nodular histiocytosis. [4] Unfortunately, for those who are affected, recurrence is possible even after treatment. [7]