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]
Signs and symptoms
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]
Diagnosis
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]
Treatment
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]
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abcGlavin, F. L.; Chhatwall, H.; Karimi, K. (October 26, 2009). "Progressive nodular histiocytosis: a case report with literature review, and discussion of differential diagnosis and classification". Journal of Cutaneous Pathology. 36 (12). Wiley: 1286–1292.
doi:
10.1111/j.1600-0560.2009.01454.x.
ISSN0303-6987.
PMID19878386.
S2CID22414587.