Progressive noduläre Histiozytose

Abstract

Progressive nodular histiocytosis (PNH) represents a very rare type of non-Langerhans cell histiocytosis (NLCH). It can be distinguished from other types by the occurrence of yellow to red-brown papules and nodules measuring a few centimeters in size. Histologically the nodules represent spindle-cell xanthogranulomas. We report on two new cases of PNH with novel clinical symptoms. In the first case, the patient had microcytic anemia due to massive iron deposition in the nodules and a progressively worsening limp due to involvement of the soles. The complex therapeutic challenge was effectively managed by successive surgical ablation of all visible lesions. The second patient had epibulbar infiltrates. The clinical variability of PNH leads to considerable overlap with other forms of NLCH.

Progressive nodular histiocytosis: an unusual disorder

“…Multiple superficial telangiectases on the surface of large nodules, as noted in our patient, have been described. Ulceration or infection is uncommon [5]. Bulky masses over 20cm derived from many coalescing pedunculated nodules have been reported [5,9,11,12].…”

“…Conjunctival, oropharyngeal, and laryngeal mucosa can be affected; involvement of the latter can lead to respiratory symptoms and hoarseness. Multiple lesions affecting the eyelids can lead to ectropion and visual disturbance [4,5,9]. Iron-storing nodules leading to anemia as a rare complication of PNH was reported in one patient [5].…”

“…Progressive nodular histiocytosis is defined by the progressive emergence of yellow-tobrown papules and pink-to-reddish nodules on the head, trunk, extremities, and mucous membranes. Facial involvement can be severe and lead to leonine facies and ophthalmological alterations [4][5]. Most reports of PNH describe adults but it can also affect the pediatric population.…”

“…Most reports of PNH describe adults but it can also affect the pediatric population. No direct association with malignancies has been reported although it can appear in patients with cancer history and may indicate recurrence of the tumor [5]. Histopathological examination of both papules and nodules is necessary for diagnosis because of differences in histiocyte morphology in both lesions.…”

Effective treatment of progressive nodular histiocytosis by intermediate-dose cytarabine: a case report and literature review

“…Although PNH, like most NLCHs, typically does not have extra-cutaneous manifestations, various associated conditions have been reported, including a male with chronic myeloid leukemia and metabolic disorders(9), a woman with thrombocytopenia and angiobromas(10) and a 9-year-old girl with a hypothalamic tumor (6). Additionally, there have been reports of microcytic anemia due to massive iron deposition in the nodules (7). However, no relevance has been confirmed so far between PNH and those systematic disorders.…”