Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report

Salunke, Aarti S.; Belgaumkar, Vasudha Abhijit; Chavan, Ravindranath B.; Dobariya, Rinkesh

doi:10.4103/2229-5178.193913

Cited by 3 publications

(4 citation statements)

References 15 publications

(16 reference statements)

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“…Since PNH normally localized in skin and/or mucosa, patients remained in good general health without visceral involvement. However, one patient with laryngeal involvement died due to airway obstruction (17).…”

Section: Resultsmentioning

confidence: 99%

“…Facial and/or mucosa involvement is a prominent feature and may cause a leonine appearance, and eyelid, conjunctival, oral, or laryngeal lesions may also occur (2,18). laryngeal involvement can be fatal sometimes due to airway obstruction (17). In addition, the widespread skin nodules and leonine face appearance are problematic for patients, especially for a young girl, and seriously affect quality of life, impair social interactions, even lead to suicidal tendencies.…”

Section: Discussionmentioning

confidence: 99%

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

Wang

Qiu

Sun

et al. 2020

Preprint

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Background Progressive nodular histiocytosis (PNH) is a rare, clinically distinct non-Langerhans cell histiocytosis, affecting the skin and mucous membranes with no signs of spontaneous regression of the lesions, without any other system involved. Patients typically have hundreds of lesions of superficial xanthomatous papules to nodules and deep larger fibrous nodules. So far, very few universally accepted reports of PNH existed and no effective treatment have been reported to reverse the progressive clinical course. Results Twenty-five PNH cases were identified so far, with a median age of 34 years. 14 patients were male. All patients presented as papules and nodules over the skin and/or mucosal surfaces, including face (39%), fingers or palm (16%), laryngeal (12%) and eyes (8%). 7 cases (28%) reported PNH with systematic diseases. Treatment efficacy using steroids, chemotherapy or surgical excision were disappointing in previous case reports. One patient with laryngeal involvement died due to airway obstruction. In our case, traditional chemotherapy achieved favorable therapeutic effect with intermediate-dose cytarabine confirmed by clinical symptoms of the patient, as well as PET/CT imaging. Conclusions PNH is a rare, proliferative disorder with disfiguring lesions and deteriorative quality of life. Intermediate-dose cytarabine should be considered as a treatment option for PNH patients.

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Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Wang

Qiu

Sun

et al. 2020

Preprint

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“…Mucosal involvement may occur [9,10], but internal organs are not usually affected [10]. Functional impairment may occur from mechanical interference brought on by critically positioned lesions on sites like the soles of the feet and eyelids [10,11]. On very rare occasions, the cutaneous lesions may directly invoke systemic effects-a case of microcytic anemia from marked intralesional iron sequestration is on record [10].…”

Section: Discussionmentioning

confidence: 99%

“…On very rare occasions, the cutaneous lesions may directly invoke systemic effects-a case of microcytic anemia from marked intralesional iron sequestration is on record [10]. Although not usually life-threatening, death has been reported from obstructive lesions in the upper airway [11].…”

Section: Discussionmentioning

confidence: 99%

Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature

Numbere

Pukhalskaya

Bowman

et al. 2021

Case Reports in Pathology

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Progressive nodular histiocytosis (PNH) is a rare condition characterized by progressive eruption of multiple yellowish-brown papules and nodules on the skin and mucous membranes. We present the case of a 37-year-old Caucasian man with gradually increased appearance of nodular lesions on the forehead and right temple. These lesions were initially diagnosed as xanthomas and did not respond to intralesional injections of triamcinolone. Additional biopsy revealed an intense dermal infiltrate of foamy mononuclear epithelioid cells with a minor admixture of plasma cells, lymphocytes, and scattered multinucleated giant cells. On immunohistochemical staining, the lesional cells were positive for CD163 and CD68 and negative for CD1a, thus confirming a mononuclear-macrophage lineage. The clinical presentation and the histological impression lead to the diagnosis of PNH. This condition could be challenging, mimicking microscopically similar lesions of the non-Langerhans cell histiocytosis group. Although uncommon, PNH stands out due to its clinical and microscopic features and should be taken into consideration in the differential diagnosis of cutaneous histiocytoses.

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X-Ray Diagnostics of Pulmonary Langerhans Cell Histiocytosis

Леншин¹,

Леншин²,

Il'in³

et al. 2017

Bulletin Physiology and Pathology of Respiration

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Laryngeal involvement with fatal outcome in progressive nodular histiocytosis: A rare case report

Cited by 3 publications

References 15 publications

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

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

Progressive Nodular Histiocytosis: Report of a Case and Review of the Literature

X-Ray Diagnostics of Pulmonary Langerhans Cell Histiocytosis

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