Multisystemic Langerhans Cell Histiocytosis Presenting As Chronic Scalp Eczema: Clinical Management and Current Concepts

Müller, Cornelia; Janssen, Eva; Schmaltz, Rebecca; Körner, Heiko; Vogt, Thomas; Pföhler, Claudia

doi:10.1200/jco.2010.33.9127

Cited by 7 publications

(6 citation statements)

References 18 publications

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“…More specifically, adult intracranial LCH cases involve infiltration of the posterior pituitary. The most common indicator that LCH has become intracranial is central diabetes insipidus (DI) [17][18][19], which occurs in about 15% of patients with PLCH [20]. These findings suggest that DI is the most common endocrine alteration in PLCH.…”

Section: Discussionmentioning

confidence: 99%

Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Miyashita

Yamazaki

Ohta

et al. 2020

Respirology Case Reports

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A 65-year-old man presented to our hospital with complaint of acute dyspnoea. He smoked 30 cigarettes per day from age 20 to 52 years. Immunocytochemical findings revealed 6.3% of positive CD1a cells in the cell fraction of bronchoalveolar lavage, thus suggesting a diagnosis of pulmonary Langerhans cell histiocytosis (PLCH), after nine years since the first suspicion of PLCH. Furthermore, he was diagnosed with secondary pulmonary hypertension (PH) caused by progressed PLCH by right heart catheterization. At 59 years of age, he was diagnosed with panhypopituitarism, and persistent hormone replacement therapy was subsequently started by an endocrinologist. After the initiation of oxygen therapy and treatment with a combination of sildenafil and warfarin, an estimated pulmonary artery systolic pressure reduced 97.9 to 64.0 mmHg. We believed this is a rare case of PLCH with irreversible central nervous system (CNS) disorder in whom severe PH developed due to a long-term burden of PLCH in a middle-aged male.

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Section: Discussionmentioning

confidence: 99%

Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Miyashita

Yamazaki

Ohta

et al. 2020

Respirology Case Reports

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“…The presence of CD1a – and CD207 – antigens can confirm the diagnosis 4, 6. LCH can be diagnosed if at least 2 stains for ATPase, S-100 protein, α-D-mannosidase, or binding of peanut lectin on lesional cells are positive 6 .…”

Section: Discussionmentioning

confidence: 99%

“…Tooth extractions should be avoided in these patients 3 . Bone lesions may be treated by surgery, intralesional injection of methylprednisolone, or radiotherapy 3, 4…”

Section: Discussionmentioning

confidence: 99%

“…The etiology of Langerhans cell histiocytosis (LCH), a mix between immune dysregulation, inflammation, and malignancy, remains unclear 1, 2, 3, 4. In half of the patients, an oncogenic BRAF mutation is found 3 .…”

Section: Introductionmentioning

confidence: 99%

“…Because of the diversity of symptoms, the diagnosis of LCH, as defined by the Histiocyte Society, is often made with considerable delay. Multisystemic LCH, affecting 2 or more organ systems and “risk organs,” like the hematopoietic system, the spleen, liver, and central nervous system, implies a worse prognosis 1, 3, 4, 5, 6. To raise the awareness of multisystemic LCH, the case of an elderly patient is presented and a new therapeutic scheme with pioglitazone (peroxisome proliferator–activated receptor-γ [PPAR-γ] agonist), etoricoxib (COX-2 inhibitor), and trofosfamide (alkylating medium) is described.…”

Section: Introductionmentioning

confidence: 99%

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Multisystemic Langerhans cell histiocytosis in an adult

Hegemann

Schreml

2017

JAAD Case Reports

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Monosystemische, oligoläsionale Langerhans-Zell-Histiozytose der Haut

et al. 2012

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A five-day-old female infant presented with congenital red-livid papules and nodules on the head, chest, back and left arm. The nodule on the chest was ulcerated at birth. The pregnancy and delivery were uneventful. There was no history of birth trauma to account for the ulcerated lesion. The parents and the three older siblings were healthy without similar skin lesions. The skin biopsy showed in the deep layer of the dermis a multinodular, granulomatous histiocytic infiltrate. The histiocytic cells expressed S-100 and CD1a. There were additionally many eosinophils. The suspected diagnosis Langerhans cell histiocytosis was confirmed by the histologic results. The patient was referred to pediatric hematology-oncology where evaluation showed no evidence of systemic involvement. The clinical, radiological, sonographic and histological results led to the diagnosis of a congenital, monosystemic, oligolesional Langerhans cell histiocytosis of the skin. In addition to the case presentation, we review the current stand of knowledge of the pathogenesis, the clinical classification and the therapy of the Langerhans cell histiocytosis.

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Multisystemic Langerhans Cell Histiocytosis Presenting As Chronic Scalp Eczema: Clinical Management and Current Concepts

Cited by 7 publications

References 18 publications

Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Secondary pulmonary hypertension due to pulmonary Langerhans cell histiocytosis accompanied with panhypopituitarism

Multisystemic Langerhans cell histiocytosis in an adult

Monosystemische, oligoläsionale Langerhans-Zell-Histiozytose der Haut

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