Congenital self-healing reticulohistiocytosis in a newborn: unusual oral and cutaneous manifestations

Abstract Background Congenital self-healing reticulohistiocytosis (CSHRH), also called Hashimoto-Pritzker disease, is a rare and benign variant of Langerhans cell histiocytosis, characterized by cutaneous lesions without extracutaneous involvement. Case presentation We present a case of CSHRH with diffuse skin lesions and erosions in the oral mucosa, present since birth and lasting for 2 months, and we perform a review of the literature on Pubmed in the last 10 years. Conclusions Our case confirm that lesions on oral mucosa, actually underestimated, may be present in patients with CSHRH. Patients affected by CSHRH require a close follow-up until the first years of life, due to the unpredictable course of Langerhans cell histiocytosis, in order to avoid missing diagnosis of more aggressive types of this disorder.

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A Dose-Response Relationship to Radiotherapy for Cutaneous Lesions of Langerhans Cell Histiocytosis

Case Reports in Oncological Medicine ◽

10.1155/2021/6680635 ◽

2021 ◽

Vol 2021 ◽

pp. 1-6

Author(s):

Mark K. Farrugia ◽

Carl Morrison ◽

Francisco Hernandez-Ilizaliturri ◽

Saif Aljabab

Keyword(s):

Pain Control ◽

Langerhans Cell Histiocytosis ◽

Metabolic Response ◽

Single Agent ◽

Skin Lesions ◽

Langerhans Cell ◽

Cutaneous Lesions ◽

Current Case ◽

Organ Systems

Langerhans cell histiocytosis (LCH) is a rare disease, afflicting approximately 4.6 and 1-2 per 1 million children and adults, respectively. While LCH can involve numerous organ systems such as the lung or bone, it is uncommon for the disease to be limited to the skin. Radiotherapy has an established role for osseous lesions. However, the efficacy and dose for nonosseous manifestations of the disease are not well described. In the current case report, we detail a 49-year-old adult male with skin-limited LCH requiring palliative radiotherapy (RT) to numerous sites for pain control. The patient was initially diagnosed and treated with single agent cytarabine for approximately 6 months. Despite treatment, he had little symptomatic response of his cutaneous lesions. We delivered a single dose of 8 Gray (Gy) to 3 separate skin lesions, including the bilateral groin, right popliteal region, and right axillary lesion, which resulted in pain reduction and partial response at four-month follow-up. Subsequently, we decided to treat the left axillary untreated lesion to a higher dose of 24 Gy in 12 fractions. At four-month follow-up, the left axilla RT resulted in complete clinical response and improved pain control compared to the right axilla. Following RT treatments, the patient was found to have a BRAF mutation, and vemurafenib was initiated. Further follow-up with positron emissions tomography demonstrated complete metabolic response in numerous disease areas, including both axillae. Based on this case report’s findings, a higher radiotherapy dose may be more effective for treating cutaneous LCH.

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Rosai-Dorfman Disease: Rare Pulmonary Involvement Mimicking Pulmonary Langerhans Cell Histiocytosis and Review of the Literature

Case Reports in Radiology ◽

10.1155/2018/2952084 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Rashid AL Umairi ◽

Danielle Blunt ◽

Wedad Hana ◽

Matthew Cheung ◽

Anastasia Oikonomou

Keyword(s):

Langerhans Cell Histiocytosis ◽

Corticosteroid Treatment ◽

Pulmonary Involvement ◽

Skin Lesions ◽

Langerhans Cell ◽

Sinus Histiocytosis ◽

Review Of The Literature ◽

Massive Lymphadenopathy ◽

Pulmonary Langerhans Cell Histiocytosis ◽

Rosai Dorfman Disease

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare nonmalignant lymphohistiocytic proliferative disorder. We report a patient with RDD who presented with multiple skin lesions, pulmonary involvement, and CT manifestations mimicking Langerhans cell histiocytosis, which improved after initiation of corticosteroid treatment.

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Langerhans cell histiocytosis of the rib presenting with pathological fracture: a case report

Journal of Cardiothoracic Surgery ◽

10.1186/s13019-020-01368-9 ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Tao Zuo ◽

Ping Jiang ◽

Junjie Yu ◽

Ke Zhao ◽

Yong Liu ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Pathological Fracture ◽

Soft Tissues ◽

Langerhans Cell ◽

Rib Fracture ◽

Unknown Etiology ◽

Case Presentation ◽

Left Chest ◽

Rib Tumor

Abstract Introduction Langerhans cell histiocytosis (LCH) is a rare neoplastic hyperplasia with an unknown etiology. It is clinically rare for patients with solitary rib lesion and pathological fracture; moreover, its diagnosis and treatment are quite difficult. The purpose of this study is to present a case for the pathogenesis, clinical features, imaging, and treatment of this disease. Case presentation A 52-year-old female patient complained of left chest pain for one week. CT showed a fracture in the left 5th rib. The rib tumor was then resected and the surrounding muscles and soft tissues were accordingly resected. The patient was diagnosed with pathological rib fracture, and the patient was pathologically diagnosed with LCH. After surgery, no local recurrence or distant metastasis was reported during the two-year follow-up. Conclusions LCH should be treated by observation, chemotherapy, radiotherapy, or surgery, or using a combination of several methods. Moreover, primary tumor should be considered when rib fracture without trauma and tumor metastasis.

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Self-Healing Congenital Langerhans Cell Histiocytosis Presenting as Neonatal Papulovesicular Eruption

Journal of Cutaneous Medicine and Surgery ◽

10.1177/120347540100500605 ◽

2001 ◽

Vol 5 (6) ◽

pp. 486-489 ◽

Cited By ~ 5

Author(s):

Kelli W. Morgan ◽

Jeffrey P. Callen

Keyword(s):

Langerhans Cell Histiocytosis ◽

Rare Condition ◽

Signs And Symptoms ◽

Male Infant ◽

Skin Lesions ◽

Langerhans Cell ◽

Self Healing ◽

Systemic Involvement ◽

S 100 ◽

Simplex Virus

Background: Congenital self-healing Langerhans cell histiocytosis (CSHLCH) is a rare condition which may present at birth or during the neonatal period. It is usually characterized by the eruption of multiple, disseminated, red-brown papules and nodules which may increase in size and number during the first few weeks of life. Systemic signs are usually absent except for occasional mild hepatomegaly. Objective: We present a 3.5-kg male infant who presented at birth with numerous diffuse, erythematous, crusted erosions. He was presumed to have congenital herpes simplex virus (HSV) and was started on IV acyclovir. Histopathologic examination revealed a mixed inflammatory infiltrate with numerous histiocytes which were S-100 and peanut agglutin positive consistent with CSHLCH. Further workup did not reveal any signs of systemic involvement. Conclusion: CSHLCH has rarely been reported to present as a papulovesicular eruption at birth. In these cases, a viral etiology is commonly entertained in the differential diagnosis. Despite the spontaneous regression of skin lesions in CSHLCH, close followup is required to evaluate for systemic signs and symptoms associated with latent Letterer–Siwe disease.

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A diagnosis more than skin deep: A case of late-stage pulmonary langerhans’ cell histiocytosis with skin lesions

Case Reports in Internal Medicine ◽

10.5430/crim.v4n1p68 ◽

2017 ◽

Vol 4 (1) ◽

pp. 68

Author(s):

Josh Verson ◽

Alexander Egusquiza ◽

David C Nguyen

Keyword(s):

Smoking Cessation ◽

Late Stage ◽

Langerhans Cell Histiocytosis ◽

Inhaled Corticosteroids ◽

Age Groups ◽

Occupational Exposures ◽

Skin Lesions ◽

Langerhans Cell ◽

Cutaneous Lesions ◽

Radiographic Findings

Introduction: Langerhans’ cell histiocytosis (LCH) is a group of rare diseases with a wide variety of presentations across age groups. We describe a case of pulmonary LCH diagnosed at a late stage despite having additional skin findings for several years.Case description: A 32-year-old male presented with acute exacerbation of chronic dyspnea resulting in markedly decreased functional capacity as well as cutaneous lesions. He had smoked cigarettes for 10 years with no occupational exposures. The patient also complained of a pruritic rash in his axillary and groin areas. His vitals were within normal limits. His exam was notable for diminished right lung sounds and expiratory wheezes bilaterally. A chest x-ray showed marked emphysema and bilateral upper lobe reticular opacities suggestive of scarring. Computerized tomography of the chest showed loculated right pneumothorax and hyperinflated lungs with destructive changes secondary to numerous irregular, thick walled cysts and scatteredirregular pulmonary nodules. Diagnosis was ultimately confirmed with biopsy of concomitant cutaneous lesions and characteristic radiographic findings on Chest CT. The patient started inhaled corticosteroids, inhaled anticholinergic, and inhaled short-acting beta agonists. He was sent home with oxygen and strongly encouraged to abstain from smoking.Discussion: LCH is characterized by end-organ infiltration of proliferating monoclonal Langerhans’ cells, a histiocyte involved in antigen presentation. The highest incidence of LCH is between ages 5-10 years; the adult incidence is estimated to be less than half as frequent than the pediatric, about one patient in 500,000. LCH most commonly involves the skin, bones, and lungs. Pulmonary LCH (PLCH) is interesting in that it has a peak incidence in ages 20-40 years and occurs almost exclusively in smokers (> 90% of cases). Conclusion: Smoking cessation is the cornerstone of management of PLCH. It has been hypothesized that tobacco smoke can incite the production of cytokines by alveolar macrophages, leading to increased recruitment of histiocytes to the lung parenchyma. In isolated pulmonary pathology, lung transplant with smoking cessation is often curative.

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Congenital self-healing Langerhans cell histiocytosis: The need for a long term follow up

International Journal of Dermatology ◽

10.1046/j.1365-4362.2003.16142.x ◽

2003 ◽

Vol 42 (3) ◽

pp. 245-246 ◽

Cited By ~ 26

Author(s):

Margarita Larralde ◽

Alicia Rositto ◽

Margarita Giardelli ◽

Carlos F. Gatti ◽

Andrea Santos-Munoz

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Self Healing ◽

Long Term Follow Up

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Disseminated crusted papules in a newborn

Vojnosanitetski pregled ◽

10.2298/vsp0607681p ◽

2006 ◽

Vol 63 (7) ◽

pp. 681-683

Author(s):

Milos Pavlovic ◽

Aleksandra Minic ◽

Lidija Zolotarevski ◽

Sonja Vesic

Keyword(s):

Langerhans Cell Histiocytosis ◽

Specific Treatment ◽

Langerhans Cell ◽

The Body ◽

Self Healing ◽

Disease Case ◽

Mucous Membranes ◽

Atrophic Scars ◽

Systemic Symptoms

Background. Congenital self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease) is the rarest form of Langerhans cell histiocytosis, usually confined to the skin and/or mucous membranes. Cutaneous eruption is mostly generalized, papular, nodular or vesicular. Despite impressive clinical presentation in a newborn it infrequently spreads to internal organs (which then portends a grave prognosis, indistinguishable from Letterer-Siwe disease). Case report. We presented a full-term newborn, female, 3.3 kg who had a multitude of erythematous and crusted papules, nodules and pseudovesicles distributed all over the body, except for the mucous membranes. A biopsy and haematoxylin ? eosin stain revealed dermal infiltration of pleomorphic histiocytes with eosinophilic ground-glass cytoplasm and round to bean-shaped nuclei. Over the next six weeks the eruption gradually subsided leaving no residues, or a few atrophic scars. Conclusion. There is no need for specific treatment of congenital self-healing Langerhans cell-histiocytosis in the absence of multiorgan involvement. However, a close and regular follow-up is necessary to evaluate the children for systemic symptoms and signs.

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Langerhans Cell Histiocytosis of the Adult Cervical Spine: A Case Report and Literature Review

Journal of Neurological Surgery Part A Central European Neurosurgery ◽

10.1055/s-0038-1655769 ◽

2018 ◽

Vol 80 (01) ◽

pp. 049-052 ◽

Cited By ~ 1

Author(s):

Ekkehard Hewer ◽

Christian Ulrich ◽

Ralph Schär

Keyword(s):

Differential Diagnosis ◽

Cervical Spine ◽

Langerhans Cell Histiocytosis ◽

Osteolytic Lesion ◽

Rare Condition ◽

Langerhans Cell ◽

Review Of The Literature ◽

Young Adult Patient ◽

Important Differential Diagnosis

AbstractA 36-year-old man was diagnosed with Langerhans cell histiocytosis (LCH) of the cervical spine with a unifocal expansive osteolytic lesion of C4. The surgical management with a 2-year follow-up and a review of the literature on LCH of the cervical spine are presented. Although a rare condition, LCH is an important differential diagnosis of any osteolytic lesion in the cervical spine with localized pain in a young adult patient. Review of the literature suggests a higher prevalence of LCH lesions affecting the cervical spine as compared with the thoracic or lumbar spine than historically reported.

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