Congenital Self-Healing Langerhans Cell Histiocytosis Presented as 'Purpura-like Skin Lesions'

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

Italian Journal of Pediatrics ◽

10.1186/s13052-021-01082-9 ◽

2021 ◽

Vol 47 (1) ◽

Author(s):

Alessandra Rizzoli ◽

Simona Giancristoforo ◽

Cristina Haass ◽

Rita De Vito ◽

Stefania Gaspari ◽

...

Keyword(s):

Oral Mucosa ◽

Langerhans Cell Histiocytosis ◽

Skin Lesions ◽

Langerhans Cell ◽

Self Healing ◽

Review Of The Literature ◽

Cutaneous Lesions ◽

Cutaneous Manifestations ◽

Case Presentation

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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Pulmonary Langerhans Cell Histiocytosis

Seminars in Respiratory and Critical Care Medicine ◽

10.1055/s-0039-1700996 ◽

2020 ◽

Vol 41 (02) ◽

pp. 269-279

Author(s):

Brian Shaw ◽

Michael Borchers ◽

Dani Zander ◽

Nishant Gupta

Keyword(s):

Langerhans Cell Histiocytosis ◽

Treatment Options ◽

Skin Lesions ◽

Langerhans Cell ◽

Mitogen Activated Protein Kinase ◽

Myeloid Neoplasm ◽

Cystic Lung ◽

Pulmonary Langerhans Cell Histiocytosis ◽

Mitogen Activated Protein ◽

The Central Nervous System

AbstractPulmonary Langerhans cell histiocytosis (PLCH) is a diffuse cystic lung disease that is strongly associated with exposure to cigarette smoke. Recently, activating pathogenic mutations in the mitogen-activated protein kinase pathway have been described in the dendritic cells in patients with PLCH and have firmly established PLCH to be an inflammatory myeloid neoplasm. Disease course and prognosis in PLCH are highly variable among individual patients, ranging from spontaneous resolution to development of pulmonary hypertension and progression to terminal respiratory failure. A subset of patients with PLCH may have extrapulmonary involvement, typically involving the skeletal system in the form of lytic lesions, skin lesions, or the central nervous system most commonly manifesting in the form of diabetes insipidus. Smoking cessation is the cornerstone of treatment in patients with PLCH and can lead to disease regression or stabilization in a substantial proportion of patients. Further insight into the underlying molecular pathogenesis of PLCH has paved the way for the future development of disease-specific biomarkers and targeted treatment options directed against the central disease-driving mutations.

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

Journal of Cutaneous Medicine and Surgery ◽

10.1007/s10227-001-0014-0 ◽

2001 ◽

Vol 5 (6) ◽

pp. 486-489 ◽

Cited By ~ 7

Author(s):

Kelli W. Morgan ◽

Jeffrey P. Callen

Keyword(s):

Langerhans Cell Histiocytosis ◽

Langerhans Cell ◽

Self Healing

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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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Acral, Pure Cutaneous, Self-Healing, Late-Onset, Cellulitis-Like Langerhans Cell Histiocytosis

Journal of Cutaneous Medicine and Surgery ◽

10.2310/7750.2008.07078 ◽

2009 ◽

Vol 13 (1) ◽

pp. 43-47

Author(s):

Prafulla Kumar Sharma ◽

Sunil Sabhnani ◽

Minakshi Bhardwaj ◽

Hemanta Kumar Kar

Keyword(s):

Langerhans Cell Histiocytosis ◽

Clinical Presentation ◽

Late Onset ◽

Early Adulthood ◽

Langerhans Cell ◽

Self Healing ◽

Diagnostic Dilemma

Background: An unusual and not yet reported clinical presentation of pure cutaneous Langerhans cell histiocytosis (LCH) that was a diagnostic dilemma. Objective: To bring to light the newer presentation of this disease. Method: The case was managed on clinical grounds initially as cellulitis. However, after the investigations and confirmation of the diagnosis, she was managed symptomatically. Results: Pure cutaneous LCH may manifest in early adulthood with features of cellulitis, erythematous edematous vesicular plaques, and nodules distributed in the flexural acral areas. Conclusion: Pure cutaneous LCH may have an acute late onset with unusual morphology as cellulitis, inverse acral distribution, and self-healing.

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