Congenital “self-healing” Langerhans cell histiocytosis (Hashimoto-Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course

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.

Download Full-text

Fulminant Multisystem Non–Langerhans Cell Histiocytic Proliferation With Hemophagocytosis

Archives of Pathology & Laboratory Medicine ◽

10.5858/2005-129-e39-fmnchp ◽

2005 ◽

Vol 129 (2) ◽

pp. e39-e43 ◽

Cited By ~ 2

Author(s):

R. Nagarjun Rao ◽

Chung-che Chang ◽

Nevin Uysal ◽

Kenneth Presberg ◽

Vinod B. Shidham ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Clinical Course ◽

Langerhans Cell ◽

Variant Form ◽

Malignant Histiocytosis ◽

Unique Case ◽

Multiple Organs ◽

Biopsy Specimens ◽

Extensive Involvement ◽

Erdheim Chester

Abstract Hemophagocytosis (HP), a feature seen in malignant histiocytosis and infection- and lymphoma-associated disorders, has not been previously emphasized in Erdheim-Chester disease (ECD). Generally, ECD is recognized as a rare, systemic, non–Langerhans cell histiocytosis with a variable clinical course. Herein, we describe a unique case of multisystem non–Langerhans cell histiocytic proliferation with a fulminant clinical course (death occurred within 3 months of presentation) that showed prominent HP and extensive involvement of multiple organs, including the lungs, resulting in respiratory failure. Hemophagocytosis led to severe anemia that required transfusion and thrombocytopenia. Antemortem lung and bone marrow biopsy specimens revealed involvement by a histiocytic infiltrate with features highly suggestive of ECD and HP. Furthermore, the autopsy documented the presence of HP and the histiocytic infiltrate in multiple other organs. This case is best categorized as a variant form of ECD. Recognizing this variant has the following important implications: (1) HP may be a marker for fulminant clinical course in ECD, (2) the presence of HP does not exclude a diagnosis of ECD, and (3) ECD should be considered in the differential diagnosis of HP.

Download Full-text

A role for ITPA variants in the clinical course of pulmonary Langerhans' cell histiocytosis?

European Respiratory Journal ◽

10.1183/09031936.00026610 ◽

2010 ◽

Vol 36 (3) ◽

pp. 684-686 ◽

Cited By ~ 7

Author(s):

J. A. Bakker ◽

J. Bierau ◽

M. Drent

Keyword(s):

Langerhans Cell Histiocytosis ◽

Clinical Course ◽

Langerhans Cell ◽

Pulmonary Langerhans Cell Histiocytosis

Download Full-text

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

Download Full-text

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.

Download Full-text

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.

Download Full-text

A Case Report of Pediatric Langerhans Cell Histiocytosis: Current Approach and Diagnostic Challenges for Dermatologist

Serbian Journal of Dermatology and Venerology ◽

10.2478/sjdv-2020-0012 ◽

2020 ◽

Vol 12 (3) ◽

pp. 79-86

Author(s):

Irwan Junawanto ◽

Khairuddin Djawad ◽

Sri Rimayani ◽

Farida Tabri ◽

Nurelly N. Waspodo ◽

...

Keyword(s):

Langerhans Cell Histiocytosis ◽

Poor Prognosis ◽

Clinical Manifestations ◽

Current Approach ◽

Myeloproliferative Disorder ◽

Langerhans Cell ◽

Histopathologic Examination ◽

Chemotherapy Treatment ◽

Cutaneous Manifestations ◽

Patient Organ

Abstract Langerhans Cell Histiocytosis (LCH) is a chronic and rare myeloproliferative disorder caused by disorders in Lang-erhans cell proliferation in various organs and tissues. LCH has a wide variety of clinical manifestations, making it difficult to diagnose. Cutaneous manifestations are polymorphic in the form of purpura, papule, vesicles and pustules. LCH can involve vital organs such as the liver and lungs as well as the hematopoiesis system that usually gives a poor prognosis. The prognosis is also influenced by the age of patient, organ dysfunction and response to the first 6 weeks of chemotherapy treatment. A 3-year-old girl reported a major complaint of an abscess-like lesion in the region of neck accompanied by an extensive purpura of scalp, neck and inguinal areas accompanied by vulvar erosions. The immunohistochemical and histopathologic examination support LCH and the clinical improvement after intravenous administration of intravenous 3 mg/m2 Vinblastine chemotherapy, 75 mg/m2 etoposide, oral 40 mg/m2 per prednisone. After the 6th cycle of chemotherapy, the patient died.

Download Full-text