Langerhans Cell Histiocytosis: A Case Report with Unusual Cutaneous Manifestation

Abstract

Langerhans cell histiocytosis (LCH) is characterized by idiopathic monoclonal infiltration of Langerhans cells in different organs such as the skeleton, skin, pituitary gland, liver, spleen, lungs, and the hematopoietic system. Skin lesions are common in LCH and affect about 40% of cases. It is reported that skin lesions are usually the first manifestation of LCH in 80% of patients. Usually, cutaneous presentations of LCH in adults are generalized or seborrhea-like lesions and it is often the first manifestation of disease. Here, we describe a 45-year old female who was known case of hypothyroidism, systemic lupus erythematosus, and diabetes insipidus. In our patient, cutaneous involvement was unusual. It was single and presented in unusual site (ankle) and before developing such lesion, she had diabetes insipidus for several years due to the involvement of pituitary gland.

Persistent purple erythematous rash: Langerhans cell histiocytosis

“…The male-to-female ratio is 2:1 [2]. The clinical presentations are highly variable, ranging from discrete, localized, mild, and asymptomatic forms to aggressive and widespread forms [4]. It is now more important to distinguish forms with multisystem involvement from those with localized monotissue lesions [5].…”

“…Histopathological examination reveals an inflammatory infiltrate containing eosinophils, T lymphocytes, neutrophils, macrophages, and large multinucleated giant cells (LCH) [4,9].…”

Dermoscopy and reflectance confocal microscopy finding of different anatomic sites of Langerhans cell histiocytosis

“…The prognosis of LCH was related to diagnosis with promptly or delayed. Cutaneous manifestations of LCH were the first presentations in approximately 80% of cases and often the leading clue for disease diagnosis, 2 , 3 including a seborrheic dermatitis, erythematous/reddish‐brown crusted/scaly papules/maculopapules/plaques/patches and eczematous lesions, petechiae/purpura, hypopigmented macules/papules, umbilicated (molluscum‐like) papules, vesicles/blisters/bullae, pustules, nodules, and blueberry muffin spots. 4 , 5 Sites of predilection included the scalp, abdomen, chest, back, and intertriginous areas, rarely involved nail and vulvar.…”

Infiltrating the Heart and Kidney: A Rare Pediatric Case of Multisystem Langerhans Cell Histiocytosis from Pakistan

“…Langerhans cell histiocytosis (LCH) is a rare, clonal disease of the monocyte-macrophage system, characterized by uncontrolled proliferation and accumulation of CD1a + /CD207 + dendritic cells (DCs) as a result of continuous immune stimulation, resulting in destruction of hard and soft tissues [1]. The proliferation of dendritic cells, in addition to histiocytes, eosinophils, neutrophils and plasma cells is the histological hallmark of this hematological disorder, the neoplastic or inflammatory nature of which remains an unsettled topic of debate [2]. Historically known as “histiocytosis X,” the disease was broadly categorized into three disorders based on the clinical presentations, each of which was staged clinically according to Greenberg et al clinical staging system before the revised classifications that are used today came to the forefront [3].…”

“…The clinical presentation of LCH is highly variable and may range from isolated, self-healing skin and bone lesions to life-threatening multi-system disease, which makes its diagnosis challenging and very essentially aided by the histologic and immunophenotypic examination of tissue lesions [2]. The most common organs to be involved are: skeleton (80%), skin (33%) and lymph nodes (30%) and amongst those less frequently involved are the liver, spleen, lungs, the hematopoietic system and central nervous system (CNS) [2].…”