Langerhans Cell Histiocytosis Clinical Presentation

  • Author: Christopher R Shea, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Oct 29, 2010
 

History

The clinical presentation of Langerhans cell histiocytosis (LCH) depends on the extent of dissemination.

Unifocal bony LCH is characterized by the development of solitary osseous lesions at any site. Unifocal bony LCH is least common in the hands and the feet. These lesions are often asymptomatic and are detected incidentally during investigation for unrelated disorders.

Patients with multisystem disease may have a protean history depending on the location of osteolytic lesions and the degree of organ dysfunction. Patients with Hand-Schüller-Christian syndrome (which occurs in 25% of patients with multifocal LCH) often present with recurrent episodes of otitis media and mastoiditis or with polyuria and polydipsia.

Letterer-Siwe disease presents with symptoms suggestive of a systemic infection or malignancy, including a generalized skin eruption, anemia, and hepatosplenomegaly.

The congenital form of LCH manifests as skin lesions at birth or during the early postnatal period. Cutaneous nodules and ulceration have onset early in life. Rarely, patients with purpuric lesions present with a blueberry-muffin appearance.[18] Symptoms of organ involvement may also occur.

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Physical

Signs of Langerhans cell histiocytosis (LCH) depend on the localization and the extent of the disease. The clinical spectrum is broad, and an individual case may differ markedly from the prototypes described.

Solitary cutaneous disease presents with noduloulcerative lesions in the oral, perineal, perivulvar, or retroauricular regions.[19] Rarely, solitary cerebral lesions may occur. In adults with LCH, the pulmonary system is the most frequently involved organ system, and solitary pulmonary lesions may be the only manifestation. Chronic cough, dyspnea, chest pain, and recurrent pneumothoraces are typical signs and symptoms of pulmonary disease.[20]

Because of its protean nature, LCH must be considered in more unusual presentations, including protein-losing enteropathy in infants and combined pulmonary-thyroid involvement in adults.[21, 22]

Chronic unifocal LCH

Chronic unifocal LCH (eosinophilic granuloma of bone) classically presents as a solitary calvarial lesion in young adults; other frequent sites of involvement include vertebra, rib, mandible, femur, ilium, and scapula.

Lesions are usually asymptomatic, but bone pain and a soft tissue mass may occur. When calvarial lesions extend into the nervous system, a variety of neurologic manifestations may be seen. Bony lesions may cause otitis media by destruction of the temporal and mastoid bones, proptosis secondary to orbital masses, loose teeth from infiltration of the mandibles, or pituitary dysfunction due to involvement of the sella turcica.

Spontaneous fractures can result from osteolytic lesions of the long bones; vertebral collapse with spinal cord compression has been described. Mono-ostotic lesions of LCH may also appear in less common sites, such as the scapula.[23]

Classic multifocal LCH (Hand-Schüller-Christian disease)

The classic multifocal form of LCH (Hand-Schüller-Christian disease) includes diabetes insipidus, exophthalmos, and bony defects, particularly of the cranium. Lesions may affect a variety of systems, including liver (20%), spleen (30%), and lymph nodes (50%). Pulmonary involvement may occur. Osteolytic lesions of the long bones can lead to spontaneous fractures.

One third of patients have mucocutaneous lesions, most frequently infiltrated nodules and ulcerated plaques, especially in the mouth, axillae, and anogenital region. Other cutaneous manifestations include extensive coalescing, scaling, or crusted papules.

Acute disseminated LCH

Patients with acute disseminated LCH (multiorgan involvement) present with fever, anemia, thrombocytopenia, pulmonary infiltrates, skin lesions, and enlargement of lymph nodes, spleen, and liver. Cutaneous abnormalities are present in almost 80% of patients, frequently as the first sign. The eruption may be extensive, involving the scalp, face, trunk, buttocks, and intertriginous areas. Lesions consist of closely set petechiae and yellow-brown papules topped with scale and crust. The papules may coalesce to form an erythematous, weeping or crusted eruption mimicking seborrheic dermatitis. Intertriginous lesions are often exudative, and secondary infection and ulceration may occur. Note the images below.

Letterer-Siwe disease. Bilateral inguinal erosive Letterer-Siwe disease. Bilateral inguinal erosive plaques and erythematous papules on the abdomen. Courtesy of Dr Neil S. Prose. Abdominal area of an infant with multiple erythemaAbdominal area of an infant with multiple erythematous papules covered by scale and/or crust. Typical purpuric lesions in Langerhans cell histioTypical purpuric lesions in Langerhans cell histiocytosis (must be distinguished from seborrheic dermatitis).

Osteolytic lesions are not common in the disseminated form of LCH, but the mastoid can be affected, resulting in a clinical picture of otitis media, which may be the presenting complaint. Aural discharge, conductive hearing loss, and postauricular swelling have been described.[24]

As described above, patients with pulmonary involvement present with chest pain, hemoptysis, dyspnea, failure to thrive, cystic changes, and pneumothorax; if lung disease is extensive, oxygen diffusion and lung capacity may be reduced.[25, 26]

Neurologic involvement may produce seizures, vertigo, headache, ataxia, and cognitive defects.

Congenital self-healing histiocytosis

Congenital self-healing histiocytosis presents at birth or during the early neonatal period with firm, red-brown, painless papulonodules (1-10 mm in diameter) or vesicles and crusts scattered over the scalp, face, and, to a lesser extent, trunk and the extremities. Lesions may ulcerate. Lesions may be solitary. Lesions may be followed by residual hypopigmented or hyperpigmented macules.

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Causes

The etiology of Langerhans cell histiocytosis (LCH) remains unknown.

Langerhans cell proliferation may be induced by a viral infection, a defect in intercellular communication (T cell–macrophage interaction), and/or a cytokine-driven process mediated by tumor necrosis factor, IL-11, and leukemia inhibitory factor.[27, 28, 29] Specifically, human herpesvirus 6 (HHV-6) has been proposed to contribute to the initiation and/or modulation of persistent LCH. However, other studies have not shown a correlation between HHV-6 and LCH, and their reported associations may represent coincidental findings.[30, 31]

Cigarette smoking may play a role as a chronic irritant in the development of eosinophilic granuloma of the lung.

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Contributor Information and Disclosures
Author

Christopher R Shea, MD  Professor and Chief, Section of Dermatology, Department of Medicine, University of Chicago

Christopher R Shea, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society of Dermatopathology, Arthur Purdy Stout Society, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois Dermatological Society, International Society of Dermatopathology, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Coauthor(s)

Markus D Boos, MD, PhD  Resident, University of Chicago Comer Children's Hospital

Markus D Boos, MD, PhD is a member of the following medical societies: Alpha Omega Alpha

Disclosure: Nothing to disclose.

Specialty Editor Board

Bernice R Krafchik, MBChB, FRCPC  Professor Emeritus, Department of Pediatrics, Section of Dermatology, University of Toronto

Bernice R Krafchik, MBChB, FRCPC is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, Canadian Medical Association, College of Physicians and Surgeons of Ontario, Royal College of Physicians and Surgeons of Canada, and Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Richard P Vinson, MD  Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA

Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas Health Science Center-San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Joel M Gelfand, MD, MSCE  Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania

Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology

Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds Investigator; Genentech Grant/research funds investigator; Centocor Consulting fee Consulting; Abbott Grant/research funds investigator; Abbott Consulting fee Consulting; Novartis investigator; Pfizer Grant/research funds investigator; Celgene Consulting fee DMC Chair; NIAMS and NHLBI Grant/research funds investigator

Chief Editor

Dirk M Elston, MD  Director, Department of Dermatology, Geisinger Medical Center

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

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Letterer-Siwe disease. Bilateral inguinal erosive plaques and erythematous papules on the abdomen. Courtesy of Dr Neil S. Prose.
Abdominal area of an infant with multiple erythematous papules covered by scale and/or crust.
Typical purpuric lesions in Langerhans cell histiocytosis (must be distinguished from seborrheic dermatitis).
On a plain skull radiograph, lesions are typically lytic, with sharp borders and a punched out appearance.
High-power views. Marked epidermotropism is noted (left). The lesional cells are large, with abundant pink cytoplasm and reniform nuclei. An admixture of inflammatory cells, including occasional eosinophils, is present (right).
High-power views. Diffuse immunoreactivity for S-100 protein (right). Langerhans cells and lymphocytes (left, hematoxylin and eosin).
Widespread positivity for CD1a. Note the presence of epidermotropism (right). Langerhans cells and lymphocytes are present in the epidermis and the papillary dermis (left, hematoxylin and eosin).
Electron microscopy. Tennis racquet form of Birbeck granules with a small terminal expansion.
 
 
 
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