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HISTIOCYTOSESBY Dr. WAJIHA NAQVI
DEFINITION An infiltrative process characterized by
accumulation of langerhan type and dermal dendrocytes,antigen presenting cells.
Histiocytes are derived from circulating monocytes
Langerhan cell is a histiocytic cell that of bone marrow origin.
Dermal dendrocytes are highly dendritic interstitial cells of the papillary and reticular dermis.
FUNCTION OF HISTIOCYTEHistiocytes can be further divided into two;
Professional phagocyte
Antigen presenting cell
Phagocyte Include majority of resident tissue
macrophages and immature macrophages
Immature cells are responsive to chemotactic stimuli and attracted to sites of inflammation
Phagocytosis is important in the removal of particulate matter and destruction of bacteria and parasites.
Antigen presenting cells Internalize antigen by endocytosis
Process it by lysosomal digestion
Express the antigen on their surface
MARKERS OF HISTIOCYTESSurface markers of langerhans cell Surface ATP ase CD1a,CD1c CD4 S100 Placental alkaline phosphatase Birbeck granule
Surface markers of dermal dendrocytes Adenosine triphosphatase(ATPase) Alpha naphthylbutyrate esterase Beta glucuronidase Acid phosphatase
CLASSIFICATION Class l:langerhans’ cell histiocytosis
Class lla:histiocytes involving cells of dermal dendrocyte lineage
Class llb:histiocytes involving cells other than langerhans’cells and dermal dendrocytes
Class lll:malignant histiocytic disorders
CLASS l HISTIOCYTOSIS Condition in which Langerhan’s cell
accumulate in various tissues and cause damage.
AETIOLOGY Tuberculosis Lipid abnornmality Immunology diminished[dec.IgA,IgG] Skin lymphocyte response[dec. T cell
activity]
PATHOLOGY If lesional cells are found to express CD1
and birbeck granules on microscopy,it is a definitive diagnosis.
Upper dermal and junctional accumulation of large histiocytic cells with homogenous pink cytoplasm is characteristic
IMMUNOCYTOCHEMISTRY Diagnostic markers are: S100 Peanut agglutinin Placental alkaline phosphatase CD1a Birbeck granules
CLINICAL FEATURES Multisystem disease with fever,malaise. SKIN Scalp involvement with seborrheic like
rash Purpura Ulceration Hashimoto Pritzker variant starts in
neonates with nodular lesions resembling healing chicken pox
Juvenile xanthogranulomas maybe seenNAILS Paronychia Nailfold destruction OnycholysisEARS Persistent aural discharge deafness
ORAL Destruction of alveolar ridges Infiltration of gums with LCH vells
resulting in teeth floating from socketsBONE MARROW Pancytopenia Splenomegaly
LUNGS Dyspnoea Tachypnoea Subcostal recessionGIT Hepatomegaly Ascites Malabsorption
CNS Cerebellar syndrome-
ataxia,dysarthria,choreoathetoid movements
BONE Bone swelling,fracture Bones affected are
calvarium,fever,scapula,vertebra
ENDOCRINE Growth hormone deficiency Diabetes insipidus HypogonadismPROGNOSIS Under 2yrs poor prognosis Inc.mortality with widespread organ
involvement
INVESTIGATION CBC,ESR ELECTROLYTES,UREA ,LFTs Skeletal survey Chest x ray MRI of brain Water deprivation test CT chest Lung function test U/S ,liver biopsy
TREATMENT Topical nitrogen mustard PUVA therapy Thalidomide
BONE DISEASE I/L steroid Radiotherapy
MULTISYSTEM DISEASE Prednisolone 2mg/kg Ciclosporin,IFN alpha Epipodophyllotoxin etoposide
CLASS ll a HISTIOCYTOSISDivided into Dermatofibroma Juvenile xanthogranuloma Benign cephalic histiocytosis Erdheim chester disease Generalized eruptive Papular xanthoma Progressive nodular histiocytosis Xanthoma disseminatum Diffuse plane xanthomatosis
JUVENILE XANTHOGRANULOMA Benign tumors of histiocytic cellsPATHOLOGY Giant cells with a wreath like
arrangement of nuclei[touton giant cell] Immunocytochemical examination shows
CD68 +ve cells
CLINICAL FEATURES Common in infants less than 6 mths Sudden appearance of lesions with
spontaneous regression Lesions involve the upper part of body Reddish yellow papulesoral mucosa
affected Ulceration Resolution occurs with atrophic scars
Other organs maybe involved but eyes and CNS involvement is common
Secondary glaucoma,hemorrhage in ant chamber
Uveitis,iritis,proptosis Lesions of cerebellum and cortex may
occur
ASSOCIATIONS
Neurofibromatosis Niemann Pick disease Leukemia Urticaria pigmentosa LCH
TREATMENT Self healing Surgery or radiotherapy for eye and CNS
lesions CO2 laser for multiple lesions Systemic steroids
BENIGN CEPHALIC HISTIOCYTOSIS
Starts in early childhood Erythematous papules,nodules and
macules on cheeks,forehead,earlobes Asymptomatic Become reddish brown Self limiting
XANTHOMA DISSEMINATUM
Proliferation of histiocytic cells in which lipid deposition is a secondary event
Affects male children and young adults Skin,mucous membranes of eyes,resp.
tract and meninges commonly involved Erythematous yellow brown papules and
nodules Symmetrically distributed on trunk,face
and proximal extremities
Lesions become confluent and verrucous Stridor,resp distress Diabetes insipidus Seizures and growth retardation Progressive bone disease can also be
seen XANTHOSIDEROHISTIOCYTOSIS-diffuse
infiltration of skin,subcutaneous tissue and muscle [Histiocytes contain iron]
Self limiting disease Co2 lasers Azathioprine,cyclophosphamide Surgery for CNS
DIFFUSE PLANE XANTHOMATOSIS
Xanthomatous lesions develop with paraproteinemia
Common in adults Large,flat plaque like lesions on
eyelids,neck,upper trunk Serum lipids normal Associated with myeloproliferative
disorder Treat the underlying cause
CLASS ll b HISTIOCYTOSIS Reticulohistiocytoma Multicentric reticulohistiocytosis Familial haemophagocytic
lymphohistiocytosis Familial sea blue histiocytosis Hereditary progressive mucinous
histiocytosis Malakoplakia Necrobiotic xanthogranuloma Sinus histiocytosis
Multicentric reticulohistiocytosis
Joints,skin and mucous membrane affected
Exposure to TB might be present
PATHOLOGY Infiltration of mononucleated and
multinucleated giant cells Giant cells are PAS +ve Immunocytochemistry shows CD68 +ve
but CD1 and S100 -ve
CLINICAL FEATURES Affects women with ratio 3:1,middle age
disease Polyarthritis affecting hands Other joints involved are
knees,shoulder,wrists Firm ,brown or yellow papules and
plaques on extensor surfaces
Coral bead like lesions around nail folds Nodular lesions also seen around affected
joints Mucosal involvement specially lips and
tongue Associated with internal malignancy
TREATMENT Systemic steroids Azathioprine Cyclophosphamide TNF alpha antagonists
MALAKOPLAKIA Immunodeficiency disease in which
macrophages fail to phagocytose and digest bacteria
Commonly GIT and urinary system affected
Cutaneous sinuses,ulcers,fluctuant masses,nodules
Tongue and cervix maybe affected
Histologically Michealis Gutmann bodies are seen[represent abnormal degradation of bacteria with calcium and iron deposition]
E.coli and staph aureus has been cultured Self limiting
NECROBIOTIC XANTHOGRANULOMA
Necrobiosis with xanthogranuloma and paraprotein
Periorbital nodular and ulcerative lesions Reddish yellow in color Limbs have subcutaneous nodules and
plaques with atrophy Eyes show conjunctivitis,keratitis,uveitis Nausea,vomiting,fatigue
IgG monoclonal protein
TREATMENT Alkylating agents like melphalan Plasmapheresis Chlorambucil Radiotherapy
SINUS HISTIOCYTOSIS Sinus histiocytosis with massive
lymphadenopathy EBV,Klebsiella,Brucella Usually in young adults Painless lymph node involvement with
90% being cervical Fever ,weightloss,malaise Extranodal involvement is common
Skin lesions are yellow or vilaceous Erythema,papules,nodules or infiltrated
plaques Scaling and telangiectasia maybe present Bone shows lytic lesions CNS involvement is common with
seizures,numbness or paraplegia
Mild anaemia Serum proteins maybe abnormal SHML cells show macrophage markers
and monocyte markers Spontaneous regression Radiotherapy Surgery
THANK YOU.
