cutaneous pseudolymphoma
DESCRIPTION
1. Cutaneous T-cell pseudolymphomas A) Primarily with stripe-like infiltration (the majority of cases) Lymphomatoid drug eruption (most cases); Lymphomatoid contact dermatitis; Actinic reticuloid; Nodular scabies (individual cases); Idiopathic forms; Clonal cutaneous T-cell pseudolymphomas. B) Primarily with nodular infiltration (a small percentage of the cases) Drug-induced – mainly by anti-convulsive drugs Persistent nodules after insect bites; Nodular scabies (the majority of cases). 2. Cutaneous B-cell pseudolymphomas (with nodular infiltration) Cutaneous lymphocytoma from Borrelia burgdorferi; Cutaneous lymphocytoma after antigens injection; Cutaneous lymphocytoma resulting from tattoo; Cutaneous lymphocytoma after Herpes zoster; Idiopathic forms; Clonal cutaneous B-cell pseudolymphomasTRANSCRIPT
![Page 1: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/1.jpg)
CUTANEOUS PSEUDOLYMPHOMA
Moderator : Dr Ram Singh Meena
![Page 2: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/2.jpg)
Historical aspects
Cutaneous pseudolymphoma(CPL) was first described under the term sarcomatosis cutis by Kaposi in 1891.
In 1923, Bilerstein coined term lymphocytoma cutis
Term lymphadenosis benigna cutis was introduced by bafverstedt in 1943
In 1967, Lever introduced term pseudolymphoma of Spiegler and Fendt
Subsequently,Caro and Helwig in 1969 introduced term cutaneous lymphoid hyperplasia
![Page 3: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/3.jpg)
DEFINITION
Pseudolymphoma is process of accumulation of lymphocytes in skin in response to a variety of known and unknown stimuli
It simulates lymphoma, primarily histologically but sometime clinically,which at time of diagnosis appear to have a benign biological behavior and do not satisfy criteria for malignant lymphoma
![Page 4: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/4.jpg)
EPIDEMIOLOGY
Mortality/Morbidity Pseudolymphoma is not associated with mortality.
Localized variants rarely result in morbidity other than minor pain or pruritus.
Race Although 90% of reported patients of CPL are white In localized CPL, F:M ratio is 2:1. No significant epidemiologic data are available CTPL Age Individuals of any age may be affected, but
localized, nodular CPL is MC in early life. Mean age of onset is 34 years. 2/3 of patients are
younger than 40 years at time of biopsy. Borrelial pseudolymphoma is more common in
children than in adults
![Page 5: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/5.jpg)
CLASSIFICATION Rijlaarsdam & Willemze’s classification based on
clinical and morphologicalcriteria. 1. Cutaneous T-cell pseudolymphomas
A) Primarily with stripe-like infiltration (the majority of cases)
Lymphomatoid drug eruption (most cases); Lymphomatoid contact dermatitis; Actinic reticuloid; Nodular scabies (individual cases); Idiopathic forms; Clonal cutaneous T-cell pseudolymphomas. B) Primarily with nodular infiltration (a small percentage of the cases) Drug-induced – mainly by anti-convulsive drugs Persistent nodules after insect bites; Nodular scabies (the majority of cases).
![Page 6: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/6.jpg)
2. Cutaneous B-cell pseudolymphomas (with nodular infiltration) Cutaneous lymphocytoma from Borrelia
burgdorferi; Cutaneous lymphocytoma after antigens
injection; Cutaneous lymphocytoma resulting from
tattoo; Cutaneous lymphocytoma after Herpes zoster; Idiopathic forms; Clonal cutaneous B-cell pseudolymphomas
![Page 7: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/7.jpg)
Classification of Cutaneous Pseudolyphomas(2012)Clinicopathologic Subtype Predominan
tLymphoid Subset
PredominantLocalization
Major Associated Findings
Cutaneous lymphoid hyperplasia
B and T cell Reticular dermis
Kimura’s disease B and T cell Subcutis Lympadenopathy
Angiolymphoid hyperplasia with eosinophilia
B and T cell Reticular dermis Eosinophilia
Castleman disease B and T cell Subcutis Lympadenopathy, POEMSsyndrome
Pseudo mycosis fungoides T cell Papillary dermis &Epidermis
Lymphomatoid contact dermatitis
T cell Papillary dermis &Epidermis
Contact allergen
Lymphocytic infiltration of the skin (Jessner’s disease)
T cell Perivascular &periadnexial dermis
![Page 8: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/8.jpg)
Clinicopathological subtypes
Depending on predominant cell type in infiltrate,cutaneous pseudolymphomas are divided into two major categories.
1.Mixed B and T cell pseudolymphoma. cutaneous lymphoid hyperplasia, Kimura's disease, angiolymphoid hyperplasia with eosinophilia, Castleman disease
2. T cell pseudolymphoma pseudo mycosis fungoides, lymphomatoidcontact dermatitis, Jessner’s lymphocytic infiltration of the skin
Wood GS. Inflamatory diseases that simulate lymphomas: Cutaneous lymphomas. Ed.Wolf K, Goldsmith L, Gilchrest B, Paller A, Leffell D. In. Fitzpatrick’s Dermatology in General Medicine. 7th edition
![Page 9: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/9.jpg)
CUTANEOUS LYMPHOID HYPERPLASIA Also Known as Spiegler-Fendt sarcoid,
Lymphocytoma cutis, lymphadenosis benigna cutis & cutaneous lymphoplasia
World-wide distribution and affects all races and ethnic groups.
Both adults and children Females >males CLH is characterized by a relatively dense
lymphoid infiltrate, centered in the reticular dermis, that is usually B-cell rich and may resemble lymphoma clinically and/or histopathologically
![Page 10: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/10.jpg)
In most cases of CLH is idiopathic but in some there is an identifiable trigger.
These include foreign agents such as: Tattoo dye,Insect bites,Scabies, Stings and spider bites
Vaccinations Desensitisation injections Trauma Acupuncture Gold earring piercing Infections with :-Borrelia burgdorferi (Lyme
disease), Varicella zoster (chickenpox) and Human immunodeficiency virus (AIDS virus).
Drugs
![Page 11: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/11.jpg)
Drugs capable of inducing cutaneous pseudolymphomas
Class Drugs
Anticonvulsants
AntidepressantsNeurolepticsTranqulizers (anxiolitics)ACE-inhibitorsβ-blockersH2- blockersCalcium antagonistsAntirheumatic drugsCytostaticsAntibioticsAntihistaminesAntiarrhythmic drugsDiureticsAntilipemic drugsSexual steroidsLocal drugs
Phenytoin, carbamazepine, phenobarbital, trimethadone, primidone, butobarbitol, methsuximide, phensuximideAmitriptyline, fluoxetine, doxepin, desipramine, lithiumChlorpromazine, thioridazine, promethazine(clonazepam, lorazepam)
Captopril, enalapril, benazeprilAtenolol, labetololCimetidine, ranitidineVerapamil, diltiazem Aspirin, phenacetin, D-penicillamin, allopurinolCyclosporin, methotrexatePenicillinDiphenhydramineProcainamideHydrochlorothiazide, ModureticLovastatinEstrogen, progesterone Menthol, etheric oils
![Page 12: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/12.jpg)
Approach to CLH Clinical history should elicit information
about Duration and symptomatology of lesions, Nature and pace of clinical progression, Past treatment, Local and systemic exposure to foreign
antigens, Including medications and personal or family
history about other lymphoproliferative disorders.
It also include review of systems focusing on so called lymphoma B symptoms
![Page 13: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/13.jpg)
GPE :-with special attention to type and distribution of skin lesions and to status of peripheral lymph nodes, liver and spleen.
Scanning tests such as a CBC, Routine biochemistry screening and chest radiography help to exclude extra cutaneous involvement.
Computed tomography of chest, abdomen and pelvis, as well as bone marrow aspiration and biopsy.
Biopsy can be performed from abnormally enlarged lymph nodes
Lesional skin biopsy is an essential part of diagnostic evaluation
![Page 14: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/14.jpg)
Cutaneous lesions of CLH present most commonly as a solitary nodule or can appear as a localized array of nodules, papules and plaques.
Head, neck, extremities, breast and genitalia are common predilection sites.
Lesions have a doughy to firm consistency & range from red- brown to violaceous in color
Lesions may be pruritic or asymptomatic
![Page 15: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/15.jpg)
HPE of CLH lesions reveals dense, nodular and diffuse lymphoid infiltrate that is concentrated in t reticular dermis.
Epidermis is normal and separated from underlying infiltrate by a narrow grenz zone of uninvolved papillary dermis.
Lesions of CLH include generally a mixed B and T cell lymphocytes. However, various types of histiocytes, including macrophages, dermal dentritic cells, Langerhans cells are scattered throughout infiltrate.
Other cells are sometimes admixed, including plasma cells, eosinophils, mast cells, neutrophils and histiocytic giant cells.
Plasma cells and eosinophils are particularly common in arthropod induced reactions
![Page 16: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/16.jpg)
CLH:-Infiltrate is wedge shaped and taper out in its deeper portion reactive lymphoid
follicles containing pale germinal centre
![Page 17: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/17.jpg)
Lyphoid follicle in the dermis contain pale germinal centers surrounded by darker
mmantle zone
![Page 18: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/18.jpg)
Algorithm for the differential diagnosis of typical CLH mixed B-cell/T-cell type, clonal CLH, and CBCL. CLH, cutaneous lymphoid hyperplasia; CBCL,
cutaneous B-cell lymphoma; IPOX, immunoperoxidase; IGH, immunoglobulin H; GR, gene rearrangement; PCR, polymerase chain reaction
![Page 19: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/19.jpg)
Histologic differentiating features between CPL &CBCL
CPL CBCL
Prominent acanthosis Minimal or no acanthosisTop heavy infiltrate (75%) dermalinfiltrate with perivascular &periappendageal preference
Bottom-heavy infiltrate(65%)diffuse infiltrate that dissects
collagen & may involve the subcutis
Organized immune response (e.g., follicles with germinal centers, etc.); heterogeneous cellular constituency
65% with germinal centers
No organized immune response cellular monomorphism often present10%-20% with germinal centers
No or few mitoses Mitoes may be numerousNo necrosis en masse Necrosis en masse (sometime)Multinuleated gaint cells No multinuleated gaint cells
&granulomaLymphoid infiltrate with no
apoptotic cells Lymphoid infiltrate puching borders with infiltrating borders apoptotic cells
Preservation of adnexae Destruction of adnexaeVascular proliferation No vascular proliferationStromal fibrosis Little or no stromal fibrosis
![Page 20: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/20.jpg)
LYMPHOMATOID DRUG ERUPTIONS
Also known as drug-induced CPL or drug- induced pseudolymphoma syndrome
Divided in two major categories:- 1. Induced by anticonvulsants 2. Induced by other drugs
![Page 21: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/21.jpg)
Anticonvulsants- induced pseudolymphoma syndrome
Develops during first 2 to 8 weeks of drug intake, but can occur from 5 daysup to 5 years of phenytoin therapy
Black>white patients Clinically, most patient have triad of fever,
lymphadenopathy, and an erythemic eruptionin association with hepatosplenomegaly, blood eosinophilia
Lymphadenopathy can localized or generalized Skin manifestations appear as single papule or
widespread erythematous papules, plaques and nodules, but occasionally, lesions may be generalized.
MC offenders are phenytoin,primidone,mephenytoin and trimethadione
![Page 22: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/22.jpg)
Anticonvulsant-induced pseudolymphoma syndrome in a 16-year-old boy
![Page 23: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/23.jpg)
erythrodermic pseudolymphoma drug-induced pseudolymphoma, secondary to anticonvulsant therapy
![Page 24: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/24.jpg)
2.CUTANEOUS PSEUDOLYMPHOMAS INDUCED BY DRUGS OTHER THAN
ANTICONVULSANTS
Occur one month to one year following the administration of therapy
M=F Localized papules together with single or
multiple nodules and plaques, generalized papulonodular lesions and exfoliating erythrodermia resembling Sezàry’s syndrome
Eg:-Neuroleptics,ACE-inhibitors,β-blockers, Antihistamines, Cytotoxic
![Page 25: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/25.jpg)
Pathogenesis of lymphomatoid drug eruptions
Immunological function is reduced and immune control is impaired, leading to abnormal proliferation of lymphocytes, increased function of T-suppressors and hypogammaglobulinemia
Studies of these patients show1. Relative increase in absolute number of
peripheral T-lymphocytes by 85-95 %2. Significant stimulation of drug-induced
blastic tranformation of lymphocytes 3. Impaired ability of T-suppressor lymphocyte
to suppress B-cell differentiation and immunoglobulin production
![Page 26: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/26.jpg)
Persistent nodular arthropod-bite reations and nodular scabies
CBPL or CTPL can develop as a result of scabies or arthropod bite
Clinically, multiple pruritic firm erythematous to red-brown papule and nodule MC on genitalia,abdomen,axillae and elbows
Nodule following scabies may persist for many months after adequate anti-scabietic therapy
It is thought to be due to delayed-type hypersensitivity reaction to componant of mite
![Page 27: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/27.jpg)
Persistent nodular arthropod-bite reations
nodular scabies
![Page 28: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/28.jpg)
Acral pseudolymphomatous angiokeratoma
It is known by its acronym APACHE – Acral Pseudolymphomatous Angiokeratoma of Children
Apache etiology remains unknown howeverpossibility that it is a hypersensitivity
reaction to insect bites Clinically characterized by presence of 10 to
40 violet-erythematic papules, diameter ranges from 1 to 4 mm, and which are asymptomatic, located unilaterally, generally with acral distribution
Occurring more often between 2 and 13 years of age
![Page 29: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/29.jpg)
Histological studies of Apache reveal an epidermis of normal aspect and a dense well-differentiated lymphocyte infiltrate in the dermis, amongst connective tissue structures, not affecting skin appendage.
Such histopathological aspect configures lymphocyte proliferation,associate with no nuclear atypia.
![Page 30: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/30.jpg)
Asymptomatic papules in the right forearm,measuring approximately 3 mm in diameter
![Page 31: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/31.jpg)
Leukocyte infiltrate with predominance oflymphocytes
![Page 32: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/32.jpg)
Treatment
Cutaneous lymphoid hyperplasia related to infection with B. Burgdorferi, antibiotic therapy with cephalosporins
Excision,glucocorticoids (topical, intralesional, and systemic), cryotherapy, antimalarials, minocycline and radiation therapy have all been used with various successes.
Laser therapy and photodynamic therapy
![Page 33: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/33.jpg)
Comparison of clinicopathological changes between Kimura’s disease and ALHE
Clinical features
Kimura’s disease ALHE
Sex Young adult male predominance
Middle-aged female
Age Mean range 27-40 Mean 35 years
Presentation & site
as solitary or multiple nodules up to 10 cm in diameter centered in subcutis, MC involving head and neck
present with smaller,more superficial intradermal papulo-nodules that are typically unilateral. Also involve Salivary glands & resional lymph nodes(reactive)
Regional L N enlargement
LN often show histological evidence of involvement
Uncommon, show reactive changes only
Peripheral bloodeosinophilia
Present in majority of cases Occurs in about 20 % of cases
Course Progressive, becoming stationary after years. Recurrence is common 15%-40%, but there is no fatality.Rarely, association with a nephrotic syndrome
Benign lesion which recurs in about 30%of cases. Recurrence is rare ifexcision is complete
![Page 34: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/34.jpg)
Histological features
Kimura’s disease ALHE
Outline Usually non-circumscribed Often circumscribed except dermal lesions
Lymphoidcomponent
Abundant lymphocytes and plasma cells; lymphoid follicles are always found
Sparse to heavy infiltrate of lymphocytes & plasma cells, with or without lymphoid Follicles
Eosinophils Moderate to abundant; eosinophilic abscesses
Sparse to abundant; eosinophilic abscess is rare
Vascular proliferation
Some stromal vascularity with unremarkable endothelial cells
Prominent vascular proliferation with large epitheloid/ histiocytoid endothetial cells,evidence of underlying vascular malformation may evident
fibrosis Prominent Absent or limited
![Page 35: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/35.jpg)
showing severe facial disfiguration from mass effect(kimura disease)
![Page 36: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/36.jpg)
Histopathologic features of Kimura's disease. A, Scanning view shows prominent germinal center formation. B,Higher magnification of a
germinal center with abundant number of eosinophils. C, Focally, blood vessels may show prominent endothelial cells with vacuolated cytoplasm
![Page 37: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/37.jpg)
Histologic section with hematoxylin and eosin staining showing nodular vascular proliferation and inflammatory infiltrate. High power (inset) shows lymphoid proliferation with plasma cells
and eosinophils
![Page 38: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/38.jpg)
![Page 39: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/39.jpg)
Vascular channels lined by epithelioid endothelial cells with abundant
pink cytoplasm(ALHE)
![Page 40: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/40.jpg)
TreatmentFIRST LINE Excision, Topical corticosteroids, Tntralesional corticosteroids (5-40 mg/ml,
monthly)SECOND LINE Topical tacrolimus ointment, Systemic corticosteroids (60/ 40/ 20 mg PO
tapers, 5 days each), Cyclosporine (2.5- 4mg/kg/day), Local radiation, Vinblastine (15 mg/week IV), Intravenous immunoglobulins
![Page 41: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/41.jpg)
Also referred to as angiofollicular lymph node hyperplasia
First described by Benjamin Castleman in 1956
follicular hyperplasia of lymph nodes with abnormally increased interfollicular vascularity
Can be associated with Kaposi's sarcoma (KS), non-Hodgkin's lymphoma, Hodgkin's lymphoma, and POEMS syndrome
![Page 42: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/42.jpg)
Types of CD
Unicentric and Multicentric Hyaline vascular , Plasmacytic and
Mixed cellularity variety based on histopathology
HIV associated
![Page 43: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/43.jpg)
Unicentric CD
More common Presents as slow growing solitary mass
typically located in the mediastinium or mesenteries.
No constitutional sm-s Not associated with progression to
malignancy Treated by surgical resection with
excellent results Histologicaly Unicentric CD is of hyaline
vascular variety
![Page 44: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/44.jpg)
Follicles show abnormal germinal centers with marked vascular proliferation and hyalinization.Center of
follicle is usually depleted of lymphocytes and shows follicular dendritic cells.Follicle is surrounded by a broad
mantle zone consisting of a concentric layering of lymphocytes resulting in an onion-skin appearance. Follicles are frequently penetrated radially by a sclerotic blood vessel
![Page 45: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/45.jpg)
Multicentric CD Median age 50-60’s ( younger if HIV+) Widespread lymphadenopathy Hepatosplenomegaly Can present with systemic sm-s: fatigue, fever,
wt loss, night sweats (overproduction of IL-6). Severe peripheral edema, anemia,
hypoalbumenia, peripheral neuropathy Also can be associated with:-autoimmune
hemolytic anemia,multiple myeloma, amyloidoisis,Pemphigus & POEMS syndrome
Diagnosis by biopsy: Histologicaly usually of the plasmacytic type or mixed cellularity variety
![Page 46: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/46.jpg)
show diffuse plasma cell proliferation in interfollicular region.Image shows a small follicle in the center with eosinophilic deposits of fibrin and immune complexes
![Page 47: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/47.jpg)
HIV association
More likely to be associated with Multicentric Castleman’s Disease
More likely to be caused by HHV8 Associated with poor prognosis and
progression to malignancy Initiation of HAART may lead to fulminant
multicentric CD
![Page 48: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/48.jpg)
Associated Malignancies
Kaposi's sarcoma (up to 70% in HIV+/HHV+)
Non-Hodgkin's lymphoma (15-20% of pt)
Hodgkin's lymphoma (both MCD & UCD)
POEMS syndrome
![Page 49: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/49.jpg)
Therapy for Multicentric CD
Steroids (15-20% eff, not in HIV+) IV Ig Antivirals (acylovir/gancyclovir/foscarnet)
in HIVand HHV8 + population Rituximab (complete remission in few
cases) CHOP or CVAD (90% eff) Anti-IL6 or anti-IL6 receptor antibody. Thalidomide (anecdotal)
![Page 50: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/50.jpg)
Pseudo Mycosis Fungoides Eruptions mimicking mycosis fungoides
occur typically in adults. Both genders can be affected. Pseudo T cell lymphomas may arise
spontaneously or may occur in association with B cell chronic lymphocytic leukemia
Also associated with ingestions of various drugs, including hydantoins, carbamazepine and antihistamines.
The lesions present clinically as one or a few plaques on the trunk or extremities.
![Page 51: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/51.jpg)
1.Lesion during drug therapy2.Lesion after discontinuation of drug
![Page 52: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/52.jpg)
Histopathologically, there is a papillary dermal, band-like infiltrate containing mostly atypical lymphocytes with clefted and cerebriform nuclei.
Compared with mycosis fungoides,epidermotropism is typically far less prominent and Pautrier’s microabscess-like aggregates are rare.
Most cases contain polyclonal T cells In the differential Lichenoid drug eruptions, Lymphomatoid contact dermatitis, Chronic radiodermatitis, Secondery syphilis
![Page 53: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/53.jpg)
Pautrier microabcess–like appearancecan be seen
![Page 54: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/54.jpg)
First line of therapy Drug discontinuation Treatment of underlying disorder when an
associated specific disease is detected Excision for isolated lesions, Topical and systemic corticosteroids, Ultraviolet B phototherapy and PUVA
therapies
![Page 55: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/55.jpg)
Lymphomatoid Contact Dermatitis LCD is a chronic and persistent allergic contact
dermatitis Lymphomatoid contact dermatitis was described
originally in four patients with persistent allergic contact dermatitis proven by patch testing
Responsible agents include gold, nickel and para-phenylenediamine.
Characterizedby generalized pruritic red, scaly papules and plaques
Histopathologically, superficial lymphocytic dermatitis that contains foci of spongiosis simulating appearance of cutaneous T cell lymphomas
![Page 56: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/56.jpg)
![Page 57: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/57.jpg)
Frequently there is edema in papillary dermis in lymphomatoid contact dermatitis, this finding is usually absent in mycosis fungoides
Avoidance of the responsible allergic agents leads to eventual resolution of condition.
A search for offending agent via patch testing
Therapy firstly Elimination of a suspected allergic agent,
later topical corticosteroids, pimecrolimus and tacrolimus ointments
![Page 58: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/58.jpg)
There is a superficial and deep perivascular infiltrate of lymphocytes and eosinophils. Some lymphocytes extend to a hyperplastic epidermis where there is spongiosis, a few individual necrotic keratinocytes, and parakeratosis with serum. There is no evidence of lymphocytic atypia
![Page 59: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/59.jpg)
Jessner Lymphocytic Infiltration of the Skin
Jessner lymphocytic infiltrate commonly present with asymptomatic, non-scaly, erythematous papules or plaques predominantly on face and neck,upper trunk or arms of several months duration
Majority of cases occur in middle aged adults
![Page 60: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/60.jpg)
Histologically ,epidermis usually is normal In dermis, a moderately dense superficial and
deep perivascular lymphocytic infiltrate.Lymphocytic infiltrate may also be perifollicular or may extend into subcutis.
Higher magnification reveals a predominance of small mature lymphocytes. Large lymphoid cells, plasma cells, or plasmacytoid monocytes may occasionally be present and eosinophils and neutrophils are absent.
Differential diagnosis:- Polymorphous light eruption, Discoid lupus erythematosus, Well-differentiated lymphocytic lymphoma & Lymphocytoma cutis
![Page 61: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/61.jpg)
Histopathologic features of Jessner's lymphocytic infiltration of skin: absence of epidermal and
dermoepidermal junction abnormalities
![Page 62: Cutaneous pseudolymphoma](https://reader034.vdocuments.us/reader034/viewer/2022052523/55506ecfb4c90524138b49e5/html5/thumbnails/62.jpg)
Thanks