cpc 1 jan 12 2008 dr ruenger

8/8/2019 CPC 1 Jan 12 2008 Dr Ruenger

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CPC

Supervisor

Dr. Thomas Ruenger

Jan. 12, 2009

Muhammad Khawar Nazir


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Case 1


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Verocay body of a neurilemoma, consisting of tight, discrete

aggregates of spindle-shaped, palisaded nuclei with a central

fibrillary area, representing collections of cytoplasmic

processes of tumorous Schwann cells


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Hypercellular areas often show nuclear palisading and Verocay

bodies.


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Schwannoma "Neurilemmoma

Tumor originating from the Schwann cells

Myelin sheath which covers peripheral nerves isproduced by the Schwann cells

Tumor cells always stay on the outside of the nerve

Relatively slow growing


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Schwannoma "Neurilemmoma

Mostly benign and less than 1% become malignant known asmalignant peripheral nerve sheath tumour or malignant

Schwannoma or neurofibrosarcoma

Schwannomas can arise from a genetic disorder calledneurofibromatosis

Schwannomas can be removed surgically, but can then recur


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A solid lesion arises within a nervecomposed of a single fascicle

The tumor is composed of Schwann cell

proliferation within the epineurium andperipherallydisplaced nerve fibers,resulting in nodular eccentric growth

No capsule is formed in the early growthphase

The larger tumor (bottom) slightlyincreases the size of the parent nerve andeventually becomes separated fromsurrounding fascicles by a capsule formedfrom the perineurium and epineurium.Occasional axons are present.


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HistopatholgyAntoni A & Antoni B Tissues

Encapsulated subcutaneous tumor

with

1) Cellular areas ( Antoni A tissue )

and / or

2)Edematous myxoid areas (Antoni B tissue)


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Vecoray Bodies

Spindle cells in Antoni A tissue line up in twoparallel rows separated by areas without

nuclei


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Case 2


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Kaposi Sarcoma

Controversial whether Kaposi's sarcoma (KS) representsneoplasia or hyperplasia;

All clinical variants are viewed as a virally induced disease

human herpesvirus-8 (HHV-8) is the implicated agent

Multifocal systemic disease with 4 principal clinicalvariants:

(1) chronic or classic KS;

(2) African endemic KS, including a fulminantlymphadenopathic type;

(3) KS in iatrogenically immunocompromised patients; and

(4) AIDS-related epidemic KS


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Key Features

Cutaneous lesions present as variably distributed pink patches,blueviolet to black nodules or plaques, and polyps, depending onthe clinical variant and stage

The histologic appearance of KS does not vary significantly

between clinical subtypes, but does vary with stage of the lesion

Most cases in childhood, with or without HIV infection, are of thelymphadenopathic type and are rapidly fatal due to visceraldissemination

Because of multifocality, treatment with chemotherapy and/orradiation is favored over surgery


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Kaposi's sarcoma

Tumor caused byHuman herpesvirus 8

(HHV8), also known as Kaposi's sarcoma-

associated herpesvirus (KSHV)

It was originally described by Moritz Kaposi, a

Hungarian dermatologist practicing at the

University of Vienna in 1872


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Kaposi sarcoma

KS is a neoplasm that often manifests withmultiple vascular nodules in the skin and otherorgans.

Although true metastases appear to occur, a

multifocal origin is most common. The pattern of KS is variable, with a course

ranging from indolent (only skin manifestations)to fulminant (extensive visceral involvement).

KS also may arise primarily in the oral mucosa,lymph nodes, and/or viscera without skininvolvement.

KS initially may be evident in any organ of thebody


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3 FORMS

1)Localized nodular

2)Locally aggressive3)Generalized


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6 stages

1. Patch

2. Plaque

3. Nodular4. Exophytic

5. Infiltrative

6. Lymphadenopathic


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Differential Diagnoses

1. BacillaryAngiomatosis

2. Blue Rubber Bleb Nevus Syndrome3. Nevi, Melanocytic

4. Pyogenic Granuloma (Lobular Capillary

Hemangioma)5. Tufted Angioma


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Histopathology

Slitlike spaces

Vessels sometimes appear as lymphangiomatosis,

without erythrocytes

Promontory sign

small blood vessel and its stroma project like a

promontory into a vascular space.


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Treatment

Surgical excision,

Radiotherapy

Intralesional Outpatient low-dose vinblastine

chemotherapy

Radiotherapy Laser


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Case 3


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Porphyria

Uncommon to rare group of porphyrin

metabolidsm disorders

Most of them present as photodermatitis with

papules or vesicles mostly on sun exposed skin

Acute and Non Acute Types


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Porphyria Cutanea Tarda

Tarda means Late ( adult onset)

Non Acute Type

Most common type of porphyria. It results from a

decreased catalytic activity of uroporphyrinogendecarboxylase, the fifth enzyme in heme biosynthesis

Cutaneous Manifestations : increased photosensitivity andskin fragility as well as blistering, erosions, crusts, milia and

scars in sun-exposed sites

Postinflammatory hyperpigmentation, hypertrichosis,scarring alopecia, and morpheaform and sclerodermoidchanges can be observed


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Types

Depending upon the major site of expression of the

uroporphyrinogen decarboxylase, at least 2 types canbe distinguished:

1) Sporadic (acquired) variant, designated type I PCT, inwhich the enzymatic deficiency is exclusively expressedin the liver

2) Autosomal dominant familial (hereditary) variant,designated type II PCT, in which the catalytic enzymaticdefect is detected in all tissues


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Histologic & Immunofluorescence Microscopy Findings

Subepidermal blister with a minimal cell-poor dermalinflammatory infiltrate

Festooning of the dermal papillae (very well preservedpapillae projecting into blister ) with PAS-positivedeposits within the walls of blood vessels (PAS stain)

Direct immunofluorescence with prominent ring-shaped staining around blood vessels and lesser linearstaining along the dermalepidermal junction.

Diff ti l Di i


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Differential Diagnosis

Other types of cutaneous porphyria that manifesting blistering,

Mild variants of congenital erythropoietic porphyria

Hepatoerythropoietic porphyria

Variegate porphyria

Hereditary coproporphyria.

Pseudoporphyria

Epidermolysis bullosa acquisita

Polymorphous light eruption

Phototoxic and bullous drug eruptions

Hydroa vacciniforme

All above diseases can easily be differentiated from PCT bymeasuring urinary and stool porphyrins


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Therapeutic Approaches

1. Photoprotection, e.g. broad-spectrum sunscreens and/orprotective clothing

2. Avoidance of sunlight exposure and trauma

3. Cease alcohol ingestion; stop estrogen therapy

4. Phlebotomy: 400500 ml every 2 weeks over 3 to 6 months

5. Low-dose hydroxychloroquine or chloroquine

6. Measurement of urinary porphyrin excretion to monitortherapeutic outcome


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Case 4


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Leishmaniasis

Synonyms:

Old World cutaneous leishmaniasis: oriental sore, Delhi

boil, Baghdad boil

New World cutaneous and mucocutaneousleishmaniasis: chiclero's ulcer (Mexico), uta and espundia(Peru), ulcera de Bauru (Brazil), bush or forest yaws, pian

boi (Guyanas)

Visceral leishmaniasis: kala-azar and Dumdum fever


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Leishmaniasis

Chronic parasitic (protozoan) disease in whichorganisms are found within phagolysosomes of themononuclear phagocyte system

Most common cutaneous finding is a papule thatdevelops at the site of inoculation and then enlarges andulcerates

Vector is a sandfly infected with promastigotes

Worldwide distribution, but endemic in LatinAmerica, the Mediterranean basin, and parts ofAsia andAfrica


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Sand Fly


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Life cycle of the Leishmania parasite


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Leishmaniasis

Leishmaniasis encompasses a spectrum ofchronic infections in humans and several animalspecies

It is caused byover 15 species ofLeishmania,flagellated protozoans belonging to the orderKinetoplastidae

Transmission is via the bite of sandflies from thegenera Phlebotomus and Lutzomyia.


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Clinical Features

Begins as a small, well-circumscribed papule at theinoculation site that may enlarge into a nodule orplaque and then become ulcerated or verrucous

Exposed sites are most commonly involved

Lesions are often solitary but they may be multiple,with the formation of satellites or lymphatic spread

Majority of acute cutaneous infections resolvespontaneously with scarring but some may becomechronic or disseminated.


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Histopatholgy

Epidermis normal, atrophic, hyperplastic, or

ulcerated

Diffuse mixed granulomatous dermal infiltrateof lymphocytes, histiocytes, plasma cells,

neutrophils, and multinucleated giant cells

Occasional caseational necrosis

Fibrosis in older lesions


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Diagnosis

Diagnosis can be confirmed by demonstrating the

presence ofAmastigotes in dermal macrophages

byskin biopsy, dermal scrapings or fine-needle

aspiration

Giemsa, Wright or Feulgen stains are used to

demonstrate the organisms in smears and tissue:the cytoplasm appears blue, the nucleus pink and

the kinetoplast a deep red


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Persistent arthropod bites

Basal cell carcinomas

Atypical mycobacterioses

Mucocutaneous leishmaniasis can resemble otherinfections such as paracoccidioidomycosis, nomaand tertiary syphilis

In addition, Wegener's granulomatosis andangiocentric NK/T-cell lymphoma must beconsidered when ulcerative mucocutaneouslesions affect the central part of the face


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Treatment

Pentavalent antimonials - Standard therapy

for New World leishmaniasis as well as more

severe cutaneous infections

Additional therapies :

Heat therapy, Cryotherapy, Pentamidine,

Itraconazole, Amphotericin B, Ketoconazole,

Allopurinol


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Prevention

Use ofinsect repellants and insecticides, as

well as the destruction of animal reservoirs

Akilled L. amazonensis promastigote vaccine

has been shown to induce a Th1 response andsignificant resistance against Leishmania


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Case 5


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Venous Lake

First described in 1956 by Bean and Walsh, whonoted how they can be easily compressed and theirtendency to occur on sun-exposed skin, especiallythe ears of elderly

Also known as "Phlebectases" is an asymptomatic ,generally solitary, soft, compressible, dark blue toviolaceous, 0.2- to 1-cm papule

Commonly found on sun-exposed surfaces of thevermilion border of the lip, face and ears

Lesions generally occur among the elderly


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Importance

ClinicallyInsignificant from a biological

standpoint

Important because of their mimicry of more

ominous lesions, such as Melanoma and

Pigmented Basal Cell Carcinoma.


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Venous Lake

Though these lesions may resemble nodularmelanoma , the lack of induration, slowgrowth, and lightening appearance upon

diascopy suggest against it, and indicate avascular lesion

Additionally, lack of pulsation distinguishesthis lesion of the lower lip from a tortuoussegment of the inferior labial artery


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Etiology

Unknown; however it is thought to be associated withsun exposure and sun damage, leading to a dilatedblood-filled vascular channel

One theory is that chronic solar damage injures thevascular adventitia and the dermal elastic tissue,permitting dilatation of superficial venous structures.

"...lined with a singled layer of flattened endothelialcells and a thin wall of fibrous tissue filled with redblood cells."


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Angiokeratoma Circumscriptum

Lentigo

Basal Cell Carcinoma

Malignant Melanoma Blue Nevi

Nevi, Melanocytic

CherryHemangioma

Pyogenic Granuloma (Lobular CapillaryHemangioma) Kaposi Sarcoma


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Work Up

Diascopy

Dermoscopy

Biopsy, If diagnosis is uncertain

Histologic Findings


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Histologic Findings Single large dilated space or several interconnecting dilated

spaces characteristically are observed in the superficial dermis

Dilated channels have very thin walls that are lined by a singlelayer of flattened endothelium and supported by a thin layerof fibrous connective tissue

Usually, no smooth muscle or elastic tissue is found in thevessel wall

In rare cases, a thin and noncircumferential area suggestive ofsmooth muscle can be found instead of the fibrous tissue

Solar elastosis and other evidence ofsun damage usually arefound in the adjacent dermis.


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Treatment

Not necessary, and patients should be

reassured of the lesion's non-malignant

nature

However, if treatment is sought for cosmetic

reasons, lesions can be treated with surgical

excision, laser therapy, infrared coagulation,cryotherapy and sclerotherapy


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Thank you

cpc 1 jan 12 2008 dr ruenger

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