1 - 50 Flashcards
An increased number of miniaturized hairs are seen in:
1 Lichen planopilaris
2 Alopecia areata
3 Trichotillomania
4 Telogen effluvium
5 Lichen planopilaris and alopecia areata
Alopecia areata
In alopecia areata, especially early stages, increased miniaturized catagen hairs can be seen in addition to the finding of peribulbar lymphocytes resembling a “swarm of bees.” Lichen planopilaris is a scarring alopecia in which vertical tracts of fibrosis are seen in place of follicles. Trichotillomania displays follicular plugging, trichomalacia, pigmented casts, hemorrhage, and increased catagen hairs on biopsy. Telogen effluvium is characterized by an increased number of telogen hairs.
Which of the following can be used to stain amyloid?
1 PAS
2 Eosin
3 Giemsa
4 Aldehyde fuchsin
5 Crystal violet
Crystal violet
Stains for amyloidosis: Congo Red, Thioflavin T, Crystal Violet, Methyl violet, Pagoda red no. 9, PAS + diastase, amyloid P-component antibody
Which of the following would suggest a diagnosis of malignant lymphoma, B-cell type over pseudolymphoma, B-cell type?
1 Mixed-cell infiltrate
2 Mitotic figures only in germinal centers
3 Histoicytic giant cells episodically
4 Larger lymphocytes predominate
5 Prominant vasculature with thick-walled blood vessels lined by plump endothelial cells
Larger lymphocytes predominate
Histological findings in pseudolymphoma, B-cell type include a mixed-cell infiltrate (important diagnostic criterion), small lymphocytes (that predominate), the presence of histiocytes and giant cells, mitotic figures limited to the germinal centers and prominant vasculature.
Which of the following histological features would be most helpful in differentiating lichenoid drug eruption from lichen planus?
1 Civatte bodies
2 Parakeratosis and eosinophils
3 Squamatization of the basal layer
4 Presence of pruritus
5 Band-like infiltrate with Saw-tooth rete ridges
Parakeratosis and eosinophils
Lichenoid drug eruptions share clinical and histopathologic features with lichen planus. Sometimes differentiation is not possible; however, eosinophil, parakeratosis, and a deeper perivascular infiltrate is more suggestive of lichenoid drug. Implicated medications include captopril, penicillamine, and chloroquine.
Blue-gray pigmentation on the legs secondary to minocycline on biopsy stains with:
1 Fontana Masson
2 Perls
3 Sudan black
4 Fontana Masson and Perls
5 All of these answers are correct
Fontana Masson and Perls
There are three types of pigmentary change that are caused by minocycline. The blue-gray pigmentation on the legs and the blue pigment in scars is thought to be secondary to a drug-protein complex deposited in the dermis. The blue-gray pigment on the legs stains with Perls and Fontana-Masson. The blue in scars (often on the face) stains with Perls. The muddy-brown discoloration on sun-exposed areas shows increased basilar pigment and melanin incontinence on biopsy. It is likely secondary to phototoxicity.
Eccrine glands are found on the:
1 Labia majora
2 Labia minora
3 Glans
4 Prepuce
5 Vermilion
Eccrine glands are present all over the body except on the vermilion of lips, glans, labia minora, nail beds, and inner prepuce.
Paraproteinemia is associated with all except:
1 Scleromyxedema
2 Necrobiotic xanthogranuloma
3 Scleredema
4 Sclerosing panniculitis
5 Plane xanthoma
Sclerosing panniculitis Sclerosing panniculitis (lipodermatosclerosis) displays characteristic changes in the fat (lipomembranous change); it is not associated with paraproteinemia. Generalized plane xanthomas, scleromyxedema, necrobiotic xanthogranuloma, scleredema, erythema elevatum diutinum, xanthoma disseminatum, and pyoderma gangrenosum have all been associated with a paraproteinemia.
Pic: Erythema Elevatum Diutinum
Paraproteinemia, or monoclonal gammopathy, is the presence of excessive amounts of a single monoclonal gammaglobulin (in this case denominated “paraprotein”) in the blood. It is usually due to an underlying immunoproliferative disorder.
Paraproteinemias may be categorized according to the type of monoclonal protein found in blood:
Light chains only (or Bence Jones protein). This may be associated with multiple myeloma or AL amyloidosis.
Heavy chains only (also known as “heavy chain disease”);
Whole immunoglobulins. In this case, the paraprotein goes under the name of “M-protein” (“M” for monoclonal). If immunoglobulins tend to precipitate within blood vessels with cold, that phenomenon takes the name of cryoglobulinaemia.
The three types of paraproteins may occur alone or in combination in a given individual. Note that while most heavy chains or whole immunoglobulins remain within blood vessels, light chains frequently escape and are excreted by the kidneys into urine, where they take the name of Bence Jones protein.
It is also possible for paraproteins (usually whole immunoglobulins) to form polymers by aggregating with each other; this takes the name of macroglobulinemia and may lead to further complications. For example, certain macroglobulins tend to precipitate within blood vessel with cold, a phenomenon known as cryoglobulinemia. Others may make blood too viscous to flow smoothly (usually with IgM macroglobulins), a phenomenon known as Waldenström macroglobulinemia.
Methyl-green pyronin stains RNA what color?
1 Pink
2 Green
3 Blue
4 Purple
5 Black
Pink
Methyl-green pyronin stains RNA pink and stain DNA green.
What is this neoplasm?
1 Hibernoma
2 Malakoplakia
3 Myospherulosis
4 Liposarcoma
5 Pleomorphic lipoma
Hibernoma
Hibernoma: This is a rare neoplasm that typically occurs in 30-40 year old males as a slowly enlarging warm mass on scapular region, trunk, axilla or thigh. There are also myxoid & spindle cell variants that occur on the posterior neck/shoulder. The lesion enhances with contrast on CT and MRI fails to reveal fat septations which differs for that seen with other lipomas. Abnormalities in 11q13 and loss of MEN1 gene on 10q22 have been noted. Histologically: The lesion is composed of large polygonal adipocytes that have with multiple vacuoles and an eosinophilic granular cytoplasm with a central nucleus and prominent nucleolus. These cells are referred to as “Mulberry cellsâ€. Admixed among these larger adipocytes are smaller cells with a granular cytoplasm, mature white fat and some times spindle cells.
This is associated with MEN IIa:
1 Neurothekeoma
2 Macular amyloidosis
3 Malignant peripheral nerve sheath tumor
4 Chondroid syringoma
5 Mucocele
Macular amyloidosis
Macular amyloidosis is associated with MEN IIa.
Multiple clear cell acanthomas are associated with:
1 Ichthyosis
2 Cowden’s
3 Immunosuppression
4 Gastrointestinal polyps
5 Breast cancer
Ichthyosis
Clear cell acanthoma is associated with ichthyosis.
Which of the following is characteristic of Birt-Hogg-Dube Syndrome?
1 Autosomal recessive mode of inheritance
2 Multiple trichoepitheliomas
3 Caused by mutation in hamartin
4 Multiple trichodiscomas
5 Colon cancer common
Multiple trichodiscomas
Birt-Hogg-Dube Syndrome (BHD) characterized by multiple small harartomas of mesodermal component of hair discs, which were indentified as trichodiscromas. It is an autosomal dominant disease caused by mutations in folliculin. Patients with multiple fibrofolliculomas may also have acrochordons, collagenomas, lipomas, and/or oral fibromas. BHDS recently has been reported in association with various types of renal tumors, such as oncocytoma and a variant of papillary renal cell carcinoma. There are no trichoepitheliomas in BHD syndrome.
Think of a Hog & Disc!!
TRICHODISCOMA
Trichodiscoma usually occurs as multiple dome-shaped papules a few millimeters in diameter. They occur either localized to one area, widely distributed, or in association with multiple fibrofolliculomas on the face and neck (Birt-Hogg-Dubé syndrome). Trichodiscoma is hypothesized to be derived from the hair disc (Haarscheibe).
Trichodiscoma: Dome-shaped papule with thinned epidermis and moderate increase in papillary dermal connective tissue. Note normal epidermis and dermis on the extreme left. (Scanning power)
Fine fibrillar connective tissue contains superficial thick-walled blood vessels with small lumina (Fig. 34-87)
Mild interstitial mucin deposition among fine collagen bundles (Fig. 34-88)
DDX:
Small angiofibroma (fibrous papule) may resemble trichodiscoma; mild perifollicular fibrosis and stellate cells favor angiofibroma.
Which fixative would best preserve the histologic features of a gout tophus?
1 Saline
2 Absolute ethanol
3 Formaldehyde
4 Michel’s fixative
5 Tissue culture media
Absolute ethanol
Gout tophi are caused by deposition of monosodium urate monohydrate crystals. Using absolute ethanol to fix the biopsy specimen will preserve the crystalline architechture. In formaldehyde fixed tissure, the crystals are less obvious.
Which of the following is sometimes used to stain BCC during Mohs micrographic surgery?
1 Carcinoembryonic Antigen
2 Factor 13A
3 Glial Fibrillary Acidic Protein
4 Desmin
5 Toluidine Blue
Toluidine Blue
The correct answer is toluidine blue. Toluidine blue staining shows dark blue BCC tumor cells and a magenta-colored stroma around the tumor cells. CEA is used to stain adenocarcinoma, extramammary Paget's disease, and eccrine neoplasms. Desmin stains muscle. GFAP stains neurologic cells including astrocytes and Schwann cells. Factor 13A is used to stain dermatofibroma.
Weibel-Palade bodies are seen in:
1 Endothelial cells
2 Spitz Nevi
3 Cells infected with MCV
4 Plasmacytoid Cells
5 Malakoplakia
Endothelial cells
Weibel-Palade bodies are seen in endothelial cells and are therefore found in vascular lesions. Kamino bodies are found in Spitz nevi. Henderson Patterson bodies are seen in molluscum. Dutcher bodies are intranuclear inclusions seen in plasmacytoid cells. Michaelis Gutmann bodies are partially digested bacteria seen in malakoplakia.
What is your best diagnosis?
1 Lichen Trichophyticus
2 Secondary Lues
3 Lichen Nitidus
4 Scabies
5 Acral Hyperkeratosis
Lichen Nitidus
A chronic inflammatory disease characterized by shiny, flat-topped, usually flesh-coloured micropapules no larger than the head of a pin. Lesions are localized in the early stages, found chiefly on the lower abdomen, penis, and inner surface of the thighs. Distribution may become generalized as the disease progresses.
The predominant location of the cleft in erythema toxicorum neonatorum is:
1 Dermal
2 Basement membrane zone
3 Basal keratinocytes
4 Suprabasal
5 Subcorneal/granular
Subcorneal/granular
Erythema toxicorum neonatorum is an idiopathic vesiculopustular eruption on the face and trunk of neonates that often resolves in a few weeks. Histologically it presents with subcorneal eosinophil rich pustules.
This tumor was excised from the scalp of an older woman, what is this neoplasm?
1 Cylindroma
2 Sebaceous carcinoma
3 Pilomatricoma
4 Basal cell carcinoma
5 Spiradenoma
Cylindroma
Cylindroma: Most often seen in middle age females on the scalp. Multiple lesions can be associated with trichoepitheliomas, spiradenoma and adenomas of parotid gland in Brooke Spieler syndrome. Histologically this is a poorly circumscribed dermal tumor with multiple small irregularly shaped basophilic islands often likened to puzzle pieces composed of a peripheral layer of smaller darker cells, with cells that have increased eosinophilic cytoplasm centrally. Each island is surrounded by a thick eosinophilic cuticle (type IV and VII collagen), with similar eosinophilic hyaline droplets seen within the island.
This lesion was excised from the face, what is this neoplasm?
1 Clear cell syringoma
2 Sebaceous carcinoma
3 Microcystic adenocarcinoma
4 Renal cell carcinoma
5 Clear cell hidradenoma
Clear cell syringoma
Syringoma: Multiple eyelids, cheeks, chests, can be eruptive and increased numbers in Down’s syndrome and a clear cell change has been associated with Diabetes. Histologically this is a dermal tumor consisting of eccrine ducts, lined by two cell layers sometimes creating a tad pole appearance, there can also be small basaloid islands or strands of cells. Within the lumens of the ducts is eosinophilic material, the lumens are CEA+. The tumor is located superficially and fails to display perineural extension as is seen in MAC. The glands are associated with a fibrous sclerotic stroma, usually no (or minimal) keratin cysts or foreign body granulomas as seen in desmoplastic trichoepithelioma. Enlargement of the glandular cells more of a clear or vacuolated cytoplasm can be seen in patients with diabetes.
Syringomas arise from or recapitulate the intradermal eccrine duct and usually occur as multiple 1 to 3 mm periorbital papules (Fig. 33-50, 33-51, 33-52). They are most commonly symmetrically distributed although rarely in a linear pattern. They appear at puberty or during young adult life and are seen in nearly one in five individuals with trisomy 21 (Down’s syndrome). They occasionally appear on the neck, trunk, axillae or genitalia and are rarely solitary. They may appear in successive crops as eruptive syringoma (Fig. 33-53).
MICROSCOPIC FEATURES:
Short epithelial strands with occasional ducts and ectatic ducts or microcysts in a fibrotic stroma in the superficial reticular dermis or papillary dermis (Fig. 33-54)
Ectatic duct may have epithelial extension giving the characteristic comma-shaped duct (Fig. 33-55,33-57) or “tadpole” sign (Fig. 33-55,33-56).
Ducts are composed of two cell layers of flattened epithelial cells (Fig. 33-57).
Fine eosinophilic granular material within the ducts (Fig. 33-57)
Superficially the ectatic ducts will often keratinize and form milia.
Clear cell syringoma is a variant with two to several layers of pale highly glycogenated or vacuolated duct cells (Fig. 33-58).
May be seen in association with intradermal nevi as an incidental finding
DIFFERENTIAL DIAGNOSIS:
Fibrotic stroma, irregular epithelial strands and occasional keratin cyst formation seen in syringoma may be difficult to separate from desmoplastic trichoepithelioma that can be excluded by lack of eccrine ducts.
Fibrosing or morphea-form basal cell carcinoma may appear similar to a syringoma; presence of ducts or lack of nuclear atypicality point to syringoma.
Lack of deep reticular dermal or subcutaneous involvement by the epithelial strands or cysts help in excluding microcystic adnexal carcinoma.
All of the following are true regarding smooth muscle hamartomas except:
1 Hyperpigmentation
2 Transient piloerection
3 Hypertrichosis
4 Association with “Michelin tire baby” in females
5 Becke’s nevus is an example
Association with “Michelin tire baby” in females
Smooth muscle hamartomas of the skin may develop from three locations: the arrector pili muscles, blood vessel walls, and genital/areolar skin. The have been associated with hyperpigmentation, tranisent piloerection (“pseudo-Darier’s sign”) and hypertrichosis. It is thought to be the underlying lesion in “Michelin tire baby” syndrome in boys, not girls.
The mutated product in Darier’s disease is:
1 SPINK5
2 SERCA2
3 ATP2A2
4 ATP2C1
5 SPINK5 and ATP2A2
SERCA2
In Darier’s disease, the ATP2A2 gene encoding the SERCA2 Ca(2+)-ATPase is mutated in some patients. SPINK5 is the gene that is mutated in some patients with Netherton’s syndrome, and this gene the serine protease inhibitor LEKTI. ATP2C1 is the gene mutated in some patients with Hailey-Hailey disease.
2 Headed Shark eating a Dairy Cow!
The organisms highlighted by this GMS stain are?
1 Blastomycosis
2 Coccidioidomycosis
3 Mucormycosis
4 Candida albicans
5 Histoplasmosis capsulatum
Blastomycosis
Blastomycosis: Gilchrist disease, Bird droppings or spores on wood along Mississippi basin, incubation 33-44 days. Most infections develop in previously healthy people. In patients with HIV, 46% will get CNS symptoms, which is 5-10x greater than non-HIV pts. 4 forms, pulmonary, disseminated (CNS, bone, skin and GU) with secondary cutaneous lesions and primary inoculation (rare-1-2 weeks after exposure-sporotrichoid pattern- mixed infiltrate numerous budding organisms). Lesions more typically are verrucous or ulcerated plaques in males on exposed skin-face. Histology: Pseudoepitheliomatosis hyperplasia overlying acute dermal inflammation usually only a few organisms in center of abscess with thick walled 7-15 microns, single broad based bud, has a WI-1 adhesion protein and are best seen with PAS or GMS.
Which of the following does not stain for mucopolysaccharides (mucin)?
1 Periodic-acid Schiff (PAS)
2 Trichome
3 Alcian blue
4 Colloidal iron
5 Mucicarmine
Trichome
Trichome does not stain acid mucopolysaccharides. Trichome stains collagen blue or green and muscle red depending on reagents used. PAS stains mucopolysaccharides red, alcian blue stains mucopolysaccharides blue, colloidal iron stains mucin blue and mucicarmine stains mucin red.
This was a deep seated large tumor in the thigh, what is it?
1 Liposarcoma
2 Nodular fascitis
3 Hibernoma
4 Spindle cell lipoma
5 Pleomorphic lipoma
Liposarcoma
A liposarcoma is one of the most common sarcomas to occur in adults, 50-70yos. Those that occur in resectable regions (like the thigh) have a good prognosis; those that occur in retroperitoneum, mediastinum or spermatic cord recur repeatedly and have a risk to dedifferentiate with a mortality approaching 80% over the following 10-20 years. It has a characteristic supernumerary ring with amplification of 12q14 MDM2 region. Histologically The adipocytes vary in size throughout the tumor. Pleomorphism is variable depending upon the degree of differentiation that the tumor displays. The nuclei of the more well-differentiated adipocytes, as well as, the spindled cells are hyperchromatic with nuclear atypia. The presence of lipoblasts, i.e. either mono-vacuolated signet ring type of lipoblast as is more commonly seen in myxoid liposarcoma and/or multi-vacuolated lipoblasts with central nuclei scalloped by vacuoles, are characteristic of a liposarcoma, but are not necessary to make the diagnosis. There is often a plexiform proliferation of blood vessels, with an appearance that has been likened to crow’s feet or chicken wire. The stroma can be loose and delicate, fibrous or myxoid. Thick ropey fibrous septae are common in liposarcoma and in lipoblastoma.
The majority of liposarcomas are found in the deep soft tissues, particularly the thigh muscles, and only a very small proportion arise in the subcutaneous fat (Fig. 37-38). However, liposarcoma arising from muscle may extend along fascia and into the subcutaneous fat. The tumor may be aggressive and exhibit metastases to lung and liver. Early wide excision is necessary for cure of these aggressive lesions (Fig. 37-39,37-40).
At least four types of liposarcoma have been characterized with different histologic features, age predominance, and prognosis. They are 1) well-differentiated, 2) myxoid, 3) round cell and 4) pleomorphic. More than one subtype may be seen in a tumor. Well-differentiated tumors are often seen in the elderly and may be subdivided into a) “lipoma like”, b) inflammatory and c) sclerosing, all of which are low grade and show occasional recurrences but no metastases. Well-differentiated tumors in the subcutis are cured by simple excision and the term atypical lipoma or atypical lipomatous tumor has been introduced. The myxoid variants are typically seen in younger individuals and also have a better prognosis. Pleomorphic liposarcomas appear in the elderly and have a worse prognosis.
MICROSCOPIC FEATURES:
Common denominator for the diagnosis of all subtypes of liposarcoma is identification of lipoblasts, age of the patient, location of tumor, poor circumscription with infiltration or replacement of normal muscle, fascia, or adipose tissue.
Variable cellularity but usually some areas with high cellularity (Fig. 37-41)
Lipoblasts exhibit characteristic sharply demarcated vacuole or several vacuoles within the cytoplasm of a spindle cell or ovoid cell that indents the nucleus (Fig. 37-42,37-43).
Fat stains of frozen sections or alcohol prepared, non-extracted specimens may reveal subtle (Fig. 37-44) to marked lipid content (Fig. 37-45).
Nuclear hyperchromasia, pleomorphism and atypical mitoses in lipoblasts are variable, depending on tumor subtype.
Well-differentiated liposarcoma (deep tissues) or atypical lipoma (subcutaneous primary lesion) exhibit broad fibrous septa dividing mature adipose tissue but containing lipoblasts.
Well-differentiated liposarcoma and atypical lipoma exhibit lipoblasts and mature adipocytes, with mild hyperchromasia and mild pleomorphism.
Myxoid liposarcoma shows lipoblasts, myxomatous loose collagenous stroma (Fig. 37-46) and a plexiform capillary pattern (Fig. 37-47).
Well-differentiated variant of myxoid liposarcoma with better prognosis is composed of signet ring cells.
Poorly differentiated variant of myxoid liposarcoma exhibits large, pleomorphic nuclei and lipoblasts with fewer and smaller vacuoles.
Round cell liposarcoma is more densely cellular with uniform round to oval cells with scant glycogenated cytoplasm as confirmed by diastase sensitive PAS staining.
Round cell liposarcoma has cells with small vacuoles and both these and the round cells show nuclear atypicality and numerous mitoses.
Transition areas to other subtypes often help identify lipoblasts in round cell liposarcoma.
Pleomorphic liposarcoma contains lipoblasts with marked pleomorphism (Fig. 37-48,37-49) and bizarre giant cells (Fig. 37-50).
Some tumors may exhibit regions of hypocellularity (Fig. 37-51) with atypical multinucleated lipoblasts (Fig. 37-52), hypercellularity (Fig. 37-53) with numerous mitoses (Fig. 37-54), and rare bizarre giant cells (Fig. 37-55).
Abrupt transitions from cellular to myxoid areas are seen (Fig. 37-56,37-57).
DIFFERENTIAL DIAGNOSIS:
Several mesenchymal and neural tumors may exhibit atypical vacuolated cells or myxomatous features; immunohistochemistry panels are necessary to help discriminate between them.
Liposarcoma and the following tumors are positive for vimentin; other markers (in parentheses) help identify the other mesenchymal tumors, namely malignant schwannoma (S-100 protein), leiomyosarcoma (desmin), rhabdomyosarcoma (myoglobin) and malignant fibrous histiocytoma (α-1-antichymotrypsin and α-1-antitrypsin).
Cutaneous extraskeletal Ewing’s sarcoma (primitive neuroectodermal tumor) resembles round cell liposarcoma due to round cell morphology with abundant cytoplasmic glycogen; immunohistochemical staining for neuron specific enolase, S-100 protein and leu 7 helps identify Ewing’s sarcoma.
Poorly differentiated metastatic adenocarcinoma of the kidney or adrenal gland exhibit lipid vacuoles and are important to differentiate from liposarcoma, particularly round cell variants that may show pseudoglandular features. This is most important in the retroperitoneum, but metastatic lesions to skin may present a differential; immunohistochemical positivity for vimentin and focal low-molecular weight cytokeratins following proteolytic digestion favor adenocarcinoma or cortical carcinoma.
Metastatic gastric signet ring carcinoma may resemble vacuolated lipoblasts; intracellular sialomucin and cytokeratin positivity are seen in this tumor.
Benign lipoblastoma may be extremely difficult by histology alone to differentiate from liposarcoma; however, the young age of the patient, location of the lesion and lack of mitoses permit a benign diagnosis.
This tumor is vimentin+ and cytokeratin+:
1 Angiosarcoma
2 Neurothekeoma
3 Nodular fasciitis
4 Dermatofibroma
5 Epithelioid sarcoma
Epithelioid sarcoma
Characteristic immunohistochemistry of epithelioid sarcoma is vimentin- and cytokeratin- positivity.
Epithelioid sarcoma is a rare mesenchymal soft tissue tumour with an epithelioid pattern. It often occurs in the extremities, and is more commonly found in young adults.[1]
Diagnosis
The diagnosis is made by examination of tissue, e.g. a biopsy. The diagnosis may be delayed because of the unusual nature of the tumour and its rarity. Its histomorphology near perfectly may mimic an adenocarcinoma, melanoma or granuloma.
Their immunohistochemical profile may mimic those of carcinomas; thus, these tumours may be misdiagnosed as such.
They typically stain for vimentin and keratins. They characteristically have loss of staining for SMARCB1 (INI1, BAF47), and typically stain for CA125.[2]
Which of the following histologic features is seen in aging skin?
1 Thickened dermal-epidermal junction
2 Increased mast cells
3 Increased number of terminal hairs
4 Fewer Langerhans cells
5 Increased sebum production
Fewer Langerhans cells
Histologic features of aging epidermis include flattened dermo-epidermal junction, occasional nuclear atypia, decrease in the number of melanocytes and Langerhans cells. Changes that are present in the dermis include atrophy, decrease in fibroblasts, mast cells and blood vessels.
Mulberry cells contain increased:
1 Phagolysosomes
2 Mitochondria
3 Golgi
4 Ribosomes
5 Phagolysosomes and mitochondria
Mitochondria
Hibernomas commonly arise in the neck, axillae, and posterior shoulder. The cells are multivacuolated and resemble mulberries; the cells are filled with mitochondria, as are the cells in normal brown fat.
All have been associated with increased risk of breast cancer except:
1 Cowden’s
2 Multicentric reticulohistiocytosis
3 Peutz-Jeghers
4 Birt-Hogg-Dube
5 ataxia telangiectasia
Birt-Hogg-Dube
Birt-Hogg-Dube is associated with renal cancer and thyroid cancer. Female carriers of a mutated ATM (homozygous mutations ATM cause ataxia telangiectasia) have an increased risk of breast cancer.
CAMP kids associated with BREAST CANCER
COWDEN’S
ATAXIA TELANGIECTASIA
MULTICENTRIC RETICULOHISTIOCYTOSIS
PEUTZ-JEGHERS
Langerhans cells express or are characterized by all of the following except:
1 Chromagranin
2 HLA-DR
3 CD1a
4 Birbeck granules
5 S-100
Chromagranin
Chromagranin stain neuroendocrine cells, Merkel cellcarcinomas and eccrine glands. They do not stain Langerhans cells.
Sebaceous carcinomas commonly arise on the eyelid. A biopsy of an ocular sebaceous carcinoma typically reveals:
1 uniform, eosinophilic cells with rare atypia
2 pagetoid cells in the epidermis
3 negative staining with Androgen Receptor (AR)
4 positive staining with Ber-Ep4
5 may be associated with overexpression of MLH-1 and MSH-2
Sebaceous carcinoma that arises at other site rather than the eyelid is associated with what syndrome?
What expression is loss in sebaceous carcinoma of this syndrome?
pagetoid cells in the epidermis
Periocular sebaceous carcinomas are histologically composed of lobules of cell that extend deep in the dermis and subcutaneous tissue. The cells are pleomorphic, with vacuolated cytoplasm with moderate atypia. A characteristic feature in ocular tumors is the pagetoid spread of the tumor in the overlying epidermis. Immunostaining for the androgen receptor has been reported as a method to determine sebaceous differentiation, and it is helpful in diagnosing poorly differentiated sebaceous carcinomas, which may lack staining for EMA and other markers. Basal cell carcinomas, nodular-type are typically positive for Ber-Ep4 while sebaceous tumors are almost always negative for this marker.
Sebaceous carcinomas may be associated with Muir-Torre syndrome, which is characterized with loss of expression of MLH-1 and MSH-2.
SEBACEOUS CARCINOMA
The majority of sebaceous carcinoma tumors arise in the elderly on the eyelids and may resemble chronic blepharoconjunctivitis or a chalazion. The tumor is derived from the meibomian glands and rarely from the glands of Zeis. Regional metastases are reported to occur in more than 20% of cases; however, visceral metastases are rare. Sebaceous carcinomas arising at other sites are often associated with the Muir-Torre syndrome, and no cases of sebaceous carcinoma metastasis in these patients have been reported.
MICROSCOPIC FEATURES:
Irregular lobular formation with infiltration of adjacent dermis by an atypical, irregular, undifferentiated epithelium or vacuolated epithelium (Fig. 35-53,35-54,35-55)
The epithelium is composed of spindle to polygonal cells with pleomorphic, hyperchromatic nuclei and pale cytoplasm (Fig. 35-54,35-55).
Vacuolization of cell cytoplasm with occasional scalloping of nuclei by vacuoles (Fig. 35-54,35-55)
Lesions may exhibit pagetoid spread of the malignant cells in the conjunctiva or epidermis.
Intra-epidermal spread may resemble lentiginous or contiguous hyperplasia along the dermo-epidermal junction (Fig. 35-56).
Some tumors show marked atypicality, clear cell change (Fig. 35-57) and extensive invasion.
Oil red 0 or Sudan black fat stains on fresh frozen sections reveal abundant lipid content in sebaceous carcinoma cells.
Immunoperoxidase staining shows tumor cells to be positive for epithelial membrane antigen (EMA) (Fig. 35-58) and negative for GCDFP-15.
DIFFERENTIAL DIAGNOSIS:
Basal cell carcinoma with sebaceous differentiation; sebaceous carcinoma has more pleomorphic nuclei and a more eosinophilic cytoplasm.
Squamous cell carcinoma with invasion and partial replacement of preexisting sebaceous glands; nuclear atypia of the sebocytes is not present.
Pagetoid spread of pale, clear atypical cells in the conjunctiva or epidermis is also seen in extramammary Paget’s disease, with positivity for GCDFP-15 on immunoperoxidase staining.
Which of the following drugs has been known to cause pyogenic granuloma?
1 Daunorubicin
2 Mithramycin
3 Isosfamide
4 Capecitabine
5 Paclitaxel
Capecitabine
Systemic retinoids, indinavir and capecitabine have all been describe to cause pyogenic granulomas
Capec Jacket covering PG
A healthy 6 month old girl has a subcutaneous nodule above her right eyebrow. A skin biopsy demonstrates a cystic lesion with adnexal structures in the wall. Your diagnosis is:
1 Steatocystoma
2 Pilar cyst
3 Nevus sebaceous
4 Epidermal inclusion cyst
5 Dermoid cyst
Dermoid cyst
Dermoid cysts present along lines of embryonic closure. The are most commonly found on the head (around the eyes) and the neck. They are lined by an epidermis that contains various epidermal appendages that are usually fully matured.
DERMOID CYST TOP
These lesions result from aberrant sequestration of the ectoderm in the dermis during embryogenesis and are typically found along the ectodermal lines of closure. They usually become apparent during childhood as a cutaneous swelling, most frequently lateral to the upper eyelid, mastoid area or midline of the neck.
MICROSCOPIC FEATURES:
Cyst contents have loose laminated keratin with intermingled hair shafts (Fig. 29-1, 29-2).
Cyst lining has stratified keratinizing squamous epithelium resembling normal epidermis with a normal granular cell layer (Fig. 29-2).
Wall is always associated with pilosebaceous components (Fig. 29-3, 29-4).
Rupture with giant-cell reaction to hair shaft is often seen (Fig. 29-5).
Frequently pilar arrector smooth muscle, eccrine or apocrine differentiation may be observed.
DIFFERENTIAL DIAGNOSIS:
Epidermal inclusion cyst epithelial lining is identical to the dermoid cyst; however, the former lacks attached epidermal appendages and hair shafts within the cyst contents.
Acquired follicular cysts in rhinophyma may have attached pilosebaceous elements.
Steatocystoma exhibits squamous epithelial lining with sebaceous gland attachment and may contain vellus hairs; however, characteristic folded eosinophilic cuticle is present.
Which of the following is more commonly seen in lichen sclerosus et atrophicus compared to morphea?
1 Inflammation and fibrosis in the subcutis
2 Marked edema in the papillary dermis
3 Minimal hydropic degeneration noted at the dermaldermal-epidermal junction
4 Epidermis relatively normal with no thinning of the rete ridges
5 No follicular plugging
Marked edema in the papillary dermis
Morphea vs. Lichen schlerosus et atrophicus: Morphea has a relatively normal epidermis without follicular plugging. Interface changes are rare. In the dermis, the collagen fibers appear homogenized. Elastic fibers are preserved. The inflammation and sclerosis can extend into the subcutaneous fat. LS&A has an atrophic epidermis with follicular plugging. There is often prominent hydropic changes with clefting. There is often edema of the papillary dermis and elastic fibers are often absent. The subcutis is uninvolved.
Which of the following is characteristic of PLEVA?
1 Alternating orthokeratosis and parakeratosis
2 Red cell extravasation
3 Dermal mucin deposition
4 Fibrinoid necrosis of medium sized vessels
5 Numerous eosinophils and neutrophils
Red cell extravasation
Pityriasis lichenoides et varioliformis acuta has characteristic findings. Parakeratosis, spongiosis with vacuolar alteration, papillary dermal edema, wedge shaped infiltrate, and extravasated RBC’s can all be seen. Occasionally, the infiltrate can include atypical lymphoid cells. Eosinophils and neutrophils are more frequently seen in LyP.
If you attended ASDP this year, this case was a poster presented by my colleague Roy King. A young adult man presented with a history of prior treatment for tinea on the face, that did not improve with therapy. He later developed multiple crusted scaly papules on his trunk and extremities. The biopsy from the arm showed parakeratosis, crust, interface changes with scattered necrotic keratinocytes in the basal and spinous layers of the epidermis.
The superficial dermis showed an interface and perivascular infiltrate of primarily small lymphocytes with few eosinophils, no plasma cells. These changes all seem to point to pityriasis lichenoides et varioliformis acuta (PLEVA). There was some clinical suspicion for secondary syphilis and so the anti-treponemal immunostain was performed to be certain. Low and behold it was loaded with spirochetes. Nothing ground breaking here, but since syphilis appears on the rise, just something to keep in mind when you’re staring down a supposed case of pityriasis lichenoides. Treponema pallidum is no friend to the dermatopathologist, or anyone for that matter.
Leukocytoclastic vasculitis can be seen in which condition?
1 Rheumatoid neutrophilic dermatosis
2 Erythema elevatum diutinum
3 Urticaria
4 Granuloma inguinale
5 Sweet’s Syndrome
Erythema elevatum diutinum
Rheumatoid neutrophilic dermatitis is seen in the setting of severe rheumatoid arthritis. The pathogenesis is not understood, but in the few reports of this rare dermatosis, a true vasculitis has not been seen.
Sweet’s syndrome also does not show evidence of a true vasculitis. Erythema elevatum diutinum histologically shows a leukocytoclastic vaculitis, but with prominent interstitial neutrophils. Other conditions with evidence of LCV include Henoch-Schonlein purpura, granuloma faciale, urticarial vasculitis, and occasionally serum sickness.
What is the best diagnosis for this vesiculobullous disorder?
1 Bullous Pemphigoid
2 Porphyria Cutanea Tarda
3 Pemphigus vulgaris
4 Dermatitis Herpetiformis
5 Arthropod Bite
Bullous Pemphigoid
Bullous Pemphigoid: Most common subepidermal blistering disease that frequently occurs on abdomen, groin, flexor surface of arms and legs of the elderly. In men an association with HLA-DQ7 has been seen. Often eosinophilia and elevated IgE is seen in the serum. The pathogenesis of this blistering disorder is due to binding of antibodies, IgG1 and 4 or IgE and rarely IgA, to BPAg I (230kd) and less commonly BP Ag II (180kd) in the hemidesmosome. Histologically there is a unilocular subepidermal blister with eosinophils. Early lesions may show eosinophilic spongiosis with papillary dermal edema. Within the dermis there is a lymphocytic eosinophilic infiltrate that is both perivascular and interstitial. DIF shows linear deposits of C3> IgG along the DEJ. Salt split skin show localization of immunoreactants in the vesicle roof. Circulating anti-BMZ antibodies are seen in 60-80% of patients but do not correlate to disease activity.
Multiple trichoepitheliomas are seen in all except:
1 Bazex’s syndrome
2 Brooke-Fordyce syndrome
3 Brooke-Spiegler syndrome
4 Gorlin’s syndrome
5 Rombo syndrome
Gorlin’s syndrome
Gorlin’s syndrome is nevoid basal cell carcinoma syndrome; multiple trichoepitheliomas are not seen. Several syndromes have been associated with multiple trichoepitheliomas: Basex, Brooke-Fordyce, Brooke-Spiegler, Rombo, and possibly Rasmussen. (Rasmussen described one family in 1975 with autosomal dominant inheritance of multiple trichoepitheliomas, milia, and cylindromas.) Basex syndrome (follicular atrophoderma, hypotrichosis, occasional trichoepitheliomas, basal cell carcinomas, and localized or generalized hypohidrosis) is inherited in an X-linked dominant manner. Brooke and Fordyce both described multiple trichoepitheliomas concurrently in 1892, and therefore multiple familial trichoepitheliomas are sometimes called “Brooke-Fordyce” sydrome. Spiegler described patients with multiple cylindromas in 1899 and also noted that many of these patients had mutiple trichoepitheliomas; more recently it has been noted that multiple spiradenomas may be seen in patients with multiple trichoepitheliomas and cylinidromas; this co-occurrence of tumors has been referred to as “Brooke-Spiegler” syndrome. (Brooke-Fordyce and Brooke-Spiegler are likely the same syndrome.) Rombo syndrome is characterized by vermiculate atrophoderma, multiple BCCs, multiple trichoepitheliomas, cyanosis and peripheral vasodilation.
TRICHOEPITHELIOMA (TRICHOBLASTOMA) TOP
Trichoepithelioma typically occurs as a solitary firm nodule on the face in children and young adults but may present at other sites (Fig. 34-113) or in older individuals (Fig. 34-114). It is usually a few millimeters in diameter but may rarely reach 1 to 2 cm in size. Multiple trichoepithelioma is associated with an autosomal dominant inheritance (Fig. 34-115,34-116, 34-117) known as epithelioma adenoides cysticum.
Use of the term trichoblastoma has been proposed to include tumors showing “immature” trichoepithelioma features with abundant basaloid buds, numerous papillary mesenchymal bodies and absent horn cysts. Others favor replacing the term trichoepithelioma with trichoblastoma, as the root word “epithelioma” is part of an ambiguous nomenclature that does not clearly specify benignity. However, “blastoma” also does not fully succeed in indicating that trichoblastoma is a benign neoplasm (e.g. retinoblastoma). Others propose, as do we, that trichoepithelioma and trichoblastoma comprise a spectrum of benign trichogenic tumors with variable degrees of differentiation towards the follicular hair bud.
MICROSCOPIC FEATURES:
Well-circumscribed, organoid, basaloid proliferation (Fig. 34-118,34-123,34-126)
Eosinophilic well-organized cellular stroma (Fig. 34-119, 34-124, 34-127)
Basaloid proliferation recapitulates hair bud that may be similar in size to normal hair bulb (Fig. 34-120) or moderately (Fig. 34-124) to extensively hyperplastic (Fig. 34-127) compared to the normal hair bulb size.
Horn cysts may be absent (Fig. 34-118,34-123) or present (Fig. 34-126).
Hair shafts are usually absent (Fig. 34-119,34-120,34-127) but minute vellus hair shafts may be seen (Fig. 34-125).
Basaloid budding, invagination of basaloid epithelium and focal condensations of plump fibroblasts (papillary mesenchymal bodies) (Fig. 34-121,34-122, 34-128) are characteristic.
Basaloid epithelium may exhibit abrupt squamatization and keratinization with granular cell layer and concentric horn cyst formation (Fig. 34-126).
Melanocytes may be present, and accumulation of melanin is seen in pigmented variant (Fig. 34-129).
DIFFERENTIAL DIAGNOSIS:
Basal cell carcinoma, keratinizing type, will usually show epidermal origin, focal mucinous stroma with separation artifact or focal necrosis.
Clinical pathologic correlation with age of patient may be necessary to help exclude basal cell carcinoma.
Squamous cell carcinoma with horn pearls; lack of abrupt keratinization, less basaloid feature and more cytologic atypia
Which of the following is true regarding pilomatricomas?
1 Calcification is rarely seen
2 Poorly demarcated tumor
3 Comprised of shadow cells and basophilic cells
4 Mutations found in the APC gene
5 Frequent malignant degeneration
Comprised of shadow cells and basophilic cells
Pilomatricomas are well-defined lobular tumor in dermis or subcutis comprised of germinative-matrical cells that are basaloid and transition to “ghost” or “shadow” cells. Calcifiation or ossification are frequently seen. Mutations are found in beta catenin.
PILOMATRICOMA (CALCIFYING EPITHELIOMA OF MALHERBE) TOP
Pilomatricoma typically presents as a several millimeters to few centimeters in diameter solitary nodule on the face or upper extremities of children and young adults (Fig. 34-55,34-56). This hair-matrix tumor differentiates towards hair cortex cells.
MICROSCOPIC FEATURES:
Well-circumscribed lobulated tumor (Fig. 34-57,34-58,34-59)
Composed of irregular islands of basophilic matrix cells and eosinophilic keratinized cells (Fig. 34-57, 34-58)
Basophilic cells appear basaloid and resemble hair-bulb matrix cells (Fig. 34-58, 34-59).
Eosinophilic sheets of keratin composed of “shadow” or “ghost” cells with delineated cell boundaries and central fenestrations resulting from nuclear degeneration of the differentiated matrix cells (Fig. 34-57,34-58, 34-59)
Stroma within the basaloid islands and keratinized regions exhibits numerous fibroblasts, chronic lymphocytic infiltrate and multinucleated giant cells (Fig. 34-60).
Basophilic cells are absent in some tumors that are fully keratinized; these lesions often show calcification or ossification (Fig. 34-61, 34-62).
Marked foreign body-type giant cell reaction to cornified “shadow” or “ghost” cells (Fig. 34-63)
Other regions may show highly irregular islands of basaloid matrix cells (Fig. 34-64,34-65) and may show “separation artifact” with the stroma, resembling basal cell carcinoma (Fig. 34-66).
Careful search of “older” pilomatricomas may show pilomatrical keratinization of the matrical basaloid cells into pearls of keratinous ghost cells (Fig. 34-67).
Pilomatricoma may appear cystic with a lining of basaloid matrix cells (Fig. 34-68, 34-69) and show occasional foci of pilomatrical differentiation (Fig. 34-70).
Melanization may be apparent in some pilomatricomas (Fig. 34-68, 34-69) and, if also cystic they may present clinically as a dark blue tumor resembling nodular melanoma.
Some pilomatricomas are superficial, exhibit a follicular architecture, and connect to the surface (Fig. 34-71).
Pilomatricoma may show rupture with acute inflammation (Fig. 34-71, 34-72).
Rarely, irregular islands of atypical cells exhibit invasion of the capsule and infiltration of the dermis, indicating malignant transformation into pilomatrix carcinoma (Fig. 34-73, 34-74, 34-75, 34-76).
DIFFERENTIAL DIAGNOSIS:
Basal cell carcinoma with focal matrical differentiation
Basal cell carcinoma exhibits focal cell necrosis, mucinous stroma, prominent basaloid palisading and connection to the epidermis.
What infectious agent is most likely responsible for a reaction of fibrin and antibodies which help to prevent phagocytosis?
1 Nocardia
2 Ricketsii species
3 Actinomycosis
4 Candida albicans
5 Anthrax
Actinomycosis
Hoeppli-Splendore reaction (BOB HOPE) BIG CHEEKS!! is characterized histologically by intensely eosinophilic material consisting of fibrin and antibodies. Causes of the phenomenon include Actinomycosis israelii, Staph aureus, Proteus, Pseudomonas and E. coli.
Mycetoma refers to a chronic and indolent granulomatous infection involving skin, subcutaneous tissue, and eventually bone, from which grains composed of the etiologic agents are eliminated through sinus tracts. Mycetoma is defined by the triad of tumefaction, draining sinuses, and grains (Fig. 13-81,13-82,13-83). The infection usually remains localized; systemic symptoms are rare but if present are the result of secondary bacterial infection. Mycetoma is common in tropical and hot temperate climates. It is caused by traumatic implantation of the potential pathogens by penetrating injuries from splinters, thorns, fish scales, fins, or other foreign materials into the body of an otherwise healthy individual. Persons walking barefoot are affected most frequently. Mycetoma affecting the foot is also known as “Madura foot” (Fig. 13-84).
Mycetoma can be classified into three categories based on the microbiology of the grains: eumycetoma, caused by true fungi; actinomycetoma; caused by filamentous bacteria of the order Actinomycetales, and botryomycosis, caused by bacteria including species of staphylococci and streptococci. The most common etiologic agents of eumycetoma are Pseudallescheria boydii and Madurella mycetomatis.
Grains should be examined for the color, approximate size and texture. Organisms producing white to yellow grains include actinomycetes species such as Nocardia and Streptomyces, eumycetes such as P. boydii and Acremonium species and eubacteria causing botryomycosis. A. israelii produces yellow grains. Brown to black grains are produced by true fungi such as the Madurella species, E. jeanselmei, P. romeroi, L. senegalensis, and L. tompkinsii. A. pelletieri produces bright red grains.
Which of the following is true of Rosai-Dorfman disease?
1 S-100 negative, CD1a positive, not characterized by emperipolesis
2 S-100 positive, CD1a positive, not characterized by emperipolesis
3 S-100 positive, CD1a negative, characterized by emperipolesis
4 S-100 positive, CD1a positive, characterized by emperipolesis
5 S-100 negative, CD1a negative, characterized by emperipolesis
S-100 positive, CD1a negative, characterized by emperipolesis
Rosai-Dofrman disease (also known as sinus histiocytosis with massive lymphadenopathy) is characterized by large “fluffy” histiocytes that are S-100 positive but CD1a negative, multinucleated giant cells, plasma cells, aggregates of lymphocytes, and emperipolesis. There is debate as to whether the condition is related to herpes type 6.
The organism on this slide stained with Warthin-Starry is responsible for what disease?
1 Bacillary Angiomatosis
2 Cellulitis
3 Madura foot
4 Pyoderma gangrenosum
5 Pyogenic granuloma
Bacillary Angiomatosis
Bacillary (epithelioid) angiomatosis is caused by Bartonella Henselae or Bartonella quintana (trench fever- from body louse). Clinically resembles PG but multiple lesions and in a HIV+ patient, usually with history of exposure to cats. Histologically there is dome shaped lesion with an epidermal collarette with a proliferation of reactive plump blood vessels similar to a PG, but the lesion is less lobular and there is PMNs and dust throughout the lesion along with extracellular clumps of purplish gram – bacilli. The organism is + for Warthin-Starry, which highlights the organisms in a characteristic Chinese letter configuration.
Immunohistochemical staining with neuron-specific enolase is positive in:
1 Anaplastic large cell lymphoma
2 Malignant firbroushistiocytoma
3 Cutaneous T cell lymphoma
4 Merkel cell carcinoma
5 Sebaceous carcinoma
Merkel cell carcinoma
Neuron-specific enolase is a cytoplasmic product produced by Schwann cells and neurons. This enzyme is present in neuroendocrine cells, neurons and tumors derived from them. Positive staining for neuron-specific enolase is found in Merkel cell carcinomas, carcinoid tumors, and malignant melanoma.
(a) Biopsy of the lesion showed nests and cords of uniform basophilic cells with high nuclear:cytoplasmic ratios and abundant mitoses that were infiltrating through the dermis. (b) large necrotic, hemorrhagic, ulcerated multilobulated tumor, (c) 5 mm papule A 79-year-old Caucasian man developed a nodular skin lesion noted on his right forearm. Biopsy of the lesion showed nests and cords of uniform basophilic cells with high nuclear:cytoplasmic ratios and abundant mitoses that were infiltrating through the dermis (figure A; courtesy of Dr. Frederic Askin). Immunostains were positive for cytokeratin (CK) 20 in a dot-like cytoplasmic pattern and negative for thyroid transcription factor-1 (TTF-1). The patient underwent repeat surgical excision to obtain negative margins and there was no evidence of distant metastases on imaging. Due to the patient’s poor performance status, a decision was made not to pursue adjuvant therapy with radiation or chemotherapy. About three months after resection of the right forearm lesion there was clinical evidence of local tumour recurrence. The lesion grew rapidly in a multinodular pattern and was erythematous to violaceous with a shiny surface. Focal areas of necrosis which oozed blood were present (figure B). Several months later a new lesion developed in the area of the right shoulder that closely resembled the initial lesion in appearance (figure C; arrow). The patient’s clinical condition deteriorated rapidly, and he died within fourteen months with the diagnosis of Merkel cell carcinoma.
The histologic finding of “shoulder parakaratosis”, parakeratosis with prediliection for the follicular ostia, is characteristic of pityriasis rubra pilaris as well as:
1 Stasis dermatitis
2 Atopic dermatitis
3 Seborrheic dermatitis
4 Nummular dermatitis
5 Allergic contact dermatitis
Seborrheic dermatitis
Parakeratosis refers to pyknotic keratinocyte nuclei in the stratum corneum, where nuclei are not usually present. It is common in diseases with changes in the epidermis. Histologically seborrheic dermatitis can shows “shoulder parakeratosis” with epidermal spongiosis. Histologically atopic, nummular and contact dermatitis present with spongiosis with or without vesicles. Stasis dermatitis presents with more dilated papillary dermal small blood vessels and hemosiderin.
A biopsy was obtained from the nasal mucosa, what are the organisms in this biopsy?
1 Rhinosporidium
2 Coccidioidomycosis
3 Prototheca wickerhamii
4 Histoplasmosis capsulatum
5 Cryptococcus neoformans
Rhinosporidium
Rhinosporidiosis originally thought to be due to a fungus Rhinosporidium seeberi, now believed to be caused by an aquatic protistan parasite or Cyanobacteria, Microcystics aeruginosa. Sri Lanka & South America polypoid nasal/mucosal lesions in males and conjunctival lesions in females. Obtained from water or soil. Histology: granulomatous dermatitis with mixed inflammatory cell infiltrate with large thick walled birefringent sporangia which mature toward the center of a cyst, the organism measures 10-200 microns contain 7-8 micron endospores which contain eosinophilic globules. Watery environments causes cyst to rupture.
Cellular neurothekeoma stains with:
1 Stromelysin-3
2 Desmin
3 S-100
4 PGP-9.5
5 Low molecular weight keratin
PGP-9.5
PGP-9.5 and S100-a6 stains cellular neurothekeoma. Stromelysin-3 is positive in dermatofibromas and negative in dermatofibrosarcoma protuberans. Desmin stains rhabdomyosarcoma. S-100 stains neural tumors and melanocytic tumors among other things, but cellular neurothekeomas are generally S100-negative.
PGA GOLF
Which disease process best describes Texier’s disease?
1 Neutrophilic dermatosis
2 Deposition disorder
3 Infectious process
4 Panniculitis
5 Granulomatous disease
Panniculitis
Texier’s disease is a panniculitis secondary to vitamin K injections causing sclerotic lesions with lilac borders on the buttocks and thighs resembling a cowboy belt and holster.
Which of the following is characteristic of pleomorphic lipoma?
1 Foreign body giant cells
2 Floret giant cells
3 Frequent mitoses
4 Exocytosis of lymphocytes
5 Virchow bodies
Floret giant cells
Pleomorphic lipomas characteristically have a mixture of variably sized fat cells with a varying number of pleomorphic enlarged cells. These cells contain nuclei arranged in a circumferential pattern that has been termed floret cells. Rare lipoblasts are found. Focal collections of lymphocytes and plasma cells within the tumor are seen.
Using the salt-split skin technique with direct immunofluorescence, epidermolysis bullosa acquisita will show linear deposition of complement in what position?
1 Roof of the split
2 Roof and floor of the split
3 Floor of the split
4 Neither the roof or floor of the split since IgA is the most common reactant
5 None of the above since the pattern is not linear
Floor of the split
Epidermolysis bullosa acquisita (EBA) is a bullous disease of adults in which minor trauma (usually on the hands and feet) leads to blisters that heal with scaring. On histology there is classically a noninflammatory subepidermal split. The blister will immunostain with IgG on the floor of salt-split skin, as the antigen is type VII collagen.
