Derm Flashcards
4 causes of pseudoepitheliomatous hyperplasia?
Prurigo nodularis, some deep fungal infections (including Blastomycosis), granular cell tumors, halogenodermas (iododerma, bromoderma).
Carney Complex. AKA (3)?
AKA LAMB syndrome (Lentigenes, Atrial myxomas, Mucocutaneous myxomas, Blue nevi), NAME syndrome (Nevi, Atrial myxomas, Myxoid neurofibroma, Ephelides), or Swiss syndrome. AD condition. The majority (Carney Complex type I) are caused by mutations in the PRKAR1-alpha gene on 17q24, which has been suggested to function as a tumor-suppressor gene. Carney Complex type II involves chromosome 2. 7% of all cardiac myxomas are associated with Carney complex. M=F. Mean age at Dx: 10-20 years.
DDx of Merkel cell carcinoma.
The histologic features of MCC are similar to those of various other tumors, such as metastatic small cell carcinoma, the small round cell tumors, blastic hematologic malignancies of skin/soft tissue, and melanoma. Immunostains can help distinguish them.
Examples of pseudoneoplastic lesions in the skin and their related neoplastic mimes.
Adnexal nevi [adnexal neoplasms]. Lymphoid hyperplasias [lymphomas]. Reactions to Monsel solution [sarcomas; sarcomatoid carcinomas]. Acroangiodermatitis AKA acroangiodermatitis of Mali AKA dermatitis hemostatica AKA Bluefarb-Stewart syndrome AKA pseudo-Kaposi sarcoma [Kaposi sarcoma]. Proliferating scars and posttraumatic spindle cell nodules [sarcomas; sarcomatoid carcinomas]. Intravascular papillary endothelial hyperplasia (Masson lesion) [angiosarcoma]. Pseudocarcinomatous (pseodoepitheliomatous) epithelial (epidermal) hyperplasia [squamous cell carcinoma]. Rudimentary meningocele [angiosarcoma]. Mycobacterial pseudotumors [mesenchymal neoplasms]. Bacillary angiomatosis [hemangiomas]. Other pseodoneoplasms include Der Wulst AKA central facial folliculocentric basaloid proliferation AKA hair follicle “bulges”, pseudosarcomatous fibroepithelial polyps (acrochordons), proliferative noninfectious granulomatous lesions, reactive angioendotheliomatosis, and intravascular histiocytopathy.
Grover disease.
AKA Transient Acantholytic Dermatosis. Is a self-limited, primary, nonfamilial, non-immune-mediated acantholytic skin disorder that manifests are pruritic, discrete, edematous papules and/or a vesiculopapular rash. The term TAD may be misleading, since this entity may be persistent, recurrent and show morphologies other than acantholysis. M:F = 2.4:1. The lesions are mostly on the trunk (99%) and proximal extremities (35%). Grover disease has been found to coexist with numerous other dermatoses in 11% of cases, hematologic malignancies in 8% of cases, and has also been associated with skin cancers. Histologically, the acantholysis seen in Grover disease occurs in a variety of different patterns in small, circumscribed foci, with the patterns occuring singly, or pathognomonically in combination. Patterns of acantholysis seen in Grover disease: pemphigus vulgaris-like (47%), Darier-like (18%), spongiotic (9%), pemphigus foliaceous-like (9%), mixed (9%), and Hailey-Hailey-like (8%). Pathogenesis is unknown, but a strong relationship with heat, sunlight, exercise, and bed confinement places the eccrine apparatus as a primary suspect.
Immunostains in melanocytic neoplasms in dermatopathology: S100, HMB-45, Melan-A/Mart-1, Tyrosinase, Ki-67.
S100: most sensitive marker for melanoma and spindle cell/desmoplastic melanoma. HMB-45: higher specificity for melanoma (primary-metastatic) can help distinguish nevi from melanoma. Melan-A/Mart-1: similar sensitivity and specificity to HMB-45, but with more diffuse and intense staining. Tyrosinase: higher sensitivity than HMB-45 and specificity of 97-100%, sensitivity decreases with increased clinical stage and in metastases. Ki-67: less than 5% of cells in nevi, and 13-30% of cells in malignant melanoma; higher percentages have been noted in Spitz nevi as well.
Lentigo maligna.
The preinvasive/in situ form of melanoma located on chronically sun-damaged skin. The appellation lentigo maligna has been used b/c of the lesion’s clinical resemblance to an actinic/solar lentigo and/or b/c of some authors’ unwillingness to label these lesions as a malignancy per se. Most patients present with a slowly enlarging macule or patch. Areas of partial regression within the lesion are not uncommon. Amelanotic lentigo maligna may develop spontaneously or may arise from a previously treated pigmented lentigo maligna. Histologic features related to chronic sun damage such as epidermal atrophy, increased pigmentation in basal keratinocytes, and prominent solar elastosis are present. The melanocytes are increased in number, and as in solar lentigenes, often are disposed as single cells along the basal layer. Nests of melanocytes may be present, but are unevenly distributed across the lesion. A few melanocytes may permeate superficial epidermal layers; involvement of cutaneous adnexal structures is common. Nuclear atypia is variable and may be subtle.
Lichen planus vs. lichenoid drug eruption?
LDE should be considered in all pts with clinical features of LP. LDE may appear clinically and histologically identical to LP. The drugs MC associated with LDE are ACEIs, thiazides, beta-blockers, gold salts, antimalarial agents, and penicillamine. The mean age of onset is ~60 yo, about 10 years later than idiopathic LP. LDE has a variable latency period of weeks to years that is dependent on multiple factors. Although the pathogenesis of LDE is unclear, offending drugs likely alter epidermal proteins, acting as haptens to induce an immune response. Idiopathic LP is characterized by monomorphic, violaceous, shiny, flat-topped papules and plaques, characteristically with a fine reticulated pattern of white scale (Wickham striae, which is the macroscopic correlate to histologic hypergranulosis). The flexural surfaces of the wrists, anterior lower legs, and mucous membranes are often affected. LDE is often more polymorphic and may include lesions with a more eczematous, scaly appearance, and is also more likely to present with an atypical distribution of lesions, esp truncal or photodistribution, and to resolve with more pronounced hyperpigmentation. Characteristic histologic features of idiopathic LP include a dense band-like lymphocytic infiltrate at the dermal-epidermal junction, damage to the basal layer, colloid bodies (eosinophilic hyaline bodies originating from apoptosed keratinocytes), irregular acanthosis with “saw-toothed” rete ridges, and overlying hypergranulosis. Although no histologic features can reliably distinguish between idiopathic LP and LDE, certain findings such as the presence of eosinophils, plasma cells, focal parakeratosis, and a deeper perivascular infiltrate may support a diagnosis of LDE.
Metastatic Crohn disease.
MCD is an entity characterized by cutaneous, noncaseating granulomas at sites anatomically separate from the GI tract. It is the least common dermatologic manifestation of Crohn disease. In adults, the age of onset of MCD ranges from approximately 29 to 39 years, and the majority of patients have a previous diagnosis of Crohn disease. Twenty percent of patients with MCD may present without classical manifestations of Crohn disease. In these patients, Crohn disease manifests in 2 months to 4 years after the initial presentation of MCD. Cutaneous lesions of MCD may present as papules, plaques, nodules, and ulcerations, which may involve the arms, legs, genitalia, and face. Lesions have also been noted to have predilection for the moist environment of skin folds, including submammary and abdominal creases as well as the perineal and inguinal regions. MCD may present as a solitary lesion or occur in multiple sites and may be painless or tender upon palpation. In the pediatric population, MCD typically presents from the ages of 10 to 14, with about 50% of these patients having concurrent Crohn disease. Of these patients, approximately one-half have active gastrointestinal symptoms. In children who present with MCD lesions without evidence of Crohn disease, subsequent onset of gastrointestinal manifestations occurs from 9 months to 14 years after the initial presentation of MCD. The genitalia appear to be the most common area of involvement in children with MCD; the most common cutaneous manifestation presents as labial, penile, and/or scrotal swelling with or without accompanying erythema. Genital ulcerations have also been reported. MCD presents microscopically as sterile, noncaseating granulomatous inflammation located primarily in the superficial papillary and deep reticular dermis with occasional extension into the subcuticular fat. The granulomas consist of Langerhans giant cells, epithelioid histiocytes, lymphocytes, and occasional plasma cells. The underlying etiology of MCD is currently unknown. It has been suggested that antigens or immune complexes stemming from the GI tract in primary Crohn disease travel through the circulatory system and deposit in the skin, creating perivascular granulomatous features seen on microscopic examination of MCD lesions. Autoimmune cross-reactivity has also been suggested, where antibodies specific to antigens in the GI tract that may be responsible for inflammatory Crohn disease react with skin antigens of similar structure. The differential diagnosis of MCD consists of any granulomatous entities that may involve the skin, including but not limited to cutaneous sarcoidosis, erythema nodosum, pyoderma gangrenosum, hidradenitis suppurativa, mycobacterial disease, and foreign body reaction.
Painful skin lesions mnemonic (variation 1 of 3).
ANGEL: Angiolipoma, Neuroma, Glomus tumor, Eccrine spiradenoma, Leiomyoma.
Painful skin lesions mnemonic (variation 2 of 3).
LEND AN EGG: Leiomyoma, Eccrine spiradenoma, Neuroma, Dermatofibroma (sometimes), Angiolipoma, Neurilemmoma, Endometrioma, Glomus tumor, Granular cell tumor.
Painful skin lesions mnemonic (variation 3 of 3).
BLUE ANGEL: BLUE rubber bleb nevus, Angiolipoma, Neuroma, Glomus tumor, Eccrine spiradenoma, Leiomyoma.
What is seen histologically in skin and GI biopsies from patients with acute GVHD?
The skin biopsy initially shows epidermal basal vacuolization and focal epidermal apoptosis with lymphoid infiltration. Bullae formation with epidermal separation can extend to erythroderma, and necrosis is observed in later stages. In grade I, there is vacuolization of the basal keratinocytes; in grade II, there are dyskeratotic keratinocytes and basal cell vacuolization; in grade III, there is increased keratinocyte necrosis and focal basal layer clefting; in grade IV, there is necrosis of the entire epidermis and complete separation from the dermis. There are various degrees of lymphocytic infiltration. The microscopic findings of early GVHD are similar to erythema multiforme, while the severe form resembles toxic epidermal necrolysis.
The GI biopsy shows diffuse edema and mucosal swelling followed by variable crypt cell apoptosis (“exploding” crypts) and a mixed chronic and predominantly lymphoplasmacytic infiltrate, and there can be crypt dropout. In grade I, there is increased crypt apoptosis; in grade II, there is apoptosis with crypt abscess; in grade III, there is crypt necrosis; in grade IV, there is total denudation of mucosal areas. The immunosuppressive drug mycophenolate mofetil can cause variable GI mucosal injury patterns, including GVHD-like changes.
In the skin and GI tissue affected by GVHD, the mechanism of apoptosis is likely similar and caused by alloreactive T lymphocytes targeted to alloantigen on recipient tissue.
Erysipelas is caused by (organism). Erysipeloid is caused by (organism).
Erysipelas is caused by S. pyogenes. Erysipeloid is caused by Erysipelothrix rhusiopathiae.
Adiaspiromycosis is caused by (organism).
Adiaspiromycosis is caused by Chrysosporium parvum.
Define the following terms: acantholysis, acanthosis, bulla, epidermolysis, lichenoid interface change, spongiosis, vacuolar interface change.
Acantholysis is disruption of desmosomes that normally join the keratinocytes of the epidermis, resulting in loss of cohesion and rounding up of the affected cells. Acanthosis is an increase in the thickness of the stratum malpighii. A bulla is an intraepidermal or subepidermal cavity. Intraepidermal bullae may be secondary to either spongiosis or acantholysis. Subepidermal bullae are formed from extensive papillary dermal edema. Epidermolysis is a distinctive alteration of the granular layer characterized by perinuclear clear spaces, swollen and irregular keratohyalin granules, and an increase in the thickness of the granular layer. Lichenoid interface change results from destruction of basal keratinocytes. Destruction and dyskeratosis result in “remodeling” of the basement membrane zone. A bandlike lymphocytic infiltrate usually accompanies the keratinocyte changes. Spongiosis is intercellular intraepidermal edema. Vacuolar interface change results from destruction of the basal keratinocytes characterized by the presence of intracytoplasmic vesicles. The vesicles enlarge, and eventually the basal keratinocytes die; as a result, the integrity of the basal zone of the epidermis is lost.
What are the 4 layers of the epidermis?
The stratum corneum is the outermost layer of the epidermis. The fully keratinized cells of this layer are flattened and devoid of nuclei. On acral surfaces, a thin stratum lucidum (clear zone) is present between the stratum corneum and the stratum granulosum. The stratum granulosum is named for the prominent deeply basophilic keratohyalin granules found in flattened keratinocytes. Deep to the stratum granulosum is the stratum spinosum, which is characterized by abundant eosinophilic cytoplasm, ovoid nuclei, and intercellular bridges. The stratum basale (basal cell layer) is the undulating row of cuboidal to columnar cells with minimal cytoplasm and contain proliferating cells for epidermal renewal. The basal cells attach to the basement membrane of the epidermis. The stratum spinosum and the basal cell layer are collectively referred to as the stratum malpighii.
What HPV types (in order of frequency) are seen mostly commonly in the following lesions? Plantar wart, common wart, flat (juvenile) wart, oral squamous papilloma, oral focal epithelial hyperplasia (Heck disease), epidermodysplasia verruciformis, laryngeal papillomas, condyloma acuminatum, cervical LSIL, cervical HSIL, cervical AIS and invasive cervical adenocarcinoma.
Plantar wart: 1, 2. Common wart: 2, 1, 4, (HPV 7 in fish and meat handlers). Flat (juvenile) wart: 3, 10. Oral squamous papilloma: 6, 11. Oral focal epithelial hyperplasia (Heck disease): 13, 32. Epidermodysplasia verruciformis: 2, 3, 10, 5, 8. Laryngeal papillomas: 6, 11. Condyloma acuminatum: 6, 11. Cervical LSIL: 6, 11. Cervical HSIL: 16, 18, 31, 33, 35. Cervical AIS: 18. Invasive cervical adenocarcinoma: HPV 16 and 18 are detected with equal prevalence in most subtypes of cervical adenocarcinoma.
What are Henderson-Patterson bodies?
Henderson-Patterson bodies = molluscum bodies, seen in molluscum contagiosum. They are eosinophilic intracytoplasmic inclusion bodies (accumulated virus particles) in keratinocytes of stratum spinosum and stratum granulosum.
What is myospherulosis?
Myospherulosis, AKA spherulocytosis AKA subcutaneous spherulocystic disease, is an iatrogenic benign mass formed by a foreign body-type granulomatous reaction to lipid-containing material and blood. Clinically, there are solid subcutaneous or dermal nodules. Grossly, is a large saccular cyst-like lesion Histologically, there are saclike structures with fungi-like spherules. The spherules are composed of erythrocytes damaged by endogenous and exogenous fat. The damaged erythrocytes are enclosed by a lipid membrane and later phagocytosed by histiocytes as part of the lipogranulomatous reaction that takes place in adipose tissue. The walls of the spherules are formed due to the physical emulsion phenomenon that occurs between lipid-containing materials and blood. Myospherulosis can be a result of fat necrosis, malignancy such as RCC, endogenous membranocystic degeneration of fat that occurs in lupus, or lipid/petrolatum-based medications being injected or applied to open wounds. It is called MYOspherulosis due to the involvement of skeletal muscle in some patients. It can be mistaken for true fungus, such as Coccidioides, because myospherulosis has parent bodies/pigmented bodies and spherules/endobodies that are similar in size and morphology to Coccidioides; however, myospherulosis has pigmented bodies, while Coccidioides is never pigmented.
What are the 3 main types of multinucleated giant cells?
Foreign body giant cells, Langhans giant cells, and Touton giant cells. All three types are transformed macrophages; mononuclear phagocytes fused under the influence of cytokines. Foreign body giant cells have nuclei that are randomly distributed but often aggregate as centrally located, overlapping nuclei. Langhans giant cells have a peripheral ring-like arrangement of nuclei in an arcuate configuration but no rim of clear cytoplasm. Touton giant cells have a ring of nuclei separating a peripheral clear or foamy rim of cytoplasm from central, more eosinophilic cytoplasm. The peripheral cytoplasm appears clear due to high lipid content. Touton giant cells are seen in lesions with high lipid content, such as xanthomas, xanthogranulomas, and fat necrosis. Touton giant cells can also be seen in dermatofibroma. Other types of multinucleated giant cells include epithelium-derived MGCs, which can be prominent in certain viral infections such as measles (Warthin-Finkeldey cells), RSV, HSV/VZV, and parainfluenza. Also, MCGs derived from neoplastic cells may be formed in a variety of neoplasms.
Does Merkel cell carcinoma stain with TTF-1?
No.
When is SLN biopsy indicated in melanoma, according to ASCO and Society of Surgical Oncology guidelines?
SLN bx is recommended for intermediate-thickness melanomas (Breslow thickness 1-4 mm) of any anatomic site for accurate staging. SLN bx may be indicated for thick melanomas (T4, Breslow thickness >4 mm) for staging and to facilitate regional disease control. Evidence does not support routine SLN bx of thin melanomas (<1 mm) except with high risk features (ulceration, mitotic rate 1/mm^2 or greater, Breslow thickness 0.75-0.99 mm) when benefits of staging outweight risks of the procedure. Complete lymph node dissection is recommended for all patients with a positive SLN bx.
What is a Triton tumor?
A benign Triton tumor is a neuromuscular hamartoma/choristoma. It is a rare developmental lesion of mature skeletal muscle and nerve. Microscopically, is made up of multiple nodules, each 3-5 mm, separated by narrow bands of connective tissue. Nodules are composed of fasicles of striated muscle of varying size with nerve fibers (myelinated or not) within same perimysial fibrous sheath. Stroma may be more cellular with bland spindle cells and resemble fibromatosis. A malignant Triton tumor is made up of both malignant schwannoma cells and malignant rhabdomyoblasts, and is classified as a MPNST with rhabdomyosarcomatous differentiation. The name “triton” was first used in reference to observation of supernumerary limbs containing bone and muscle growing the backs of tritons (a name given to some species of sea snails) after the implantation of the sciatic nerve into the soft tissues of the back.