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Dermatology > Disorders of Hyperpigmentation > Flashcards

Disorders of Hyperpigmentation Flashcards

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Dermatology Board Prep flashcards
1
Q

Lichen Planus Pigmentosus

A

favors Skin Types III-V; oval round/brown/gray macules and patches in sun-exposed or intertriginous zones; NO erythematous border (whereas EDP does)

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2
Q

Nevus of Ota

A

blue/back or gray/brown at 1st or 2nd branch of trigeminal nerve; distinguish bilateral from Hori nevus (acquired bilateral nevus of Ota-like macules; no mucosal involvement and less pigmented)

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3
Q

Nevus of Ito

A

skin of acromioclavicular and deltoid region

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4
Q

Mongolian Spots

A

dermal melanocytosis; preferentially in sacral area; most show spontaneous regression; histo w/ spindle-shaped melanocytes (in lower dermis; failed to migrate to DEJ)

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5
Q

Dermal Melanocyte Hamartoma

A

gray-blue pigmentation in dermatomal pattern

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6
Q

Peutz-Jeghers Syndrome

A

Gene: (AD) STK11
Features: mucocutaneous pigmentation and intestinal hamartomatous polyposis; assoc with GI malignancies; begins in childhood

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7
Q

LEOPARD Syndrome

A 
Gene:  (AD) PTPN11
Lentigines
EKG abnormalities
Ocular hypertelorism
Pulmonary stenosis
Abnormal genitalia
Retardation of growth
Deafness sensorineural
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8
Q

Carney Complex (NAME/LAMB)

A 
Gene: (AD) PRKAR-1alpha
Triad of  1. primary adrenal hypercortisolism; 2. lentigenes, ephelides, and blue nevi of skin/mucosa; 3. variety of tumors of endocrine and non-endocrine origin (cardiac, cutan and mammary myxomas; overactive endocrine and adrenocortical disease; psammomatous melanotic schwannoma)
Nevi
Atrial myxoma
Myxoid neurofibroma
Ephelides 
aka
Lentigenes
Atrial myxoma
Mucocutaneous myxoma
Blue nevi
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9
Q

McCune Albright Syndrome

A

Gene: GNAS1 (activating G protein –> const cAMP)
Features: 1. poly/monostotic fibrous dysplasia
2. CALMs
3. Hyperfxning endocrinopathies (precocious puberty, hyperthyroidism, hypercortisolism, hypersomatotropism, hypophosphatemic rickets)

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10
Q

Russell Silver Syndrome

A

low birth weight, short stature, small triangular face, clinodactyly of 5th finger; CALMs variable feature

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11
Q

Primary Cutaneous Amyloidosis

A
  1. Macular (upper back)
  2. Lichenoid (extensor surface lower extremities)
  3. Nodular
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12
Q

Atrophoderma of Pasini and Pierini

A

oval hyperpigmented patches on posterior trunk w/ subtle depression of entire lesion and “cliff sign”

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13
Q

Bleomycin Hyperpigmentation

A

Flagellate bands on chest/back; pigmented banding of nails

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14
Q

Adriamycin Hyperpigmentation

A

pigmented patches on oral mucosa (esp lateral tongue)

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15
Q

5-FU Hyperpigmentation

A

hyperpigmentation of sun-exposed skin

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16
Q

Argyria

A

silver exposure (occupational, use of silver sulfadiaine): diffuse slate-gray discoloration w/ accentuation in sun-exposed areas, nails, sclera

17
Q

Chrysiasis

A

gold exposures; blue grey hyperpigmentation on sun-exposed areas

18
Q

Drugs that can cause hyperpigmentation

A

Amiodarone, AZT (mucous membranes/nails), Clofazimine, Hydroquinone, minocycline, psoralens, hydroxychloroquine/chloroquine, quinacrine

19
Q

Exogenous Ochronosis

A

hyperpigmentation following hydroquinone applications

20
Q

Endocrinopathies leading to Diffuse Non-Figured Hypermelanosis

A

Addisons, Cushing Syndrome, Nelson Syndrome, Pheochromocytoma, Carcinoid, Hyperthyroidism, pregnancy, acanthosis nigricans, diabetes

21
Q

Nutritional Hyperpigmentation

A

Kwashiorkor, Vit B12 def, Folic acid def, pellagra

22
Q

Metabolic Conditions causing Hyperpigmentation

A

PCT, hemochromatosis

23
Q

Causes of Linear Hyperpigmentation

A
  • Phytophotodermatitis
  • Pigmentary Demarcation Lines
  • Flagellate Pigmentation from Bleomycin
  • Flagellate Mushroom Dermatitis (raw shiitake mushrooms)
  • If along Lines of Blaschko:
    • Linear and Whorled Nevoid Hypermelanosis
    • Incontinentia Pigmenti (x-linked dominant)
24
Q

Incontinentia Pigmenti

A

Gene: (X-linked dominant) NEMO (nf kappa b anti-apoptotic); most males embryonic lethal
Features: 4 stages:
1. Vesicles (from birth or shortly after)
2. Verrucous lesions (2-8 weeks)
3. Hyperpigmentation (sev months - adulthood)
4. Hypopigmentation (infancy to adulthood in stage IV)
Many of ocular, dental, skeletal, CNS anomalies

25
Q

Common causes of Reticulated Hyperpigmentation

A

CARP, Erythema ab igne, livedo reticularis, atopic dermatitis

26
Q

Dyskeratosis Congenita

A

Gene: (X-linked recessive) DKC1 (dyskerin, interacts w/ telomerase; mut leads to defective telomerase maintenance; melanocytes become senescent and make more melanin); 90% male
Features:
TRIAD: reticulated hyperpigmentation of neck; nail dystrophy; premalignant leukoplakia (all dev in 1st decade)
Other: malformed teeth, epiphora (continuous lacrimation), bone marrow failure, malignancy (SCC) increased risk of myelodysplasia, AML, Hodgkin’s disease, and GI carcinoma

27
Q

Naegeli-Franceschetti-Jadassohn Syndrome

A

Gene: (AD) Keratin 14
Features: reticulated hyperpigmentation (primarily abd, periocular, perioral) in 1-2yrs of life (fades during adolescence)
Also: dental anomalies (early total loss of teeth), heat intolerance, hypohidrosis; absent or hypoplastic dermatoglyphics, palmoplantar hyperkeratosis, onychodystrophy

28
Q

Dermatopathia Pigmentosa Reticularis

A 
Gene:  (AD) Keratin 14
Features:  TRIAD
1.  persistent reticulated hyperpigmentation w/ truncal predominance
2.  nonscarring alopecia
3.  onychodystrophy
29
Q

X-Linked Reticulate Pigmentary Disorder

A
  • reticulated hyperpigmentation (along lines of Blaschko in girls; generalized in males)
  • frequent infxns and systemic manifestations
  • blonde unruly hair w/ frontal upsweep
30
Q

Dowling-Degos Disease

A

Gene: Keratin 5 (AD); onset 3rd-4th decade
Features:
-Acquired reticulated hyperpigmentation beginning in axillae and inguinal folds (later others like flexures)
-Comedone-like lesions on back/neck
-Pitted facial scars
-Epidermoid cysts
[Assoc with HS]

31
Q

Galli-Galli

A

equivalent to Dowling-Degos but with suprabasilar non-dyskeratotic acantholysis on path
DD:
Gene: Keratin 5 (AD); onset 3rd-4th decade
Features:
-Acquired reticulated hyperpigmentation beginning in axillae and inguinal folds (later others like flexures)
-Comedone-like lesions on back/neck
-Pitted facial scars
-Epidermoid cysts

Dermatology (17 decks)

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