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56 Cards in this Set
- Front
- Back
Pathogenesis of epidermal post-inflammatory hyperpigmentation? |
Increased melanin production and/or transfer to keratinocytes |
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Pathogenesis of dermal post-inflammatory hyperpigmentation? |
Damaged basement membrane allowing "falling" of melanin and subsequent phagocytosis by dermal macrophages |
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Color seen with increased epidermal melanin? |
Tan to dark brown |
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Color seen with increased dermal melanin? |
Gray-blue to gray-brown |
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Which lasers (3) may aid in removal of dermal pigment? |
1. Nd:YAG 2. Q-switched ruby 3. Alexandrite |
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Clinical presentation of erythema dyschromicum perstans? |
Irregularly shaped macules and patches with slate-gray to blue-brown color seen in a symmetric pattern on the trunk with spread to neck, proximal upper extremities, and face |
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Disease course of erythema dyschromicum perstans? |
Slowly progressive; usually does not regress Usually asymptomatic, however can be mildly pruritic |
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Histopathologic findings in erythema dyschromicum perstans? |
1. Vacuolar degeneration of the basal layer 2. Perivascular mononuclear cell infiltrate in upper dermis 3. Increased epidermal melanin and dermal melanophages |
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Two potential drugs that can help treat erythema dyschromicum perstans? |
1. Clofazimine 2. Dapsone |
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Most commonly affected individuals in lichen planus pigmentosus? |
Young to middle-aged adults with skin phototypes III-V |
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Clinical presentation of lichen planus pigmentosus? |
Brown to gray-brown macules and patches in sun-exposed areas or intertriginous zones |
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What can potentially cause lightening of lesions in lichen planus pigmentosus? |
Topical tacrolimus |
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Clinical presentation of melasma? |
Symmetric hyperpigmented patches with irregular outline on the face (also uncommonly on upper chest, forearms) Exacerbated by pregnancy, OCPs, sun exposure |
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Melasma is seen in increased prevalence in what 3 ethnic groups? |
1. Hispanic 2. African 3. Asian/Middle Eastern (i.e. darkly pigmented skin) |
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Pathogenesis of melasma? |
Hyperfunctional melanocytes produce increased amounts of melanin in response to an inducer (hormones, UV radiation) |
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Most common topical regimen to treat melasma? |
Hydroquinone + tretinoin + corticosteroid 2 - 6 month course |
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Clinical presentation of primary (localized) cutaneous amyloidosis? |
Rippled pattern of parallel bands or ridges of hyperpigmentation + pruritus |
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What form of mastocytosis is classically associated with hyperpigmentation? How does it present? |
Urticaria pigmentosa Few to several hundred brown to red-brown papules which urticate after stroking (Darier's sign) |
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What are common medications (4) that cause hyperpigmentation / discoloration? |
1. Chemotherapeutic agents (gleevec, bleomycin) 2. Antimalarials 3. Minocycline 4. Zidovudine i.e. AZT |
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Most common presentation of minocycline-induced hyperpigmentation? |
Gray-blue hyperpigmentation on the b/l shins |
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Where are pigmentary demarcation lines most commonly seen? |
Anterolateral upper arm, posteromedial thigh (demarcation b/w the dorsal and ventral surfaces) Lines are perfectly symmetric and stable over time |
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What percentage of patients taking bleomycin develop flagellate hyperpigmentation? |
10-20% (fairly common side effect) Pigmentation (brown streaks) appears to be dose-dependent |
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Most common location for flagellate hyperpigmentation caused by bleomycin? |
Chest and back |
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What food can cause a flagellate dermatitis that presents as linear streaks of papules or petechiae? |
Shitake mushrooms; raw or partially cooked Pruritus is initially seen; scratching leads to long, flagellate streaks |
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What does hyperpigmentation along the lines of Blaschko indicate? |
Mosaicism (normal variant) |
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Term for hyperpigmentation along the lines of Blaschko? |
Linear and whorled nevoid hypermelanosis (LWNH) |
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What is clinically important about Linear and whorled nevoid hypermelanosis? |
Up to 25% of patients present to pediatrician with extracutaneous findings (neurologic, MSK, cardiac) |
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Inheritance pattern for incontinentia pigmenti? |
X-linked dominant |
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What are the 4 stages of incontinentia pigmenti? |
1. Linear erythema and blisters 2. Verrucous lesions 3. Hyperpigmentation along lines of Blaschko 4. Hypopigmentation along lines of Blaschko |
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What gene is responsible for incontinentia pigmenti? |
Mutations in the NEMO gene |
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Clinical presentation of prurigo pigmentosa? |
Markedly pruritic eruption of erythematous papules and papulovesicles that develop rapidly and involute within a week, leaving macular reticulated hyperpigmentation |
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Markedly pruritic eruption of erythematous papules and papulovesicles that develop rapidly and involute within a week, leaving macular reticulated hyperpigmentation? |
Prurigo pigmentosa |
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Typical patient profile for prurigo pigmentosa? |
Young females in Japan |
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Treatment options for prurigo pigmentosa (3)? |
1. Minocycline 2. Doxycycline 3. Dapsone |
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Clinical presentation of dyskeratosis congenita? |
1. Progressive bone marrow failure 2. Reticulated hyperpigmentation 3. Nail dystrophy 4. Leukoplakia 5. Increased risk of SCC (esp mucosal), AML, and Hodgkin disease |
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Genodermatosis that presents with mucocutaneous triad of reticulated hyperpigmentation + nail dystrophy + leukoplakia, along with progressive bone marrow failure and increased risk of SCC? |
Dyskeratosis congenita |
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Inheritance pattern of dyskeratosis congenita? |
X-linked recessive |
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What enzyme is implicated in dyskeratosis congenita? |
Telomerase mutation is in DKC1 gene which encodes dyskerin, which interacts with telomerase Tissues with high replication rates (bone marrow, skin) are affected the most when telomerase is defective |
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Best method to diagnose dyskeratosis congenita? |
Flow fluorescence in situ hybridization (FISH) of leukocytes detecting short telomere length |
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Inheritance pattern of Naegeli-Franceschetti-Jadassohn syndrome? |
Autosomal dominant |
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What is the mutation responsible for Naegeli-Franceschetti-Jadassohn syndrome? |
Truncating mutations of keratin 14 |
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Clinical features of Naegeli-Franceschetti-Jadassohn syndrome (4)? |
1. Brown or gray-brown reticulated hyperpigmentation of the trunk and periocular/perioral regions 2. Decreased sweat gland function + heat intolerance 3. Palmoplantar keratoderma 4. Dental anomalies |
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Genodermatosis that presents with reticular hyperpigmentation, decreased sweat gland function, palmoplantar keratoderma, and dental anomalies? |
Naegeli-Franceschetti-Jadassohn syndrome |
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What is the mutation responsible for dermatopathia pigmentosa reticularis? |
Heterozygous truncating mutation of keratin 14 gene |
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Clinical features of dermatopathia pigmentosa reticularis? |
1. Reticulated hyperpigmentation 2. Non-scarring alopecia 3. Onychodystrophy |
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Genodermatosis that presents with reticulated hyperpigmentation, non-scarring alopecia, and onychodystrophy? |
Dermatopathia pigmentosa reticularis |
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Genodermatosis with reticulated brown hyperpigmentation that is generalized in male patients and along the lines of Blaschko in female patients? |
X-linked reticulate pigmentary disorder |
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Inheritance pattern in Dowling-Degos disease? |
Autosomal dominant |
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Clinical features in Dowling-Degos disease? |
Reticulated hyperpigmentation in the intertriginous areas (groin, intercluteal, inframammary, neck, inner arms/thighs) |
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Genodermatosis that presents with reticulated hyperpigmentation in the intertriginous areas by the 3rd or 4th decade of life? |
Dowling-Degos disease |
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Genodermatosis that presents as slightly depressed lentigo-like hyperpigmented macules in a reticular pattern on dorsal aspects of hands and feet? |
Reticulate acropigmentation of Kitamura |
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Hypermelanosis that presents as both hyper- and hypopigmented macules of varying size on the dorsal surfaces of distal extremities, mostly in Asians? |
Dyschromatosis symmetrica hereditaria (DSH) |
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Inheritance pattern of dyschromatosis symmetrica hereditaria? |
Autosomal dominant Caused by mutations in ds-RNA specific adenosince deaminase |
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Primary difference between reticulate acropigmentation of Kitamura and dyschromatosis symmetrica hereditaria? |
The former does NOT have hypopigmented macules |
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Hypermelanosis that presents as both hyper- and hypopigmented macules in a generalized distribution, most commonly in Japanese? |
Dyschromatosis universalis hereditaria (DUH) no seasonal change or spontaneous regression with age is seen |
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Hypermelanosis that presents as chronic, asymptomatic gray-brown patches interspersed with hypopigmented macules on the dorsal forearms, seen most commonly in Caucasian women in 5th to 7th decades? |
Acquired brachial cutaneous dyschromatosis Likely 2/2 chronic sun exposure |