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56 Cards in this Set

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Pathogenesis of epidermal post-inflammatory hyperpigmentation?

Increased melanin production and/or transfer to keratinocytes

Pathogenesis of dermal post-inflammatory hyperpigmentation?

Damaged basement membrane allowing "falling" of melanin and subsequent phagocytosis by dermal macrophages

Color seen with increased epidermal melanin?

Tan to dark brown

Color seen with increased dermal melanin?

Gray-blue to gray-brown

Which lasers (3) may aid in removal of dermal pigment?

1. Nd:YAG




2. Q-switched ruby




3. Alexandrite

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

Disease course of erythema dyschromicum perstans?

Slowly progressive; usually does not regress




Usually asymptomatic, however can be mildly pruritic

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

Two potential drugs that can help treat erythema dyschromicum perstans?

1. Clofazimine




2. Dapsone

Most commonly affected individuals in lichen planus pigmentosus?

Young to middle-aged adults with skin phototypes III-V

Clinical presentation of lichen planus pigmentosus?

Brown to gray-brown macules and patches in sun-exposed areas or intertriginous zones

What can potentially cause lightening of lesions in lichen planus pigmentosus?

Topical tacrolimus

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

Melasma is seen in increased prevalence in what 3 ethnic groups?

1. Hispanic




2. African




3. Asian/Middle Eastern




(i.e. darkly pigmented skin)

Pathogenesis of melasma?

Hyperfunctional melanocytes produce increased amounts of melanin in response to an inducer (hormones, UV radiation)

Most common topical regimen to treat melasma?

Hydroquinone + tretinoin + corticosteroid




2 - 6 month course

Clinical presentation of primary (localized) cutaneous amyloidosis?

Rippled pattern of parallel bands or ridges of hyperpigmentation + pruritus

Rippled pattern of parallel bands or ridges of hyperpigmentation + pruritus

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)

What are common medications (4) that cause hyperpigmentation / discoloration?

1. Chemotherapeutic agents (gleevec, bleomycin)




2. Antimalarials




3. Minocycline




4. Zidovudine i.e. AZT

Most common presentation of minocycline-induced hyperpigmentation?

Gray-blue hyperpigmentation on the b/l shins

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

What percentage of patients taking bleomycin develop flagellate hyperpigmentation?

10-20% (fairly common side effect)




Pigmentation (brown streaks) appears to be dose-dependent

Most common location for flagellate hyperpigmentation caused by bleomycin?

Chest and back

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

What does hyperpigmentation along the lines of Blaschko indicate?

Mosaicism (normal variant)

Term for hyperpigmentation along the lines of Blaschko?

Linear and whorled nevoid hypermelanosis (LWNH)

What is clinically important about Linear and whorled nevoid hypermelanosis?

Up to 25% of patients present to pediatrician with extracutaneous findings




(neurologic, MSK, cardiac)

Inheritance pattern for incontinentia pigmenti?

X-linked dominant

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

What gene is responsible for incontinentia pigmenti?

Mutations in the NEMO gene

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

Markedly pruritic eruption of erythematous papules and papulovesicles that develop rapidly and involute within a week, leaving macular reticulated hyperpigmentation?

Prurigo pigmentosa

Typical patient profile for prurigo pigmentosa?

Young females in Japan

Treatment options for prurigo pigmentosa (3)?

1. Minocycline




2. Doxycycline




3. Dapsone

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

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

Inheritance pattern of dyskeratosis congenita?

X-linked recessive

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

Best method to diagnose dyskeratosis congenita?

Flow fluorescence in situ hybridization (FISH) of leukocytes detecting short telomere length

Inheritance pattern of Naegeli-Franceschetti-Jadassohn syndrome?

Autosomal dominant

What is the mutation responsible for Naegeli-Franceschetti-Jadassohn syndrome?

Truncating mutations of keratin 14

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

Genodermatosis that presents with reticular hyperpigmentation, decreased sweat gland function, palmoplantar keratoderma, and dental anomalies?

Naegeli-Franceschetti-Jadassohn syndrome

What is the mutation responsible for dermatopathia pigmentosa reticularis?

Heterozygous truncating mutation of keratin 14 gene

Clinical features of dermatopathia pigmentosa reticularis?

1. Reticulated hyperpigmentation




2. Non-scarring alopecia




3. Onychodystrophy

Genodermatosis that presents with reticulated hyperpigmentation, non-scarring alopecia, and onychodystrophy?

Dermatopathia pigmentosa reticularis

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

Inheritance pattern in Dowling-Degos disease?

Autosomal dominant

Clinical features in Dowling-Degos disease?

Reticulated hyperpigmentation in the intertriginous areas (groin, intercluteal, inframammary, neck, inner arms/thighs)

Genodermatosis that presents with reticulated hyperpigmentation in the intertriginous areas by the 3rd or 4th decade of life?

Dowling-Degos disease

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

Reticulate acropigmentation of Kitamura

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)

Dyschromatosis symmetrica hereditaria (DSH)

Inheritance pattern of dyschromatosis symmetrica hereditaria?

Autosomal dominant




Caused by mutations in ds-RNA specific adenosince deaminase

Primary difference between reticulate acropigmentation of Kitamura and dyschromatosis symmetrica hereditaria?

The former does NOT have hypopigmented macules

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

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

Acquired brachial cutaneous dyschromatosis




Likely 2/2 chronic sun exposure