Pigmentary Disorders:
Hyperpigmentation
Mohamed Aidaros
Items that we will discuss
1. Facial Melanoses:
➢Melasma
➢Poikiloderma of Civatte
➢Peri buccal pigmentation of Broq
➢Erythromelanosis Follicularis
➢Rheil’s melanosis (Melanodermatitis toxica)
2. LPP, Ashy Dermatosis
3. Postinflammatory hyperpigmentation
4. Drug-induced hyperpigmentation or discoloration
5. Linear hyperpigmentation
6. Reticulated hyperpigmentation
➢Confluent and reticulated papillomatosis
➢Erythema ab igne
➢Prurigo pigmentosa
➢Dyskeratosis congenita
➢Naegeli–Franceschetti–Jadassohn syndrome
➢Dowling–Degos disease
➢Reticulate acropigmentation of Kitamura
7. Dyschromatoses
➢Dyschromatosis symmetrica hereditarian
➢Dyschromatosis universalis hereditaria
Melasma
Melasma
• Most prevalent among
young to middle-aged
women
• Hispanic or of Asian,
African, or Middle Eastern
descent
• Exacerbating factors
1. Sun exposure
2. Pregnancy
3. Use of oral contraceptives
• Primarily on the face
• Light to dark brown or brown–gray patches
• Irregular borders
• Distributed symmetrically
1. Centrofacial (most common):
➢Involving the forehead, cheeks, nose, upper lip (sparing the philtrum and
nasolabial folds), and chin
2. Malar
➢Affecting the cheeks and nose
3. Mandibular
➢Along the jawline
4. Less common sites:
➢Include the extensor aspect of the forearms and mid upper chest
• In lightly pigmented individuals, this “mask of pregnancy”
frequently diminishes or disappears after parturition, but it tends to
persist in women with more darkly pigmented skin
Subdivided into four types based upon the primary location of the
pigment:
1. Epidermal
➢Lesions accentuated with woods light
➢Respond better to topical therapy
2. Dermal
➢Becomes less obvious with woods light (blend with surrounding)
3. Mixed
4. Indeterminate
➢In patients with very dark skin pigmentation
Pathology
• Increased melanin deposition is observed in all layers of the
epidermis
• Epidermal melanocytes are normal to slightly increased in number,
and they are enlarged with prominent dendrites
Poikiloderma of Civatte
Poikiloderma of Civatte
• Atrophy, telangiectasia, hyper‐ and
hypopigmentation
• Sides of the face and neck and on
the upper anterior chest
• Submandibular and submental
areas are spared
• After years of repeated UV
exposure
• Treatment:
➢Photoprotection with a high SPF
sunscreen
➢Avoiding perfumes
➢IPL
Submental sparing
Peribuccal pigmentation of Brocq
Peribuccal pigmentation of Brocq
• Diffuse brownish‐red
pigmentation around the mouth
but sparing a narrow perioral
ring
• Middle‐aged women
• DD: A similar
post‐inflammatory
hyperpigmentation is seen in
some patients with perioral
dermatitis and may be the
result of topical steroid therapy
Erythromelanosis follicularis
faciei et colli
Erythromelanosis follicularis faciei et colli
• Background of reddish brown
pigmentation and telangiectasia
is studded with pale follicular
papules
• Affecting the lateral aspects of
the cheeks
• Spreads slowly, is persistent and
is not influenced by treatment
• Treatment:
➢Keratolytics eg urea 10% or
tretinoin
➢Peeling eg salicylic or glycolic
Riehl melanosis
(Melanodermatitis toxica)
Riehl melanosis (Melanodermatitis toxica)
• Phototoxic reaction to skin
contact with photoactive agents
• Tar derivatives and fragrances
are suspected to be the cause
• Brownish‐grey pigmentation
• Develops quite rapidly over the
greater part of the face but is
more intense on the forehead
and temples
Lichen Planus Pigmentosus
Lichen Planus Pigmentosus
• Uncommon variant of lichen planus
• Favors young to middle-aged adults
• Skin phototypes III–V
• Irregularly shaped or oval, brown to
gray–brown macules and patches
• Distribution mostly symmetric
• Either:
➢Sun-exposed areas (especially the
forehead, temples and neck)
➢Or intertriginous zones
• Usually asymptomatic
Lichen Planus Pigmentosus
• Usually sparing: palms, soles,
nails, and oral mucosa
• Chronic disorder with
exacerbations and remissions
• Pathology:
➢Flattened epidermis with basal cell
degeneration
➢Variably dense band-like or
perivascular infiltrate in the upper
dermis admixed with
melanophages
• DD:
➢Actinic and inverse variants lichen
planus
➢A lichenoid drug eruption
➢Ashy Dermatosis
➢Melasma
Ashy Dermatosis
(ERYTHEMA DYSCHROMICUM
PERSTANS)
• Usually during the second to third
decade
• skin phototypes III–IV
• Gray–brown to blue–gray macules
and patches in a symmetric
distribution
• Favors the neck, trunk, and proximal
extremities
• Mucous membranes are spared
• Occasionally, early lesions have a thin,
raised, erythematous border, which
tends to resolve over a few months
• Lesions may follow skin cleavage lines
• Usually asymptomatic
• There is no consistently effective
therapy (may try UV, topical steroids,
antimalarials)
PIH
PIH
• An acquired excess of melanin pigment following cutaneous
inflammation or injury
• It can occur anywhere on the skin surface, including the mucous
membranes and the nail unit
• Individuals with darkly pigmented skin tend to have a greater
frequency, severity, and duration of PIH than those with lighter
complexions
• Primary lesions of the underlying inflammatory disorder may or may not
be evident admixed with the hyperpigmentation or elsewhere
• When primary lesions are absent, the size, shape, and distribution
pattern of the hyperpigmented lesions may provide clues to the
underlying etiology
Types of PIH
1) Epidermal PIH:
• Increased melanin production and/or transfer to keratinocytes
• Inflammatory mediators that enhance pigment production may
play a role in this process
• Examples: acne, insect bites, pyodermas, atopic dermatitis,
psoriasis and pityriasis rosea
2) Dermal PIH:
• Melanin enters (“falls into”) the dermis via a damaged basement
membrane, where it is phagocytosed by and subsequently resides
within dermal macrophages (referred to as melanophages)
• Melanin within dermal melanophages tends to persist for long
periods of time (e.g. years)
• Examples: lichen planus, lichenoid drug reactions, lupus
erythematosus, and fixed drug eruptions
Treatment
• Provided that the underlying dermatosis is successfully treated, PIH
eventually improves in most patients, especially those with
epidermal hypermelanosis
• Kligman Formula
• (Same as Melasma)
Drug Induced
Hyperpigmentation
• The underlying mechanisms vary from
➢Induction of melanin production
➢To deposition of drug complexes or heavy metals within the
dermis
• The pigmentation can be circumscribed or generalized
• Although it usually resolves with discontinuation of the
offending drug, the course may be prolonged
Cyclophosphamide
• Diffuse hyperpigmentation of the skin and mucous
membranes
• Cutaneous hyperpigmentation usually regresses within 6 to
12 months after therapy is discontinued
• NB: it also causes hemorrhagic cystitis
Antimalarials
• Gray to blue–black pigment, usually pretibial
• May affect face
(exogenous ochronosis, but not as common as hydroquinone)
• Dermal deposition of melanin–drug complexes
• May fade after discontinuation of drug, but rarely resolves
completely
Iron
• Permanent brown pigment at injection sites
• Dermal hemosiderin deposits (due to lysis of extravasated red
blood cells and release of their iron stores)
are commonly observed in:
1. The setting of venous hypertension
2. Pigmented purpuric dermatoses
3. As a side effect of sclerotherapy of superficial veins
Silver (argyria)
• Diffuse slate-gray discoloration increased in sun-exposed areas;
occurs in settings of occupational exposure
• Sites of topical application, e.g. of silver sulfadiazine to burns or
ulcers
Oral contraceptives
• Melasma
• Increased pigmentation of nipples and nevi
Clofazimine
• Diffuse red to red–brown discoloration of skin, conjunctivae
Hydroquinone
• Hyperpigmentation in areas of application due to
1. Exogenous ochronosis
2. Irritant contact dermatitis (i.e. Postinflammatory)
• In exogenous ochronosis, yellow–brown banana-shaped fibers in
papillary dermis
• May fade upon discontinuation of hydroquinone; variable
improvement with pigment-directed Q-switched lasers
Minocycline
• Type I: blue–black discoloration in sites of inflammation and scars,
including those due to acne or ablative laser therapy
• Type II: blue–gray macules/patches (1 mm–10 cm in size) within
previously normal skin, most often on the shins; sometimes
misdiagnosed as ecchymoses
• Type III: diffuse “muddy brown” pigmentation that is most
prominent in sun-exposed areas
Psoralens
• Diffuse hyperpigmentation after exposure to UVA light following
oral administration (PUVA)
• Circumscribed or linear streaks of hyperpigmentation can be seen
with topical exposure, e.g. topical PUVA or phytophotodermatitis
(exposure to psoralen-containing plants plus sunlight)
Linear Hyperpigmentation
DISORDERS WITH LINEAR HYPERPIGMENTATION
DISORDERS WITH LINEAR HYPERPIGMENTATION
Pigmentary Demarcation Lines
Pigmentary Demarcation Lines
• More often apparent in individuals with darkly pigmented skin
• Lines of demarcation between dorsal surfaces (which are
relatively hyperpigmented) and ventral surfaces
• Physiologic pattern of human pigmentation
• Most commonly seen on the anterolateral upper arm and
posteromedial thigh
Confluent And Reticulated
Papillomatosis Of Gougerot And
Carteaud
Confluent and reticulated papillomatosis of
Gougerot and Carteaud
• Onset typically during puberty
• first appearing in the
intermammary area or less
frequently the interscapular or
epigastric regions
• Multiple brown, verrucous,
thin papules or plaques
• Confluent and/or reticulated
pattern
• Can involve neck, upper trunk,
and other flexural areas
• An abnormal host response to Malassezia furfur has been
suggested
DD:
1. Tinea Versicolor
2. Acanthosis Nigricans
3. Darier Disease
4. Terra Firma-forme
Treatment:
➢Often frustrating because it may not respond to therapeutic interventions
or recurs after cessation of therapy
➢Minocycline has been reported to be effective in ~50%
➢Antifungals, tetracyclines, retinoids
Erythema ab igne
• Localized areas of reticulated
erythema and hyperpigmentation
• Chronic exposure to heat below
the threshold for a thermal burn
• Initial presentation is transient
macular erythema in a broad
reticulated pattern that easily
blanches
• With recurrent heat exposure, the
erythema evolves into a dusky
hyperpigmentation; lesions
become fixed and are no longer
blanchable
• Anterior thighs or abdomen in
those with prolonged use of laptop
computers
• Risk of cutaneous malignancy,
particularly squamous cell
carcinoma
Treatment:
• Removal of the
offending heat source
• Use of heat to treat
symptoms of pain
should prompt an
evaluation to
determine its etiology
• If atypia present: 5FU
Others
Prurigo pigmentosa
• Markedly pruritic eruption of
erythematous papules and
papulovesicles on the back,
neck, and chest
• Develop rapidly and then
involute within a week,
leaving macular reticulated
hyperpigmentation
• Treatment with oral
minocycline, doxycycline, or
dapsone is often effective for
the inflammatory component
of prurigo pigmentosa
Dyskeratosis Congenita
• Triad of:
1. Reticulated hyperpigmentation
2. Nail dystrophy
3. Leukoplakia
• Predisposition to malignancy,
especially mucosal squamous cell
carcinoma (e.g. mouth, anus)
NAEGELI–FRANCESCHETTI–JADASSOHN SYNDROME
• Mutation in keratin 14 gene
1. Reticulated hyperpigmentation
beginning by 2 years of age
and often fading during
adolescence
(most often observed on the
abdomen and periocular and
perioral regions)
2. Ectodermal dysplasia with
hypohidrosis and heat
intolerance as well as dental
anomalies, including early loss
of teeth
3. Palmoplantar keratoderma,
with absent or hypoplastic
dermatoglyphs
DOWLING–DEGOS DISEASE
• Mutation in keratin 5 gene
1. Onset is typically during
the third to fourth
decade of life
2. Reticulated
hyperpigmentation in
flexural sites
3. Comedone-like lesions on
the back and neck
4. Pitted facial scars
Reticulate acropigmentation of Kitamura
• Atrophic hyperpigmented
macules
• Appear during childhood
• Acral distribution
Dyschromatosis symmetrica hereditaria
• Small, irregular
hypopigmented and
hyperpigmented macules
on the dorsal aspects of
the distal extremities,
especially the hands and
feet
Dyschromatosis universalis hereditaria
• Hyperpigmented and
hypopigmented macules in a
generalized distribution, with
onset in early childhood
Items that we have discussed
1. Facial Melanoses:
➢Melasma
➢Poikiloderma of Civatte
➢Peri buccal pigmentation of Broq
➢Erythromelanosis Follicularis
➢Rheil’s melanosis (Melanodermatitis toxica)
2. LPP, Ashy Dermatosis
3. Postinflammatory hyperpigmentation
4. Drug-induced hyperpigmentation or discoloration
5. Linear hyperpigmentation
6. Reticulated hyperpigmentation
➢Confluent and reticulated papillomatosis
➢Erythema ab igne
➢Prurigo pigmentosa
➢Dyskeratosis congenita
➢Naegeli–Franceschetti–Jadassohn syndrome
➢Dowling–Degos disease
➢Reticulate acropigmentation of Kitamura
7. Dyschromatoses
➢Dyschromatosis symmetrica hereditarian
➢Dyschromatosis universalis hereditaria
Thank You
