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Dr.Rula jafari
‫الرحيم‬ ‫الرحمن‬ ‫هللا‬ ‫بسم‬
What is a Sign…?
Objective physical finding found by the
examiner.
Two types:
1- De novo
2- Physician induced
● If the superficial silvery white scales are removed via
curettage a characteristic coherence is observed, as if one has
scratched on a wax candle (“signe de la tache de bougie”
Candle sign).
● Subsequently, a surface membrane is seen, The lesion
remains dry until this last level is reached (sign of the last
Häutchen) which will also come off as a whole.
● If the latter is removed, then a wet surface is seen with
characteristic pinpoint bleeding. This finding,is called Auspitz
sign.
Auspitz sign, is
the clinical
reflection of
elongated
vessels in the
dermal papillae
together with
thinning of the
suprapapillary
epidermis
‫ك‬
- Knuckle dimple sign.
- Due to short 4th and 5th metacarpals.
- Enhanced by making a fist.
- Described by Fuller Albright .
- Found in Albright hereditary
osteodysrtrophy.
- Short stature.
- Obesity.
- Round face.
- Mental retardation.
- Brachydactaly
- Soft tissue ossification.
- Short 4th and 5th metacarpals.
- Pseudohypoparathyroidism (PHP):
low calcium
high phosphate
high PTH
non responding kidneys
•Some Share them the habitus of AHO
but are not resistant to PTH. The latter
group are said to have pseudo-PHP.
McCune Albright Syndrome Albright Hereditary Osteodystrophy
AD XLD Inheritance
Polyostotic fibrous dysplasia osteodystrophy Bony lesions
Large café –au-lait macule
(cost of Maine) with
ipsilateral bony changes.
Precocious puberty.
Short stature.
Obesity.
Round face.
Mental retardation.
Brachydactaly
Soft tissue
calcification.
Short 4th and 5th
metacarpals.
Pseudohypoparathyroidism
Other symptoms
McCune Albright
Syndrome
-
-Named after an
English surgeon
William Henry
Battle.
-Indicates fracture
base of skull at
the posterior
fossa.
- Blood dissects
through fascia
and collects at
mastoid process.
- The ability to invaginate the tumor/lesion into the dermal
defect with digital pressure.
- Found in neurofibromas in NF1.
- others:
- Anetoderma
- Old pigmented nevi
- syphilitic chancre
●
● AKA Fitzpatrick’s sign, seen in dermatofibromas.
● Lateral compression with the thumb and index finger
leads to depression of the lesion.
● This dimpling effect is secondary to the lesion being
attached to the subcutaneous fat
Crowe’s sign
● Named after Frank W. Crowe, an American physician.He noticed that axillary freckles are present in about
20-30% of patients with neurofibromatosis, but he did not see any in patients who did not have
neurofibromatosis.
- Axillary freckling in NF-1
-Occurs at a later stage compared to the Café au lait lesions.
- Can also be found in the perenium.
skeletal
defects
- wheeling, circumscribed erythema and
localzed pruritis caused by scratching or
rubbing the lesions.
- Named after the French dermatologist :
Ferninand Jean Darier (1856-1938).
-Classically in mastocytosis. (which types ?)
--explanation ?
- Also seen in : insect bite, NF , JXG, acute
neonatal lymphoblastic leukemia.
- Transient piloerection and elevation or increased induration of
a lesion induced by rubbing.
- observed in congenital smooth muscle hamartomas.
What translocation is most likely present
within this lesion?
What are the histopathologic findings?
Identify the immunohistochemical pattern
classic to this diagnosis.
What is the recommended standard treatment
option with the highest cure rate without
recurrence?
What treatment is recommended for patients
with recurrent or metastatic lesions?
Complete surgical excision, including Mohs micrographic
surgery, is the standard treatment for DFSP
Imatinib mesylate is currently FDA-approved for adults
with unresectable, recurrent and/or metastatic DFSP (800
mg daily).
Darier-Roussy subcutaneous
sarcoidosis
- Central depression surrounded by an elevated skin
rim.
- Seen in scleromyxedema.
- On the extended proximal intelphalenges.
What stains would you use for a skin biopsy of
a scleromyxedema case?
● Symmetric confluent violacous erythema
over the knuckles, wrists, knees is called
Gottron’s sign
●Violaceous papules over MP joints
(Gottron’s papules)
What are the other features
of dermatomyositis?
Photodistributed
poikiloderma
involving the “V” of the
chest and the upper
back is often referred to
as the “shawl sign”
(Samitz sign)
cuticular dystrophy and nail fold
telangiectasias.
The cuticles are “ragged” and within the
proximal nail fold,
dilated capillary loops alternate with
vessel dropout
Heliotrope sign
(most characteristic)
Holster sign
CALCINOSIS CUTIS
• BLEOMYCIN treatment
•Adult -onset Still's
disease
• Shiitake mushroom
dermatitis.
.Juvenille DM vs adult DM – list 5 clinical differences
Juvenile Adult
Calcinosis cutis No calcinosis
No malignancy Malignancy (ovarian)
No sex predominance F:M = 2-7:1
10% overlap with other CTD 20% overlap with other CTD
More vasculitis Less vasculitis
what is in adults but not children ?
malignancy (12% vs 0%)
serology in adults not in children ?
anti-synthetase ab (Jo-1)
Two medications can induce DM
Statin – type lipid lowering agents.
Hydroxyurea
Two important clinical features differ lesion
of DM from LE
Violaceouse hue
Pruritus
- After the English surgeon George Grey Turner ( 1877 – 1951 ).
- Induration and brusing of skin over the costovertebral angel
secondary to the spread of blood from the anterior pararenal
space.
- Commonly with acute hemorrhagic pancereatitis.
- Can be with any other causes of retroperitoneal hemorrhage.
What other signs are seen in acute
hemorrhagic pancereatitis ?
- Cullen's sign :
hemorrhagic discoloration
of the umbilicus.
- Around 20% of lymphogranuloma venereum.
- Caused by a mass of femoral and inguinal nodes
separated by the femoral ligament.
LYMPHOGRANULOMA VENEREUM
Incubation
Period ??
3-21 days
Chlamydia
trachomatis L1, L2, or
L3
Ccc
histology??
Gamna-Favre bodies -
Basophilic inclusion bodies
located in the cytoplasm of
endothelial cells
Doxycycline 100 mg PO bid
for 21 d
Erythromycin base 500 mg
PO qid for 21 d
- A cutaneous sign of neural tube defect on the scalp.
- Coarse dark hair surrounding the underlying defect.
- Can be seen in : aplasia cutus congenita, encephalocele,
meningocele, ectopic brain tissue.
Aplasia Cutis Congenita (ACC )
• Onset before birth; localized defect in epidermis, dermis and/or
fat; variable appearance, typically along midline
• Presents with erosion, ulceration, scar, or membranous defect
(ovoid lesion covered by an epithelial membrane)
• Hair collar sign
IV
Group Main criterion Associations
1
Scalp: ACC without or with only isolated
anomalies
CLP, CMTC, PDA, tracheoesophageal fistula
2 Scalp ACC with limb reduction defect Adams-Oliver syndrome
3 Scalp ACC with associated epidermal nevi Sebaceous nevus , nevus verrucosus
4 ACC with underlying embryonic malformations
Encephalocele, meningocele, spina bifida,
omphalocele
5 ACC associated with fetus papyraceus
Scalp, chest, flanks, axillae and/ or extremities
Multiple, symmetric
Stellate/angulated configuration
6 ACC with epidermolysis bullosa
Lower extremities (“Bart syndrome”) or large areas of the trunk and
extremities
7 ACC in the extremities
Pretibial areas, extensor forearms, dorsal hands and feet
Radial dysplasia (associated with ACC on the extensor forearm)
8 ACC in conjunction with specific teratogens
Methimazole, Misoprostol, Low-molecular-
weight heparin ,Valproic acid
Maternal antiphospholipid syndrome
intrauterine HSV or VZV infection
9
ACC in conjunction with Malformations
- Loss of lateral third of eye brow.
-Seen in a list of diseses:
-AA, trichotelemania, leprosy, ectodermal dysplasia, syphilis, KP,
alopecia mucinosa, systemic sclerosis and hypothyroidism.
● Symmetric, prominent horizontal fold(s) (single or double) just
beneath the margin of the lower lid, originating at or near the inner
canthus and extending one-half to two-thirds the width of the lid
● Peri oral pallor due to
cheilitis ( inflammation of
the skin around the lips)
Periorbital darkening (“allergic
shiners”)
• Skin around the eyes appears
gray to violet–brown, while the
rest of the facial skin is rather
pale
• Periorbital edema and
lichenification may also be seen
● Refers to reticulate pigmentation of the neck
seen in patients with chronic atopic dermatitis.
● The condition was described in 1987 by two
different groups, Manabe and colleagues and
Colver and colleagues.
● The label ‘‘dirty neck’’ was given because of
the resemblance to the appearance of
unwashed skin, with the anterolateral aspects
of the neck typically affected.
● The pigmentary changes are secondary to
melanin incontinence
Can you remember the criteria to dx atopic
dermatitis ???
MAJOR FEATURES (3 OF 4 PRESENT)
• Pruritus
• Typical morphology and
distribution of skin lesions
• Chronic or chronically
relapsing dermatitis
• Personal or family history of
atopy
MINOR FEATURES (3 OF 23 PRESENT)
• Xerosis
• Ichthyosis/palmar hyperlinearity/keratosis pilaris
• Immediate (type I) skin test reactivity
• Elevated serum IgE
• Early age of onset
• Tendency toward cutaneous infections/impaired cell-
mediated immunity
• Tendency toward nonspecific hand or foot dermatitis
• Nipple eczema
• Cheilitis
• Recurrent conjunctivitis
• Dennie–Morgan infraorbital fold
• Keratoconus
• Anterior subcapsular cataract
• Orbital darkening
• Facial pallor/erythema
• Pityriasis alba
• Anterior neck folds
• Pruritus when sweating
• Intolerance to wool and lipid solvents
• Perifollicular accentuation
• Food intolerance
• Course influenced by environmental/emotional factors
• White dermographism/delayed blanch
● It is seen in
keratosis pilaris in
which individual
follicles show a
long strand of
keratin glinting
when examined in
tangentially
incident light
-Almost a certain sign of subungual malignant
melanoma.
The possibility of melanoma should be considered for
all pigmented nail bands in fair-skinned individuals,
especially if they are
● darkly pigmented,
● have irregular pigmentation and/or
● have a width ≥3 mm.
What other “ hutchinsosns” do you know ?
- In congenital syphilis:
Hutchinsons teeth
Eighth nerve palsy
Keratistis
● (V1; ~10–15% of zoster patients)
● Hutchinson’s sign refers to increased
risk of ocular involvement if skin
lesions are in the distribution of the
nasociliary branch, which supplies the
nasal tip, dorsum and root of the nose
and the medial canthus as well as the
cornea
● ~50% of patients have ocular
involvement, which can include
conjunctivitis, (epi)scleritis, keratitis,
uveitis, acute retinal necrosis and
optic neuritis
● May lead to ocular scarring and visual
loss
Herpes zoster Ophthalmic
division of the trigeminal nerve
Other “Hutchinsons
Hutchinson’s
summer
● Severe form common in Native Americans , often with cheilitis
and conjunctivitis
● Can occur in all races, including Caucasians
● Childhood onset; often resolution by adolescence, but can
persist indefinitely
● Intensely pruritic, crusted papules or nodules in sun-exposed
sites
● Strong association with HLA-DR4 (DRB1*0401) and subtype
DRB1*0407
● Management: photoprotection, NB-UVB, PUVA, thalidomide,
topical calcineurin inhibitors
●Deck-chair sign
● Classically described in Papulo-erythroderma of Ofuji, wherein
there is flat-topped red papules that become generalized
erythrodermic plaques without the involvement of abdominal skin
folds
- After the European surgeon Edmund Leser
( 1853 – 1916 ).
● Rapid appearance or growth of multiple seborrheic keratoses;
● Often these patients have acanthosis nigricans and generalized
pruritus.
● Adenocarcinomas are typically reported, particularly of the
stomach and colon but also of the breast, uterus, esophagus, and
pancreas
● Eruptive seborrheic keratoses can also develop in erythrodermic
patients who do not have an underlying malignancy
● Pseudo sign of Leser Trelat
(Patton et al J. Drugs
dermatology 3:565, 2OO4):
● Increase in size of seborrheic
keratosis which can be seen
after the use of cytarabene for
the the treatment of acute
myelogenous leukemia.
● Cytarabene causes inflammation
of existing seborrheic keratosises
1)Both conditions began simultaneously (neoplasia and paraneoplasia)
2) Development of a parallel course *
3) The skin lesion is not associated with a genetic syndrome
4) There is a specific type of neoplasia that occurs with paraneoplasia
5) The dermatosis is rare in the general population
6) There is a high frequency of association between both conditions
* Treatment of the neoplasia results in regression of the skin lesion;
recurrence of the neoplasia implies recurrence of the skin lesion.
Association with adenocarcinoma of the stomach
or other sites within the GI or GU tracts
How do you know its
MALIGNANT acanthosis
nigricans ?
•If it is :
• Sudden onset
• With constitutional symptoms and
weight loss
• Extensive and involves sites such
as the palms and soles and mucous
membaranes .
• With other paraneoplastic features
● Tripe palms :
● Ridged velvety lesions on the palms with
prominence of dermatoglyphics
● adenocarcinoma of the gastrointestinal tract.
● squamous cell carcinoma of the lung
Florid cutaneous papillomatosis
(gastric adenocarcinoma)
Dermatoglyphics are absent/hypoplastic in
what genodermatoses ?
• Dermatopathia pigmentosa reticularis
•Naegeli–Franceschetti–Jadassohn syndrome
•Profuse congenital familial milia with
absent dermatoglyphics (Basan's
Syndrome)
Paraneoplastic
Pemphigus (PNP)
• Associated with
•Non-Hodgkin’s
lymphoma (most
common),
•Chronic lymphocytic
leukemia,
•Castleman’s
disease,
•Thymoma ,
•Waldenström
macroglobulinemia ,
•Sarcoma
Benign
Autoantigen : various
desmosomal proteins
including
•desmoglein 3,
•periplakin,
•envoplakin,
• desmoplakin 1/2,
•BPAG1,
•plectin,
•170 kDa Ag,
•rarely desmoglein 1
Bazex syndrome (acrokeratosis
paraneoplastica)
•Acral psoriasiform plaques, typically
with involvement of the nose and
helices; often the lesions are
violaceous .
• Longitudinal and horizontal ridging of
the nails occurs in 75% of patients
•By definition, this condition is linked to
malignancy, generally occurring in the
upper aerodigestive tract (pharynx,
larynx or esophagus)
Erythema gyratum repens
Variable sites and types of malignancy
Glucagonoma syndrome
(Necrolytic migratory erythema),
•With angular cheilitis, glossitis
•Due to a glucagon-secreting tumor of the pancreas.
•Patients are often treated for intertrigo before the syndrome is
diagnosed.
•Weight loss and diabetes mellitus accompany the dermatosis
Don’t mix between
Necrolytic MIGRATORY erythema ( paraneoplastic )
with
Necrolytic ACRAL erythema ( associated with HCV and
zinc def )
Acquired hypertrichosis
lanuginosa
(malignant down)
•Growth of fine lanugo
hairs in a generalized
distribution or
•localized to the face.
With time, these hairs
may become
coarser
•Associated with a
variety of internal
malignancies, most
often carcinoma of the
lung, colon or breast
Acquired ichthyosis
Resembles ichthyosis vulgaris; most often
located on the legs
Lymphoma typically predates the diagnosis of
the ichthyosis
Sister Mary Joseph sign, refers to a palpable nodule
bulging into the umbilicus as a result of metastasis of a
malignant cancer in the pelvis or abdomen.
Gastrointestinal malignancies account for about half of
underlying sources (most commonly gastric
cancer, colonic cancer or pancreatic cancer,
Pitryasis rotunda
associated with HCC
(in type 1)
Sweet’s syndrome
● Associated with hematologic
malignancies (10–20% of
cases), in particular acute
myelogenous leukemia; solid
tumors, e.g. carcinoma of
the genitourinary tract,
breast and colon.
Trousseau syndrome
Migratory superficial thrombophlebitis’
with Pancreatic cancer
- Hyperpigmentation of the neck occurring in pellagra
patients.
What is deficient in pellagra ????????????
Niacin
- Blue sclera.
-Seen in multiple cases:
● Osteogenesis imperfecta,
● Ehler danlos syndrome,
● nevus of Ota,
● argyria and
● Alkaptinuria.
What are the other features of Alkaptinuria ??
It is a rare condition in which a person's urine turns a
dark brownish-black color when exposed to air
defect : Homogenestic acid oxidase deficiency.
- AKA : endogenous Ochranosis.
- AR.
● Osler sign,
● blue pigment in the skin,
● cartilage destruction ( ears),
● brown sweat,
● dark urine,
● arthropathy, intervertebral disc calcification.
Features that may present
early in life include :
brownish discoloration
of diapers (due to the
dark urine) and cerumen
that is more brown to
black in color than
normal
Slight rubbing of the skin results in exfoliation
of the outermost layer.
- Seen in :
●Autoimmune condition (pemphigus vulgaris).
●Bacterial infection ( scalded skin syndrome).
- Negative in BP.
● Seen in Steven Johnson syndrome, toxic epidermal
necrolysis and in some cases of burns and bullous
icthyosiform erythroderma.
● The underlying mechanism here is the necrosis of epidermal
cells and not acantholysis, as seen in true Nikolskiy's sign.
● It is elicited the same way as the true sign. However, unlike
Nikolskiy's sign, pseudo-Nikolskiy's sign is elicited only on
clinically involved or erythematous areas.
What is the name of the sign positive in BP?
- Named for Gustav Asboe-Hansen (1917–1989),
Danish physician.
- AKA "indirect Nikolsky sign" or "Nikolsky II sign“.
- Extension of a blister to adjacent unblistered skin
when pressure is put on the top of the bulla.
- The new blisters arises around the
old leasions forming linear
sequential lesions.
- Seen in CBDC.
•Target antigen:
•9 7 kDa Ag ( LAD-1 or LABD97 ):
cleaved ectodomain of
BPAG2
Treatment of choice ???
IgG staining IgA staining
linear deposition of IgA along the BMZ
Direct IF
(DIF) :
Detects in
vivo
antibodies
bound to
tissue
antigens in
perilesional
skin
Indirect IF (IIF)
Detects circulating
serum antibodies
(substrate
sections react with
serially diluted serum
from
patient → incubated
with anti-IgG or other
specific
fluorescent dye-
tagged antibody)
Best substrate :
monkey esophagus
(PV), guinea
pig esophagus (PF),
transitional rat
bladder
Salt-split-skin (SSS) Technique
● Variant of IIF allowing distinction between different subepidermal
blistering conditions with similar DIF findings;
● Normal human skin incubated in 1 M NaCl for 48–72 h resulting in
split at lamina lucida level; location of antibody binding to split (
epidermal or dermal side ) distinguishes different diseases
B- Bullous
pemphigoid
- A diagonal ear lobe crease extending diagonally
from the tragus across the lobule to the rear
edge of the auricle.
- It has been hypothesized that Frank's sign is
indicative of cardiovascular disease and/or
diabetes.
- have no predictive value in Native American
Indian and Asian patients.
● Sometimes used for scale crust of pemphigus foliaceous.
● Cornflake sign seen in Flegel's disease is characterized
by 2-3 mm keratotic scaly papules with discrete
irregular margins.
● The scale separates from many lesions, leaving a non-
exudative red base.
What other types of scale do you know?
.
Ichthyosiform scale
Describes large,
polygonal scales - as
in fish scales.
Example: Ichthyosis
vulgaris.
Thin adherent mica-like scale attached at the center of a lichenoid firm
reddish brown papule and free at the periphery. Example: Pityriasis
lichenoides chronica.
In clear cell acanthoma, wafer-like scale is seen
adherent at the periphery, which leaves a moist
or bleeding surface when removed.
Double-edged scale
Describes erythematous, exfoliating or scaly, annular or polycyclic,
flat patch with an incomplete advancing double edge of peeling
scale. Example: Ichthyosis linearis circumflexa
ICHTHYOSIS HYSTRIX
descriptive term for massive hyperkeratosis that has a verrucous surface or forms protruding, porcupine-like
spines;
Observed :
• In patients with EI (most common )
•Verrucous epidermolytic nevi
•Ichthyosis hystrix Curth-Macklin
• Hystri -like ichthyosis with deafness (HID) syndrome (mutations in the GJB2 gene)
Mauserung desquamation
Mutations:
keratin 2 (KRT2),
Plate-like scale (Armor
plate)
LAMELLAR ICHTHYOSIS
Transglutaminase-1 deficiency due to deleterious mutations
in both copies of the TGM1 gene
LAMELLAR ICHTHYOSIS
● This sign is diagnostic of Discoid
Lupus Erythematosus [DLE].
● Removal of an adherent scale from
a lesion of DLE reveals the
undersurface to be covered with
horny plugs [resembling carpet
tacks] overlying dilated hair
follicular openings
● This is pathognomic of Congenital Syphilis.
● An irregular thickening or enlargement of the
sternal end of clavicle, usually unilateral
V
● This is seen in Kwashiorkor and other diseases leading to
severe protein malnutrition like extensive bowel
resection, severe ulcerative colitis etc.
● Presence of alternating white and dark bands along
individual hair shafts SEEN BY NAKED EYES
● The bands are formed due to intermittent severe protein
malnutrition.
What else can cause this sign
???????
?
Tiger tail appearance : alternating
light and dark bands under
polarizing light
Seen in Trichothiodystrophy
(autosomal recessive disorder
characterized
by sulfur-deficient hair)
Actinic keratosis
●alternating areas
of orthokeratosis
and parakeratosis.
●It is the ability of patients of Ehlers-
Danlos syndrome to touch the tip of
the nose with the tip of their tongue.
● This sign is seen in Rubella and was originally
described by Forschheimer.
● The name is given to an enanthem [mucosal rash]
that is confined to the soft palate during the
prodromal period in Rubella
Friar-Tuck signFriar-Tuck sign
● This is seen in Trichotillomania.
● In severe forms of the disease, when the whole
scalp is involved the hair at the margins is
characteristically spared.
● The bites of bed bugs (Cimex
lectularius) usually follow a
linear pathway in a group of
three to five blood meals and
are often referred to as
“Breakfast, lunch, and
dinner” or “Breakfast, lunch,
and supper” sign
31+ 32
sign
Chagas–Mazza–Romaña's sign
● Chagas’ disease
(American
trypanosomiasis),
conjunctiva is the
portal of entry
for Trypanosoma
Cruzi.
● Unilateral swelling
of eyelids and orbit
after conjunctival
inoculation is called
as eye-sign or
Chagas–Mazza–
Romaña's sign or
Romaña's sign
● It is seen in early
stages of African
trypanosomiasis
caused
by Trypanosoma
brucei
rhodensiense and
Trypanosoma brucei
gambiense known
Sleeping sickness.
● Winterbottom's sign
is enlargement of
lymph nodes in the
posterior cervical
chain
Winterbottom's sign
● In 1877, Heinrich Koebner described the appearance of psoriatic lesions in the
uninvolved skin of psoriatic patients as a consequence of trauma.
● In general, the interval to koebnerization is between 10 and 20 days, but may be
as short as 3 days or as long as 2 years.
● The original classification of Koebner phenomenon is :
Koebner/Isomorphic phenomenon
● Reverse Koebner
phenomenon
This phenomenon is
explained, in which an area of
psoriasis clears after injury
● Remote reverse
Koebner phenomenon
It is defined in vitiligo
patients, in which
spontaneous
repigmentation is seen
in distant patches after
autologous skin graft
surgery.
Punch grafting on right leg (shown with blue arrow). Spontaneous
repigmentation over left leg (shown with yellow arrow)
● Pseudokoebner
phenomenon :
● Seen skin infections like
MC and warts
● Due to auto inoculation
Ugly duckling sign
● Refers to the observation that a nevus that does not resemble a patient’s
other nevi is more likely to represent a melanoma
A few other signs …..
Drip sign
● Found in dermatitis
artefacta produced by
corrosive liquids.
● Patterned burned
areas correspond to
the areas of dripping
of the liquid when
applied by the patient.
Enamel paint sign
● Seen in patients with kwashiorkor
● Sharply demarcated
hyperpigmented desquamating
patches and plaques resembling
enamel paint occur on the skin,
predominantly in areas of pressure
and irritation.
● The underlying skin is inflamed and
raw.
Hoagland’s sign
● is early and transient
bilateral upper lid edema
occurring in patients with
infectious mononucleosis.
● The sign is usually present
only for the first few days
of the clinical presentation
of the illness.
Foot prints in
snow'
appearance in
Pseudopelade
of Brocq
Asbestos-like
appearance Pityriasis
amiantacea
Cauliflower
appearance
Relapsing
polychondritis
Cauliflower appearance
condylomata acuminata
Cayenne pepper appearance
Schamberg disease
Cigarette paper like scars
The skin in patients with Ehler-Danlos syndrome is hyperextensible which
gives rise to gaping "fish-mouth wounds" over bony prominences like the
shins, knees, and elbows following minor trauma. Such wide, thin,
papyraceous scars over the knees and elbows are also called "cigarette
paper scars."
cigarette paper"-like wrinkled
appearance in parapsoriasis
Cliff drop appearance
Atrophoderma of Pasini and Pierini
Cobblestone appearance
In Darier's
disease
In crohn's disease
Cowden’s disease (AD)
In Heck's disease
What is Hecks
disease ?
Focal epithelial hyperplasia:
papules on buccal, gingival,
labial mucosa resembling flat
warts
What is the causative
agent ?
HPV 13, 32
Following punch grafting for vitiligo
Confetti-like appearance
Amyloidosis cutis dyschromica
(Primary cutaneous amyliodosis),
‫الورق‬ ‫قصاصات‬
‫الملونة‬
Dyschromatosis symmetrica
hereditaria (Acropigmentation
of Dohi)
The skin findings are
characterized by hypo- and
hyperpigmented macules on
the dorsal and ventral aspects
of the hands and feet, which
may extend to the proximal
portions of the limbs (knees
and elbows). Macules appear
in the first to second decade
and are typically non-
progressive. Similar lesions
(freckle-like macules) can be
found on the face.
Dyschromatosis universalis hereditaria
The disease begins in early childhood and is characterized by mottled pigmentation which
originates from the hands and can progress to involve the trunk, extremities and face. The lesions
are characterized by hyperpigmented macules admixed with hypopigmented lesions and can also
involve the palms, soles and oral mucosa.
Tuberous Sclerosis Complex
Vitiligo ponctué, an unusual
clinical presentation of
vitiligo, is characterized
by multiple small (confetti-
like), discrete amelanotic
macules,
sometimes superimposed
upon a hyperpigmented
macule
Purse string appearance of oral aperture in a case of systemic sclerosis
Figure of 8/hour glass appearance of vulva in lichen
sclerosus
Glazed appearance
Juvenile plantar dermatosis
‫مصقول‬
Inverted wine bottle
appearance in
lipodermatosclerosis
A child with diffuse
cutaneous
mastocytosis giving a
leather grain
appearance to the
skin
Nutmeg grater appearance
Pityriasis rubra pilaris (PRP)
Pebbly appearance
Hunter syndrome
mucopolysaccharidosis II
(MPS II), is a lysosomal
storage disease caused by a
deficient (or absent) enzyme,
iduronate-2-sulfatase (I2S).
The accumulated substrates
in Hunter syndrome are
heparan sulfate and dermatan
sulfate. The syndrome has
X-linked recessive
inheritance.
Pseudoxanthom a
elasticum
showing the
characteristic
'plucked chicken'
appearance
Rippled appearance
Macular amyloidosis
Salt and pepper'
appearance
on upper back in a
female with
systemic sclerosis
Sandpaper appearanc
trachyonychia
Speckled appearance
Speckled lentiginous nevus
Tapir nose appearance
Mucocutaneous leishmaniasis caused by Leishmania braziliensis and L.
mexicana is termed as "espundia," which is characterized by mutilation of the
nasal apparatus. It spares the nasal bones. When the septum is destroyed, the
nasal bridge and tip of the nose collapse, giving the appearance of a parrot beak,
camel nose, or tapir nose appearance
Vermiculate
atrophoderma in a
young patient with
'worm-eaten' picture
Zebra-like appearance
Linear and whorled nevoid hypermelanosis
Thank you

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Signs in dermatology.pptx

  • 2.
  • 3.
  • 4. What is a Sign…? Objective physical finding found by the examiner. Two types: 1- De novo 2- Physician induced
  • 5.
  • 6.
  • 7.
  • 8.
  • 9. ● If the superficial silvery white scales are removed via curettage a characteristic coherence is observed, as if one has scratched on a wax candle (“signe de la tache de bougie” Candle sign). ● Subsequently, a surface membrane is seen, The lesion remains dry until this last level is reached (sign of the last Häutchen) which will also come off as a whole. ● If the latter is removed, then a wet surface is seen with characteristic pinpoint bleeding. This finding,is called Auspitz sign.
  • 10. Auspitz sign, is the clinical reflection of elongated vessels in the dermal papillae together with thinning of the suprapapillary epidermis
  • 11.
  • 12.
  • 13.
  • 15. - Knuckle dimple sign. - Due to short 4th and 5th metacarpals. - Enhanced by making a fist. - Described by Fuller Albright . - Found in Albright hereditary osteodysrtrophy.
  • 16. - Short stature. - Obesity. - Round face. - Mental retardation. - Brachydactaly - Soft tissue ossification. - Short 4th and 5th metacarpals. - Pseudohypoparathyroidism (PHP): low calcium high phosphate high PTH non responding kidneys •Some Share them the habitus of AHO but are not resistant to PTH. The latter group are said to have pseudo-PHP.
  • 17.
  • 18. McCune Albright Syndrome Albright Hereditary Osteodystrophy AD XLD Inheritance Polyostotic fibrous dysplasia osteodystrophy Bony lesions Large café –au-lait macule (cost of Maine) with ipsilateral bony changes. Precocious puberty. Short stature. Obesity. Round face. Mental retardation. Brachydactaly Soft tissue calcification. Short 4th and 5th metacarpals. Pseudohypoparathyroidism Other symptoms
  • 20.
  • 21.
  • 22. - -Named after an English surgeon William Henry Battle. -Indicates fracture base of skull at the posterior fossa. - Blood dissects through fascia and collects at mastoid process.
  • 23.
  • 24.
  • 25. - The ability to invaginate the tumor/lesion into the dermal defect with digital pressure. - Found in neurofibromas in NF1. - others: - Anetoderma - Old pigmented nevi - syphilitic chancre
  • 26.
  • 27.
  • 28. ● AKA Fitzpatrick’s sign, seen in dermatofibromas. ● Lateral compression with the thumb and index finger leads to depression of the lesion. ● This dimpling effect is secondary to the lesion being attached to the subcutaneous fat
  • 29.
  • 31. ● Named after Frank W. Crowe, an American physician.He noticed that axillary freckles are present in about 20-30% of patients with neurofibromatosis, but he did not see any in patients who did not have neurofibromatosis. - Axillary freckling in NF-1 -Occurs at a later stage compared to the Café au lait lesions. - Can also be found in the perenium.
  • 32.
  • 33.
  • 34.
  • 35.
  • 37.
  • 38.
  • 39. - wheeling, circumscribed erythema and localzed pruritis caused by scratching or rubbing the lesions. - Named after the French dermatologist : Ferninand Jean Darier (1856-1938). -Classically in mastocytosis. (which types ?) --explanation ? - Also seen in : insect bite, NF , JXG, acute neonatal lymphoblastic leukemia.
  • 40.
  • 41.
  • 42. - Transient piloerection and elevation or increased induration of a lesion induced by rubbing. - observed in congenital smooth muscle hamartomas.
  • 43.
  • 44.
  • 45. What translocation is most likely present within this lesion? What are the histopathologic findings? Identify the immunohistochemical pattern classic to this diagnosis. What is the recommended standard treatment option with the highest cure rate without recurrence? What treatment is recommended for patients with recurrent or metastatic lesions? Complete surgical excision, including Mohs micrographic surgery, is the standard treatment for DFSP Imatinib mesylate is currently FDA-approved for adults with unresectable, recurrent and/or metastatic DFSP (800 mg daily).
  • 47.
  • 48.
  • 49.
  • 50. - Central depression surrounded by an elevated skin rim. - Seen in scleromyxedema. - On the extended proximal intelphalenges.
  • 51. What stains would you use for a skin biopsy of a scleromyxedema case?
  • 52.
  • 53.
  • 54. ● Symmetric confluent violacous erythema over the knuckles, wrists, knees is called Gottron’s sign ●Violaceous papules over MP joints (Gottron’s papules)
  • 55. What are the other features of dermatomyositis?
  • 56. Photodistributed poikiloderma involving the “V” of the chest and the upper back is often referred to as the “shawl sign” (Samitz sign) cuticular dystrophy and nail fold telangiectasias. The cuticles are “ragged” and within the proximal nail fold, dilated capillary loops alternate with vessel dropout Heliotrope sign (most characteristic)
  • 57.
  • 60. • BLEOMYCIN treatment •Adult -onset Still's disease • Shiitake mushroom dermatitis.
  • 61.
  • 62. .Juvenille DM vs adult DM – list 5 clinical differences Juvenile Adult Calcinosis cutis No calcinosis No malignancy Malignancy (ovarian) No sex predominance F:M = 2-7:1 10% overlap with other CTD 20% overlap with other CTD More vasculitis Less vasculitis what is in adults but not children ? malignancy (12% vs 0%) serology in adults not in children ? anti-synthetase ab (Jo-1)
  • 63. Two medications can induce DM Statin – type lipid lowering agents. Hydroxyurea Two important clinical features differ lesion of DM from LE Violaceouse hue Pruritus
  • 64.
  • 65.
  • 66. - After the English surgeon George Grey Turner ( 1877 – 1951 ). - Induration and brusing of skin over the costovertebral angel secondary to the spread of blood from the anterior pararenal space. - Commonly with acute hemorrhagic pancereatitis. - Can be with any other causes of retroperitoneal hemorrhage.
  • 67. What other signs are seen in acute hemorrhagic pancereatitis ?
  • 68. - Cullen's sign : hemorrhagic discoloration of the umbilicus.
  • 69.
  • 70.
  • 71. - Around 20% of lymphogranuloma venereum. - Caused by a mass of femoral and inguinal nodes separated by the femoral ligament.
  • 72. LYMPHOGRANULOMA VENEREUM Incubation Period ?? 3-21 days Chlamydia trachomatis L1, L2, or L3 Ccc histology?? Gamna-Favre bodies - Basophilic inclusion bodies located in the cytoplasm of endothelial cells Doxycycline 100 mg PO bid for 21 d Erythromycin base 500 mg PO qid for 21 d
  • 73.
  • 74.
  • 75. - A cutaneous sign of neural tube defect on the scalp. - Coarse dark hair surrounding the underlying defect. - Can be seen in : aplasia cutus congenita, encephalocele, meningocele, ectopic brain tissue.
  • 76. Aplasia Cutis Congenita (ACC ) • Onset before birth; localized defect in epidermis, dermis and/or fat; variable appearance, typically along midline • Presents with erosion, ulceration, scar, or membranous defect (ovoid lesion covered by an epithelial membrane) • Hair collar sign
  • 77. IV
  • 78. Group Main criterion Associations 1 Scalp: ACC without or with only isolated anomalies CLP, CMTC, PDA, tracheoesophageal fistula 2 Scalp ACC with limb reduction defect Adams-Oliver syndrome 3 Scalp ACC with associated epidermal nevi Sebaceous nevus , nevus verrucosus 4 ACC with underlying embryonic malformations Encephalocele, meningocele, spina bifida, omphalocele 5 ACC associated with fetus papyraceus Scalp, chest, flanks, axillae and/ or extremities Multiple, symmetric Stellate/angulated configuration 6 ACC with epidermolysis bullosa Lower extremities (“Bart syndrome”) or large areas of the trunk and extremities 7 ACC in the extremities Pretibial areas, extensor forearms, dorsal hands and feet Radial dysplasia (associated with ACC on the extensor forearm) 8 ACC in conjunction with specific teratogens Methimazole, Misoprostol, Low-molecular- weight heparin ,Valproic acid Maternal antiphospholipid syndrome intrauterine HSV or VZV infection 9 ACC in conjunction with Malformations
  • 79.
  • 80.
  • 81.
  • 82.
  • 83. - Loss of lateral third of eye brow. -Seen in a list of diseses: -AA, trichotelemania, leprosy, ectodermal dysplasia, syphilis, KP, alopecia mucinosa, systemic sclerosis and hypothyroidism.
  • 84. ● Symmetric, prominent horizontal fold(s) (single or double) just beneath the margin of the lower lid, originating at or near the inner canthus and extending one-half to two-thirds the width of the lid
  • 85. ● Peri oral pallor due to cheilitis ( inflammation of the skin around the lips) Periorbital darkening (“allergic shiners”) • Skin around the eyes appears gray to violet–brown, while the rest of the facial skin is rather pale • Periorbital edema and lichenification may also be seen
  • 86. ● Refers to reticulate pigmentation of the neck seen in patients with chronic atopic dermatitis. ● The condition was described in 1987 by two different groups, Manabe and colleagues and Colver and colleagues. ● The label ‘‘dirty neck’’ was given because of the resemblance to the appearance of unwashed skin, with the anterolateral aspects of the neck typically affected. ● The pigmentary changes are secondary to melanin incontinence
  • 87. Can you remember the criteria to dx atopic dermatitis ???
  • 88. MAJOR FEATURES (3 OF 4 PRESENT) • Pruritus • Typical morphology and distribution of skin lesions • Chronic or chronically relapsing dermatitis • Personal or family history of atopy MINOR FEATURES (3 OF 23 PRESENT) • Xerosis • Ichthyosis/palmar hyperlinearity/keratosis pilaris • Immediate (type I) skin test reactivity • Elevated serum IgE • Early age of onset • Tendency toward cutaneous infections/impaired cell- mediated immunity • Tendency toward nonspecific hand or foot dermatitis • Nipple eczema • Cheilitis • Recurrent conjunctivitis • Dennie–Morgan infraorbital fold • Keratoconus • Anterior subcapsular cataract • Orbital darkening • Facial pallor/erythema • Pityriasis alba • Anterior neck folds • Pruritus when sweating • Intolerance to wool and lipid solvents • Perifollicular accentuation • Food intolerance • Course influenced by environmental/emotional factors • White dermographism/delayed blanch
  • 89.
  • 90. ● It is seen in keratosis pilaris in which individual follicles show a long strand of keratin glinting when examined in tangentially incident light
  • 91.
  • 92.
  • 93.
  • 94.
  • 95. -Almost a certain sign of subungual malignant melanoma. The possibility of melanoma should be considered for all pigmented nail bands in fair-skinned individuals, especially if they are ● darkly pigmented, ● have irregular pigmentation and/or ● have a width ≥3 mm.
  • 96. What other “ hutchinsosns” do you know ?
  • 97. - In congenital syphilis: Hutchinsons teeth Eighth nerve palsy Keratistis
  • 98. ● (V1; ~10–15% of zoster patients) ● Hutchinson’s sign refers to increased risk of ocular involvement if skin lesions are in the distribution of the nasociliary branch, which supplies the nasal tip, dorsum and root of the nose and the medial canthus as well as the cornea ● ~50% of patients have ocular involvement, which can include conjunctivitis, (epi)scleritis, keratitis, uveitis, acute retinal necrosis and optic neuritis ● May lead to ocular scarring and visual loss Herpes zoster Ophthalmic division of the trigeminal nerve
  • 99.
  • 101.
  • 103. ● Severe form common in Native Americans , often with cheilitis and conjunctivitis ● Can occur in all races, including Caucasians ● Childhood onset; often resolution by adolescence, but can persist indefinitely ● Intensely pruritic, crusted papules or nodules in sun-exposed sites ● Strong association with HLA-DR4 (DRB1*0401) and subtype DRB1*0407 ● Management: photoprotection, NB-UVB, PUVA, thalidomide, topical calcineurin inhibitors
  • 104.
  • 105.
  • 106. ●Deck-chair sign ● Classically described in Papulo-erythroderma of Ofuji, wherein there is flat-topped red papules that become generalized erythrodermic plaques without the involvement of abdominal skin folds
  • 107.
  • 108.
  • 109. - After the European surgeon Edmund Leser ( 1853 – 1916 ). ● Rapid appearance or growth of multiple seborrheic keratoses; ● Often these patients have acanthosis nigricans and generalized pruritus. ● Adenocarcinomas are typically reported, particularly of the stomach and colon but also of the breast, uterus, esophagus, and pancreas ● Eruptive seborrheic keratoses can also develop in erythrodermic patients who do not have an underlying malignancy
  • 110.
  • 111. ● Pseudo sign of Leser Trelat (Patton et al J. Drugs dermatology 3:565, 2OO4): ● Increase in size of seborrheic keratosis which can be seen after the use of cytarabene for the the treatment of acute myelogenous leukemia. ● Cytarabene causes inflammation of existing seborrheic keratosises
  • 112.
  • 113. 1)Both conditions began simultaneously (neoplasia and paraneoplasia) 2) Development of a parallel course * 3) The skin lesion is not associated with a genetic syndrome 4) There is a specific type of neoplasia that occurs with paraneoplasia 5) The dermatosis is rare in the general population 6) There is a high frequency of association between both conditions * Treatment of the neoplasia results in regression of the skin lesion; recurrence of the neoplasia implies recurrence of the skin lesion.
  • 114.
  • 115. Association with adenocarcinoma of the stomach or other sites within the GI or GU tracts How do you know its MALIGNANT acanthosis nigricans ? •If it is : • Sudden onset • With constitutional symptoms and weight loss • Extensive and involves sites such as the palms and soles and mucous membaranes . • With other paraneoplastic features
  • 116. ● Tripe palms : ● Ridged velvety lesions on the palms with prominence of dermatoglyphics ● adenocarcinoma of the gastrointestinal tract. ● squamous cell carcinoma of the lung Florid cutaneous papillomatosis (gastric adenocarcinoma) Dermatoglyphics are absent/hypoplastic in what genodermatoses ? • Dermatopathia pigmentosa reticularis •Naegeli–Franceschetti–Jadassohn syndrome •Profuse congenital familial milia with absent dermatoglyphics (Basan's Syndrome)
  • 117. Paraneoplastic Pemphigus (PNP) • Associated with •Non-Hodgkin’s lymphoma (most common), •Chronic lymphocytic leukemia, •Castleman’s disease, •Thymoma , •Waldenström macroglobulinemia , •Sarcoma Benign Autoantigen : various desmosomal proteins including •desmoglein 3, •periplakin, •envoplakin, • desmoplakin 1/2, •BPAG1, •plectin, •170 kDa Ag, •rarely desmoglein 1
  • 118.
  • 119. Bazex syndrome (acrokeratosis paraneoplastica) •Acral psoriasiform plaques, typically with involvement of the nose and helices; often the lesions are violaceous . • Longitudinal and horizontal ridging of the nails occurs in 75% of patients •By definition, this condition is linked to malignancy, generally occurring in the upper aerodigestive tract (pharynx, larynx or esophagus)
  • 120. Erythema gyratum repens Variable sites and types of malignancy
  • 121. Glucagonoma syndrome (Necrolytic migratory erythema), •With angular cheilitis, glossitis •Due to a glucagon-secreting tumor of the pancreas. •Patients are often treated for intertrigo before the syndrome is diagnosed. •Weight loss and diabetes mellitus accompany the dermatosis
  • 122. Don’t mix between Necrolytic MIGRATORY erythema ( paraneoplastic ) with Necrolytic ACRAL erythema ( associated with HCV and zinc def )
  • 123. Acquired hypertrichosis lanuginosa (malignant down) •Growth of fine lanugo hairs in a generalized distribution or •localized to the face. With time, these hairs may become coarser •Associated with a variety of internal malignancies, most often carcinoma of the lung, colon or breast
  • 124. Acquired ichthyosis Resembles ichthyosis vulgaris; most often located on the legs Lymphoma typically predates the diagnosis of the ichthyosis
  • 125. Sister Mary Joseph sign, refers to a palpable nodule bulging into the umbilicus as a result of metastasis of a malignant cancer in the pelvis or abdomen. Gastrointestinal malignancies account for about half of underlying sources (most commonly gastric cancer, colonic cancer or pancreatic cancer,
  • 127. Sweet’s syndrome ● Associated with hematologic malignancies (10–20% of cases), in particular acute myelogenous leukemia; solid tumors, e.g. carcinoma of the genitourinary tract, breast and colon.
  • 128. Trousseau syndrome Migratory superficial thrombophlebitis’ with Pancreatic cancer
  • 129.
  • 130.
  • 131.
  • 132. - Hyperpigmentation of the neck occurring in pellagra patients. What is deficient in pellagra ???????????? Niacin
  • 133.
  • 134.
  • 135. - Blue sclera. -Seen in multiple cases: ● Osteogenesis imperfecta, ● Ehler danlos syndrome, ● nevus of Ota, ● argyria and ● Alkaptinuria.
  • 136. What are the other features of Alkaptinuria ??
  • 137. It is a rare condition in which a person's urine turns a dark brownish-black color when exposed to air defect : Homogenestic acid oxidase deficiency. - AKA : endogenous Ochranosis. - AR. ● Osler sign, ● blue pigment in the skin, ● cartilage destruction ( ears), ● brown sweat, ● dark urine, ● arthropathy, intervertebral disc calcification.
  • 138. Features that may present early in life include : brownish discoloration of diapers (due to the dark urine) and cerumen that is more brown to black in color than normal
  • 139.
  • 140.
  • 141. Slight rubbing of the skin results in exfoliation of the outermost layer. - Seen in : ●Autoimmune condition (pemphigus vulgaris). ●Bacterial infection ( scalded skin syndrome). - Negative in BP.
  • 142.
  • 143. ● Seen in Steven Johnson syndrome, toxic epidermal necrolysis and in some cases of burns and bullous icthyosiform erythroderma. ● The underlying mechanism here is the necrosis of epidermal cells and not acantholysis, as seen in true Nikolskiy's sign. ● It is elicited the same way as the true sign. However, unlike Nikolskiy's sign, pseudo-Nikolskiy's sign is elicited only on clinically involved or erythematous areas.
  • 144. What is the name of the sign positive in BP?
  • 145. - Named for Gustav Asboe-Hansen (1917–1989), Danish physician. - AKA "indirect Nikolsky sign" or "Nikolsky II sign“. - Extension of a blister to adjacent unblistered skin when pressure is put on the top of the bulla.
  • 146.
  • 147.
  • 148.
  • 149. - The new blisters arises around the old leasions forming linear sequential lesions. - Seen in CBDC. •Target antigen: •9 7 kDa Ag ( LAD-1 or LABD97 ): cleaved ectodomain of BPAG2 Treatment of choice ???
  • 150. IgG staining IgA staining linear deposition of IgA along the BMZ
  • 151.
  • 152.
  • 153. Direct IF (DIF) : Detects in vivo antibodies bound to tissue antigens in perilesional skin
  • 154. Indirect IF (IIF) Detects circulating serum antibodies (substrate sections react with serially diluted serum from patient → incubated with anti-IgG or other specific fluorescent dye- tagged antibody) Best substrate : monkey esophagus (PV), guinea pig esophagus (PF), transitional rat bladder
  • 155. Salt-split-skin (SSS) Technique ● Variant of IIF allowing distinction between different subepidermal blistering conditions with similar DIF findings; ● Normal human skin incubated in 1 M NaCl for 48–72 h resulting in split at lamina lucida level; location of antibody binding to split ( epidermal or dermal side ) distinguishes different diseases
  • 157.
  • 158.
  • 159.
  • 160.
  • 161. - A diagonal ear lobe crease extending diagonally from the tragus across the lobule to the rear edge of the auricle. - It has been hypothesized that Frank's sign is indicative of cardiovascular disease and/or diabetes. - have no predictive value in Native American Indian and Asian patients.
  • 162.
  • 163.
  • 164. ● Sometimes used for scale crust of pemphigus foliaceous. ● Cornflake sign seen in Flegel's disease is characterized by 2-3 mm keratotic scaly papules with discrete irregular margins. ● The scale separates from many lesions, leaving a non- exudative red base.
  • 165. What other types of scale do you know?
  • 166. .
  • 167. Ichthyosiform scale Describes large, polygonal scales - as in fish scales. Example: Ichthyosis vulgaris.
  • 168.
  • 169. Thin adherent mica-like scale attached at the center of a lichenoid firm reddish brown papule and free at the periphery. Example: Pityriasis lichenoides chronica.
  • 170. In clear cell acanthoma, wafer-like scale is seen adherent at the periphery, which leaves a moist or bleeding surface when removed.
  • 171. Double-edged scale Describes erythematous, exfoliating or scaly, annular or polycyclic, flat patch with an incomplete advancing double edge of peeling scale. Example: Ichthyosis linearis circumflexa
  • 172. ICHTHYOSIS HYSTRIX descriptive term for massive hyperkeratosis that has a verrucous surface or forms protruding, porcupine-like spines; Observed : • In patients with EI (most common ) •Verrucous epidermolytic nevi •Ichthyosis hystrix Curth-Macklin • Hystri -like ichthyosis with deafness (HID) syndrome (mutations in the GJB2 gene)
  • 174. Plate-like scale (Armor plate) LAMELLAR ICHTHYOSIS Transglutaminase-1 deficiency due to deleterious mutations in both copies of the TGM1 gene LAMELLAR ICHTHYOSIS
  • 175.
  • 176.
  • 177.
  • 178. ● This sign is diagnostic of Discoid Lupus Erythematosus [DLE]. ● Removal of an adherent scale from a lesion of DLE reveals the undersurface to be covered with horny plugs [resembling carpet tacks] overlying dilated hair follicular openings
  • 179.
  • 180.
  • 181.
  • 182.
  • 183. ● This is pathognomic of Congenital Syphilis. ● An irregular thickening or enlargement of the sternal end of clavicle, usually unilateral
  • 184.
  • 185.
  • 186. V
  • 187.
  • 188.
  • 189. ● This is seen in Kwashiorkor and other diseases leading to severe protein malnutrition like extensive bowel resection, severe ulcerative colitis etc. ● Presence of alternating white and dark bands along individual hair shafts SEEN BY NAKED EYES ● The bands are formed due to intermittent severe protein malnutrition.
  • 190. What else can cause this sign ???????
  • 191. ? Tiger tail appearance : alternating light and dark bands under polarizing light Seen in Trichothiodystrophy (autosomal recessive disorder characterized by sulfur-deficient hair)
  • 192.
  • 193. Actinic keratosis ●alternating areas of orthokeratosis and parakeratosis.
  • 194.
  • 195.
  • 196. ●It is the ability of patients of Ehlers- Danlos syndrome to touch the tip of the nose with the tip of their tongue.
  • 197.
  • 198.
  • 199. ● This sign is seen in Rubella and was originally described by Forschheimer. ● The name is given to an enanthem [mucosal rash] that is confined to the soft palate during the prodromal period in Rubella
  • 200.
  • 201.
  • 202.
  • 204. ● This is seen in Trichotillomania. ● In severe forms of the disease, when the whole scalp is involved the hair at the margins is characteristically spared.
  • 205.
  • 206.
  • 207. ● The bites of bed bugs (Cimex lectularius) usually follow a linear pathway in a group of three to five blood meals and are often referred to as “Breakfast, lunch, and dinner” or “Breakfast, lunch, and supper” sign
  • 208. 31+ 32
  • 209. sign
  • 210. Chagas–Mazza–Romaña's sign ● Chagas’ disease (American trypanosomiasis), conjunctiva is the portal of entry for Trypanosoma Cruzi. ● Unilateral swelling of eyelids and orbit after conjunctival inoculation is called as eye-sign or Chagas–Mazza– Romaña's sign or Romaña's sign ● It is seen in early stages of African trypanosomiasis caused by Trypanosoma brucei rhodensiense and Trypanosoma brucei gambiense known Sleeping sickness. ● Winterbottom's sign is enlargement of lymph nodes in the posterior cervical chain Winterbottom's sign
  • 211.
  • 212.
  • 213.
  • 214.
  • 215. ● In 1877, Heinrich Koebner described the appearance of psoriatic lesions in the uninvolved skin of psoriatic patients as a consequence of trauma. ● In general, the interval to koebnerization is between 10 and 20 days, but may be as short as 3 days or as long as 2 years. ● The original classification of Koebner phenomenon is : Koebner/Isomorphic phenomenon
  • 216. ● Reverse Koebner phenomenon This phenomenon is explained, in which an area of psoriasis clears after injury
  • 217. ● Remote reverse Koebner phenomenon It is defined in vitiligo patients, in which spontaneous repigmentation is seen in distant patches after autologous skin graft surgery. Punch grafting on right leg (shown with blue arrow). Spontaneous repigmentation over left leg (shown with yellow arrow)
  • 218. ● Pseudokoebner phenomenon : ● Seen skin infections like MC and warts ● Due to auto inoculation
  • 219.
  • 220.
  • 221. Ugly duckling sign ● Refers to the observation that a nevus that does not resemble a patient’s other nevi is more likely to represent a melanoma
  • 222.
  • 223. A few other signs …..
  • 224. Drip sign ● Found in dermatitis artefacta produced by corrosive liquids. ● Patterned burned areas correspond to the areas of dripping of the liquid when applied by the patient.
  • 225. Enamel paint sign ● Seen in patients with kwashiorkor ● Sharply demarcated hyperpigmented desquamating patches and plaques resembling enamel paint occur on the skin, predominantly in areas of pressure and irritation. ● The underlying skin is inflamed and raw.
  • 226. Hoagland’s sign ● is early and transient bilateral upper lid edema occurring in patients with infectious mononucleosis. ● The sign is usually present only for the first few days of the clinical presentation of the illness.
  • 227.
  • 228. Foot prints in snow' appearance in Pseudopelade of Brocq
  • 232. Cigarette paper like scars The skin in patients with Ehler-Danlos syndrome is hyperextensible which gives rise to gaping "fish-mouth wounds" over bony prominences like the shins, knees, and elbows following minor trauma. Such wide, thin, papyraceous scars over the knees and elbows are also called "cigarette paper scars."
  • 234. Cliff drop appearance Atrophoderma of Pasini and Pierini
  • 238. In Heck's disease What is Hecks disease ? Focal epithelial hyperplasia: papules on buccal, gingival, labial mucosa resembling flat warts What is the causative agent ? HPV 13, 32
  • 239. Following punch grafting for vitiligo
  • 240. Confetti-like appearance Amyloidosis cutis dyschromica (Primary cutaneous amyliodosis), ‫الورق‬ ‫قصاصات‬ ‫الملونة‬
  • 241. Dyschromatosis symmetrica hereditaria (Acropigmentation of Dohi) The skin findings are characterized by hypo- and hyperpigmented macules on the dorsal and ventral aspects of the hands and feet, which may extend to the proximal portions of the limbs (knees and elbows). Macules appear in the first to second decade and are typically non- progressive. Similar lesions (freckle-like macules) can be found on the face.
  • 242. Dyschromatosis universalis hereditaria The disease begins in early childhood and is characterized by mottled pigmentation which originates from the hands and can progress to involve the trunk, extremities and face. The lesions are characterized by hyperpigmented macules admixed with hypopigmented lesions and can also involve the palms, soles and oral mucosa.
  • 244. Vitiligo ponctué, an unusual clinical presentation of vitiligo, is characterized by multiple small (confetti- like), discrete amelanotic macules, sometimes superimposed upon a hyperpigmented macule
  • 245. Purse string appearance of oral aperture in a case of systemic sclerosis
  • 246. Figure of 8/hour glass appearance of vulva in lichen sclerosus
  • 247. Glazed appearance Juvenile plantar dermatosis ‫مصقول‬
  • 248. Inverted wine bottle appearance in lipodermatosclerosis
  • 249. A child with diffuse cutaneous mastocytosis giving a leather grain appearance to the skin
  • 250. Nutmeg grater appearance Pityriasis rubra pilaris (PRP)
  • 251. Pebbly appearance Hunter syndrome mucopolysaccharidosis II (MPS II), is a lysosomal storage disease caused by a deficient (or absent) enzyme, iduronate-2-sulfatase (I2S). The accumulated substrates in Hunter syndrome are heparan sulfate and dermatan sulfate. The syndrome has X-linked recessive inheritance.
  • 254. Salt and pepper' appearance on upper back in a female with systemic sclerosis
  • 257. Tapir nose appearance Mucocutaneous leishmaniasis caused by Leishmania braziliensis and L. mexicana is termed as "espundia," which is characterized by mutilation of the nasal apparatus. It spares the nasal bones. When the septum is destroyed, the nasal bridge and tip of the nose collapse, giving the appearance of a parrot beak, camel nose, or tapir nose appearance
  • 258. Vermiculate atrophoderma in a young patient with 'worm-eaten' picture
  • 259. Zebra-like appearance Linear and whorled nevoid hypermelanosis
  • 260.