What is a Sign…?
Objective physical finding found by the
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”
● 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
Auspitz sign, is
vessels in the
thinning of the
- Knuckle dimple sign.
- Due to short 4th and 5th metacarpals.
- Enhanced by making a fist.
- Described by Fuller Albright .
- Found in Albright hereditary
- Short stature.
- Round face.
- Mental retardation.
- Soft tissue ossification.
- Short 4th and 5th metacarpals.
- Pseudohypoparathyroidism (PHP):
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.
Short 4th and 5th
● 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
● 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
- Axillary freckling in NF-1
-Occurs at a later stage compared to the Café au lait lesions.
- Can also be found in the perenium.
- 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 ?)
- 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
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
- Central depression surrounded by an elevated skin
- 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
●Violaceous papules over MP joints
What are the other features
involving the “V” of the
chest and the upper
back is often referred to
as the “shawl sign”
cuticular dystrophy and nail fold
The cuticles are “ragged” and within the
proximal nail fold,
dilated capillary loops alternate with
.Juvenille DM vs adult DM – list 5 clinical differences
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.
Two important clinical features differ lesion
of DM from LE
- 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
- 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 :
of the umbilicus.
- Around 20% of lymphogranuloma venereum.
- Caused by a mass of femoral and inguinal nodes
separated by the femoral ligament.
trachomatis L1, L2, or
Gamna-Favre bodies -
Basophilic inclusion bodies
located in the cytoplasm of
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
Group Main criterion Associations
Scalp: ACC without or with only isolated
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,
5 ACC associated with fetus papyraceus
Scalp, chest, flanks, axillae and/ or extremities
6 ACC with epidermolysis bullosa
Lower extremities (“Bart syndrome”) or large areas of the trunk and
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
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
• Skin around the eyes appears
gray to violet–brown, while the
rest of the facial skin is rather
• 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
Can you remember the criteria to dx atopic
MAJOR FEATURES (3 OF 4 PRESENT)
• Typical morphology and
distribution of skin lesions
• Chronic or chronically
• Personal or family history of
MINOR FEATURES (3 OF 23 PRESENT)
• Ichthyosis/palmar hyperlinearity/keratosis pilaris
• Immediate (type I) skin test reactivity
• Elevated serum IgE
• Early age of onset
• Tendency toward cutaneous infections/impaired cell-
• Tendency toward nonspecific hand or foot dermatitis
• Nipple eczema
• Recurrent conjunctivitis
• Dennie–Morgan infraorbital fold
• 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
follicles show a
long strand of
when examined in
-Almost a certain sign of subungual malignant
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.
● (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
● ~50% of patients have ocular
involvement, which can include
conjunctivitis, (epi)scleritis, keratitis,
uveitis, acute retinal necrosis and
● May lead to ocular scarring and visual
Herpes zoster Ophthalmic
division of the trigeminal nerve
● Severe form common in Native Americans , often with cheilitis
● Can occur in all races, including Caucasians
● Childhood onset; often resolution by adolescence, but can
● Intensely pruritic, crusted papules or nodules in sun-exposed
● Strong association with HLA-DR4 (DRB1*0401) and subtype
● Management: photoprotection, NB-UVB, PUVA, thalidomide,
topical calcineurin inhibitors
● 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
- After the European surgeon Edmund Leser
( 1853 – 1916 ).
● Rapid appearance or growth of multiple seborrheic keratoses;
● Often these patients have acanthosis nigricans and generalized
● Adenocarcinomas are typically reported, particularly of the
stomach and colon but also of the breast, uterus, esophagus, and
● 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
● 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
•If it is :
• Sudden onset
• With constitutional symptoms and
• Extensive and involves sites such
as the palms and soles and mucous
• 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
Dermatoglyphics are absent/hypoplastic in
what genodermatoses ?
• Dermatopathia pigmentosa reticularis
•Profuse congenital familial milia with
absent dermatoglyphics (Basan's
Bazex syndrome (acrokeratosis
•Acral psoriasiform plaques, typically
with involvement of the nose and
helices; often the lesions are
• 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
(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
•Weight loss and diabetes mellitus accompany the dermatosis
Don’t mix between
Necrolytic MIGRATORY erythema ( paraneoplastic )
Necrolytic ACRAL erythema ( associated with HCV and
zinc def )
•Growth of fine lanugo
hairs in a generalized
•localized to the face.
With time, these hairs
•Associated with a
variety of internal
often carcinoma of the
lung, colon or breast
Resembles ichthyosis vulgaris; most often
located on the legs
Lymphoma typically predates the diagnosis of
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,
associated with HCC
(in type 1)
● 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.
Migratory superficial thrombophlebitis’
with Pancreatic cancer
- Hyperpigmentation of the neck occurring in pellagra
What is deficient in pellagra ????????????
- Blue sclera.
-Seen in multiple cases:
● Osteogenesis imperfecta,
● Ehler danlos syndrome,
● nevus of Ota,
● argyria and
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.
● 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 :
of diapers (due to the
dark urine) and cerumen
that is more brown to
black in color than
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
● 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.
- Named for Gustav Asboe-Hansen (1917–1989),
- 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
- Seen in CBDC.
•9 7 kDa Ag ( LAD-1 or LABD97 ):
cleaved ectodomain of
Treatment of choice ???
IgG staining IgA staining
linear deposition of IgA along the BMZ
Indirect IF (IIF)
sections react with
serially diluted serum
patient → incubated
with anti-IgG or other
Best substrate :
pig esophagus (PF),
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
- 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
- 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
● The scale separates from many lesions, leaving a non-
exudative red base.
polygonal scales - as
in fish scales.
Thin adherent mica-like scale attached at the center of a lichenoid firm
reddish brown papule and free at the periphery. Example: Pityriasis
In clear cell acanthoma, wafer-like scale is seen
adherent at the periphery, which leaves a moist
or bleeding surface when removed.
Describes erythematous, exfoliating or scaly, annular or polycyclic,
flat patch with an incomplete advancing double edge of peeling
scale. Example: Ichthyosis linearis circumflexa
descriptive term for massive hyperkeratosis that has a verrucous surface or forms protruding, porcupine-like
• 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)
Plate-like scale (Armor
Transglutaminase-1 deficiency due to deleterious mutations
in both copies of the TGM1 gene
● 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
● This is pathognomic of Congenital Syphilis.
● An irregular thickening or enlargement of the
sternal end of clavicle, usually unilateral
● 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
Tiger tail appearance : alternating
light and dark bands under
Seen in Trichothiodystrophy
(autosomal recessive disorder
by sulfur-deficient hair)
●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
● This is seen in Trichotillomania.
● In severe forms of the disease, when the whole
scalp is involved the hair at the margins is
● 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
● Chagas’ disease
conjunctiva is the
portal of entry
● Unilateral swelling
of eyelids and orbit
inoculation is called
as eye-sign or
Romaña's sign or
● It is seen in early
stages of African
● Winterbottom's sign
is enlargement of
lymph nodes in the
● 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 :
● Reverse Koebner
This phenomenon is
explained, in which an area of
psoriasis clears after injury
● Remote reverse
It is defined in vitiligo
patients, in which
repigmentation is seen
in distant patches after
autologous skin graft
Punch grafting on right leg (shown with blue arrow). Spontaneous
repigmentation over left leg (shown with yellow arrow)
● 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
● Found in dermatitis
artefacta produced by
● 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
patches and plaques resembling
enamel paint occur on the skin,
predominantly in areas of pressure
● The underlying skin is inflamed and
● is early and transient
bilateral upper lid edema
occurring in patients with
● The sign is usually present
only for the first few days
of the clinical presentation
of the illness.
Foot prints in
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
cigarette paper"-like wrinkled
appearance in parapsoriasis
Cliff drop appearance
Atrophoderma of Pasini and Pierini
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.
Vitiligo ponctué, an unusual
clinical presentation of
vitiligo, is characterized
by multiple small (confetti-
like), discrete amelanotic
upon a hyperpigmented
Purse string appearance of oral aperture in a case of systemic sclerosis
Figure of 8/hour glass appearance of vulva in lichen
(MPS II), is a lysosomal
storage disease caused by a
deficient (or absent) enzyme,
The accumulated substrates
in Hunter syndrome are
heparan sulfate and dermatan
sulfate. The syndrome has
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
atrophoderma in a
young patient with
Linear and whorled nevoid hypermelanosis