2. Points will be discussed in this presentation:
• Skin manifestation in paraneoplastic syndrome
• Skin manifestation in endocrine diseases
• Skin manifestation in rheumatologically diseases
• Skin manifestation in cardiovascular/cardiopulmonary diseases
• Skin manifestation for other systems
3. skin
• Mirror of the human body
• It the greatest system that can reveal the secrets of all body
systems
6. • Adherent polygonal, dryness, roughness scale/keratosis on lower
extremities (favors extensors); spares flexural creases
• Most commonly a/w Hodgkin and non-Hodgkin lymphoma
• Other associations: sarcoidosis, lupus, thyroid dysfunction, drugs,
lymphoma, breast and lung cancer
• Usually diagnosed after malignancy and course follows that of
underlying malignancy
8. • Initially p/w symmetric erythematous to violaceous psoriasiform
plaques on nasal bridge, helices, distal extremities, +/−
palmoplantar keratoderma; eruption gradually extends proximally
to knees, legs, arms, and scalp
• Other clinical findings include xanthonychia, subungual
hyperkeratosis, onycholysis, and horizontal and longitudinal nail
ridging (seen in 75%)
• Most commonly a/w cancer of upper aerodigestive tract (oral
cavity, pharynx, larynx, esophagus)
• M>F;averageage=40
• Skin findings precede the diagnosis by 2–6 months
11. • Scalp metastases usually take the form of solitary or multiple
nodules or plaques and spread hematogenously from a primary
breast, lung, or genitourinary cancer to dermis of scalp.
• May be scaring like that from breast cancer metastsis, or may be
non-scaring and appear clinically similar to alopecia areata
• Extramammary Paget's disease can presenting as alopecia
neoplastica
16. • Dermatomyositis is a rare acquired muscle disease that is
accompanied by a rash
• In many patients, the first sign of dermatomyositis is itchy or burning
rash.
• The rash often, but not always, develops before the muscle
weakness.
• Reddish or bluish-purple patches mostly affect sun-exposed areas.
• A violaceous rash may also affect cheeks, nose, shoulders, upper chest
and elbows.
• Most commonly a/w ovarian cancer
• Other associated malignancies: lung, colorectal, pancreatic, and non-
Hodgkin lymphoma
18. • Carcinoma en cuirasse: indurated skin w/ orange peel-like (peau
d’orange) appearance
• Carcinoma en cuirasse, also called sclerodermoid carcinoma:
indurated scar-like plaques due to cancer cells infiltrating collagen
• Carcinoma erysipeloides: well-demarcated, raised red plaque
• Both typically present on the chest wall, but can also involve axilla
and upper extremities
• As a result of metastatic breast carcinoma infiltration into
lymphatic vessels
21. • Tripe palms are characterized by thickened velvety palms that have the
appearance of tripe, the stomach lining of beef, pork, or sheep.
• Approximately 90% of cases of tripe palms are associated with internal
malignancy.This skin disease is very rare. It usually occurs before the
diagnosis of the cancer, but may arise during any point in the course of the
malignancy.
• Tripe palms are also known as acanthosis palmaris and acquired
pachydermatoglyphia.
•
Tripe palms are frequently seen in conjunction with acanthosis nigricans.
In these cases, the underlying malignancy is most commonly stomach
(35%) or lung (11%) cancer.
• In cases where tripe palms occur without acanthosis nigricans, lung
cancers are usually responsible
23. • Sudden increase in the size and number of seborrheic keratoses
• Seborrhoeic keratosis is also called basal cell papilloma, senile wart,
brown wart or barnacle
• Most commonly a/w underlying gastric adenocarcinoma (> colon,
breast, others)
• A/w pruritus and inflammation; may improve with treatment of
underlying malignancy
• May be a/w acanthosis nigricans and tripe palms
24. • Eruptive and irritated seborrhoeic keratoses may also arise as an
adverse reaction to a medication, such as adalimumab,
vemurafenib, dabrafenib, 5-fluorouracil and many chemotherapy
drugs.
• Eruptive seborrhoeic keratoses that are not associated with cancer
are sometimes described as having pseudo-sign of Leser-Trélat
26. • Sudden onset with extensive and severe lesions; p/w symmetric
hyperpigmented velvety plaques typically in intertriginous areas
• Typically a/w GI cancer (esp. stomach)
• Can occur simultaneously, before, or after cancer diagnosis
• Improves with treatment of underlying malignancy
• 25% of patients also have tripe palms (tripe palms in absence of AN is
more commonly a/w lung cancer)
28. • Arcuate and polycyclic, erosive, erythematous patches +/−
vesicles/bullae often on genital region, buttocks/ anal region,
lower extremities, and intertriginous areas
• Initially there is a ring-shaped red area that blisters, erodes and
crusts over. It can be quite itchy and painful. As it heals, it may leave
behind a brown mark
• Glucagonoma syndrome consists of NME, glucose intolerance,
weight loss, glossitis, and glucagon-secreting carcinoma
30. • Indurated yellow plaques w/ frequent ulceration and necrosis , most
commonly periorbital
• A/w paraproteinemia (most often IgG-κ); occasionally a/w multiple
myeloma and other lymphoproliferative malignancies
• Monoclonal gammopathy, also known as paraproteinemia, is the
presence of excessive amounts of myeloma protein or monoclonal
gamma globulin in the blood
32. • Yellow patches and thin plaques
• Tends to affect trunk, periorbital skin, and body folds
• Often a/w paraproteinemia, multiple myeloma, and
lymphoproliferative malignancies
34. • Severe erosive disease of mucous membranes leading to painful oral
stomatitis; polymorphous bullous skin eruption
• It may be confused with several other blistering skin conditions including
pemphigus vulgaris, erythema multiforme, bullous pemphigoid, and lichen
planus.
• 90% mortality; most common causes of death: underlying malignancy,
bronchiolitis obliterans, and sepsis
• Most commonly a/w non-Hodgkin lymphoma or CLL
• Other associated malignancies: Castleman disease (most common association
in children), thymoma, sarcoma
• The condition can resolve if the cancer can be removed or cured.Treatment is
otherwise difficult and largely just supportive, aimed at dressing the areas, pain
relief and managing secondary infections
36. • Diffuse waxy keratoderma of high pressure areas on plantar
surface (i.e. heel, ball of foot) oral leukokeratosis
• Autosomal dominant inheritance
• Also called tylosis
• Childhood onset of focal keratoderma
• a/w Oesophageal cancer in middle age
• White areas may be seen inside the mouth (leukokeratosis)
37. Treatment of focal keratoderma
• Emollients
• Keratolytics (e.g. 6% salicylic acid in 70% propylene glycol)
• Topical retinoids
• Topical vitamin D ointment (calcipotriol)
• Systemic retinoids (acitretin)
39. • Sudden onset of long, thin, soft, lanugo-like hair initially on the
face and ears, which can spread in craniocaudal manner
• A/w lung, colorectal, and breast cancer; anorexia nervosa
• F > M (3:1); average age 40–70 years
• Tumortreatmentusuallyleadstoregressionofhairgrowth
41. • Sweet syndrome also called acute febrile neutrophilic dermatosis
• “Juicy” red-violaceous papules/plaques that can have overlying
pustules and pseudovesicles; favors head/neck, and upper
extremities
• F > M (except in cases a/w underlying malignancy, where M = F)
• Also have fever, malaise, and leukocytosis
• A/w IBD, URI infection, malignancy (most common is AML), and
polycythemia vera
• Rx: steroids, potassium iodide, clofazimine, and colchicine
44. Treatment of urticarial rash in Schnitzler
syndrome
• Anakinra is an effective treatment for Schnitzler syndrome. It
is an interleukin 1 antagonist,
• Antihistamines
• Nonsteroidal anti-inflammatory agents
• Systemic steroids, e.g., prednisoneHydroxychloroquine
• Colchicine
• Dapsone
46. diabetes
• It is estimated that 30% of patients with diabetes mellitus will experience
a skin problem at some stage throughout the course of their disease.
• Several skin disorders are more common in diabetic patients, particularly
those due to infection such as candida and impetigo.
• Patients with type 2 diabetes also have twice the risk of developing the
common scaly disease, psoriasis, as non-diabetics.
48. • Bullosis diabeticorum are blister-like lesions that occur spontaneously on the feet
and hands of diabetic patients
• Diabetic bullae appear to occur more commonly in men than women and between
the ages of 17-84 years.
• It is also more common in patients whom have long-standing diabetes or with
multiple diabetic complications.
• Blisters can be from 0.5 to 17 centimetres in size.They often have an irregular
shape.
• Two types of diabetic bullae have been defined.
Intraepidermal bullae – these are blisters filled with a clear, sterile viscous fluid
and normally heal spontaneously within 2-5 weeks without scarring and
atrophy.
Subepidermal bullae – these are less common and may be filled with blood.
Healed blisters may show scarring and atrophy.
50. • Diabetic dermopathy is a skin condition characterised by light brown or reddish,
oval or round, slightly indented scaly patches most often appearing on the shins.
• Although these lesions may appear in anyone, particularly after an injury or
trauma to the area, they are one of the most common skin problems found in
patients with diabetes mellitus.
• It has been found to occur in up to 30% of patients with diabetes.
• Diabetic dermopathy is sometimes also referred to as shin spots and pigmented
pretibial patches.
• The presence of four or more lesions is almost always limited to patients with
diabetes. People presenting with shin spots not already diagnosed with diabetes
should undergo further investigation to rule out the possibility of early diabetes.
• These harmless shin spots usually do not require any treatment and tend to go
away after a few years, particularly following improved blood glucose control.
52. • Necrobiosis lipoidica is a rare granulomatous skin disorder which can affect
the shin of insulin dependent diabetics,
• although it may occur in non-diabetic subjects as well.
• The cause is unknown.
• Typically in necrobiosis lipoidica, one or more tender yellowish brown
patches develop slowly on the lower legs over several months.They may
persist for years.
• They may be round, oval or an irregular shape.The centre of the patch
becomes shiny, pale, thinned, with prominent blood vessels
(telangiectasia). A minor injury to an established patch can cause it to
ulcerate.
• This is often painless, but is at risk of secondary bacterial infection and
delayed healing.
53. • A/w diabetic nephropathy, retinopathy, and smoking
• 30% of NLD pts have diabetes, but only 0.3% of diabetics have NLD
• F>M
• The diagnosis of necrobiosis lipoidica may be made clinically
• histopathology is characteristic; it shows an granulomatous inflammatory
reaction around destroyed collagen.This is known as necrobiosis or
collagenolyis.
• Not all cases require treatment.The following are sometimes effective:
Topical steroids, usually under a plastic occlusive dressing
Intralesional steroid injections or steroid tablets
Aspirin and dipyridamole combination
Oxypentifylline tablets
55. • Scleredema is a type of cutaneous mucinosis of unknown cause
• Scleroedema should not be confused with ‘scleroderma’, in which the skin
is fibrotic (morphoea and systemic sclerosis).
• Scleredema affect any adult,These include:
Diabetes mellitus
Hyperparathyroidism
Sjögren syndrome
Rheumatoid arthritis
Multiple myeloma
Malignant insulinoma
HIV infection.
56. • Scleroedema presents with symmetrical hardening and thickening of the skin.
• The affected areas are firm and woody plaques, sometimes slightly red or
brown and often with a ‘peau d'orange’ (orange-skin) appearance due to skin
induration.
• The face may appear expressionless and there can be difficulty opening the
mouth.
• The best way to treat scleroedema is unknown, because of its rarity. Some
benefit has been reported with the following:
PUVA
Cyclophosphamide
Oral corticosteroids
Ciclosporin
60. • Pretibial myxoedema is a form of diffuse mucinosis in which there is an
accumulation of excess glycosaminoglycans in the dermis and subcutis of
the skin. Glycosaminoglycans, also called mucopolysaccharides, are
complex carbohydrates that are important for tissue hydration and
lubrication
• Affects 0.5–4.3% of patients with Graves disease;
• it is seen in up to 13% in those with severe eye disease
• Has also been seen in patients with Hashimoto thyroiditis, primary
hypothyroidism (underactive thyroid),
• associated with high serum concentrations ofTSH-R antibodiesIs
• most common in people between the ages of 40 and 60
• More commonly affects females, with a female:male ratio of 3.5:1
61. • Pretibial myxoedema can appear before, during, or after the thyrotoxic state. It
is not related to thyroid function. It is generally seen 12–24 months after
diagnosis.
• clinical features of pretibial myxoedema:
It is most commonly found on the pretibial areas, the dorsum of the feet, or
in sites of prior trauma.
It is usually asymptomatic and more of a cosmetic concern, but can be itchy
or sore.
Early lesions are bilateral, firm, non-pitting, asymmetrical plaques or nodules;
they may coalesce to form scaly, thickened and hardened skin areas.
Hair follicles are often prominent giving a peau d'orange (orange peel)
texture.
The overlying skin may be discoloured, in colours that range from a violet
tinge to a slightly pigmented yellow-brown
There may be localised hyperhidrosis (increased sweating) and/or
hypertrichosis (increased hair growth) over the affected skin.
62. Treatment of Pretibial myxedema
• Pretibial myxoedema is often asymptomatic and mild, and may require
no treatment at all.
• If symptomatic, treatment options include:
• Minimisation of risk factors:
Avoid tobacco
Reduce weight
Normalise thyroid function
• Compression stockings, which can be worn to improve lymphoedema
• Mid to high potency topical corticosteroid, which are usually
recommended under occlusion (eg plastic cling-film wrap) nightly or
every other night to enhance effect
63. Hypothyroidism
• Coarse, dry, scaly, cold, boggy, and edematous skin
• Generalized myxedema
• Dull, brittle, and coarse hair; diffuse alopecia
• Madarosis (loss of eyebrows/eyelashes), hypohidrosis,
• onycholysis, and striated/brittle/slow-growing nails
• Cutaneous pallor or yellowing of skin secondary to
• carotenemia
• Thickened skin with enlargement of the lips and
• tongue
67. cutaneous lupus erythematosus
• LE-specific cutaneous LE has been classified as:
acute,
subacute,
intermittent and
chronic.
Lesions may be localised or generalised. In
LE-specific cutaneous LE, lesions are often induced by exposure to
sunlight.
• LE-nonspecific cutaneous LE may relate to systemic LE or other
autoimmune disease.
68. Acute cutaneous lupus erythematosus:
• Acute cutaneous LE affects at least 50% of patients with systemic lupus
erythematosus (SLE). Many are sick, young, fair-skinned females.
• Specific features of acute cutaneous LE may include:
Malar eruption or ‘butterfly rash’ (erythema and oedema of cheeks,
sparing nasolabial folds) lasting hours to days
Erythematous papular rash on arms, sometimes forming
large plaques and spreading widely
Photosensitivity (a rash on all recently sun-exposed skin)
Cheilitis and mouth ulcers
Blisters (bullous LE) and erosions
69.
70. Subacute cutaneous lupus erythematosus
• About 15% of patients with cutaneous LE have subacute cutaneous LE.
• One-third of cases are due to previous drug exposure.
• most have antibodies to Ro (SS-A)
• SCLE may occur in patients with:
systemic lupus erythematosus (SLE),
Sjögren syndrome,
deficiency of the second component of complement (C2d),
drug-induced.
SCLE is the most common subtype of CLE associated with Sjögren
syndrome
71. • Features of subacute cutaneous LE include:
Precipitation or aggravation by sun exposure
Non-itchy psoriasis-like papulosquamous rash on
the upper back, chest and upper arms
Annular or polycyclic plaques that clear centrally
Usually symmetrical distribution
Absence of scarring or depigmentation on
resolution
72.
73.
74. Drug-induced subacute cutaneous LE
• More than 100 drugs have been associated with the onset of
subacute cutaneous LE.They include:
Terbinafine
Tumour necrosis factor-alpha (TNF-α) inhibitors (biologics)
Anticonvulsants
Proton-pump inhibitors
75.
76. Neonatal cutaneous lupus erythematosus
• Neonatal cutaneous LE arises within 2 months of birth to mothers with known or
subclinical subacute cutaneous LE.
• A paediatrician should assess all babies born to mothers with subacute LE (or
carrying anti-Ro/La) at birth.
• Mortality in babies with heart block is up to 20%, despite pacemaker implantation.
• Features of neonatal cutaneous LE may include:
An annular erythematous rash, which slowly resolves over 6 months
Rash is most often periorbital
Photosensitivity
Blood count abnormalities: haemolytic anaemia, leukopenia, thrombocytopenia
Hepatobiliary disease
Persistent congenital heart block
77.
78. Intermittent cutaneous lupus
erythematosus
• Intermittent cutaneous LE, more often known as lupus
tumidus, is a dermal form of lupus.
• Lupus tumidus is similar to Jessner lymphocytic infiltrate, in
which diagnostic criteria for lupus are absent.
• Jessner lymphocytic infiltrate is an uncommon skin condition
that presents as non-scaly red patches and lumps on the face,
neck and upper back.They are usually painless and do not itch.
Lesions may go through periods of remission and
exacerbation over months or years.Total spontaneous
resolution has also occurred in some cases.
79. • Features of lupus tumidus include:
Affects sun-exposed sites such as cheeks, neck, anterior chest
Erythematous, urticaria-like patches and plaques with a smooth
surface
Round or annular shapes
Clears during the winter months
Non-scarring
81. Chronic cutaneous lupus erythematosus
• Chronic cutaneous LE accounts for 80% of presentations with
cutaneous LE.
• About 25% of patients with chronic cutaneous LE also have systemic
LE.
• Types of Chronic cutaneous lupus erythematosus:
Discoid LE
Hypertrophic LE
Mucosal LE
Lupus profundus
82. Discoid LE
• Discoid LE is the most common form of chronic cutaneous LE.
• Only 5% of people with DLE have SLE (although half have positive ANA); however,
among individuals with SLE, as many as 20% have DLE
• Discoid LE is confined to the skin above the neck in most patients, but can spread
below the neck to affect upper back,V of neck, forearms and backs of hands.
• Scalp, ears, cheeks, nose are the most common sites.
• Most patients have photosensitivity.
• New lesions are destructive, erythematous scaly plaques with follicular prominence.
• Scalp discoid LE presents as red, scaly and bald plaques.
• Slow healing leads to postinflammatory pigmentation and white scars.
• Hair growth may partially or completely recover with
treatment. Cicatricial (scarring) alopecia can be permanent.
83.
84. • Hypertrophic LE
Hypertrophic LE is a variant of discoid LE in which there are thickened
and warty plaques resembling viral warts or skin cancers.
Hypertophic LE can occur on palms and/or soles.
This is also called palmoplantar LE, and is a form
of acquired keratoderma.
• Mucosal LE
Mucosal LE presents with plaques, ulcers and scaling. Mucosal lesions
may predispose to squamous cell carcinoma.
Lips and inside the mouth
Lower eyelid with madarosis (loss of eyelashes)
Rarely, vulva/penis
85. Lupus profundus
• Lupus profundus affects subcutaneous tissue. Other names for
lupus profundus are lupus panniculitis .
• Lupus profundus may develop at any age, including childhood.
• It may involve face, buttocks, limbs or anywhere.
• Firm deep and tender nodules persist for some months.
• Lesions resolve leaving dented, atrophic scars (lipoatrophy).
86.
87. nonspecific cutaneous features of lupus
erythematosus
• LE nonspecific cutaneous features are most often associated with SLE.They include:
• Diffuse hair thinning
• Urticaria
• Raynaud phenomenon: abnormal blanching of fingers and toes in response to cold weather, followed
by numbness and slow rewarming by the fingers which go blue then red.
• Lupus chilblains: painful erythematous nodules on fingers and toes during cooler months.
• Dilated periungual telangiectasia, ragged cuticles and nail dystrophy
• Digital ulcers and pitting scars
• Thrombophlebitis
• Papular and nodular mucinosis on cheeks, upper chest, upper arms or back.
• Vasculitis: small vessel vasculitis, urticarial vasculitis and less often, vasculitis of medium and large
vessels
• Livedo reticularis and antiphospholipid syndrome
89. treatment for cutaneous lupus erythematosus
• Local therapy:
Potent or ultrapotent topical steroids are applied to chronic discoid LE
plaques
Calcineurin inhibitors, pimecrolimus cream or tacrolimus ointment can
be used instead of topical steroids
Intralesional corticosteroid can be injected into small lesions resistant
to topical therapy
Topical retinoids, calcipotriol and imiquimod have also been reported
to be helpful in a few patients.
Cosmetic camouflage may be used to disguise unsightly
90. • Systemic therapyTreatment for cutaneous and systemic LE may include:
Antimalarials especially hydroxychloroquine
Immune modulators such as methotrexate, mycophenolate, dapsone,
ciclosporin
Retinoids, i.e acitretin, isotretinoinSystemic corticosteroids
If sun protection is strict, vitamin D supplementation.
• Severe disease may require more aggressive treatment:
Cyclophosphamide
Thalidomide
Photopheresis
Intravenous immunoglobulin
Monoclonal antibodies targetingT and B cells and cytokines: rituximab
91. Systemic sclerosis
• Systemic sclerosis has been subdivided into 2 main subtypes, according to the
distribution of skin involvement, to:
Diffuse cutaneous systemic sclerosis (dcSSc)
Limited cutaneous systemic sclerosis (lcSSc)
• Limited cutaneous systemic sclerosis was previously referred to as CREST
syndrome to denote key features :
Calcinosis
Raynaud phenomenon(o)
Esophageal dysmotility
Sclerodactyly
Telangiectases.
92. Key features of systemic sclerosis
• Skin thickening of the fingers and toes (sclerodactyly)
• Specific autoantibodies in the blood (anti-Scl70 or anti-centromere
antibody and others)
• Abnormal nail fold capillaries
• Internal organ fibrosis and/or vascular damage (involving the lungs,
heart, gastrointestinal tract and/or kidneys)
93.
94. Cutaneous features of systemic sclerosis
• Skin sclerosis:
Sclerodactyly: thickening and tightness of the skin of the fingers (or toes). May be spindle-
shaped
• Hands:
Puffy fingers; early inflammatory phase of disease
Raynaud phenomena
Abnormal nail fold capillaries
Palmar erythema affecting thenar / hypothenar eminence
Smaller fragile nails with ragged cuticles
Digital pitted scars
Digital ulcers
Ulceration can lead to dry gangrene and eventual loss of the tips of the fingers (like frost bite).
95. • Face:
Matt telangiectases on face, chest, palms
Peri-oral furrowing (fat loss)
Microstomia (limited oral aperture defined as interlabial distance < 4.5
cm)
Beaked nose
• Other:
Calcinosis affecting digits, extensor surfaces of limbs. Skin can
breakdown and discharge chalky material (calcium)
Salt and
pepper dyspigmentation (hyperpigmentation and hypopigmentation)
Pruritus
107. Pyoderma gangrenosum
• Pyoderma gangrenosum presents as a rapidly enlarging, very painful ulcer.
• It is one of a group of autoinflammatory disorders known
as neutrophilic dermatoses.
• Associated with following diseases:
Inflammatory bowel disease (ulcerative colitis and Crohn disease)
Rheumatoid arthritis
Myeloid blood dyscrasias including leukaemia
Monoclonal gammopathy (usually IgA)
Chronic active hepatitis
Granulomatosis with polyangiitis
108. Clinical features of pyoderma gangrenosum
• Pyoderma gangrenosum usually starts quite suddenly, often at the
site of a minor injury.
• It may start as a small pustule, red bump or blood-blister.
• The skin then breaks down resulting in an ulcer.
• The ulcer can deepen and widen rapidly.
• Characteristically, the edge of the ulcer is purple and undermined.
• Pyoderma gangrenosum is usually very painful
109.
110. • pyoderma gangrenosum can be diagnosed if there are 2 major crtieria and
2 minor criteria as follows:
• Major criteria:
Rapid progression of painful, necrolytic, cutaneous ulcer with an irregular,
violaceous and undermined border
Other causes of cutaneous ulceration have been excluded
• Minor criteria:
History suggestive of pathergy or clinical finding of cribriform scarring
Systemic diseases associated with pyoderma gangrenosum
Histopathological findings (sterile dermal neutrophilia +/- mixed
inflammation +/- lymphocytic vasculitis
Treatment response (rapid response to systemic steroid treatment)
111.
112. Treatment of pyoderma gangrenosum
• Treatment is non-surgical.
• The necrotic tissue should be gently removed.
• Wide surgical debridement should be avoided during the active
stage of pyoderma gangrenosum because it may result in
enlargement of the ulcer.
• Skin grafting and other surgical procedures may be performed
when the active disease phase has settled, with care to minimise
trauma.
113. Small ulcers are often treated with:
• Potent topical steroid ointment
• Tacrolimus ointment
• Intralesional steroid injections
• Ciclosporin solution
• Special dressings
• Oral anti-inflammatory antibiotics such as doxycycline or
minocycline
• If tolerated, careful compression bandaging to reduce swelling.
114. Systemic treatment for larger ulcers:
• Oral prednisone for several weeks or longer, or intermittent
intravenous methyl prednisolone for 3–5 days
• Ciclosporin
• Biologic agents: success with infliximab, adalimumab,
etanercept and ustekinumab is reported in a small number of
cases (used off-label).
115. Dermatitis herpetiformis (Duhring’s disease)
• immunobullous disease that has
been linked to coeliac disease.
• Some patients have a personal or
family history of other
autoimmune disorders including
thyroid disease, pernicious
anaemia, type 1 diabetes,
vitiligo, Addison disease and
alopecia areata.
116. the clinical features of dermatitis
herpetiformis
• Dermatitis herpetiformis has a symmetrical distribution.
• Lesions most commonly appear on scalp, shoulders, buttocks, elbows and knees.
• It is characterised by prurigo (extremely itchy papules) and vesicles on normal or reddened skin.
• They often appear in groups or serpiginous clusters.
• Blisters are often eroded and crusted due to immediate scratching.
• Dermatitis herpetiformis may also present initially as digital petechiae.
• Flat red patches, thickened plaques and weals may occur resembling other inflammatory skin
conditions such as dermatitis, scabies and papular urticaria.
• Lesions resolve to leavepostinflammatory hypopigmentation and hyperpigmentation.
Editor's Notes
The skin is the mirror that see us what is hidden inside our bodies, there are many diseases, one part of their journey will be through the skin, so, let’s travel to discover through the window that reveal the secrets of our systems. My gun will be done, and I hope your mind be remind to me for last moment in this presentation.
Extramammary Paget's disease (EMPD) is a rare malignant neoplasm with a predilection for the apocrine-rich anogenital skin and less commonly for the axilla. The tumor rarely occurs in non-apocrine bearing regions where it is referred to as ectopic EMPD
A/w IgM-κ paraproteinemia and lymphoplasmacytic malignancies
Urticaria (hives) describes red raised patches of skin. These are rarely itchy in Schnitzler syndrome, unlike ordinary urticaria. Weals may last longer than ordinary urticaria, often persisting for 12 hours to 3 days. A skin biopsy may or may not show features of urticarial vasculitis. Angioedema (deep swelling) may also occur in some patients.
In most cases diabetic bullae heal spontaneously without treatment. Patients should make sure the blister remains unbroken to avoid secondary infection.
Ulcerating necrobiosis lipoidica may require aggressive treatment.
Up to 50% of patients with subacute cutaneous LE may also have a mild form of SLE, resulting in arthralgia (painful joints) or arthritis (joint disease) and low blood counts. Severe SLE is rare in patients with subacute cutaneous LE.
Terbinafine is an allylamine medicine used to treat fungal infections. It is especially effective against dermatophytes (tineainfections).
Jessner lymphocytic infiltrate plaques usually start off as defined, small, slightly raised red spots that gradually enlarge peripherally to create a well demarcated red plaque. Sometimes the centre of the lesion is left clear, ie forming an annular and arciform shape
Serotonin periphery work as vasodilation and centrally work as vasoconstriction.
About 50% of those affected by pyoderma gangrenosum have none of the associated risk factors.
Drugs are occasionally implicated as triggers of pyoderma gangrenosum, especially cocaine, isotretinoin, propylthiouracil and sunitinib.
Sue et al proposed that
IgA tissue transglutaminase antibody
IgA and IgG gliadin assay
Dapsone is the treatment of choice for dermatitis herpetiformis, as it usually reduces itch within 3 days