Acquired Disorders Of Epidermal Keratinisation Flashcards
Lichen spinulosis
Idiopathic
Clinical dx, supported by histology
? variant of KP (same features on histo - dilated hair follicles with thick keratin plug, surrounded by mild to mod chronic inflammatory infiltrate)
Grouped hyperkeratotic follicular papules with keratosis spine coalescing into large plaques
Coarse to touch
Appear suddenly in crops
Grow rapidly , then remain stationary
Asymptomatic
Distributed symmetrically extensor surfaces
Limbs, knees, elbows, trunk, buttocks, neck
Face, hands, feet spared - though there is a facial variant presenting with tiny keratosis spicules on the cheeks
Children, young adults
Not usually assoc with underlying systemic disease
Drug assoc - gold, thallium, diphtheria toxin
Case reports - HIV, crohns, alcoholism, Hodgkin disease, syphilis
DDx follicular papules - KP, lichen nitidus, phrynoderma, PRP
Chronic, persist for many years but most resolve spontaneously within 1-2 years
Purely cosmetic
Aim of Mx —> improve cosmesis
Topical retinoids, keratolytics
First line Rx —> Keratolytic agents -
Lactic acid 5-12%
Salicylic acid 3-5%
Urea 10-20%
Second line Rx —> topical retinoids
Third line Rx —> tacalcitol (synthetic vit D analog)
Porokeratoses
Clonal expansion of keratinocytes that differentiate abnormally
Not hyperproliferative
Thin column of parakeratosis arising from invagination of underlying epidermis, with absent/attenuation of granular layer = cornoid lamella = active border
Chronic
No spontaneous resolution
SCC may develop within lesions
Papules and plaques —> annular lesions with thin thread-like elevated rim
CLASSIFICATION
Localised
- Porokeratosis of mibelli —> infancy/childhood, M
- Linear porokeratosis —> infancy/childhood, M/F, genetic mosaicism, higher risk malignancy change
- Punctate palmoplantar porokeratosis —> adolescence, M
- Genital porokeratosis —> M, scrotum > penis
- Perianal porokeratosis
Disseminated
- Disseminated superficial actinic porokeratosis —> adulthood, F, lower legs and arms, worsening after sun exposure
- Disseminated superficial porokeratosis —> not necessarily related to sun exposure, sun exposed + sun protected sites incl oral mucosa, genitalia, immunodeficiency (I.e. transplant, HIV, malignancy) vs sporadic during childhood
- Systematisized linear porokeratosis (ant of linear porokeratosis)
- Disseminated palmoplantar porokeratosis —> adolescence, generalisation of punctate palmoplantar porokeratosis to involve sun exposed + sun protected sites incl oral mucosa, genitalia following blaschko lines
ASSOCIATED DISEASES HIV (if suddent onset porokeratosis) Crohn disease Diabetes Liver disease Chronic renal failure Haem and solid tumours (if sudden onset porokeratosis)
PREDISPOSING FACTORS
drug induced immunosuppression including organ transplant
UVR —> DSAP, porokeratosis of Mibelli
Familial, AD patterns with variable penetrance
Genetic mosaicism —> linear porokeratosis
MANAGEMENT
Monitoring for skin cancer
Principle of Rx -
- target abnormal clone of cells I.e. same approach as for actinic keratosis
- target abnormal keratinisation with topical/systemic retinoids
First line - LN2 Efudix Imiquimod Solaraze Calcipotriol Cholesterol + 2% lovastatin cream C&C PDT CO2 laser ablation
Second line -
Oral retinoids I.e. isotretinoin, acitretin —> immunosuppresse, linear porokeratosis —> reduce risk malignancy change
Acquired ichthyosis
Arises in adulthood
But clinically and histologidally similar to imheroted ichthyosis vulgaris
Rare —> should raise suspicion on an assoc internal disease
ASSOCIATED DISEASES Malignancy - Hodgkin dosease - MF - Multiple myeloma - Kaposi sarcoma - Other sarcomas - Carcinomas i.e. lung, breast, ovary, cervix
Meds
- Statins
- Nicotinic acid
- Cimetidine
- Clofazimine
Endocrinopathies
- Thyroid disease
- Diabetes
Infections
- Leprosy
- TB
- HIV
- HTLV-1 assoc myelopathy
AICTD
- Dermatomyositis
- SLE
- Scleroderma
Nutritional
- Malnutrition
- Malabsorption syndromes
- Essential fatty acid deficiency
- Pancreatic insuffiency
- Anorexia nervosa
Others
- Chronic renal failure
- BMAT
- Sarcoidosis
CLINICAL FEATURES
Can be itchy
Sudden onset
Initial involvement lower limbs —> may generalise
Symmetrical dark thick scaling on the legs
Arms
Trunk esp back
Scalp (fine scales)
+/- PPK with fissures —> complicated by secondary infection
Spares flexures d/t high humidity in these areas
Face unaffected usually d/t size and number of sebaceous glands
DDX Xeroderma Asteatotic eczema Atopic eczema Drug eruption Hereditry ichthyoses
IX
Clinical dx
Search for underlying cause esp occult malignancy i.e. lymphoma
CXR
HISTO
Compact hyperkeratosis
Granular layer absent or thinned
GENERAL MX
Identify underlying cause
TOPICALS
First line - Retinoids i.e. tretinoin, tazarotene (reduce cohesiveness of keratinocytes)
Second line - Beta hydroxyacids Keratolytic i.e. salicylic acid (to disaggregate corneocytes)
Third line - AHA keratolytic i.e. glycolic acid, lactic acid BD (to loosen and desquamate corneocytes)
Third line - humectant i.e. urea 10-20%
Third line - propylene glycol 20% im aqueous cream (hydrates stratum corneum)
COURSE/PROGNOSIS
May improve with treatment of underlying disease/cessation of drug
Acanthosis nigricans (AN)
Asymptomatic
Symmetrical darkening and thickening
Velvet texture
May be studded by skin tags
Intertriginous skin
- Neck (back of neck most common site in children)
- Axillae
- Groin
- Submammary folds
+/- eyelid and conjunctivae involvement
Nail changes
- Leukonychia
- Subungual hyperkeratosis
More common in darker skin
Isolated skin conditon or assoc with other conditions
Benign (common) vs malignant (rare) AN
Warty thickening of oral margins (sign of malignant AN)
CLINICAL VARIANTS HAIR-AN syndrome - Familial - Young black girls - HyperAndrogenaemia —> PCOS, hirsutism, clitoral hypertrophy, high plasma testosterone levels - Insulin Resistance - Acanthosis Nigricans
AN assoc with diabetes, hyperandrogenism, AICTD
Familial AN
- Rare
- AD inheritance with variable penetrance
- Stabilises im teenage years —> improves with age
Drug-induced AN —> resolves after cessation
- Hormones i.e. testosterone, exogenous oestrogens (OCP)
- Insulin
- Systemic CS
- Nicotinic acid
Generalised AN
- Rare
- Only in children
- Extensive Ix fail to show assoc systemic abnormality
Acral AN
- FST 5, 6
- Velvety thickening and hyperpigmentation
- Dorsal hands, feet esp the knuckles
- Not assoc with systemic disease
Unilateral AN (naevoid AN) Rare Somatic mutation during embryogenesis Onset infancy or childhood or adulthood Pigmented plaque Solitary vs in lines of Blaschko Resembles epidermal naevus Typical histo features of AN Anywhere on body (rather than flexural)
Malignant/paraneoplastic AN
DDX (can be excluded with bloods)
Addison disease (hyperpigmentation) —> U/Es (hyponatraemia, hyperkalaemia), serum glucose (hypoglycaemia), ACTH, cortisol
Pellagra (vit B3 deficiency) —> urinary niacin (deficiency)
Haemochromatosis —> iron studies, HFE gene
PREDISPOSING FACTORS
Sweat
Friction
Familial form —> AD inheritance —> FGFR3 mutations
ASSOCIATED DISEASES (benign AN) Obesity —> AN can regress with weight loss
Endocrinopathy
- Insulin resistance
- Diabetes
- Acromegaly and gigantism
- PCOS
- HAIR-AN syndrome
- Hashimoto thyroiditis
AICTD
- Dermatomyositis
- LE
- Systemic sclerosis
Meds
- Hormones i.e. testosterone, exogenous oestrogens (OCP)
- Insulin
- Systemic CS
- Nicotinic acid
ASSOCIATED INTERNAL MALIGNANCIES (malignant AN) GIT - Gastric CA *** - Gallbladder and bile duct CA - Liver CA - Pancreatic CA
Gender specific
- Breast CA
- Gynaecological CA
- Prostate CA
- Testicular CA
GUT
- Bladder CA
Renal -
- Renal CA
- Wilm’s tumour (in children)
Endocrine
- Thyroid CA
MSK
- Osteogenic sarcoma (in children)
Haem
- Hodgkin disease
IX for adult onset
Screen for underlying endocrinopathy
- Serum insulin (insulin resistance)
Screen for underlying malignancy
GENERAL MX
Mx underlying condition
If no underlying condition —> Rx aim is to improve cosmetic appearance
TOPICALS
First line - retinoids (may reduce hyperkeratosis)
Second line - AHAs and keratolytics i.e. salicylic cid (may improve appearance by reducing hyperkeratosis)
SYSTEMICS
Third line - Oral isotretinoin (extensive cases)
HISTO
No or minimal acanthosis or hyperpigmentation (name is misnomer)
Hyperkeratosis —> causes pigmentation
Papillomatosis with finger-like upward projections of dermal papillae
No increase in melanocyte numbers or melanin production
+/- pseudohorn cysts
Parakeratosis (mucosal lesions)
COURSE/PROGNOSIS
Benign AN
- Generally persists
- May be significant cosmetic problem
AN assoc with metabolic abnormalities/insulin resistance
- May improve with Rx of underlyong condition
- Weight loss
- Dietary restriction
Malignant AN
- Poor prognosis
Confluent and reticulated papillomatosis (CARP)
Young adults
Asymptomatic
Small Hyperkeratotic papules
Coalescing into greyish-blue confluent (at the centre) and reticulated (towards periphery) plaques
No mucous membrane involvement
Trunk
- Presternal
- Epigastric
- Interscapular
Spread to lower abdomen and pubic areas
+/- limbs
+/- face
Localised forms i.e. limbs only, face only reported
DDX Acanthosis nigricans Macular amyloidosis Darier disease Epidermal naevus Plane warts Pityriasis versicolor Seb K Retention hyperkeratosis (deficient personal hygiene)
PATHOGENESIS
Abnormal reaction to commensal microorganisms i.e. malassezia, actinimycete bacteria
CAUSATIVE ORGANISMS
Malassezia (yeast) —> topical azoles
Dietzia papillomatosis (bacteria) —> tetracyclines, erythromycin (though response to ABs more likely anti-inflammatory)
ASSOCIATED DISEASES (Endocrinopathies) Obesity Insulin resistance Cushing disease Thyroid disease
IX
Fungal scrapings —> may show malassezia yeasts
HISTO
Hyperkeratosis
Papillomatosis
Granular layer absent or thinned
+/- increased melanin in basal cell layer and stratum corneum —> contributes to hyperpigmentation
+/- Mild non-specific inflammatory infiltrate
GENERAL MX
Rx Purely cosmetic
If asymptomatic and not bothering patient —> no Rx is an option
TOPICALS Mupirocin ointment (first line) Antifungals (seond line) i.e. Selenium sulphide shampoo Retinoids (second line) Vit D analogues (second line)
SYSTEMICS
Oral ABs (first line)
- Minocycline (most effective)
High and low dose isotretinoin (third line)
COURSE/PROGNOSIS
Chronic
Overweight patients —> weight reduction
May improve during pregnancy/with OCP
Pityriasis rotunda
Dx of exclusion
CLINICAL FEATURES Asymptomatic —> diagnosed incidentally Perfectly round Slightly erythematous/pink (fair skin individuals) or hyperpigmented (in dark skin individuals) plaques with fine scaling Sharply demarcated, can coalesce —> polycyclic plaques A few to numerous Sites - - Trunk - Buttocks - Arms - Legs
Rare
TYPE 1 (most common)
Older East Asian (Japan) or African individuals 60s
Sporadic
Assoc with underlying systemic disease/malignancy
May resolve with Rx of the underlying condition
TYPE 2 Younger white individuals 40s Familial AD inheritance Not assoc with underlying disease
DDX PIH following - Fixed drug eruption Erythrasma Tinea corporis Pityriasis versicolor Psoriasis
ASSOCIATED DISEASES (type 1, esp in African patients) Malignancies - Hepatocellular CA*** (most common) - CML - SCC of the hard palate - Other malignancies
Systemic diseases/Infections
- TB
- Cardiac disease
- Lung disease
- Liver disease
- Chronic renal failure
- Chronic diarrhoea
- Osteitis
- Nutritional disease
- Systemic sclerosis
PATHOGENESIS
Unknown cause
IX
Skin bx (exclude other conditions) —> Dx of exclusion
Skin scrapings for fungal MCS (exclude dermatophyte, pityriasis versicolor)
HISTO (shares characteristics with ichthyosis vulgaris)
Epidermal hyperkeratosis
Loss of granular cell layer
MX
Resistant to Rx
TOPICALS
Retinoids (first line)
- Tretinoin
- Tazarotene
Keratolytics
- Lactic acid 10% cream (second line)
- Salicylic acid 5% ointment (third line)
SYSTEMICS
Isotretinoin
COURSE/PROGNOSIS
Type 1 (assoc with underlying disease)
Rx of disease —> resolution
Type 2 (familial) Persists lifelong
Keratosis pilaris
Inherited abnormality of keratinisation
Affects follicular orifices with varying degrees of keratotic follicular plugging, perifolliclar erythema, follicular atrophy
CLINICAL FEATURES Often presents in 1st decade of life, worsening around puberty —> tends to improve with age F > M Small Follicular keratotic papules Perifollicular erythema “Goose bump” appearance Rough texture Can become pustular with superficial pustules developing in affected follicles (d/t rubbing on clothing)
Extensor surfaces -
- limbs esp upper arms and thighs
- Lumbar area
- Buttocks (nodules, deeper inflammatory lesions may develop d/t rubbing on clothing)
Common
Variant of normal, rather than a disease (up to 50% of the po;utation has this to an extent)
Seasonal variation, improves in summer
PATHOGENESIS
Plug of excess keratin formed d/t defect of corneocyte adhesion at the follicular orifice —> prevents hair from emerging —> ingrown hair —> inflammation
GENETICS
AD with variable penetrance
CLINICAL VARIANT - ERYTHROMELANOSIS FOLLICULARIS FACIEI ET COLLI
India, Far East, Middle East
Follicular hyperkeratosis accompanied by erythema and hyperpigmentation
Face, esp cheeks
Neck
CLINICAL VARIANT - KP ATROPHICANS (more inflamamtory —> follicular fibrosis, atrophy —> scarring alopecia)
KP atrophicans faciei/ulerythema ophryogenes
- Fixed erythema
- Follicular plugging
- Pitted scarring
- Hair loss
- Cheeks
- Lateral eyebrows
- May be assoc with common KP elsewhere
- May be inherited as AD trait
- DDx rosacea
Keratosis follicularis spinulosa decalvans
- Follicular plugging —> follicular atrophy
- Onset infancy
- Cheeks
- Nose
+/- scalp —> scarring alopecia
- May be assoc with palmoplantar hyperkeratosis
Atrophoderma vermiculatum Follicular plugging —> reticulated atrophy of the skin Late childhood Cheeks Preauricular
ASSOCIATED DISEASES Ichthyosis vulgaris (may be coincidental) Atopic eczema (may be coincidental) Obesity Insulin dependant type 1 and 2 DM Renal failure and hypervitaminosis A
Genoderm
- Noonan syndrome
- Down syndrome
- Olmstead syndrome
- Monilethrix
- Pachyonychia congenita
- Ectodermal dysplasias
Drugs
- Vemurafenib (BRAFi)
- Sorafenib (BRAFi)
- Lithium
- Systemic CS
DDX Darier disease PRP Atopic eczema Lichen nitidus Eruptive vellous hair cysts
If inflammed -
Acne
Folliculitis
Rosacea (for KP atrophicams faciei)
IX —> nil (Clinical dx)
HISTO Hyperkeratosis hypergranulosis Follicular plugging Mild perivascular lymphocytic infoltrate upper dermis
GENERAL MX
If it does not bother the patient, Rx is unnecessary
TOPICALS continued for years Keratolytics (first line) - Salicylic acid*** - Lactic acid - Glycolic acid - Urea 10% cream moisturisers (combined with topical retinoids)
Retinoids (second line) —> particularly useful for KP atrophicans faciei
SYSTEMICS for severe KP (third line)
oral isotretinoin —> relapse on cessation
LASER (fourth line)
PDL useful for fixed erythema in KP atrophicans faciei
COMPLICATIONS
Hyperpigmentation, hypopigmentation, scarring may occur
COURSE/PROGNOSIS
Mild cosmetic disorder
Improves with age
Keratosis circumscripta
Rare
Skin type 6
First described in African tribe
Sporadic with clustering of cases —> supports genetic predisposition
CLINICAL FEATURES
First develops in childhood —> persistent thereafter
Onset age 3-5 yrs
Lesions develop quickly over 2-3 weeks
Well defined Circumscribed areas of diffuse and follicular hyperkeratosis
Lesions may become -
- thickened
- hyperpigmented
- violaceous in colour
Extensor surfaces of the arms, legs, trunk
DDX
Type IV corcumscribed juvenile PRP
Lichen spinulosis
Psoriasis
PATHOGENESIS
Unknown
HISTO
Follicular plugging
Moderate hyperkeratosis
No dermis involvement
MX = TOPICALS
Keratolytics —> 40% urea in WSP most successful
TCS (generally not helpful)
Retinoids (generally not helpful)
COURSE/PROGNOSIS
Condition presists
Tend not to develop new lesions once stabilised, but existing lesions may slowly increase in size and become more keratotic
Poorly responsive to Rx
Phrynoderma (“toad skin”)
Classically skin manifestation of Vit A deficiency May also be assoc with other nutritional deficiencies - - Vit A - B complex - Vit B2 (Riboflavin) - Vit C - Vit E - Essential fatty acids
In economically deprived countries —> commonest in chuldren age 5-15yrs
CLINICAL FEATURES
Starts gradually
Mostly as non-pruritic lesions on the elbows
Groups of small follicular based skin coloured or hyperpigmented papules 3-4mm with central keratotic plug
Skin has rough texture
Sites -
- Elbows and knees (most common)
- Buttocks
- Extensor surfaces of limbs
- Trunk, face (in generalised disease)
Associated with ocular signs -
- Night blindness
- Conjunctival xerosis
- Conjunctival ulceration
ASSOCIATED DISEASES (outcome of vit A/nutritional deficiency)
Liver cirrhosis
ETOH abuse
Malabsorption syndromes
Anorexia nervosa
Nutritional deficiency following bariatric surgery
DDX Keratosis pilaris Lichen nitidus Lichen spinulosis Perforating disorders PRP
IX
Vit A levels (hypovitaminosis)
Hypoproteinaemia in context of nutritional deficiency —> vit A levels can appear reduced despite adequate Vit A stores
HISTO
Dilated follicles with compact keratin
Patchy parakeratosis
No dermal reaction
MX
Correct poor diet
Oral (preferred over IV) Multivitamin containing vit A (poor diet often also result in other concurrent deficiencies) chold 8 yrs and above + adults -
- 100,000 units daily for 3 days then
- 50,000 units daily for 2 weeks then
- 10,000 units daily for 2 months until liver storage adequate
Manage underlying disease
COMPLICATIONS Ocular involvement - - Bitot spots - Conjunctival xerosis - Blindness
Trichodysplasia spinulosa
CLINICAL FEATURES Immunocompromised patients Asymptomatic or mildly itchy Shiny follicular papules with central spiny keratotic spikes on facial skin Alopecia eyebrows, scalp Progresses rapidly
CAUSE
Infection of the hair inner root sheath keratinocytes by polyoma virus in immunocompromised patients
ASSOCIATED DISEASES
Immunosuppression following organ transplant
Leukaemia
SLE
DDX of early lesions
Keratosis pilaris
Lichen nitidus
Follicular mucinosis
IX
Skin bx for histo and molecular identification of polyoma virus
HISTO
Hair follicles show abnormal maturation
dilatation of the follicular infundibulum —> Plugged by cornified eosinophilic keratinocytes containing large trichohyalin granules
EM —> intracellular polyoma viral particles
MX
Antivirals (Rx of choice)
Surgery + post op retinoid
COURSE/PROGNOSIS
Persistent
Progresses unless immunosuppresion withdrawn, or specifically treated
Flegel disease
@
Hyperkeratosis lenticularis perstans
CLINICAL FEATURES
Older adults
Occasionally younger persons
Most common in white population
Asymptomatic
Flat keratotic papules 1-5mm
Topped by horny keratotic scale —> removal of scale causes bleeding points
Red brown colour
Common Sites -
- Lower legs
- Dorsa feet
Slowly spread up the legs
Rare sites -
- Outer ear lobes
- Arms
- Palms, soles
- Oral mucosa
- Generalised form
ASSOCIATED DISEASES
Endocrinopathies esp. diabetes
CAUSE
Unknown
Familial (AD trait) and non-familial variant
ENVIRONMENTAL FACTORS
Sun exposure
DDX (localised areas hyperkeratosis) AKs Arsenical keratosis Darier disease Acquired reactive perforatimg dermatosis (Kyrle disease) Porokeratosis Stucco keratosis
IX
Skin bx —> characteristic features —> confirm dx
HISTO Papillomatosis SC Thickened, eosinophilic, compact Patchy parakeratosis SG absent or thinned Underlying SS compressed Band-like lymphocytic infiltrate beneath affected epidermis
TOPICAL RX
5-FU for several months (first line —> most consistent results)
Vit D analogue/calcipotriol (third line —> variable inconsistent results)
Retinoids (third line —> not helpful)
Keratolytics (third line —> not helpful)
PHYSICAL RX
Dermabrasion (first line)
SYSTEMIC RX
Retinoids i.e. acitretin, isotretinoin (second line —> tends to recur on cessation)
SURGICAL RX Local excision (first line)
Transient acantholytic dermatosis
@
Grover disease
CLINICA. FEATURES
Common
Older white M
Sudden onset itchy papules on the trunk
monomorphous papulovesicular erythematous eruption Quickly crusts Keratotic erosions Mutiple excoriations Mainly affects trunk +/- proximal limbs Very itchy Some asymptomatic
transient lasting 2-4 weeks or PERSISTENT for months/years with a chromic relapsing course —> name is a misnomer
DDX Folliculitis Papular urticaria Scabies HZV Galli-Galli disease (acantholytic vriant of Dowling-Degos disease, usually more widespread, hands/groins woth retoculate pigmentation)
PATHOGENESIS
Unknown
PREDISPOSING FACTORS Natural (not artificial) UVR Heat, sweating In some reports, more common in winter (? All rugged up in warm clothing Hospitalisation Bedridden patients
ASSOCIATED DISEASES Psoriasis Asteatotic eczema ACD ICD Skin cancer Haem malignancies
IX
Skin bx to confirm Dx
Haem work-up (given possible association/complication)
HISTO Small foci of acantholytic dyskeratosis Intraepidermal clefting Sometimes vesicle formation 5 patterns of acantholysis - - Pemphigus vulgaris like - Darier like - Spongiotic - Pemphigus foliaceus like - Hailey-Hailey like Sparse lymphohistiocytic perivascular infiltrate ? Lichenoid change assoc with basal vacuolation \+/- eos \+/- neuts
PEMPHIGUS VULGARIS LIKE
Suprabasal acantholysis often with large numbers of eos
DARIER LIKE
Suprabasal acantholysis
scattered corp ronds and grains (dyskeratotic and apoptotic keratinocytes)
SPONGIOTIC
Epidermal oedema causing separation of keratinocytes and prominent desmosomes
PEMPHIGUS FOLIACEUS LIKE
Superficial clefting in the superficial epidermis
HAILEY-HAILEY LIKE
Acantholysis throughout stratum spinosum
Hyperplastic epidermis
No significant dyskeratosis
GENERAL MEASURES
Avoid sunlight
Avoid strenuous exercise
Avoid heat
TOPICALS (first line)
Potent TCS (symptomatic relief)
Calcipotriol
Topical calcineurin inhibitors
SYSTEMICS
Antihistamines (first line, pruritus)
Short course oral pred (second line, severe cases, risk of rebound)
Retinoids i.e. acitretin (second line, severe cases)
MTX (second line, severe cases)
Etanercept (third line, severe cases)
PHOTOTHERAPY (second line)
PDT (third line)
COMPLICATIONS
Skin cancer
? Haem malignancy
COURSE/PROGNOSIS
May resolve spontaneously
May persist
No clinical or histological prognostic signs
Keratolysis exfoliativa
Acquired form of non-inflammatory skin peeling
CLINICAL FEATURES
Common
Young adults
Sudden onset of discrete scaling on the palms
Initial small superficial blister-like air-filled pockets
Palms, palmar fingers
+/- feet
D/t focal separation of corneocytes from SC
Roofs of pockets rupture centrally as they expand centrifugally —> ragged rim of residual scale surrounding an irregular superficial dry erosion
Discrete areas of superficial skin peeling —> lack barrier functio —> dry and fissured esp on fingertips
No irritation
No vesicles
DDX Pompholyx Psoriasis Tinea manuum EB simplex Acral skin peeling syndrome
PATHOGENESIS
Premature corneodesmolysis
No assoc with atopy or fillagrin mutation
PREDISPOSING FACTORS Warm weather Detergents Solvents Irritants
ASSOCIATED DISEASES
Local Hyperhidrosis
IX
None
GENERAL MEASURES
Avoid contact with detergents/irritants
Avoid TCS —> exacerbate condition by further drying skin
TOPICALS
Emolients containing urea, salicylic acid
COURSE/PROGNOSIS
Self-limiting
May recur each summer
Acute epidermal distension and acute oedema blisters
CLINICAL FEATURES
Elderly susceptible
Tense unilocular non-pruritic bullae in areas of acute oedema
No itch
Lack of spread to pther areas of body
Bullae occurs as a result of rapid onset oedema
Speed of development of acute oedema most important risk factor
DDX Bullous pemphigoid (itchy) Diabetic bullae (blisters not so tense, more extensive on the lower legs, irregular in shape, not assoc with acute oedema)
PREDISPOSITING FACTORS (causes of acute onset oedema)
Attacks of angioedema
Acute CCF
ASSOCIATIONS
Acute cutaneous distension —> disrupted barrier function —> Eczema craquele
IX
Clinical dx
No specific tests
If in doubt, skin bx for histo and perilesional for DIF + anti-skin ABs to exclude autoimmune bullous disorders
HISTO
Marked epidermal spongiosis
Slight lymphohistiocytic inflammatory infiltrate
DIF neg
MX
Reduction of oedema via managing underlying disease
Aseptic puncture of individual bullae to reduce risk of ulceration
COURSE/PROGNOSIS
Blisters respond rapidly to reduction of oedema
No residual scars
Multiple minute digitate keratoses
Adults
Heterogeneous cause
CLINICAL FEATURES Sudden appearance of asymptomatic tiny flesh coloured spiny keratoses —> may be hundreds Non-follicular Trunk Proximal limbs Cases of lesions only on palms and soles
FORMS AND THEIR ASSOCIATED DISEASES
Genetic/familial -
- AD inheritance
- Becomes climically apparent in the 2nd and 3rd decades
Post inflammatory/reactive -
- Solar damage
- RTX
- Drugs i.e. acitretin, simvastatin, CSA
Sporadic cases -
- Systemic diseases
- Paraneoplastic
DDX Lichen spinulosis Phrynoderma Multiple filiform viral warts Trichodysplsia spinulosa Arsenical keratoses
IX FOR SPORADIC CASES
Age-related malignancy screen
HISTO
Discrete columns of compact hyperkeratosis arising from acanthotic inter follicular epidermis
Granular layer intact (unlike porokeratosis where thee is loss of granular layer)
Dermis normal
TOPICALS
Keratocytics esp salicylic acid, lactic acid + emolients (first line)
Retinoids + emolients + urea moisturisers (second line)
5-FU (third line)
