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Med Derm Flashcards > Cutaneous Mucinoses > Flashcards

Cutaneous Mucinoses Flashcards

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1
Q

Special stains for mucin

A

Alcian blue pH 2.5
Colloidal iron
Toluidine blue pH 4.0

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2
Q

What is associated with a Scleromyxoedema

A 
Monoclonal gammopathy (immunoglobulin G lambda)
No thyroid disease
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3
Q

What is the microscopic triad of scleromyxoedema

A

Diffuse Mucin deposition (confirmed with a mucin stain)
Fibroblast proliferation (irregularly arranged fibroblasts)
Fibrosis @ increased collagen deposition

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4
Q

Clinical features of scleromyxoedema

A

Widespread eruption 2-3mm firm waxy closely spaced dome-shaped/flat topped papules - can be itchy
Arranged linear pattern
Surrounding skin sclerodermoid (shiny and thick)
Leonine facies (deep furrowing forehead)
Shar-pei sign (deep furrowing trunk, shoulder, limbs)
Sparse eyebrow, axillary, pubic hair
Doughnut sign over PIP joints
Sclerodactyly
Rarely Raynaud’s phenomenon
Lacks telengiectasia/calcinosis (scleroderma features)
No mucosal lesions

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5
Q

DDx of scleromyxoedema and differences with scleromyxoedema

A

Scleroderma/systemic sclerosis (no papules, positive ANA, anticentromere, antiScl70)

Sclerodema (no papules, assoc URTI, diabetes)

Nephrogenic systemic fibrosis (no facial involvement/monoclonal gammopathy, renal dysfunction + exposure to gadolinium)

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6
Q

Systemic involvement of scleromyxoedema

A

Haem - 100% monoclonal gammopathy, rarely others I.e. myeloma, lymphoma etc
Neuro - carpal tunnel syndrome, peripheral neuropathy, memory loss, stroke, seizures, psychosis, gait issues, vertigo, dermatoneuro syndrome)
Rheum - Arthralgias/arthritis, myopathy, myalgia
Cardiovascular - CCF, myocardial ischaemia, heart block
Pulmonary - obstructive or restrictive
GI - dysphagia
Renal - acute RF

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7
Q

Prognosis of scleromyxoedema

A

Chronic, progressive, unpredictable course

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8
Q

Causes of death in scleromyxoedema

A

Dermatoneuro syndrome (concomitant fever, convulsions, coma)
Cardiovascular
Haem malignancies

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9
Q

Baseline investigations for scleromyxoedema

A

Skin biopsy
Serum and urine protein electrophoresis with immunofixation
TFT normal
Little value in imaging

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10
Q

Treatment ladder for scleromyxoedema

A

1st line -
IVIg 2g/kg for 5 days alone or in combo with Lenalidomide/systemic steroids - skin and extra cutaneous, esp for acute deterioration with neuro symptoms, response not permanent, maintenance infusions required every 6-8 weeks

2nd line (combined with IVIg rather than as mono therapy) -
Lenalidomide 100-400mg/day +/-
Systemic steroids

3rd line -
Autologous peripheral blood stem cell transplant
Bortezomib and dexamethesone (for recurrences and dermatoneuro syndrome)

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11
Q

Additional treatment for scleromyxoedema

A 
Medical - 
Oral retinoids
Cyclosporin 
Interferon alpha
Hydroxychloroquine

Chemo -
Melphalan
Cyclophosphamide
MTX

Physical - 
Plasmapheresis
PUVA, UVA1
Electron beam
Extracorporeal photochemo
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12
Q

Cutaneous mucinoses classification

A 
PRIMARY MUCINOSES
Dermal
- lichen myxoedematosus (scleromyxoedema, localised lichen myxoedematosus)
- reticular erythematous mucinosis
- scleroedema 
- myxoedema in thyroid diseases (pretibial myxoedema)
- papular and nodular mucinosis in CTD
- self healing cutaneous mucinosis
- cutaneous focal mucinosis
- digital myxoid cyst

Follicular

  • Pinkus follicular mucinosis
  • Urticaria-like mucinosis

SECONDARY MUCINOSES

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13
Q

Localised lichen myxoedematosus clinical features

A 
Small firm waxy papules, nodules, plaques confined to few sites
No sclerotic features
No paraproteinaemia
No systemic involvement
No association with thyroid disease
4 subtypes 
- acral persistent papular mucinosis
- papular mucinosis of infancy 
- discrete papular lichen myxoedematosus
- nodular lichen myxoedematosus
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14
Q

4 subtypes of localised lichen myxoedematosus

A

Acral persistent papular mucinosis

Papular mucinosis of infancy

Discrete papular lichen myxoedematosus

Nodular lichen myxoedematosus

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15
Q

Localised lichen myxoedematosus pathology

A

Mucin in the upper and mid reticular dermis
Variable fibroblast proliferation
Fibrosis not marked/absent
Grenz zone (acral persistent papular mucinosis)

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16
Q

Acral persistent papular mucinosis (subtype of localised lichen myxoedematosus)

A

Multiple ivory to skin coloured papules

Exclusive on dorsal hands, distal forearms

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17
Q

Discrete papular lichen myxoedematosus (subtype of localised lichen myxoedematosus)

A

Small reddish, violaceous or skin coloured papules
Trunk and limbs
Symmetrical distribution

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18
Q

Papular mucinosis of infancy (subtype of localised lichen myxoedematosus)

A

Firm opalescent papules
Neck
Trunk
Upper arms

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19
Q

Nodular lichen myxoedematosus (subtype of localised lichen myxoedematosus)

A

Multiple nodules
Mild/absent papular component
Trunk, limbs

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20
Q

Localised lichen myxoedematosus associations

A

HIV
Hep C
Exposure to toxic oil/L-tryptophan

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21
Q

DDx of localised lichen myxoedematosus

A

Granuloma annulare
Lichen amyloidosus
Lichen planus
Eruptive collagenoma

22
Q

Disease course of localised lichen myxoedematosus

A

Chronic but benign course, in the absence of systemic involvement

23
Q

Prognosis for localised lichen myxoedematosus

A

Progression to scleromyxoedema has never been proven

24
Q

Reticular erythematous mucinosis (REM) clinical features

A 
Chronic persistent macular erythema, indurated erythematous papules/plaques
Reticular configuration 
Lack of scale
Midline back, chest
Occasionally itchy 
Sometimes face, abdomen, arms, legs
25
Q

Reticular erythematous mucinosis (REM) associations

A

Generally not related to systemic diseases
Not associated with abnormal lab tests

Malignancies - haem, breast, lung, colon
HIV
Thyroid dysfunction
Diabetes
Idiopathic thrombocytopenic Purpura
SLE
26
Q

Reticular erythematous mucinosis (REM) aggravating factors

A 
OCP
Pregnancy
Menses
Heat, sweat
X-ray therapy
Role of sunlight is controversial

Family Hx

27
Q

Reticular erythematous mucinosis (REM) pathology

A

Epidermis normal
Mucin in upper dermis
Slight vascular dilatation
Perivascular (sometimes perifollicular) T cell infiltrate, with variable deep extension
DIF negative (rarely IgM, IgA, C3 at DEJ)

28
Q

DDx for reticular erythematous mucinosis (REM)

A

Tumid lupus - clinical and histo similarities, lack immune serological abnormalities, respond well to HCQ, resolve without residual lesions (tumid lupus without reticulated lesions on the midline are strongly photosensitive, higher positive DIF, higher recurrence rate, present with other lupus features)

Seb derm - central chest, scale

Pityriasis versicolor - central chest, scale

Confluent and reticulated papillomatosis of Gougerot-Carteaud (CARP) - scale

29
Q

Disease course of reticular erythematous mucinosis (REM)

A

Chronic
Prolonged duration of untreated
May clear spontaneously

30
Q

Treatment ladder for reticular erythematous mucinosis (REM)

A

First line
HCQ 400mg daily - improvement/healing within 1-2 months

Second line
TCS
Systemic steroid
Topical calcineurin inhibitors I.e. tacrolimus, pimecrolimus

Third line
Phototherapy I.e. UVA1, UVB (potential for exacerbation, but successful results reported)
PDL

Other
Oral antihistamines
Tetracycline
CSA

31
Q

Scleroedema clinical features

A

Non-pitting swelling/induration
Upper part of body (neck, shoulders, upper back) - typically begin on posterior neck, spread to scalp, shoulders, upper back
Spares hands and feet
Erythema
Peau d’orange appearance
Symmetrical, diffuse
Progressive
Limited body mobility/movement restriction
Subtypes
- Diabetic
- Non diabetic (idiopathic, with preceding febrile illness/post-strep, with monoclonal gammopathy/multiple myeloma, misc conditions I.e. HIV, internal malignancy, autoimmune disorder)

32
Q

Subtypes of scleroedema

A

Diabetic (poorly controlled insulin-dependent) - slowly progressive, non-resolving

Non diabetic

  • idiopathic
  • with preceding febrile illness/post-strep URTI (also influenza, measles, mumps, chickenpox, CMV, diphtheria, encephalitis, dental abscess) - acute onset, complete resolution 6 months-2 years
  • with monoclonal gammopathy/multiple myeloma - slowly progressive, non-resolving
  • misc conditions I.e. HIV, internal malignancy, autoimmune disorder (RA, primary biliary cirrhosis, sjogren syndrome, dermatomyositis)
33
Q

Scleroedema path

A

Normal epidermis
Thickened dermis
Swollen collagen fibres separated by wide spaces
Mucin
Subcut fat replaced by coarse collagen fibres
No fibroblast proliferation

34
Q

DDx of scleroedema

A 
Systemic sclerosis (has additional sclerodactyly, Raynaud phenomenon, nail fold capillary changes, positive serum autoantibodies)
Dermatomyositis
Other infiltrates - 
- Myxoedema (mucin)
- Amyloidosis (amyloid)
Lymphoedema
Cellulitis
35
Q

Extracutaenous complications of scleroedema

A 
Serositis 
Myositis 
Parotitis
Hepatosplenomegaly
Ocular
Cardiac
Dysphasia 
Dysarthria
36
Q

Scleroedema course/prognosis

A

Post-infectious scleroedema - benign course, self-limiting, resolves spontaneously 6 months - 2 years

Diabetes/monoclonal gammopathy associated scleroedema - chronic, little chance of remission

37
Q

Initial investigations for scleroedema

A

To detect underlying disorder

Throat swab, ASOT - recent strep infection
Fasting blood glucose, HBA1C - diabetic control
Serum, urine protein electrophoresis and immunofixation - monoclonal gammopathy, multiple myeloma
USS - evaluate skin thickness at baseline, after treatment
MRI - extent of disease progression (better than USS for this purpose)

38
Q

Treatment of scleroedema

A

Difficult, limited success
No effective treatment
Some patients (but not all) diabetes patients improve with better glucose control

First line -
Treat underlying cause
UVA1, PUVA, nb-UVB (for disabling manifestations)
Physio MSK rehab (for motion/respiratory disability)

Second line -
Electron beam radiotherapy
Extracorporeal photopheresis
IVIg

Third line (immunosuppressants) -
CSA
MTX
TCS, ILCS, pred

39
Q

Pretibial myxoedema clinical features

A

Bilateral shins, dorsum feet
Thickened and indurated skin
Orange peel appearance/texture due to prominent hair follicles
Overlying hyperhidrosis or hypertrichosis

4 variants -

  • diffuse non-pitting oedema
  • plaque type
  • nodular type
  • elephantiasis

Hyperthyroidism esp Graves’ disease, sometimes Hashimoto’s thyroiditis
Sometimes no past/present Hx of thyroid dysfunction

40
Q

Pretibial myxoedema path

A 
Hyperkeratosis
Papillomatosis
Acanthosis
Follicular plugging
Mid to lower dermis separation of collagen bundles by mucin
Perivascular lymphocytic infiltrate
Increase mast cells
Normal/increased fibroblasts
Reduced elastic fibres
41
Q

DDx for pretibial myxoedema

A

LSC
Hypertrophic lichen planus
Obesity-associated lymphoedematous mucinosis (no thyroid disease)

42
Q

Pretibial myxoedema course/prognosis

A

Elephantiasis variant less likely to have remission

Entrapment of peroneal nerve by mucinous connective tissue —> foot drop, faulty dorsiflexion

43
Q

Initial investigations for pretibial myxoedema

A

Biopsy

Anti-TSH antibodies - high

TSH - low —> high thyroid hormone to stimulate more TSH production

44
Q

Treatment of pretibial myxoedema

A

General measures -
Reduce weight
Stop smoking
Normalise thyroid function (though pretibial myxoedema can still develop after treatment started)

First line -
Medium to high potency TCS under occlusion
ILCS
Compression stockings/gradient pneumatic compression (improves lymphoedema)

Second/third line (only for elephantiasis variant) - 
Rituximab 
Extracorporeal plasmapheresis
IVIg
Octreotide
\+/- shave removal
N.B relapse after skin graft
45
Q

Papular and nodular mucinosis in connective tissue diseases features

A

Skin coloured papules, nodules, plaques
Trunk, upper extremities

Accompany/antedate connective tissue disease I.e. LE (cutaneous lupus mucinosis, usually associated with SLE but may also be associated with DLE/SCLE), dermatomyositis, scleroderma

Clinical course may/may not be related to the underlying connective tissue disease activity
Path - abundant mucin throughout dermis, sometimes subcut fat, slight/moderate perivascular lymphocytic infiltrate, positive lupus band

General measures - sunscreen
First line treatment - TCS, ILCS, HCQ
Resistant cases - Pred, oral tacrolimus

46
Q

Self-healing juvenile cutaneous mucinosis

A

13 months - 15 years old

Acute eruption of multiple papules, sometimes linear infiltrated plaques scalp, face, neck, abdomen, thighs

Subcut nodules face, periarticular areas on limbs, periorbital swelling

Systemic symptoms - fevers, arthralgias, weakness, muscle tenderness

No paraproteinaemia/bone marrow plasmocytosis/thyroid dysfunction

Spontaneous resolution few weeks - months

Avoid aggressive therapy as heals spontaneously

47
Q

Cutaneous focal mucinosis

A

Benign localised cutaneous dermal mucinosis

No systemic manifestations
Anecdotal associations - hypothyroidism, myxoedema, scleromyxoedema, REM, anti-TNF Rx

Reactive lesion
Trauma may act as a trigger
Asymptomatic solitary skin coloured papule/nodule, sometimes with cystic appearance
Anywhere except in proximity of the joints of the hands, wrists, feet

Treatment - surgical excision

Path -

  • Diffuse ill-defined dermal mucin
  • Spares subcut tissue
  • Normal/slight increase fibroblast (spindle-shaped cells)
  • No inflammation
  • No increased vascularity
  • No elastic fibres
  • Normal/hyperplastic epidermis, sometimes forming collarette

DDx - angiomyxoma (benign >1cm, subcut involvement by mucin + increased vascularity)

48
Q

Digital myxoid cyst

A

Benign ganglion cyst of digits

Translucent dome shaped soft/fluctuate nodule
+/- visible semi transparent contents
Dorsal skin/near distal interphalangeal joint
Surface smooth or verrucous
Clinical/radio graphic evidence of osteoarthritis
Grooving of the nail may precede/associate the cyst by up to 6 months
Antecedent trauma in minority of cases
MRI can denote Connection of cyst to underlying joint

2 variants -
Over joints - ganglion type derived from joint fluid and synovial cells
Between interphalangeal joints - myxomatous type derived from dermal fibroblasts

Histo -
Large deposit mucin containing stellate fibroblasts
Multiple clefts
Vascular spaces
Epidermis - atrophied centrally, acanthotic laterally
Sometimes transepidermal elimination of mucin

Treatment -
No consistently successful treatment
Excision (relapse not uncommon)
Multiple needling/aspiration followed by ILCS/cryo/sclerosant/CO2 laser (favoured as a conservative alternative to excision)

49
Q

Pinkus follicular mucinosis

A

Children, adults in 30s/40s
Familial cases ? Genetic predisposition

Acute/subacute eruption
1 or several sharply demarcated erythematous plaques with follicular prominence (more reassuring if solitary lesion on the head/neck)
Alopecia
Scaling

Differentiation with MF-associated follicular mucinosis is difficult

Histo -
Mucin within follicular epithelium, sebaceous glands abusing keratinocytes to disconnect from each other
Perifollicular infiltrate of lymphocytes, histiocytes, eosinophils
Absence of epidermotropism/atypical lymphocytes (favour MF-associated follicular mucinosis)
T cell gene rearrangement not always useful to exclude MF-associated follicular mucinosis

Treatment-
No specific Rx
Consider wait and see - many cases heal spontaneously 2-24 months
Topicals - TCS, topical calcineurin inhibitor, topical retinoids/bexarotene, imiquimod
ILCS
Systemic - Pred, isotretinoin, minocycline, dapsone, HCQ, interferon alpha-2b
Physical - PDT, UVA1

50
Q

Urticaria-like follicular mucinosis

A

Cyclic eruption of itchy urticaria-like papules/plaques
Head/face/neck of middle-aged men
Rosaceiform/seborrhoeic background skin
Red macules persist for a few weeks as lesions resolve
No follicular plugging
No alopecia
No systemic manifestations

DDx - 
Urticaria
Seb derm
Rosacea
Tumid lupus

Histo -
Mucin deposition within hair follicles
Perivascular and perifollicular infiltrate of lymphocytes and eosinophils
T-cell gene rearrangement polyclonal

Treatment -
Chronic
Difficult to treat
Systemic - HCQ, Dapsone

Med Derm Flashcards (35 decks)

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