Mucinoses, Lichen Planus and Related Conditions

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Mucinoses, Lichen Planus and Related Conditions

• Adam Wray, D.O.
• September 28, 2004

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What is Mucin?

• Fibrillar matrix in dermis called ground
  substance, created by fibroblasts.
• GS acid glycosaminoglycans (formerly called acid
  mucopolysaccharides) that bind 1000 times their
  own volume in water
• Adding sulfates to the acid GAGs creates
  chondroitin sulfate and dermatan sulfate, the 2
  primary dermal mucins

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Classification

• Primary mucinoses
• Mucin deposition is the main histologic feature
• Secondary mucinoses
• Mucin deposition is an additional finding

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HE GA Wispy bluish, feathery material between
collagen bundles
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Colloidal Iron Staining GA
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Mucin wispy, feathery
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Mucin Stains

• Colloidal Iron
• Alcian Blue
• Toluidine Blue
• Incubation of tissue in hyaluronidase eliminates
  the staining, confirming the presence of mucin

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Lichen Myxedematosus
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Lichen Myxedematosus

• Aka Papular Mucinosis
• Generalized Scleromyxedema
• Onset age 30-80
• Multiple waxy 2-4mm dome shaped or flat topped
  papules, usu. linear array
• Acral dorsal hands, face, elbows, and extensor
  extremities

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Acral Persistent Papular Mucinosis

• Subtype of localized LM
• Usually women
• Few to 100 bilaterally symmetrical 2 to 5mm,
  flesh colored papules
• Always on hands and wrists.
• Rarely elbows and knees
• No paraprotein, no systemic dz
• HE Increased mucin, but not fibroblasts

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Cutaneous Mucinosis of Infancy

• Subtype of localized LM
• 3 cases, onset birth-3 months of age
• 2-8mm papules trunk, extremities, especially
  backs of hands
• TSH, Paraprotein negative
• There are neither systemic symptoms nor
  spontaneous resolution
• HE papillary dermal mucin, no fibroblasts

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• Histopathology of lichen myxedematosus (discrete
  type). Mucin deposits splay collagen bundles in
  the dermis, but there is only slight fibroblast
  proliferation and no sclerosis.

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• Lichen myxedematosus of the discrete type
  demonstrating mucin accumulation in the dermis.
  Alcian blue stain (pH 2.5).

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Scleromyxedema

• Woody, fibrous sclerosis of skin
• Lesions coalesce to form leonine facies
• Decreased ROM of hands, lips and extremities
• Dysphagia is most common GI symptom

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Scleromyxedema Systemic

• Pulmonary
• Proximal Muscle weakness
• Carpal Tunnel Synd.
• Peripheral Neurop.
• Raynauds
• IgG lambda paraproteinemia
• Myeloma

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Scleromyxedema

• Widespread eruption of 2-3 mm, firm, waxy,
  closely spaced papules
• Papules often arranged in a linear array
• Surrounding skin is shiny and indurated
  (sclerodermoid)
• Doughnut sign central depression surrounded
  by an elevated rim (due to skin thickening) is
  seen on the PIP joints.

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Scleromyxedema Histopathology

• Triad
• Diffuse deposit of mucin in the upper and
  mid-reticular dermis
• Increase in collagen deposition
• Marked proliferation of irregularly arranged
  fibroblasts

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Increased fibroblasts and mucin in the reticular
dermis
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Differential diagnosis

• Granuloma annulare
• Lichen amyloidosis
• Lichen planus
• Eruptive collagenoma
• Systemic scleroderma
• Scleredema

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Treatment

• Oral retinoids
• Systemic corticosteroids
• Extracorporeal photochemotherapy
• Melaphan
• Topical and intralesional hyaluronidase
• Electron-beam radiation
• Plasmapharesis
• PUVA
• Dermabrasion
• Topical DMSO
• Overall prognosis is poor.

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Papular Mucinosis and AIDS

• Widespread
• Not associated with a paraprotein
• Always seen in advanced HIV disease
• If assoc. with eczematoid dermatitis, clears when
  eczema clears
• Oral retinoids

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Scleredema

• Stiffening and hardening of the subcutaneous
  tissues as if they were infiltrated with
  paraffin.
• With DM II (men) without DM II (women)
• Begins on the neck, may spread symmetrically to
  arms, shoulders and back
• Skin waxy, wood-like.
• Demarcation poor if no DM, good if DM

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Scleredema without DM II

• FgtM 21, Poorly demarcated edges
• 25 Streptococcal infection
• Paraproteinemia usually IgG, Myeloma
• Cardiac arrhythmias.
• Upper GI dysphagia, tongue involvement
• Pleural, pericardial or peritoneal effusion
• If infection precedes it, 50 resolve 2-3 yrs.

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Scleredema with DM II

• Most common, well demarcated edges
• MgtF 101, usually obese
• Sharp step off at lesion edge
• Persistent erythema folliculitis possible
• Controlling DM II has no effect on skin
• No visceral disease or paraprotein

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Scleredema Clinical Photo
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Treatment

• PUVA
• Pulse therapy with cyclophosphamide and
  corticosteroids
• Electron-beam therapy
• Cyclosporine
• Therapy has little benefit, however
• Systemic and intralesional steroids,
  intralesional hyaluronidase, MTX, antibx, and
  penicillamine have not proved helpful

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REM Syndrome

• Reticular Erythematous Mucinosis
• Aka Plaque-like Cutaneous Mucinosis
• Women, 3rd -4th decades of life
• Frequently follows intense sun exposure, and
  often pregnancy, OCPs, menses
• DIF, ANA negative
• MC midline chest back
• Reticulate or plaque-like appearance
• Antimalarials often helpful

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REM SYNDROME
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Cutaneous Focal Mucinosis

• Benign, symptomless, skin colored solitary
  lesions less than 1 cm
• Resemble neurofibromas, cyst, BCC, and
  angiomyxoma
• Can occur anywhere on the the body, except over
  the joints of the hands and feet
• Adulthood
• Histopath mucin throughout upper and mid-dermis
  sparing subQ fat. Cleft like spaces, but no
  cysts, are seen. Numerous vimentin-positive
  dendritic shaped fibroblasts are seen
• Tx Surgical excision

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Self-healing Juvenile Cutaneous Mucinosis

• Sudden onset, age 5 to 15
• Skin lesions and polyarthritis x a few mos.
• Ivory white papules head, neck, trunk and
  periarticular
• Hard edema of periorbital area/face
• TSH, IgG normal
• HE dermal mucin
• Prognosis excellent

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Follicular Mucinosis

• Aka Alopecia Mucinosa, 3 groups
• MC young, head, neck arms, resolves in 2 mos to 2
  years
• 2nd group, larger more numerous lesions, takes
  several years to resolve
• 3rd group, older, CTLC
• 30 of FM patients have MF
• Any patient gt30 yo with FM, look for MF

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Follicular papules merging into a scaly plaque
with alopecia
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MF associated follicular mucinosis HE asterisks
mark large collections of mucin within cells of
the sebaceous gland and outer root sheath with
mixed dermal infiltrate
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• Treatments for Follicular Mucinosis after you
  have ruled out MF
• Topical or oral corticosteroids
• Dapsone
• PUVA, Radiation therapy
• Interferon alfa-2b
• Mepacrine
• Indomethacin
• Minocycline
• Oral isotretinoin

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Myxoid Cysts

• MC terminal dorsal or lateral fingers
• Solitary 5-7mm opalescent or skin colored
  asymptomatic swellings of proximal nail fold or
  DIP
• NO CYSTIC LINING, not a true cyst
• Synovial origin still debated
• TX ID, Cryo, IL steroids, Fulguration of the
  base after draining. Meticulous excision
  including stalk by Salasche in 11 pts, no recur.

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• Groove of nail plate secondary to lesional
  pressure to the nail matrix

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Case of dermatomyositis presenting with cutaneous
mucinosis as the sole manifestation. A malignancy
screen revealed an underlying nasopharyngeal
carcinoma. (J Am Acad Dermatol 200348S41-2.)
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Lichen Planus

• A disease of the skin, nails, hair follicles and
  mucous membranes.
• Mnemonic the 5 Ps
• Wickhams Striae, flat topped papules
• Pruritis is paroxysmal, rubbing gt scratching
• Flexor wrists, trunk, medial thighs, shins,
  dorsal hands, glans penis

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Plentiful Purple Pruritic Polygonal Papules
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Wickhams striae gray or white puncta or streaks
that cross the lesion
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5-10 of LP cases have nail changes including
pterygium, longitudinal grooving, onycholysis,
splitting and peculiar midline fissure.
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• Oral LP
• Ulcerative
• Reticulate
• Atrophic
• Risk of SCC
• 1/200 cutaneous LP pts develop oral SCC
• 1/100 oral LP pts develop SCC over 3 year period
• Mouth, anus are risk areas too.

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A - Annular LP most commonly found on lips or
penis. Central hyperpigmentation is
characteristic
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Bullous LP Usually shins, as individual lesions
merge, they vesiculate centrally. Often
resolves spontaneously
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Lichen Planus Pemphigoides

• Rare
• Typical LP patients who experience bullae on top
  of their LP plaques
• DIF positive for 180kd BPAg2 antigen
• Treat like Bullous Pemphigoid

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Hypertrophic LP

• AKA LP verrucosus
• Extremely pruritic
• Usually symmetric
• Chronic venous stasis frequently contributes to
  this condition
• SCC, which must be distinguished from
  pseudoepitheliomatous hyperplasia, has been
  reported to arise within these lesions

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Hypertrophic LP
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Linear LP Aka Zosteriform 1 of LP
cases Blaschkos lines, not dermatomal
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Hepatitis C

• Prevalence in LP pts between 4 and 38
• Japan Hep C 8, LP w/ HepC 60
• LP patients with Hep C are more likely to have
  erosive mucous membrane dz.
• TX of Hep C w/ Alpha INF may induce LP.
• Primary biliary cirrhosis LP may coexist ?
  risk of lichenoid rxn to D-Penicillamine

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Lichen Planopilaris - scalp only Graham-Little-Pic
ardi-Lasseur Syndrome - scalp hair-bearing body
sites Females gt Males 41 Tufts of normal hair
appear in cicatricial areas of alopecia Perifollic
ular erythema and keratotic spiny papules Tx
Difficult, IL or oral steroids, antimalarials.
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Interface dermatitis with saw-tooth pattern of
epidermal hyperplasia together with
orthokeratosis and hyper-granulosis. Basal layer
is lost or squamatized with vacuolar interface
change, necrotic keratinocytes. Band-like
infiltrate in the papillary dermis. Civatte
bodies are necrotic keratinocytes in the dermis
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Hyperkeratosis, irregular (saw-tooth) acanthosis,
dyskeratosis, basal vacuolization, and a
band-like infiltrate of mononuclear cells
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LICHEN PLANOPILARIS
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LP Pathology

• Hypertrophic LP LP LSC
• LPP and GLPL LP histology centered on
  follicular epithelium
• Civatte bodies reveal IgM on DIF
• Lichenoid drug reaction may mimic LP. Look for
  eosinophils, photodistribution

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Lichen Planus Treatment

• Prednisone, Isotretinoin
• PUVA, LMW Heparin, Cyclosporine
• Oral potent topical steroids in Orabase w/ vinyl
  dental tray TID and 20 mins qHS
• Plaquenil 400mg daily
• Inhaled forms of corticosteroid are sometimes
  helpful for oral LP
• Vaginal mix steroids with Replens (bioadhesive
  moisurizer)

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Lichen Planus Actinicus

• MC Africa, Middle East, Indian, Asians
• MC Spring, Summer, Quiescent in Winter
• Photodistributed
• Mild or no pruritis
• Hyperpigmented macules or plaques with the
  blue-gray tinge of dermal melanin
• May resemble Melasma

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Erythema Dyschromicum Perstans
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Erythema Dyschromicum Perstans

• Onset before age 40.
• Chronic generalized, symmetrical
• Various sizes shapes, ashy-gray macules,
  sometimes with a palpable non-scaling border.
  Feels like a small cord
• Pruritus is not reported
• Nail and mucosal involvement is not found

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Erythema dyschromicum perstans

• Occasionally there is a peripheral rim of
  erythema
• As in pityriasis rosea, the long axis of oval
  lesions can follow skin cleavage lines
• Histology lichenoid infiltrate with pigment
  laden macrophages in the dermis

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EDP Treatment

• Sun protection
• Topical retinoids
• Topical vitamin C
• Chemical peels
• Oral antibx
• Oral vitamin A

• Dapsone
• Antimalarials
• Griseofulvin
• Anecdotal report with clofazamine
• Systemic and topical steroids

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Lichenoid Contact Dermatitis

• Dorsal hands - Paraphenylenediamine (color film)
• Oral due to Amalgan fillings
• Patch testing recommended

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Keratosis Lichenoides Chronica

• Rare, acral buttocks, onset childhood
• Violaceous papulonodular, hyperkeratotic lesions
  covered with gray scales discrete or may
  coalesce to form reticulate or linear arrays,
  keratotic plugs and prominent telangiectasias
• Associated facial seb derm pattern
• Nail thickening, longitudinal ridging,
  onycholysis, warty periungual lesions
• Painful oral apthae, keratoconjunctivitis
• Tx symptomatic usually unsatisfactory

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Keratosis Lichenoides Chronica
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Lichen Nitidus
Minute, shiny, flat-topped, pale, exquisitely
discrete, uniform papules
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Lichen Nitidus HE
CHARACTERISTIC PATH EPIDERMIS GRASPS
LYMPHOHISTIOCYTIC INFILTRATE WITH CLAW-LIKE
COLLARETTE
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LICHEN NITIDUS
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• Lichen Nitidus
• Linear Koebnerization
• Lower abdomen and penis, inner thighs, flexor
  wrists and forearms
• Actinic Lichen Nitidis in black, Middle Eastern
  and Indian subcontinent
• Slowly progressive with tendency to remissions
• Tx Top. Steroids, PUVA, DNCB, Oral Retinoids

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Lichen Striatus

• Common, Linear, erythematous or hypopigmented
  papules, scaly, asymptomatic, follow Blaschkos
  lines, nails may be involved
• Children age 3, females gt males
• Active lesions last for months, then resolve
• Histology varies, lichenoid or spongiotic,
  possibly even granulomatous. Dense eccrine
  infiltrate helps differentiate from LP

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Lichen Sclerosis et Atrophicus

• Females gt Males, Itching may be severe
• Anogenital hourglass or figure eight with
  dyspareunia
• Glans penis (balanitis xerotica obliterans)
• Chest, breasts, back, oral mucosa, tongue
• Histology may have features of morphea
• Genital SCC risk higher than general population,
  but lifetime risk is lt 5

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LSA IN A PERIUMBILICAL SKIN FOLD
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LSA Etiology

• Unknown.
• Borrelia burgdorferi assoc. Japan, Europe
• 20 of pts have autoimmune disease, usually
  vitiligo, alopecia areata or thyroid

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LSA Treatment

• Steroids (clobetasol)
• Topical testosterone (emollient)
• Topical retinoids
• CO2 laser ablation
• Excisional bx
• Oral calcitriol (used in morphea and scleroderma)
• Pimecrolimus/tacrolimus
• PDT
• Topical estrogens

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Childhood LSA

• Childhood onset in 10-15, usually genital
• Boys present with phimosis, circumcision may
  improve/resolve
• Girls present with pain with defecation, dysuria
  and 50 have spontaneous resolution

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LSA Histology
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LATE LSA RETE RIDGES EFFACED, EDEMA FADED,
WASHED OUT LOOK, TELANGIECTATIC VESSELS
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• JAAD, Volume 48, Number 6 Pages 935-937,June
  2003Tacrolimus ointment for the treatment of
  vulvar lichen sclerosis.
• Assmann T, Becker-Wegerich P, Grewe M, Megahed M,
  Ruzicka T.
• Department of Dermatology,Heinrich-Heine-Universi
  ty,Düsseldorf, Germany.
• The treatment of vulvar lichen sclerosus is
  generally considered difficult. Ultrapotent
  corticosteroids represent the most effective
  topical treatment, but carry the risk of side
  effects such as skin atrophy. We describe a
  71-year-old woman with long-standing vulvar
  lichen sclerosus refractory to conventional
  treatment. After 6 consecutive weeks of treatment
  with tacrolimus ointment 0.1 (Protopic) twice
  daily, signs and symptoms of lichen sclerosus
  resolved. To our knowledge, this is the first
  report of the use of topical tacrolimus, which
  does not induce skin atrophy, in the treatment of
  vulvar lichen sclerosus.
• PMID 12789187 PubMed - indexed for MEDLINE

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The End