Dermatology
Mucinoses
Built from Dermatology, 5th Edition
What’s inside
12 sections · 129 slides
What is a cutaneous mucinosis
What mucin is, why it builds up, how it is detected, and how the mucinoses are classified.
- What mucin actually is
- Why mucin builds up
- Spotting mucin under the microscope
- Staining characteristics of acid glycosaminoglycans (mucopolysaccharides)
- Two families of glycosaminoglycan
- Primary versus secondary mucinosis
- Two shapes of primary mucinosis
- The three branches at a glance
- Reading the classification table
- Classification of the primary cutaneous mucinoses
- Classification of the primary cutaneous mucinoses (continued)
- Approach to the patient with suspected primary dermal mucinosis
Scleromyxedema
A generalized papular disease with sclerodermoid skin, almost always tied to a monoclonal gammopathy, that can turn systemic.
- What scleromyxedema is
- Telling it apart from localized disease
- History
- Epidemiology and cause
- Cytokines and possible triggers
- Clinical picture: the papules
- Linear papules of scleromyxedema
- Two named skin signs
- Leonine facies and the Shar-Pei sign in scleromyxedema
- More clinical features
- Linear papules on the neck and upper back in scleromyxedema
- Deeply furrowed "elephant-like" trunk skin in scleromyxedema
- Hyperpigmented linear papules in scleromyxedema
- Paraproteinemia and the bone marrow
- Systemic (whole-body) involvement
- Dermato-neuro syndrome
- Pathology: the diagnostic triad
- Fibrosis, fibroblast proliferation, and mucin in scleromyxedema
- More pathology detail
- Differential diagnosis
- Comparing scleromyxedema to localized disease
- Diagnostic criteria of scleromyxedema versus localized variants of lichen myxedematosus
- Leonine facies across different skin diseases
- Treatment: first steps
- IVIg and modern therapy
- Advanced and future therapy
- Recognizing a medical emergency
Localized lichen myxedematosus
Skin-only variants of papular mucinosis, without sclerosis, paraproteinemia, or systemic disease.
- What makes it different from scleromyxedema
- Four subtypes
- Discrete papular lichen myxedematosus
- Acral persistent papular mucinosis
- Cutaneous mucinosis of infancy
- Nodular and atypical tuberous forms
- Localized disease in HIV infection
- Toxic-syndrome associated forms
- Hepatitis C, drugs, and trauma
- Atypical or overlap forms
- Pathology of the localized forms
- Differential diagnosis
- Treatment
Self-healing cutaneous mucinosis
A distinct, rare mucinosis that resolves on its own, mostly seen in children.
- A distinct, resolving disease
- How it presents
- Pathology and follow-up
Scleredema
A symmetric hardening of the upper body's skin, in post-infectious, chronic, and diabetes-associated forms.
- What scleredema is
- History
- Who gets it, and why
- Type 1: post-infectious
- Type 2: chronic, paraprotein-linked
- Type 3: diabetes-associated
- Systemic associations
- Pathology
- Fenestrated dermis and mucin deposits in scleredema
- Differential diagnosis
- Treatment
- Other tried treatments
Thyroid-associated mucinoses
How Graves disease and hypothyroidism each produce a distinct pattern of cutaneous mucin deposition.
- Localized (pretibial) myxedema: definition
- Epidemiology
- What drives it
- Clinical appearance
- Pretibial myxedema with eye involvement in Graves disease
- Complications and pathology
- Differential diagnosis
- Treatment
- More on treatment and course
- Generalized myxedema: definition and cause
- Congenital hypothyroidism
- Juvenile and adult hypothyroidism
- Clinical picture of adult hypothyroidism
- Systemic features and diagnosis
- Treatment
Reticular erythematous mucinosis
Persistent pink, net-like plaques on the midline back or chest, overlapping with lupus erythematosus tumidus.
- What reticular erythematous mucinosis is
- History and epidemiology
- Triggers and pathogenesis
- Clinical features
- Reticular erythematous mucinosis on the central chest and abdomen
- Reticular erythematous mucinosis with pink plaques
- Annular reticulated plaques in reticular erythematous mucinosis
- Pathology
- Differential diagnosis
- Treatment
Mucinosis in autoimmune disease and focal mucinosis
Papulonodular mucinosis linked to lupus, dermatomyositis, and systemic sclerosis, plus the reactive lesion cutaneous focal mucinosis.
- Mucinosis linked to lupus and other CTDs
- Clinical picture
- Course and systemic link
- Pathology and other CTD links
- Treatment
- Cutaneous focal mucinosis
- Telling it apart from true tumors
Primary follicular mucinoses
When mucin builds up inside the hair follicle instead of the dermis - a benign form, and one that can shadow a lymphoma.
- Follicular mucin: two primary forms
- Follicular mucinosis: background
- Primary (benign) clinical form
- The other, secondary-leaning type
- Scaly plaques of follicular mucinosis with hair loss
- Facial plaques of follicular mucinosis in a child
- Pathology
- Telling primary from lymphoma-associated
- Treatment
- Urticaria-like follicular mucinosis
- Pathology and treatment
Hamartomatous-neoplastic mucinoses
Two rare growths where mucin itself is the defining feature: mucinous nevus and superficial (angio)myxoma.
- When mucin defines a growth, not a disease
- Mucinous nevus
- Superficial (angio)myxoma
- Carney complex and pathology
- Distinguishing myxoma from its mimics
Secondary cutaneous mucinoses
When mucin is only an extra finding, a clue hiding inside an already-named disease rather than the disease itself.
- What counts as a secondary mucinosis
- Major disorders associated with histologic deposition of mucin (secondary mucinoses)
- Major disorders associated with histologic deposition of mucin (secondary mucinoses) (continued)
- Reading the secondary mucinosis table
Key takeaways
Pulling the primary and secondary mucinoses together.
- Key takeaways
- Key takeaways, continued
- References
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- Dermatology, 5th Edition (2-Volume Set)