Pigmented Purpuric Dermatoses + Misc Purpura Flashcards
What is a pigmented purpuric dermatoses?
A group of diseases
Characterised by petechial haemorrhage
Thought to be secondary to capillaritis
No systemic features
Which blood vessels to pigmented purpuric dermatoses affect?
Superficial papillary dermal vessels (usually capillaries)
List the subtypes of pigmented purpuric dermatoses (7)
Shamberg Disease
Purpura annularis telangectodes of Majocchi
Pigmented purpuric lichenoid dermatitis of Gougerot and Blum
Eczematoid like purpura of Doucas and Kapetanakis
Lichen aureus
Linear pigmented purpura
Granulomatous pigmetned purpura
What is Schamberg disease? What are the clinical features?
The most common pigmented purpuric dermatoses
Normally in middle aged / older men.
Favours the lower legs. Also thighs, buttocks, trunk, arms.
Yellow / Brown patches with an oval irregular outline
Pinpoint petechiae within the patches - “cayenne pepper”
What is pupura annularis telangectodes of Majochhi? what are the clinical features?
A pigmented purpuric dermatoses
Occurs in adolescents and young adults (women)
Occurs on the trunk
1 - 3cm annular plaques that slowly expand
Punctate petechiae and petechiae within the plaques
What is a pigmented purpuric lichenoid dermatisis of Gougerot and Blum? what are the clinical features?
a RARE pigmented purpuric dermatoses
occurs in middle age men on the LLs
Admixture of
- yellow - brown cayenne pepper lesions
- purpuric / red / brown lichenoid papules
What is Eczematoid like purpura of Doucas and Kapeanakis?
A pigmented purpuric dermatoses
Occurs in middle age / older men
LLs
Scaly petechial or purpuric macules, papules and patches
What is lichen aureus?
A pigmented purpuric dermatoses
Rare
Solitary patch
Golden / Rust to purple / brown
Over a perforator vein
What is linear pigmented purpura
A pigmented purpuric dermatoses
occuring in a linear array, normally on a single extremity
Can occur in children
What is granulatous pigmented purpura
A pigmented purpuric dermatoses
Very rare
Brown patches with superimposed haemorrhagic papules on the LLs
What is the expected histology findings for a pigmented purpuric dermatoses
Red cell extravasation
Endothelial cell swelling
Haemosiderin containing macrophages
A perivascular lymphocytic infiltrate
+ lichenoid infiltrate in PP lichenoid dermatisis of gougerot and blum and lichen aureus
+ epidermal spongiosis and parakertosis in Lichen aureus and Eczematid - like purpura of Doucas and Kapetanakis
what is the treatment of a pigmented purpuric dermatoses
Topical CS if itchy
Otherwise minimal evidence for
- nbUVA
- PUVA
- ascorbic acid (500mg BD)
- rutoside (50mg BD)
What is Hypergammaglobulinic purpura of Waldenstrom?
Also called recurrent macular vasculitis in hypergammaglobulinaemia
A condition characterised by
- Recurrent crops of petichiae and larger purpuric macules on the lower extremitis
- Ass with pruritis / burning / stinging
Ass with
- hypergammaglobulinaemia
+/- elevated IgA or IgG RF (not IgM so not picked up on standard rheum test)
+/- anti SSA/Ro or anti SSB/La
Often in women
Sometimes ass with AI-CTD (Sjrojens > RA and lupus)
Rarely ass with monoclonal gammopathy / lymphoma / myeloproliferative disorders
Expected histo and lab findings in Hypergammaglobulinaemic purpura of Waldenstrom?
Bloods:
- Hypergammaglobulinaeamia
- Elevated ESR
- Elevated IgG or IgA RF (not a standard pannel)
- Anti SSA/Ro
- Anti SSB / La
Histo:
- Simple haemorrhage
- Mild perivascular lymphocytic infiltrate
What is the treatment for Hypergammaglobulinaemic purpura of Waldenstrom?
Primary
- support stockings (can exacerbate)
- aspirin (can exacerbate)
- avoid precipitating factors (alcohol, prolonged standing)
Secondary
- Rx underlying AI conditions
What is Gardner Diamond Syndrome?
Factitial disorder that typically affects women with an underlying psychiatric illness
Characterised by
- Painful, swollen ecchymoses
- observed at sites of trauma
- usually resolve within a 2-week period and then typically recur.
What is Onylai?
A condition that presents with haemorrhagic blisters in the mouth
Due to consumption of Sorghum grains containated with Epicoccum Sorphinum (fungus)
This fungus inhibits platelet aggregation and angiogenesis
What is Mondor disease?
A superficial thrombophlebitis affecting the anteriorlateral thoracoabdominal wall
Characterised buy sudden onset pain, followed by the appearance of palpable cords
Usually unilateral
What causes Mondor disease?
trauma,
excessive physical activity,
a surgical procedure on the breast,
mastitis,
a breast abscess
pendulous breasts
Up to 10% of patients with Mondor syndrome have an underlying breast carcinoma.
What is a paroxysmal finger hematoma?
Recurrent acute episodes of painful echymoses on the palmar surface of ones fingers
Normally in middle age women
Ix are negative
No specifc treatment
Resolves within 2 weeks
