last authored:
last reviewed:
Prurutic, purple, polygonal papules
General self-limiting and resolves spontaneously 1-2 years after onset, often leaving zones of postinflammatory hyperpigmentation.
Oral lesions may last for years.
Pathogenesis is not well known, though may be related to cell-mediated immune reactions following antigenic release at levels of basal cell layer and dermoepidermal junction.
Malginancy emerging from lichen planus areas has been observed, but direct pathogenic relationship is not clear.
Dermomyositis is an autoimmune connective tissue disease of unkown cause characterized by inflammation of muscle and skin. adult and juvenile forms.
may be paraneoplastic, especially later in life: 20-30% chance of malignancy
characteristic skin findings
proximal muscle weakeness
elevated serum levels of muscle enzymes
abnormal muscle biopsy
abnormal electromyogram
Elderly people
tense blisters, can be hemorrhagic
autoimmune: IgG, C3l subepidermal blisters
Negative Nikolsy's sign
usually very itchy at first
oral steroids; can use lower doses, ie 0.25-0.5 mg/kg
can also use other immunosuppressants
can also use topical steroids
treat with vitamin D and bisphosphonate
Often diagnosis is profoundly delayed
INTENSE pruitus
almost everyone has mild celiac disease
Elbows, knees, lumbar spine
Tissue transglutaminase is almost always positive
autoimmune IgA mediated
neutrophils
Refractory to steroids.
Dapsone 100mg po OD, works within 24 hours. Can cause leukopenia, hypersensivity, and peripheral neuropathy (long term use).
Gluten-free diet takes 6 monhts.
Drug-induced. Stephens-Johnsons Sundrome
Sulfa, NASIDs
less than 10% blistering
EN is the most common form of panniculitis, inflammation affecting subcutaneous fat lobules or the connective etissue separating them.
Subcutaneous non-ulcerative nodules are present on extensor surfaces - usually the shins but sometimes the arms.
They are:
fever, malaise, and joint pain may accompany.
Over weeks, lesions usually flatten and become bruise-like, leaving no residual scar.
Many times the causes cannot be identified.
Erythema nodosum may be caused by various agents:
treat symptoms with NSAIDs
Also known as [prickly heat'.
Obstruction of sweat, as in miliaria crystalina, but located in the mid-epidermis.
It worsens with creams/ointments, with heat (overdressing etc).
Due to excessive scratching/picking
Morgellons
group of autoimmune disorders characterized by intraepidermal blister formation
Beofre glucocorticoids, disease was uniformly fatal. Still signigicant morbidity and mortality.
take edge of blister for histology, and para-blister region for immunofluorescence
Glucocorticoid treatment is tarted early, with good control
Characteristic eruption of pseudovesicular papules and plaques on face, upper
extremities and trunk with
Drug-induced.
More than 30% of skin blixyered.
Mortality in TEN
very painful
sheetlike loss of skin
genetic predisposition
allopurinol
anticonvulsants: phenytoin, lamotrgine, carbamazepine, phenobarbital
sulfa
NSAIDs
stop offending drug
IV IgG
admit to burn unit