Tutorial 10

 

Topics discussed in this Tutorial

The development of lesions

Vasculitis as plaques, nodules, ulcers and guttate

Lichen planus erythematosus

The DD of white macules

Parakeratosis variegata

Various ichthyotic skin syndromes

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The development of lesions

        The early phases of various diseases and the pathophysiology involved. Note it usually depends on severity and time.

Vasculitis under the headings of
systemic features
plaques
nodules
ulcers and
guttate
all due to damage to blood vessels but is a matter of degree and time.

Panniculitis drug reaction

Vasculitis

Vasculitis legs

Note the earliest feature may be urticaria.
Acute vasculitis gives rise to vesicles, bullae, haemorrhage, necrosis and ulcers.
Subacute to papules, urticaria, plaques and macules and
Chronic to nodules.
(a)        Systemic, mainly hypersensitivity angiitis, which is usually monomorphic with rapid onset and death
(b)        Polyarteritis nodosa
(c)        Henoch-Schloenlein, and
(d)        Other syndromes, for example rheumatoid arthritis, lupus erythematosus and scleroderma.

Plaques Granuloma faciale, erythema elevatum diutinum, Sweet's Syndrome and systemic lupus erythematosus.

Nodules Nodular vasculitis, erythrocyanosis and cold, erythema induratum, erythema nodosum, erythema nodosum migrans and cutaneous polyarteritis nodosa.

Ulcers Livedo with ulcers, lymphomatoid papulosis, lymphomatoid granulomatosis, papulo necrotic tuberculid, midline granuloma and Wegner's.

Guttate Pityriasis lichenoides and malignant atrophic papulosis.

These pictures can all be seen with leukocytoclastic vasculitis. Lymphocytic vasculitis is usually seen with the more chronic diseases such as pityriasis lichenoides and lymphomatoid papulosis and perniosis.
GlobalSkinAtlas     PubMed   eMedicine

The Development of Lesions

Morphology is really a manifestation of severity and time. For example, lichen planus initially gives rise to dilated vessels with a sluggish blood flow, basal cell damage and epidermal reaction and pigmentation. Hence, erythema, bluish, oedema, bullae. Papules nodules if chronic and heals with pigmentation. Bullae occur early and are part of an acute reaction whereas nodules are part of a chronic reaction and late.

Lichen Planus Erythematosus

This is a more erythematous variant of lichen planus with cherry red papules on the forearms and legs, usually in women. The papules sometimes are confluent, there are no Wickham striae. There may be a suggestion of atrophy in some of them. On pressure the red colour goes but a brownish stain underneath remains. Note well that these lesions are symptomless. They are not itchy like lichen planus. They may persist for an indefinite period.
There is also an overlap syndrome showing features of lichen planus and lupus erythematosus which also goes under this name. See this article.

Compare lichen planus with lichen sclerosus atrophicus.
In the latter there are red papules then white papules due to oedema, then bullae or blisters, then perhaps hemorrhage into weakened skin.

Other causes of white macules include Dagos's Disease, Darier's Disease, lichen sclerosis et atrophicus and albo-papuloid variant of epidermolysis bullosa. In lichen sclerosis there are bullae early with erosions and purpura later. Note its major characteristics of its location and the follicular plugging seen with it. Also there is no post inflammatory hyperpigmentation in lichen sclerosis et atrophicus. Follicles may be accentuated in an area of palor because of their different blood supply, hence they look redder.

Other disorders covered

Poikiloderma vasculare atrophicans. In this condition you can see atrophy, scaling with underlying erythema, telangiectasia and both hypo and hyperpigmentation.

Parakeratosis Variegata Try This Link

You get striped areas of scaly erythema forming a zebra like pattern on the trunk, shoulders and thighs. There may be an increased incidence of lymphoma associated with it.
Dermatology is plagued with different names for essentially the same condition!

SYNDROME        CLINICAL FEATURES        SKIN FINDINGS        DEFECT

Conradi-Hünermann disease (chondrodysplasia punctata)        Chondrodysplasia punctata, limb defects, cataracts, cardiovascular and renal abnormalities, mental retardation

        
Congenital ichthyosiform erythroderma (CIE), whorled hyperpigmentation, palmoplantar keratoderma        Sterol isomerase emopamil–binding protein

CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome        Hemidysplasia and limb defects with sharp midline demarcation        CIE        NAD(P)H steroid dehydrogenase–like protein

Sjögren-Larsson syndrome        Spasticity, mental retardation, retinal degeneration        Lamellar scales        Fatty aldehyde dehydrogenase

Chanarin-Dorfman syndrome (neutral lipid storage disease)        Fatty liver, myopathy, cataracts, deafness, CNS defects        CIE        Impaired long-chain fatty acid oxidation

Netherton’s syndrome        Trichorrhexis invaginata (bamboo hairs), atopy, aminoaciduria        Ichthyosis linearis circumflexa        SPINK5 gene

Trichothiodystrophy (PIBIDS)        Photosensitivity, ichthyosis, brittle hair, intellectual impairment, decreased fertility, short stature        CIE        Xeroderma pigmentosa D or B gene

KID (keratitis-ichthyosis-deafness) syndrome        Keratitis, neurosensory deafness, alopecia        Grainy, spiculated scaling        Connexin 26 gene

Below images of the double scale and skin lesions of Netherton's syndrome

Below image of Sjogren Larsson disease