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Topics covered in this tutorial
1. Linear papules
2. Follicular papules
3. Erythema and papules
4. Pigmented papules
Questions on the following conditions-
Kyrles disease
Pityriasis rubra pilaris
Urticaria pigmentosa
Fabrys disease
Follicular mucinoses
Degos disease
Polyarteritis nodosa
Follicular Papules
Keratosis pilaris, lichen spinulosus, lichen planopilaris, pityriasis rubra pilaris, follicular mucinosis, lichen sclerosis et atrophicus and discoid lupus erythematosus.
Using the SIGNDIP and the mnemonic NIGL, N is for nutritional particularly vitamin A, vitamin C and essential fatty acid deficiencies, I is for infiltrates, G is for the genodermatoses particularly Darier's, Downs syndrome and Kyrle's disease and L is lichenoid.
A Spiny eruption on the neck
Pityriasis Rubra Pilaris
Red slightly scaly papules are a fundamental feature of pityriasis rubra pilaris. They are usually perifollicular. They occur initially on the backs of the hands and the neck but gradually spread and coalesce. Islands of sparing within affected areas are a characteristic feature. The disease is classified in respect to its onset in childhood or in adulthood and as to whether it is familial or acquired. Childhood cases are rare.
eMedicine-Pityriasis Rubra Pilaris GSA-Pityriasis Rubra Pilaris
A spiny eruption on the neck
The photo below is of a case of pityriasis rubra pilaris
Question 1. How common are the juvenile onset forms of PRP?
Question 2. Which vitamin deficiency will give similar morphological lesions as PRP?
Answer 1. The three types of juvenile forms account for about 40% of cases with a poor prognosis for involution, hence the highest incidences for PRP are in the first five years of life or between the ages of 50 and 55.
2. Vitamin A deficiecy
Linear Papules
These are best differentiated as those showing the Koebner effect and then linear developmental, linear contact and linear anatomical. These include contact dermatitis, Darier,s disease, Epidermal nevi, Focal dermal hypoplasia, Herpes zoster, Incontinentia pigmenti, Lichen striatus, ILVEN,and also Papular Mucinosis, Psoriasis, Sarcoidosis, Warts and Molluscum contagiosum.
Linear warty lesions in a child Pruritic linear eruption in a child Linear atrophy,telangiectasia and soft nodules along the lines of Blaschko
The photo below is an ILVEN.
Pigmented Papules
Urticaria pigmentosa, xeroderma pigmentosum (papules in light exposed and photo damaged areas), fibrous papule of the nose, this may be on the tongue as well and Gottron's papules. The others include lichen planus, melanoma, lymphoma, leukaemia, Sweet's syndrome, mycosis fungoides, Kaposi's sarcoma.
Urticaria Pigmentosa (Mastocytosis)
Urticaria Pigmentosa is a proliferation of mast cells in the skin giving hyperpigmented macules of varying sizes which urticate on pressure or rubbing. Other accumulations of mast cells may give macules, papules, nodules, plaques, vesicles or bullae as presenting features and sometimes generalised telangiectasia. Occasionally there will be virtually no skin lesions although biopsies may show high numbers of mast cells. Cases that come on in childhood are likely to spontaneously involute, often by the age of 5 to 8 years old.
eMedicine-Mastocytosis GSA-Mastocytosis
Inguinal pigmented papules
The photo below is urticaria pigmentosa
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| This is a very extensive case Check tryptase levels and organise a bone marrow examination |
Question 1-What are the major primary perforating disorders?
Question 2. Is urticaria pigmentosa a neoplastic proliferation of mast cells?
Question 3. Is urticaria pigmentosa spontaneous or is it a genetic disorder in some cases?
Answer 1.-Reactive perforating collagenosis is 1 of 4 primary perforating disorders; the other 3 primary perforating disorders are Kyrle disease, elastosis perforans serpiginosa, and perforating folliculitis.
eMedicine-Perforating folliculitis eMedicine-Reactive perforating collagenosis
eMedicine-Elastosis Perforans Serpiginosum Image of EPS
Answer 2. Clonality studies and an analysis of mutation suggest that some adult cases are indeed a neoplastic proliferation of mast cells. However in contradistinction childhood mastocytosis is mainly driven by cytokine induced hyperplasia.
Answer 3. It is very rarely familial but when it does occur it is usually as an autosomal dominant with reduced expressivity.
Erythema and Papules
All erythema with a few papules - scarlatina- Scarlet Fever
All erythema plus short lived little papules - cholinergic urticaria
Erythema and chronic papules - pityriasis rubra pilaris
Chronic papules with no erythema - lichen myxoedematosus
Erythema with non-infiltrating papules - Sezary's syndrome
Circinate Papules
The mnemonic used SIGNDIP LEPPP (lichen sclerosis et atrophicus, elastosis perforans serpiginosa, pustules, pemphigus and porokeratoses).
Pustules above would include necrolytic migratory erythema, subcorneal pustular dermatosis, acrodermatitis enteropathica, impetigo.
Fabry's Disease (Angiokeratoma corporis diffusum)
The skin lesions are small, punctate, vascular papules with slight hyperkeratotic tops, particularly on the lower legs and around the scrotum and penis. Sometimes they will also curiously congregate in the mid line of the lower lip. See Image Patients may also present with a curious burning sensation of the hands and feet and reduced sweating. The burning sensation is reduced by elevating the arms.
eMedicine-Fabry's Disease Dermis Atlas-Fabry's Disease
A long standing keratotic papular eruption
Question What is the cause of Fabry's disease and what is the inheritance?
Answer It is an x linked recessive caused by a deficiency of alpha-galactosidase A. Females who may be heterozygotes may show varying degrees of the disease.
Kyrle's Disease
This is a papular disorder affecting the lower legs where a horny cone projects into the dermis. When this is removed there is a pit that extends deeply into the skin. The lesions may coalesce to form a plaque. The condition is associated with diabetes mellitus often with accompanying renal failure.
eMedicine-Kyrles disease eMedicine-Reactive perforating collagenosis
Asymptomatic papules in a child
The photo below is reactive perforating collagenosis
Question 1. Does Kyrle's Disease show the Koebner Phenomenon?
Question 2. How does reactive perforating collagenosis differ from Kyrle's Disease?
Question 3. What proportion of chronic renal failure patients on dialysis develop perforating disorders?
Answer 1. Yes, particularly in the antecubital and popliteal spaces.
Answer 2. It is usually seen in children, the papules are much smaller, the lesions regress spontaneously in six to eight weeks.
Answer 3. Between 4 and 10% usually on the legs. Later lesions resembling those of prurigo nodularis.
Malignant Atrophic Papulosis (Degos' Disease) Image
This is a papular disorder where the centre of the lesion is a pale white scar. The lesions may be umbilicated with a central depression. The centre of the lesion is also described as being porcelain white while the periphery becomes red and telangiectatic. Atrophy occurs eventually. This condition crops. As well as affecting the skin this condition may also affect intestines giving rise to abdominal bleeding and perforation, cerebral infarction can also occur. The condition is a necrotizing vasculitis. Inherited forms have also been reported.
eMedicine-Malignant Atrophic Papulosis
Multiple pink papules with white depressed centres
View the image below at this site
Question Are steroids effective in this condition?
Answer No they are not. Aspirin and Persantin may be effective in some cases.
Polyarteritis Nodosa
In the systemic form of this condition the commonest lesions are subcutaneous nodules, along the course of blood vessels on the lower legs. They may occur singly or in groups. The skin in this area may show evidence of livedo. The nodules are often painful. Two major forms of the condition are described, the benign cutaneous form and the systemic form. Polyarteritis nodosa if late in diagnosis will present with cutaneous ulceration and infarcts. Pulp infarcts on the fingers are also common. Cutaneous polyarteritis nodosa as the name implies has no systemic involvement.
GSA-Polyarteritis Nodosa Dermis Atlas-Polyarteritis Nodosa
Necrotising levido reticularis Purpuric nodules and macules on the extremities of a young woman
The photo below is of polyarteritis nodosa-a large vessel vasculitis
Follicular Mucinosis
This condition presents as one or more plaques, particularly on the face and neck. The plaques are generally skin coloured but they may be red or even hypopigmented with a slight eczematous look. Pressure on the plaque may exude some mucinous material from the hair follicle openings. The hairs themselves may be damaged and broken off at the skin surface. The surface sensation may be diminished to light touch mimicking leprosy.
Childhood presentation is usually on the head and neck, generally solitary and spontaneously resolve in up to two years. Multiple lesions in an older patient can be a manifestation of T-cell lymphoma of the skin. Histologically there is mucin within the cells of sebaceous gland in the outer root sheath. In those associated with mycosis fungoides the cellular infiltrate is atypical.
eMedicine-Follicular Mucinosis GSA-Follicular Mucinosis Gibson on Follicular Mucinosis
Asymptomatic follicular papules with alopecia on the lower part of the leg
The photo below shows alopecia mucinosis and its histology
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| Note the pale mucin in the follicular lining cells |
Question 1. How does lichen spinulosus differ from keratosis pilaris?
Question 2. What are the clinical variants of Porokeratoses?
Question 3. What histologic test may help in differentiating linear lichen planus from the other linear disorders?
Question 4. Skin lesions are seen in virtually all cases of Focal Dermal Hypoplasia-What are they?
Question 5. What conditions may be seen in association with Elastosis perforans serpiginosa?
Answer 1. Histologically, LS is most similar to keratosis pilaris, but differs clinically by its more rapid onset and its tendency to occur in more sharply grouped lesions. Keratosis pilaris is, of course, far more common than LS.
Answer 2. classic porokeratosis of Mibelli; disseminated superficial actinic porokeratosis; linear porokeratosis; porokeratosis palmaris, plantaris, et disseminata; and punctate porokeratosis.
Answer 3. Direct immunofluorescence may be helpful, as it may show staining of Civatte bodies with IgM, lgG, and C3 in lichen planus, with negative results in the other entities listed in the differential diagnosis.
Answer 4. Pigmentation and depigmentation may be seen along with atrophic lesions, telangiectasia, yellowish lipomatous nodules, and verrucous papillomas with a linear arrangement that follows the lines of Blaschko.
Answer 5. Down syndrome, Ehlers-Danlos syndrome type IV, osteogenesis imperfecta, Marfan syndrome, acrogeria, and Rothmund-Thomson syndrome
