Tutorial 4

These tutorials are particularly directed at third and fourth year registrars.They are meant to give you a broad introduction to clinical dermatology particularly aspects of differential diagnosis. They will use online atlases ,PubMed and eMedicine websites as resource materials as well as the cases in the Dermconsult website itself and Off-Centre Fold cases from the Archives. The format is based on one used by Ken Paver some years ago.

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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

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

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

Tutorial 23

Topics covered in this Tutorial

Acrokeratosis verruciformis

Plugging in the skin

Fox Fordyce disease

Perforating disorders

Follicular papules

Plaques of micropapules

Subcutaneous Phycomycosis

Jessners lymphocytic infiltrate

White plaques in the mouth

Bald areas in the scalp

Keratosis Pilaris atrophicans

Acrokeratosis verruciformis

This is an autosomal dominant disease, which particularly affects the hands, feet, knees, elbows and forearms. There are often palmar pits and friction blisters. The nails may be thickened and white. There are also hypopigmented spots.

View Dermnet on Darier's disease

The image below is of acrokeratosis 

Plugging

Plugging can be due to a comedone which can be both superficial and deep or a milium, which is seen in normal skin. The follicular keratoses are a primary phenomenon of plugging. Plugging can be a secondary phenomenon in conditions such as lupus erythematosus of the discoid variant when it involves hair bearing areas and conditions such as lichen plano pilaris and lichen sclerosis.  

Fox Fordyce Disease

The skin colour is slightly pigmented with dome shaped papules. They come on at puberty particularly in females. It can affect the breasts and inner genital areas with pruritus, it is persistent and it is particularly a risk in pregnancy.

Lichen spinulosus is a cutaneous reaction pattern. Lichen scrofulosorum is an id reaction associated with tuberculosis. Plugging can be simulated by miliary papular syphillis and also drug reactions.

Lichen spinulosus

Lichen scrofulosorum

Reactive Perforating Collagenosis

Here you may have a dome shaped 5mm papule with a central keratin core. Particularly occurs on the hands after trauma. It may regress with temporary hypopigmentation and you can see lesions at different stages. It exhibits the Koebner phenomenon and it occurs over a period of years. See cases in GSA

Elastosis Perforans Serpiginosa

There are various diseases that it can occur with. It is one of the elastic tissue diseases. It presents with annular or linear lesions particularly on the neck and cheeks or the antecubital fossae or the upper thighs.

The image below is of Elastosis perforans serpiginosa

Follicular Papules

Consider the keratosis pilaris group. Then the id group, particularly lichen scrofulosorum and lichen spinulosus. The perforating group particularly perforating collagenosis, porokeratosis, elastosis perforans serpiginosa and perforating granuloma annulare and then there is a nutritional group with Vitamin D or Vitamin C deficiency. Follicular papules are also presenting features of pityriasis rubra pilaris and Darier’s disease

The image below shows the follicular papules of early Pityriasis rubra pilaris

Plaques of Micropapules

Consider sarcoidosis, granuloma annulare, lichen planus, lichen scrofulosorum, lichen nitidus, lichen plano pilaris, eczema, follicular mucinosis, lichen myxedematosus, lichen amyloid, acrokeratosis verruciformis, epidermodysplasia verruciformis.

Follicular Accentuation   

If you get infiltrates around follicles consider either mucin, sarcoid or xanthomata.

Annular Serpiginous Lesions   

Consider Sign Dip and the four Ps and L. Consider pemphigus, porokeratosis, the pustuloses, the perforating diseases and lichen sclerosis et atrophicus.

The image below is lichen nitidus

Subcutaneous Phycomycosis

This is granuloma due to fungi in fatty tissue. It is disc shaped and can have a firm rubber like consistency. The overlying skin may be tense, oedematous, desquamating. It can be hyperpigmented or normal and the regional nodes are not enlarged. The differential diagnosis is lymphatic oedema but this does not have an edge. Subcutaneous malignant lymphoma, which grows rapidly and also induration around an injection site are other possibilities. Particularly nurses injecting Pentazocine. Acanthamoeba  infection  is as rare cause of firm facial plaques.

Rare example of a firm facial plaque due to acanthamoeba infection

See web site associated with this image

Jessners Lymphocytic Infiltrate

This is often pink to red brown with a central clearing tendency particularly on the face and neck and it is relapsing. Many people consider it a variant of lupus erythematosus.

Lymphocytoma Cutis This is a collection of benign normal looking lymphocytes in the skin. It particularly follows insect bite reactions. Classic sites for it though are the scrotum, nipples and the earlobes. It can create alopecia.

Granuloma Faciale Occurs particularly on the face. There are prominent follicular openings, it is painless and it is soft. Usually red or violaceous in colour. Often seen on the nose.

The image below is of Jessners Lymphocytic infiltrate

White Plaques in the Mouth

Dyskeratosis congenita and pachyonychia congenita both occur on the tongue. In contrast white sponge nevus does not occur on the tongue but on the gingival surfaces or buccal surfaces of the cheek and can also be seen on the anus and the vagina.

Anthrax   

There is no lymphadenopathy but there is toxaemia. Lymphangitis is unusual. You get haemorrhage and necrosis and sometimes a gelatinous oedema. The differential diagnosis can be ecthyma due to pseudomonas, a staph infection, accidental vaccinia, cowpox, cat scratch disease, North American blastomycosis and sporotrichosis.

Bald Areas in the Scalp    

Consider a variety of diseases as to whether this is a scarring alopecia or a non-scarring. There are the simple conditions such as fungal infections, alopecia areata and diseases such as lupus or lichen plano pilaris. Three others to consider include pseudopelade, folliculitis decalvans and the Graham Little syndrome. Pseudopelade is a specific reaction pattern on its own. There are three variants, the localised, the diffuse and the type in Negro women. It occurs particularly on the vertex with baldness and slow progression. A biopsy shows inflammation in the upper two-thirds of the follicle. Folliculitis decalvans is a slow balding condition particularly in the temple area in males. It is unilateral but can be bilateral. There are brown or red lupoid like papules or pustules in the advancing edge. All hair areas can be affected. The differential diagnosis is favus, tinea and follicular mucinosis.   The Graham Little Syndrome is a variant of lichen plano pilaris particularly in women. Frontal fibrosing alopecia is seen in post menopausal women with slow receding of the frontal hair line with perifollicular redness and scale.

The image below is listed as pseudopelade

Keratosis Pilaris Atrophicans   

There are several variants of this condition. Keratosis pilaris atrophicans faceii is usually confined to the eyebrows. This is sometimes known as Ulerythema oopherogenes. The second type is atrophoderma vermiculatum. This affects the cheeks and it is an autosomal dominant disorder. The third type is keratosis pilaris decalvans, which is very extensive keratosis pilaris with a scarring alopecia of the scalp and other regions. It is probably an autosomal recessive whereas ordinary keratosis pilaris is autosomal dominant. Regarding atrophoderma vermiculatum it occurs between the ages of five and 12 on the preauricular or cheek areas. There is erythema and pinhead plugs and when the plugs fall out there is a reticulate atrophy with variable milia.

Keratosis pilaris atrophicans