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Although lichen planus can present with a variety of lesions, the most common presentation is as a well defined area of purple-coloured, itchy, flat-topped papules with interspersed lacy white lines (Wickham's striae). This description is known as the characteristic "6 Ps" of lichen planus: planar (flat-topped), purple, polygonal, pruritic, papules, and plaques. This rash, after regressing, is likely to leave an area of hyperpigmentation that slowly fades. That said, a variety of other lesions can also occur.
Variants of cutaneous lichen planus are distinguished based upon the appearance of the lesions and/or their distribution. Lesions can affect the:
- Extremities (face, dorsal hands, arms, and nape of neck). This is more common in Middle Eastern countries in spring and summer, where sunlight appears to have a precipitating effect.
- Palms and soles
- Intertriginous areas of the skin. This is also known as "Inverse lichen planus."
- Nails characterized by irregular longitudinal grooving and ridging of the nail plate, thinning of the nail plate, pterygium formation, shedding of the nail plate with atrophy of the nail bed, subungual keratosis, longitudinal erthronychia (red streaks), and subungual hyperpigmentation. A sand-papered appearance is present in around 10% of individuals with nail lichen planus.
- Hair and Scalp. The scalp is rarely affected by a condition known as lichen planopilaris, acuminatus, follicular lichen planus, and peripilaris, characterised by violaceous, adherent follicular scale with progressive scarring alopecia. While lichen planus and lichen planopilaris may occur together, aside from sharing the term ‘lichen’ and revealing inflammation on skin biopsy, there is neither established data on their co-occurrence nor data to suggest a common etiology. Lichen planopilaris is considered an orphan disease with no definitive prevalence data and no proven effective treatments.
Other variants may include:
- "Lichen planus pemphigoides" characterized by the development of tense blisters atop lesions of lichen planus or the development vesicles de novo on uninvolved skin.
- "Keratosis lichenoides chronica" (also known as "Nekam's disease") is a rare dermatosis characterized by violaceous papular and nodular lesions, often arranged in a linear or reticulate pattern on the dorsal hands and feet, extremities, and buttock, and some cases manifest by sorrheic dermatitis-like eruption on the scalp and face; also palmo plantar keratosis has been reported.
- "Lichenoid keratoses" (also known as "Benign lichenoid keratosis," and "Solitary lichen planus") is a cutaneous condition characterized by brown to red scaling maculopapules, found on sun-exposed skin of extremities. Restated, this is a cutaneous condition usually characterized by a solitary dusky-red to violaceous papular skin lesion.
- "Lichenoid dermatitis" represents a wide range of cutaneous disorders characterized by lichen planus-like skin lesions.
The dry scaling appearance is most noticeable during the winter as a result of dry air inside people's homes. During the summer, tanning of the surrounding normal skin makes the pale patches of pityriasis alba more prominent.
Individual lesions develop through 3 stages and sometimes are itchy:
1. Raised and red – although the redness is often mild and not noticed by parents
2. Raised and pale
3. Smooth flat pale patches
Lesions are round or oval raised or flat, of 0.5–2 cm in size although may be larger if they occur on the body (up to 4 cm), and usually number from 4 or 5 to over 20. The patches are dry with very fine scales. They most commonly occur on the face (cheeks), but in 20% appear also on the upper arms, neck, or shoulders.
The diagnostic differential should consider tinea and vitiligo amongst other causative factors.
The symptoms of this condition include:
- An upper respiratory tract infection may precede all other symptoms in as many as 69% of patients.
- A single, 2- to (rarely) 10-cm oval red "herald" patch appears, classically on the abdomen. Occasionally, the "herald" patch may occur in a 'hidden' position (in the armpit, for example) and not be noticed immediately. The "herald" patch may also appear as a cluster of smaller oval spots, and be mistaken for acne. Rarely, it does not become present at all.
- 7–14 days after the herald patch, many small (5–10 mm) patches of pink or red, flaky, oval-shaped rash appear on the torso. The more numerous oval patches generally spread widely across the chest first, following the rib-line in a characteristic "christmas-tree" distribution. Small, circular patches may appear on the back and neck several days later.
- In 6% of cases an "inverse" distribution may occur, with rash mostly on the extremities. In children, presentation can be atypical or inverse, and the course is typically milder.
- About one in four people with PR have mild to severe symptomatic itching. (Moderate itching due to skin over-dryness is much more common, especially if soap is used to cleanse the affected areas.) The itching is often non-specific, and worsens if scratched. This tends to fade as the rash develops and does not usually last through the entire course of the disease.
- The rash may be accompanied by low-grade fever, headache, nausea and fatigue.
Types include:
- Pityriasis alba
- Pityriasis lichenoides chronica
- Pityriasis lichenoides et varioliformis acuta
- Pityriasis rosea
- Pityriasis circinata
- Pityriasis rubra pilaris
- Pityriasis versicolor
- Dandruff, historically called "Pityriasis capitis"
- Pityriasis amiantacea
Erythroderma is generalized exfoliative dermatitis, which involves 90% or more of the patient's skin. The most common cause of erythroderma is exacerbation of an underlying skin disease, such as psoriasis, contact dermatitis, seborrheic dermatitis, lichen planus, pityriasis rubra pilaris or a drug reaction. Primary erythroderma is less frequent and is usually seen in cases of cutaneous T-cell lymphoma, in particular in Sézary's disease.
The most common causes of exfoliative dermatitis are best remembered by the mnemonic device ID-SCALP. The causes and their frequencies are as follows:
- Idiopathic - 30%
- Drug allergy - 28%
- Lymphoma and leukemia - 14%
- Atopic dermatitis - 10%
- Psoriasis - 8%
- Contact dermatitis - 3%
- Seborrheic dermatitis - 2%
Differential diagnosis in patients with erythroderma may be difficult.
Pityriasis lichenoides is a form of pityriasis.
Types include:
- Pityriasis lichenoides et varioliformis acuta
- Pityriasis lichenoides chronica
Pityriasis commonly refers to flaking (or scaling) of the skin. The word comes from the Greek πίτυρον "bran".
Cradle cap is seborrheic dermatitis that affects infants. It presents on the scalp as greasy patches of scaling, which appear thick, crusty, yellow, white or brown. The affected regions are not usually itchy and do not bother the child. Other affected areas can include the eyelids, ear, around the nose, and in the groin. Hair loss can also occur.
Erythroderma (also known as "Exfoliative dermatitis," "Dermatitis exfoliativa") is an inflammatory skin disease with erythema and scaling that affects nearly the entire cutaneous surface.
In ICD-10, a distinction is made between "exfoliative dermatitis" at L26, and "erythroderma" at L53.9.
The symptoms of this condition include:
- Occasional fine scaling of the skin producing a very superficial ash-like scale
- Pale, dark tan, or pink in color, with a reddish undertone that can darken when the patient is overheated, such as in a hot shower or during/after exercise. Tanning typically makes the affected areas contrast more starkly with the surrounding skin.
- Sharp border
Pityriasis versicolor is more common in hot, humid climates or in those who sweat heavily, so it may recur each summer.
The yeasts can often be seen under the microscope within the lesions and typically have a so-called "spaghetti and meatball appearance" as the round yeasts produce filaments.
In people with dark skin tones, pigmentary changes such as hypopigmentation (loss of color) are common, while in those with lighter skin color, hyperpigmentation (increase in skin color) is more common. These discolorations have led to the term "sun fungus".
Lichenoid trikeratosis is a cutaneous condition that may be related to keratosis lichenoides chronica.
Pityriasis rosea is a type of skin rash. Classically, it begins with a single red and slightly scaly area known as a "herald patch". This is then followed, days to weeks later, by a pink whole body rash. It typically lasts less than three months and goes away without treatment. Sometime a fever may occur before the start of the rash or itchiness may be present, but often there are few other symptoms.
While the cause is not entirely clear, it is believed to be related to human herpesvirus 6 (HHV6) or human herpesvirus 7 (HHV7). It does not appear to be contagious. Certain medications may result in a similar rash. Diagnosis is based on the symptoms.
Evidence for specific treatment is limited. About 1.3% of people are affected at some point in time. It most often occurs in those between the ages of 10 and 35. The condition was described at least as early as 1798.
A papulosquamous disorder is a condition which presents with both papules and scales, or both scaly papules and plaques.
Examples include psoriasis, lichen planus, and pityriasis rosea.
Pityriasis alba is a common skin condition mostly occurring in children and usually seen as dry, fine-scaled, pale patches on the face. It is self-limiting and usually only requires use of moisturizer creams.
The condition is so named for the fine scaly appearance initially present (pityriasis) and the pallor of the patches that develop (while "alba" is Latin for white, the patches in this condition are not totally depigmented).
Pityriasis amiantacea (also known as "Tinea amiantacea") is an eczematous condition of the scalp in which thick tenaciously adherent scale infiltrates and surrounds the base of a group of scalp hairs. It does not result in scarring or alopecia.
Pityriasis amiantacea was first described by Alibert in 1832. Pityriasis amiantacea affects the scalp as shiny asbestos-like (amiantaceus) thick scales attached in layers to the hair shaft. The scales surround and bind down tufts of hair. The condition can be localised or covering over the entire scalp. Temporary alopecia and scarring alopecia may occur due to repeated removal of hairs attached to the scale. It is a rare disease with a female predilection.
Pityriasis amiantacea can easily be misdiagnosed due its close resemblance to other scalp diseases such as psoriasis, seborrhoeic dermatitis or lichen planus. However in pityriasis amiantacea the scales are attached to both the hair shaft and the scalp. Pityriasis amiantacea may be present with other inflammatory conditions such as atopic dermatitis or seborrhoeic dermatitis and sebaceous scales and alopecia can occur. According to the dermatology text Bolognia this condition is most often seen in psoriasis, but may also be seen in secondarily infected atopic dermatitis, seborrheic dermatitis, and tinea capitis.
Tinea versicolor is a condition characterized by a skin eruption on the trunk and proximal extremities. The majority of tinea versicolor is caused by the fungus "Malassezia globosa", although "Malassezia furfur" is responsible for a small number of cases. These yeasts are normally found on the human skin and become troublesome only under certain circumstances, such as a warm and humid environment, although the exact conditions that cause initiation of the disease process are poorly understood.
The condition pityriasis versicolor was first identified in 1846. Versicolor comes from the Latin, from "" to turn + color. It is also commonly referred to as Peter Elam's disease in many parts of South Asia.
Cradle cap is a yellowish, patchy, greasy, scaly and crusty skin rash that occurs on the scalp of recently born babies. It is usually not itchy and does not bother the baby. Cradle cap most commonly begins sometime in the first three months but can occur in later years. Similar symptoms in older children are more likely to be dandruff than cradle cap. The rash is often prominent around the ear, the eyebrows or the eyelids. It may appear in other locations as well, where it is called seborrhoeic dermatitis rather than cradle cap. Some countries use the term "pityriasis capitis" for cradle cap. It is extremely common, with about half of all babies affected. Most of them have a mild version of the disorder. Severe cradle cap is rare.
Although other forms of the disease occur at younger ages, some individuals start having long term symptoms at thirty years of age. This disease also affects adolescents and young adults. This also affects the immune system which therefore results in rashes. The symptoms rarely affect the face or scalp, but occurs at other sites of the body. The duration may last for months or even several years. For instance, new crops of lesions appear every few weeks.
The bacteria staphylococci are present in the majority of cases. Treatment with systemic antibiotics and coal tar shampoo can completely clear the condition when Staphylococcus aureus bacteria are found. Fungal infections such as tinea capitis are known to mimic the symptoms of the condition and can be cleared with antifungal treatment.
It is commonly misdiagnosed as chickenpox or rosacea. Pleva is also often misidentified as a form of staph. The most accurate way to diagnose it is by biopsy. This disease has not been known to be life-threatening. However, there may be mutations of the disease that can cause ulcers on the exterior.
Pityriasis lichenoides chronica is an uncommon, idiopathic, acquired dermatosis, characterized by evolving groups of erythematous, scaly papules that may persist for months.
The parapsoriasis groups, described and debated for nearly a century, has spawned a confusing nomenclature. There are some authors who prefer to limit the term "parapsoriasis" to large- and small-plaque variants only. However, the following classification scheme is now generally accepted:
- Large-plaque parapsoriasis
- Small-plaque parapsoriasis
- Pityriasis lichenoides
- Pityriasis lichenoides chronica
- Pityriasis lichenoides et varioliformis acuta
- Lymphomatoid papulosis
Parapsoriasis refers to one of a group of skin disorders that are characterized primarily by their resemblance to psoriasis (red, scaly lesions), rather than by their underlying cause.
Neoplasms can develop from parapsoriasis. For example, it can develop into cutaneous T-cell lymphoma.
The word "parapsoriasis" was formed in 1902.
PVA can be characterized by speckled, combined hyper- and hypopigmentation in the plaques or patches of affected skin. Hyperpigmentation is excess coloration, or darkening of the skin, while hypopigmentation is a diminished or pallid coloring to the skin. Pigmentation changes in PVA, apparent in the epidermal (outermost) skin layer, may be attributed to incontinence (leaking out) of melanin from melanocytes into the dermal skin layer below. Inflammation of the skin and cutaneous tissue, common with PVA, also contributes to color changes in the skin, typified by redness. Telangiectasia, the visible "vascular" element of PVA, is the of small blood vessels near the skin surface. Skin atrophy, a wasting-away of the tissue comprising the skin, is a prominent part of PVA and effects the dermal, and particularly the epidermal layer. This, in part, is the result of degenerative of the stratum basale (bottom cell-layer) of the epidermis. Atrophy of the skin gives it a thin, dry and wrinkled appearance, which in PVA-affected individuals has been described as "cigarette paper". Hyperkeratosis, a thickening of the stratum corneum (top cell-layer of the epidermis), has also been reported.