5.2 Lichen Planus, Lichenoid Eruptions & Other Papulosquamous Disorders
Key Takeaways
- Lichen planus shows compact orthohyperkeratosis, wedge-shaped hypergranulosis, saw-tooth rete ridges, a band-like lymphocytic infiltrate, and Civatte bodies on histology.
- Oral lichen planus carries a small risk of squamous cell carcinoma, about 1% in meta-analyses and higher in erosive disease, so long-term oral review is advised.
- Pityriasis rosea is a self-limiting eruption linked to HHV-6 and HHV-7 reactivation, and syphilis serology should be checked when the diagnosis is uncertain.
- Pityriasis rubra pilaris causes orange-red erythroderma with islands of sparing, follicular papules, and an orange waxy palmoplantar keratoderma.
- Large plaque parapsoriasis can progress to, or represent early, mycosis fungoides, whereas small plaque (digitate) parapsoriasis is usually benign.
5.2 Lichen Planus, Lichenoid Eruptions & Other Papulosquamous Disorders
Papulosquamous disorders present as scaly papules and plaques. Many look alike clinically, and the examination often uses a clinical image plus a histology clue to separate them. This section covers the main conditions other than psoriasis.
Lichen Planus (LP)
LP is a T-cell-mediated interface dermatitis affecting roughly 0.5–1% of adults, with oral involvement in many patients. The classic lesion follows the "6 Ps": planar (flat-topped), purple, polygonal, pruritic papules and plaques. Fine white lines on the surface are Wickham striae. Common sites are the flexor wrists, forearms, shins, lower back, and genitals. The Koebner phenomenon is frequent.
| Variant | Features |
|---|---|
| Hypertrophic | Thick, itchy plaques on the shins; risk of squamous cell carcinoma in long-standing lesions |
| Annular, atrophic, linear | Variants by shape; linear LP may follow Blaschko lines |
| Bullous and LP pemphigoides | Blisters within LP lesions (bullous LP) or on normal skin with anti-BP180 antibodies (LP pemphigoides) |
| LP pigmentosus and actinic LP | Grey-brown macules on the face and flexures; more common in darker skin types |
| Oral LP | Reticular white lacy striae on the buccal mucosa (most common); erosive and atrophic forms are painful |
| Genital LP and vulvovaginal-gingival syndrome | Erosive disease of the vulva, vagina, and gums; can scar and cause vaginal stenosis |
| Lichen planopilaris and frontal fibrosing alopecia | Scarring alopecia with perifollicular erythema and scale |
| Nail LP | Thinning, longitudinal ridging, dorsal pterygium, trachyonychia; can destroy the nail |
Associations: hepatitis C virus (the association varies by region and is strongest in Southern Europe and Japan), and other autoimmune disease.
Histology: compact orthohyperkeratosis, wedge-shaped hypergranulosis, saw-tooth rete ridges, vacuolar change of the basal layer, a band-like lymphocytic infiltrate hugging the dermo-epidermal junction, Civatte (colloid) bodies, and sometimes clefts (Max-Joseph spaces). Direct immunofluorescence shows shaggy fibrinogen along the basement membrane zone and IgM on colloid bodies.
Oral LP and cancer: oral LP carries a small risk of oral squamous cell carcinoma, about 1% in meta-analyses, higher in erosive disease, smokers, and tongue lesions. Persistent ulcers or thickened areas need biopsy, and patients need long-term review.
Treatment: potent or very potent topical corticosteroids for skin lesions; topical steroids or tacrolimus for oral disease; and dental hygiene, including removal of irritant restorations where a lichenoid reaction to amalgam is suspected. Extensive or refractory disease may need a short course of oral prednisolone, acitretin, narrowband UVB, methotrexate, or other immunosuppressants. Hydroxychloroquine is used for lichen planopilaris.
Lichenoid Drug Eruption
Drugs can cause an LP-like eruption, typically weeks to months after starting. Culprits include ACE inhibitors, beta-blockers, thiazides, antimalarials, gold, and immune checkpoint inhibitors. Clues are photodistribution, a more eczematous appearance, and absence of Wickham striae. Histology may show parakeratosis, eosinophils, and a deeper perivascular infiltrate. Resolution after stopping the drug can take months, often with pigmentation.
Lichen Nitidus and Lichen Striatus
- Lichen nitidus: tiny (1–2 mm) shiny skin-coloured papules, often in children. Histology shows a small infiltrate held by rete ridges like a "ball in claw".
- Lichen striatus: a self-limiting linear band of papules along Blaschko lines in children. It clears within about a year, sometimes leaving pigment change.
Pityriasis Rubra Pilaris (PRP)
PRP is rare. The Griffiths classification has six types: type I (classic adult, the most common, usually resolving within about 3 years), type II (atypical adult), types III–V (juvenile forms; familial type V is linked to CARD14), and type VI (HIV-associated).
- Clinical: follicular hyperkeratotic papules (often starting on the dorsal fingers), spreading orange-red erythroderma with well-defined islands of sparing, and an orange, waxy palmoplantar keratoderma ("sandal").
- Histology: alternating orthokeratosis and parakeratosis in vertical and horizontal directions (checkerboard), "shoulder" parakeratosis around follicles, and follicular plugging.
- Treatment: oral retinoids (acitretin or isotretinoin), methotrexate, and, in case series, TNF, IL-17, and IL-23 inhibitors.
Pityriasis Rosea
- Clinical: a single larger herald patch, followed after days to 2 weeks by smaller oval pink patches with a collarette of scale. They follow skin cleavage lines on the trunk in a "Christmas-tree" pattern. It mainly affects adolescents and young adults.
- Cause: reactivation of HHV-6 and HHV-7 is the most accepted explanation.
- Course: self-limiting over about 6–8 weeks; treat itch with emollients and mild steroids.
- Pregnancy: pityriasis rosea in early pregnancy has been associated with miscarriage, so affected pregnant women should be told about this and followed up.
- Key differential: secondary syphilis. Palm and sole lesions, lymphadenopathy, mucosal lesions, or no herald patch should prompt syphilis serology. Also consider drug-induced pityriasis rosea-like eruptions and tinea corporis.
Seborrhoeic Dermatitis
A common inflammatory response linked to Malassezia yeasts in sebum-rich areas: scalp (dandruff), eyebrows, nasolabial folds, ears, and presternal skin.
- Infants: "cradle cap" and flexural disease in the first months, usually self-limiting.
- Adults: chronic and relapsing. It is more severe in HIV infection and Parkinson disease and other neurological conditions.
- Treatment: ketoconazole shampoo or cream, other antifungals, short courses of low-potency corticosteroids, and topical calcineurin inhibitors for the face.
Parapsoriasis and Pityriasis Lichenoides
| Condition | Features | Behaviour |
|---|---|---|
| Small plaque parapsoriasis (digitate dermatosis) | Finger-like yellow-pink patches under 5 cm along the flanks | Usually benign and chronic |
| Large plaque parapsoriasis | Patches over 5 cm on the buttocks and flexures, sometimes with poikiloderma | May progress to, or already be, early mycosis fungoides; biopsy and follow-up |
| Pityriasis lichenoides et varioliformis acuta (PLEVA) | Crops of papules with haemorrhagic crust and varioliform scars | Acute; the rare febrile ulceronecrotic form (Mucha-Habermann) is serious |
| Pityriasis lichenoides chronica (PLC) | Red-brown papules with an adherent "mica" scale | Chronic, relapsing; phototherapy or oral macrolides and tetracyclines are used |
A biopsy of itchy violaceous papules on the wrists shows compact orthohyperkeratosis, wedge-shaped hypergranulosis, saw-tooth rete ridges, and a band-like lymphocytic infiltrate at the dermo-epidermal junction. What will direct immunofluorescence most likely show?
A 22-year-old man has oval pink scaly patches along the trunk in a Christmas-tree pattern. He did not notice a herald patch, and he also has scaly macules on the palms and enlarged lymph nodes. What is the most important next investigation?
A 55-year-old man develops follicular hyperkeratotic papules on the backs of his fingers, then orange-red erythroderma with sharply defined islands of normal skin and an orange waxy keratoderma of the palms and soles. What is the most likely diagnosis?
A 60-year-old woman has several large, slightly atrophic patches over 5 cm on the buttocks and breasts with fine wrinkling and mottled pigmentation. Why is careful follow-up with biopsy recommended?