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Image from the • Febrile ulceronecrotic PLEVA: a severe, rare form of PLEVA with constitutional symptoms like fever and which is a dermatological emergency much like Stevens-Johnson syndrome. Also known as febrile ulceronecrotic Mucha-Habermann disease.

Fewer than 100 cases have been reported in the literature. Epidemiology • The exact frequency of the three types of pityriasis lichenoides is not known because it is often not formally diagnosed []; however, it is likely to be rare. • In adults it tends to come on in the 20s or 30s, with a slight male predominance. • It can occur in children, however, with an average age of onset of 6 years [].

• The cause of pityriasis lichenoides is not yet known but two major theories exist []: • A hypersensitivity reaction to an infectious agent such as parvovirus. • A relatively benign form of a T-cell lymphoproliferative disorder. • Despite resembling a vasculitis, the histology does not show features of vasculitis such as fibrin or thrombi in the walls or lumen of vessels. • Infections that have been associated with both PLC and PLEVA include Toxoplasma gondii, Epstein-Barr virus, HIV, cytomegalovirus, parvovirus, Staphylococcus aureus and group A beta-haemolytic streptococcus. Presentation Pityriasis lichenoides chronica (PLC) • PLC usually presents with small pink papules that turn a reddish-brown colour. It resembles pityriasis rosea.

• The eruption is often polymorphic, with lesions at different stages of development. • PLC is usually distributed over the trunk, buttocks and proximal arms or legs. It may occur elsewhere but usually spares the face and the palms of the hands, unlike chickenpox.

• The lesions are covered with a firm shiny scale ('frosted glass') which can be peeled off to reveal a red-brown colour underneath. • The lesions usually flatten out over a period of weeks or months. Management For the relatively quiescent, asymptomatic condition of PLC, often no treatment is required.

If the patient requests treatment, or would like to try to expedite resolution of the lesions, the following first-line therapies are described in the literature: • Topical steroids to reduce irritation. • Topical immunomodulators - eg, tacrolimus or pimecrolimus.

• Oral antibiotics; the most common being erythromycin and tetracycline [], with an 80% response rate to three months of erythromycin. Second-line therapies for either PLC or PLEVA include: • Monotherapy using narrow-band UVB (NB-UVB). This has been shown to achieve a 90% complete response rate over eight weeks, obviating the need for systemic therapies [].

• For resistant cases, systemic steroids, methotrexate, acitretin, dapsone or ciclosporin may be used. For the much more serious condition of febrile ulceronecrotic PLEVA: • Aggressive treatment with immunosuppressant and/or immunomodulating agents as well as intensive supportive care are recommended for febrile ulceronecrotic PLEVA. [] • TNF-alpha inhibitors (eg, infliximab) may be useful for patients with febrile ulceronecrotic PLEVA, particularly in resistant cases [].

• Treatment is likely to take place on a burns intensive care unit. Prognosis • With the right treatment almost all children will have full resolution at three months []but in total median time to resolution is reported as 21 months for children [].

• In adults, for both PLC and PLEVA, prognosis is usually good. Resolution takes a few months for most patients, and a few years in others, with a median time to cure of eight months.

• Two thirds affected by a first outbreak, even if reaching full resolution, will have a relapse []. • Ulceronecrotic PLEVA carries a high morbidity and is potentially fatal []but with expert treatment it has been reported to be responsive to prednisolone, NB-UVB light therapy or methotrexate [].

• For chronic lesions that seem resistant to standard therapy, there is the risk of transformation to a cutaneous T-cell lymphoma such as mycosis fungoides. Even if this has occurred histologically it does not necessarily mean they will progress []. •; DermNet NZ •; Pityriasis lichenoides et varioliformis acuta: a disease spectrum. Int J Dermatol. 2010 Mar49(3):257-61.

Doi: 10.1111/j.1365-4632.2008.03915.x. •; Pityriasis Lichenoides in Childhood: Review of Clinical Presentation and Treatment Options.

Pediatr Dermatol. 2015 Sep-Oct32(5):579-92. Doi: 10.1111/pde.12581. Epub 2015 Mar 26. •; Pityriasis lichenoides: pathophysiology, classification, and treatment. Am J Clin Dermatol. •; Childhood pityriasis lichenoides and oral erythromycin.

Pediatr Dermatol. 2012 Nov-Dec29(6):719-24. Doi: 10.1111/j.1525-1470.2012.01765.x. Epub 2012 May 29. •; Is narrowband ultraviolet B monotherapy effective in the treatment of pityriasis lichenoides? Int J Dermatol. 2013 Aug52(8):1013-8.

Doi: 10.1111/j.1365-4632.2012.05814.x. Epub 2013 Jun 20. •; Febrile ulceronecrotic Mucha-Habermann disease: treatment with infliximab and intravenous immunoglobulins and review of the literature. Doi: 10.1145. Epub 2013 Jan 31. •; Pityriasis lichenoides in an Asian population. Int J Dermatol.

Doi: 10.1111/j.1365-4632.2012.05608.x. •; Pityriasis lichenoides in childhood: a retrospective review of 124 patients. J Am Acad Dermatol. 2007 Feb56(2):205-10. Epub 2006 Oct 13.

•; Febrile ulceronecrotic Mucha-Habermann disease (pityriasis lichenoides et varioliformis acuta fulminans) associated with parvovirus infection. Am J Dermatopathol. 2013 Jun35(4):503-6. Doi: 10.1097/DAD.0b0626. •; Febrile ulceronecrotic Mucha-Habermann disease: a case report and review of the literature. Acta Derm Venereol.

Doi: 10.235-0441. •; Adult pityriasis lichenoides-like mycosis fungoides: a clinical variant of mycosis fungoides. Int J Dermatol. 2014 Nov53(11):1331-8. Doi: 10.1111/ijd.12135. Epub 2014 Jun 25. • Last Checked 30 November 2016 • Next Review 29 November 2021 • Document ID 2616 (v23) • Author • Peer reviewer Dr Adrian Bonsall Disclaimer: This article is for information only and should not be used for the diagnosis or treatment of medical conditions.

Patient Platform Limited has used all reasonable care in compiling the information but make no warranty as to its accuracy. Consult a doctor or other health care professional for diagnosis and treatment of medical conditions. For details see our.