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Pityriasis lichenoides et varioliformis acuta
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Pityriasis lichenoides et varioliformis acuta (PLEVA) is an eruption of small, erythematous papules that can become vesicular and hemorrhagic. Some ulcerate and necrose, leaving pitted scars. The name refers to the morphology, not the duration, of the condition because a significant proportion of cases regress, with or without treatment, only to recur. Patients should be warned that relapse is common and that recurrent courses of therapy may be required. Febrile ulceronecrotic Mucha–Habermann disease (FUMHD) is a rare and severe form of PLEVA characterized by an abrupt onset of an ulceronecrotic eruption associated with a high fever and systemic symptoms.
An infective etiology for PLEVA is suggested by reports of clustering of cases, resolution after tonsillectomy, and occurrence in five members of a family. Varicella zoster virus (VZV) has been isolated from lesional skin, and DNA from VZV has been demonstrated using polymerase chain reaction in skin samples of patients with PLEVA. Case reports have associated PLEVA with parvovirus, adenovirus in the urine, staphylococci from throat cultures, Epstein–Barr virus, toxoplasmosis, human immunodeficiency virus (HIV), and human herpesvirus 7 (HHV-7); therefore, investigation for an infective trigger may be useful.
Drug causes are unusual, although one case has been reported of an association with Nivolumab, an immunomodulatory agent targeting PD1-A, and another case associated with injected vaccines against measles–rubella (MR), Haemophilus influenza type b (Hib), and pneumococcus.
There have been case reports of patients with a history of PLEVA developing mycosis fungoides (MF) and, therefore, some authorities recommend long-term follow-up of these patients.
Management Strategy
There are only a handful of controlled trials for this condition, and large series are rare. Unfortunately, in many therapeutic trials PLEVA is often reported with and alongside pityriasis lichenoides chronica (PLC), and management strategies are therefore often similar or interchangeable.
Although a ‘wait and see’ approach is justifiable in infants, children should be given a 6-week course of high-dose erythromycin . Tetracycline should not be given because of its effects on dentition.
Topical corticosteroids are only reported anecdotally in textbooks rather than in studies. They are used with oral antihistamines to reduce pruritus but have no reported effect on disease course.
Second-line therapy in children, and possibly first-line in adults, is ultraviolet B ( UVB) or psoralen plus ultraviolet A (UVA) (PUVA). The only comparative study has shown PUVA to be more effective.
In more extensive or symptomatic disease low-dose methotrexate is useful, and systemic corticosteroids or ciclosporin have also been used. Patients who develop gastrointestinal side effects with methotrexate can be transferred to subcutaneous methotrexate.
In Mucha–Habermann disease, given its severe nature, combination therapy is often prescribed. Intravenous immunoglobulin (IVIG) has been utilized recently.
A diagnostic skin biopsy is unnecessary in clinically obvious cases but may be useful to exclude lymphomatoid papulosis or before commencing aggressive systemic therapy. Classically a lichenoid infiltrate is seen in the superficial dermis with parakeratosis in the stratum corneum and keratinocyte necrosis in the epidermis. There may be red cell extravasation, including trapped red cells in the epidermis, and a lymphocytic infiltration around the dermal vascular plexus and the dermoepidermal junction.
Pityriasis lichenoides in childhood
Alcala R, Vicente A, Gonzalez-Ensenat MA, et al. In: Proceedings of the 17th Annual Meeting of the European Society for Pediatric Dermatology, ESPD 2017. Blackwell Publishing Inc.; 2017; 34 (Suppl 2): S32.
This is a retrospective longitudinal study in Spain of 57 patients aged <18 years old with pityriasis lichenoides from 2006 to 2016 confirmed histologically. Of these, 39% had PLEVA and 61% PLC. Oral erythromycin was most commonly prescribed for PLEVA. Three cases manifested mycosis fungoides and they concluded that patients with active disease should be followed up long term as pityriasis lichenoides can progress to CTCL.
Relationship between pityriasis lichenoides and mycosis fungoides: a clinicopathological, immunohistochemical, and molecular study
Zaaroura H, Sahar D, Bick T, et al. Am J Dermatopathol 2018; 40(6): 409–15.
In this study of 58 patients with pityriasis lichenoides, three patients developed MF following 3–11 years of persistent disease. They concluded that pointers to progression to MF include persistent disease, progression into patches and plaques, reduction of apoptotic keratinocytes and CD7+ and CD8+ lymphocytes, significant lymphocytic nuclear atypia, and gene rearrangement of clonal T-lymphocyte receptors.
Pityriasis lichenoides et varioliformis acuta after vaccination
Moriwaki M, Tanaka A, Tanaka M, et al. J Cutan Immunol Allergy 2018; 1: 37–8.
This is a case report of a 13-month-old female who developed PLEVA 7 days after receiving her first vaccine injection against measles–rubella (MR) and fourth vaccine injection against Haemophilus influenzae b (Hib) and pneumococcus. She was treated with topical difluprednate 0.05% ointment and her skin improved gradually over 12 weeks without recurrence.
Varicella zoster virus as a possible trigger for the development of pityriasis lichenoides et varioliformis acuta: retrospective analysis of our institutional cases
Horie C, Mizukawa Y, Yamazaki Y, et al. Clin Exp Dermatol 2018; 43(6): 703–7.
This study analyzed the endothelial cells and eccrine epithelium of PLEVA patients to see if VZV glycoprotein (g)E is detected. This was identified in 5/6 cases of PLEVA, suggesting that VZV could be a possible cause of PLEVA.
Pityriasis lichenoides et varioliformis acuta as presenting feature of acute human immunodeficiency virus infection
Relvas M, Santiago L, Cardoso JC, et al. BMJ Case Rep 2019; 26; 12(8).
This is a case report of a 38-year-old female who presented with PLEVA and was also found to have an acute HIV infection. One week after, she was treated with topical corticosteroid and antiretroviral therapy, resulting in improvement of her skin. The authors recommend testing for HIV in cases of PLEVA as acute HIV infection may be a cause of PLEVA.
Febrile ulceronecrotic Mucha–Habermann disease: proposed diagnostic criteria and therapeutic evaluation
Nofal A, Assaf M, Alakad R, et al. Int J Dermatol 2016; 55: 729–38.
The authors propose diagnostic features of FUMHD including fever, acute onset of generalized ulceronecrotic papules and plaques, rapid and progressive course, and histopathology consistent with PLEVA.
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