Pityriasis Lichenoides

Pityriasis Lichenoides

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Pityriasis lichenoides is a rare cutaneous disorder of unknown etiology. Pityriasis lichenoides encompasses a spectrum of clinical presentations ranging from acute papular lesions that rapidly evolve into pseudovesicles and central necrosis (pityriasis lichenoides et varioliformis acuta or PLEVA) to small, scaling, benign-appearing papules (pityriasis lichenoides chronica or PLC). [1, 2] Although historically, the term Mucha-Habermann disease has referred only to PLEVA, the term applies broadly to the entire spectrum of disease including PLC. A rare febrile ulceronecrotic variant has been reported, which is a severe form of PLEVA with high fever and marked constitutional symptoms. Lesions may self-involute and resolve completely over weeks, or new lesions occasionally may appear in crops, waxing and waning spontaneously for months to years thereafter.

Mucha-Habermann disease is not a vasculitic process despite reports of immunoglobulin and complement deposition in vessels. Fibrin is not present in the walls of vessels, and thrombi are not found in the lumen. A cell-mediated mechanism has been proposed based on a T-lymphocytic infiltrate with a cytotoxic/suppressor phenotype, diminished epidermal Langerhans cells, and a reduction of the CD4/CD8 ratio. CD30 (Ki-1) cells, which are associated with large cell lymphoma, have been identified in the infiltrate of both lymphomatoid papulosis and Mucha-Habermann disease, leading some authors to view this as a self-limited self-healing lymphoproliferative disease. [3, 4] One study suggests that pityriasis lichenoides is a form of a T-cell dyscrasia, based on the presence of intraepithelial atypical lymphocytes, phenotypic abnormalities, and TCR-gamma rearrangements. [5]

The exact etiology of PLC and PLEVA remains elusive; many cases resolve without the discovery of an identifiable culprit. Consideration has been given to the possibility of a low-grade or self-limited lymphoproliferative disorder, a response to an infectious agent, or an inflammatory reaction to an unknown epitope.

A number of acute exanthems (eg, Mucha-Habermann disease, pityriasis rosea, acute lichen planus, guttate psoriasis, erythema multiforme) are believed to be caused by a hypersensitivity reaction to infectious agents. Familial outbreaks, clustering of cases, and comorbid symptoms have been used to support these relationships in Mucha-Habermann disease, although clear causality is lacking. Elevations of pathogen-specific antibody titers also have been offered as proof of causality, but such immunologic responses may represent epiphenomena caused by nonspecific immune responses to unknown pathogens. The most commonly reported associated pathogens are Epstein-Barr virus (EBV), Toxoplasma gondii, and human immunodeficiency virus (HIV).

Two studies implicate EBV as an etiologic factor in Mucha-Habermann disease. The cases indicate that EBV may be a trigger in PLEVA, but neither study necessarily illustrates well-characterized comorbid EBV-mediated disease. Note the following:

In 1977, Boss et al reported a cluster of 10 cases seen over 1 year, in which eruptions were clinically consistent with PLEVA. [6] Of these, 4 demonstrated elevated immunoglobulin G (IgG) complement-fixing antibodies to EBV. During resolution of the eruption, 3 of 4 patients demonstrated 4-fold or greater decrements in antibody titers.

In 1989, Edwards et al described a child with a 3-week history of migratory arthralgias, monoarticular arthritis, acute pharyngitis, otitis media, and fevers to 104ºF. [7] The girl developed a vesicular eruption localized primarily to the extremities, which clinically and histopathologically was consistent with Mucha-Habermann disease, with the exception of necrotic fibrin thrombi in the superficial and mid dermis. A Monospot test result was positive, and acute and convalescent serologies were consistent with a reactivation of EBV. Liver function tests were within normal limits. The patient’s condition improved with treatment using oral tetracycline.

Elevated Toxoplasma gondii titers have been demonstrated in some patients with Mucha-Habermann disease. Despite an absence of clinical infection in case reports and series, more than 80% of primary Toxoplasma infections are asymptomatic, and toxoplasmosis cannot necessarily be dismissed as a causative agent. Note the following:

In 1969, Andreev et al were the first to suggest a link between toxoplasmosis and a recurrent PLEVA-like skin eruption in a patient with positive Toxoplasma serologies. [8] Cutaneous lesions reportedly responded favorably to pyrimethamine.

In 1972, Zlatkov and Andreev reported 11 patients with PLC and found that test results were positive for toxoplasmosis in 6 patients (55%) using complement-fixations test, intradermal test with toxoplasmin, and Sabin-Feldman dye test. [9] Patients in whom test results were seropositive responded favorably to pyrimethamine, while no improvement was noted in the cutaneous lesions of 3 patients in whom results were seronegative.

In 1987, Rongioletti et al described a patient who presented with acute onset of cutaneous lesions and histopathologic findings consistent with PLEVA. [10] Serologic examination demonstrated enzyme-linked immunosorbent assay positivity for IgG and immunoglobulin M (IgM) and weak positive indirect fluorescence test results for IgM (1:16). Giemsa stain on the biopsy specimen failed to demonstrate Toxoplasma cysts. Spiramycin treatment was initiated, and lesions subsided over a few weeks. Convalescent serologies failed to demonstrate IgM 2 months later, although the authors still concluded that Toxoplasma species may have caused the cutaneous eruption.

In 1997, Nassef and Hammam reported 22 patients diagnosed clinically and histopathologically with PLC and 20 healthy control subjects. [11] Clinical examination for signs of toxoplasmosis only revealed axillary lymphadenopathy in 2 patients. Eight patients with PLC (36%) had a positive serodiagnosis by indirect hemagglutination versus 10% in the control group, and this difference was statistically significant. Using indirect immunofluorescence antibody tests, the difference was 36% versus 15%, respectively, but the difference was not statistically significant. All 22 patients with PLC were treated with pyrimethamine and trisulfapyrimidine, and lesions in 5 of 8 patients with seropositive results cleared completely within 2 months. None of the patients with seronegative results responded to treatment.

The first association between Mucha-Habermann disease and HIV infection was reported in 1991 by Ostlere et al. [12] A patient with asymptomatic disease and a CD4+ T-cell count of 208 cells per microliter, diagnosed 6 months previously, presented with lesions consistent clinically and histopathologically with PLEVA. Note the following:

In 1997, Smith et al reported a series of 5 patients with HIV infection in the early stage of disease, with CD4+ T-cell counts exceeding 200 cells per microliter and/or absolute lymphocyte counts within normal limits. [13] The authors suggested that PLEVA serves as a marker of early–to–mid stage HIV disease.

In 1998, Griffiths reported a patient who presented with a severely pruritic, erythematous, papular eruption that worsened as the CD4+ T-cell count fell from 200 to 20 cells/μ L. [14] Biopsy confirmed PLC, and the disease progressed to febrile ulceronecrotic PLEVA. Dramatic improvement was attained using cyclosporine, and mild PLC-like lesions remained on maintenance doses. On saquinavir and lamivudine, the viral load became undetectable with a concomitant rise in the CD4+ count and a complete resolution of skin lesions. That the inherent immunologic dysregulation of HIV may play a role in Mucha-Habermann disease has been suggested.

In addition to EBV, Toxoplasma gondii, and HIV, a number of other infectious agents have been implicated. The following observations are provocative but may be chance associations. Note the following:

Case reports have suggested that parvovirus B19 and adenovirus can trigger Mucha-Habermann disease.

Herpes simplex has been associated with onset of the disease.

One case report also describes resolution of PLC after tonsillectomy, with throat cultures yielding Staphylococcus aureus and group A beta hemolytic streptococci.

Piamphongsant similarly found coagulase-positive staphylococci on throat cultures in 4 of 10 patients, with some improvement of cutaneous lesions using oral tetracycline. [15]

Freeze-dried live attenuated measles vaccine administered by injection has been associated with Mucha-Habermann disease [16] .

A 12-year-old boy presented with PLEVA five days following influenza vaccination. [17]  

Interestingly, with the increased usage of antitumor necrosis factor-α agents, several reports of PLC induction in patients have been published. [18, 19, 20, 21]  These events remain rare and in patients with underlying inflammatory conditions such as Crohn disease. Additionally, it is not clear if a preceding illness was found in these immunosuppressed patients.

The incidence of Mucha-Habermann disease in the United States has not been reported. In approximately 44,000 patients seen over 10 years in 3 catchment areas in Great Britain, 17 cases of PLEVA were diagnosed.

All races are affected. A racial predisposition has not been reported.

A male predominance has been reported in the pediatric population and in patients presenting with febrile ulceronecrotic Mucha-Habermann disease.

Most patients present during the first 3 decades of life. Studies of children have shown a variable age of onset from 3-15 years, with a mean age of 9.3 years. The chronic form is more common in children. [22]

No clear consensus has been formed regarding duration of the disease, but most cases tend to resolve over time. Patients must be told that lesions may take time to resolve and that the duration of the disease cannot be predicted. The skin-limited form of pityriasis lichenoides is a self-limited disease.

A case series of 22 children revealed a mean duration in PLEVA of 1.6 months to complete resolution and a mean duration in PLC of 7.5 months. The natural tendency of the disease is to remit spontaneously, but some cases may wax and wane over years. Disease duration may be longer in adults. A rare severe variant of PLEVA presents with a sudden eruption of diffuse coalescent necrotic ulcerations associated with high fever. [23] Patients may develop complications such as interstitial pneumonitis, abdominal pain, malabsorption, central nervous system involvement, bacteremia, sepsis, and rheumatic manifestations. T-cell receptor clonal rearrangements of lymphocytic infiltrates have been detected in patients with PLEVA. Occasional cases (< 2%) have been reported to evolve into cutaneous lymphoma, although some reports may have represented misdiagnosis of lymphomatoid papulosis. [24]

The febrile-ulceronecrotic variant may arise de novo or from a preexisting case of pityriasis lichenoides. Rare reports of death from the febrile-ulceronecrotic variant have been attributed to secondary pulmonary thromboembolism, pneumonia, cardiac arrest, and sepsis, among others. [25]

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Panhans A, Bodemer C, Macinthyre E, Fraitag S, Paul C, de Prost Y. Pityriasis lichenoides of childhood with atypical CD30-positive cells and clonal T-cell receptor gene rearrangements. J Am Acad Dermatol. 1996 Sep. 35(3 Pt 1):489-90. [Medline].

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Boss JM, Boxley JD, Summerly R, Sutton RN. The detection of Epstein Barr virus antibody in ‘exanthematic’ dermatoses with special reference to pityriasis lichenoides. A preliminary survey. Clin Exp Dermatol. 1978 Mar. 3(1):51-6. [Medline].

Edwards BL, Bonagura VR, Valacer DJ, Ilowite NT. Mucha-Habermann’s disease and arthritis: possible association with reactivated Epstein-Barr virus infection. J Rheumatol. 1989 Mar. 16(3):387-9. [Medline].

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Rongioletti F, Rivara G, Rebora A. Pityriasis lichenoides et varioliformis acuta and acquired toxoplasmosis. Dermatologica. 1987. 175(1):41-4. [Medline].

Nassef NE, Hammam MA. The relation between toxoplasmosis and pityriasis lichenoides chronica. J Egypt Soc Parasitol. 1997 Apr. 27(1):93-9. [Medline].

Ostlere LS, Langtry JA, Branfoot AC, Staughton RC. HIV seropositivity in association with pityriasis lichenoides et varioliformis acuta. Clin Exp Dermatol. 1992 Jan. 17(1):36-7. [Medline].

Smith KJ, Nelson A, Skelton H, Yeager J, Wagner KF. Pityriasis lichenoides et varioliformis acuta in HIV-1+ patients: a marker of early stage disease. The Military Medical Consortium for the Advancement of Retroviral Research (MMCARR). Int J Dermatol. 1997 Feb. 36(2):104-9. [Medline].

Griffiths JK. Successful long-term use of cyclosporin A in HIV-induced pityriasis lichenoides chronica. J Acquir Immune Defic Syndr Hum Retrovirol. 1998 Aug 1. 18(4):396-7. [Medline].

Piamphongsant T. Tetracycline for the treatment of pityriasis lichenoides. Br J Dermatol. 1974 Sep. 91(3):319-22. [Medline].

Gil-Bistes D, Kluger N, Bessis D, Guillot B, Raison-Peyron N. Pityriasis lichenoides chronic after measles-mumps-rubella vaccination. J Dermatol. 2012 May. 39 (5):492-3. [Medline].

Castro BA, Pereira JM, Meyer RL, Trindade FM, Pedrosa MS, Piancastelli AC. Pityriasis lichenoides et varioliformis acuta after influenza vaccine. An Bras Dermatol. 2015 May-Jun. 90 (3 Suppl 1):181-4. [Medline].

Echeverri AF, Vidal A, Cañas CA, Agualimpia A, Tobón GJ, Bonilla-Abadía F. Etanercept-induced pityriasis lichenoides chronica in a patient with rheumatoid arthritis. Case Rep Dermatol Med. 2015. 2015:168063. [Medline].

Said BB, Kanitakis J, Graber I, Nicolas JF, Saurin JC, Berard F. Pityriasis lichenoides chronica induced by adalimumab therapy for Crohn’s disease: report of 2 cases successfully treated with methotrexate. Inflamm Bowel Dis. 2010 Jun. 16 (6):912-3. [Medline].

Newell EL, Jain S, Stephens C, Martland G. Infliximab-induced pityriasis lichenoides chronica in a patient with psoriasis. J Eur Acad Dermatol Venereol. 2009 Feb. 23 (2):230-1. [Medline].

Martínez-Peinado C, Galán-Gutiérrez M, Ruiz-Villaverde R, Solorzano-Mariscal R. Adalimumab-Induced Pityriasis Lichenoides Chronica That Responded Well to Methotrexate in a Patient With Psoriasis. Actas Dermosifiliogr. 2015 Oct 29. [Medline].

Zang JB, Coates SJ, Huang J, Vonderheid EC, Cohen BA. Pityriasis lichenoides: Long-term follow-up study. Pediatr Dermatol. 2018 Mar. 35 (2):213-219. [Medline].

Smith JJ, Oliver GF. Febrile ulceronecrotic Mucha-Habermann disease associated with herpes simplex virus type 2. J Am Acad Dermatol. 2009 Jan. 60(1):149-52. [Medline].

Panizzon RG, Speich R, Dazzi H. Atypical manifestations of pityriasis lichenoides chronica: development into paraneoplasia and non-Hodgkin lymphomas of the skin. Dermatology. 1992. 184(1):65-9. [Medline].

Geller L, Antonov NK, Lauren CT, Morel KD, Garzon MC. Pityriasis Lichenoides in Childhood: Review of Clinical Presentation and Treatment Options. Pediatr Dermatol. 2015 Sep-Oct. 32 (5):579-92. [Medline].

Clayton R, Warin A. Pityriasis lichenoides chronica presenting as hypopigmentation. Br J Dermatol. 1979 Mar. 100(3):297-302. [Medline].

Musumeci ML, Lacarrubba F, Verzì AE, Micali G. Evaluation of the vascular pattern in psoriatic plaques in children using videodermatoscopy: an open comparative study. Pediatr Dermatol. 2014 Sep-Oct. 31 (5):570-4. [Medline].

Errichetti E, Lacarrubba F, Micali G, Piccirillo A, Stinco G. Differentiation of pityriasis lichenoides chronica from guttate psoriasis by dermoscopy. Clin Exp Dermatol. 2015 Feb 16. [Medline].

Magro C, Guo R, Nguyen GH, Tsang H, Momtahen S. Pityriasis lichenoides-like drug reaction: A clinical histopathologic study of 10 cases. Dermatol Online J. 2017 Nov 15. 23 (11):[Medline].

Weinberg JM, Kristal L, Chooback L, Honig PJ, Kramer EM, Lessin SR. The clonal nature of pityriasis lichenoides. Arch Dermatol. 2002 Aug. 138 (8):1063-7. [Medline].

Ackerman AB, Chongchitnant N, Sanchez J, et al. Histologic diagnosis of inflammatory skin diseases: an algorithmic method based on pattern analysis. Baltimore, Md: Lippincott Williams & Wilkins; 1997: 553-60.

Martinez-Escala ME, Sidiropoulos M, Deonizio J, Gerami P, Kadin ME, Guitart J. γδ T cell-rich variants of pityriasis lichenoides and lymphomatoid papulosis: benign cutaneous disorders to be distinguished from aggressive cutaneous ?d T cell lymphomas. Br J Dermatol. 2014 Aug 21. [Medline].

King RL, Yan AC, Sekiguchi DR, Choi JK. Atypical cutaneous γδ T cell proliferation with morphologic features of lymphoma but with clinical features and course of PLEVA or lymphomatoid papulosis. J Cutan Pathol. 2015 Aug 12. [Medline].

Luo DQ. Febrile ulceronecrotic Mucha-Habermann disease. Cutis. 2013 Nov. 92(5):E9-E12. [Medline].

Aydogan K, Saricaoglu H, Turan H. Narrowband UVB (311 nm, TL01) phototherapy for pityriasis lichenoides. Photodermatol Photoimmunol Photomed. 2008 Jun. 24(3):128-33. [Medline].

Eustace K, Dolman S, Alsharqi A, Sharpe G, Parslew R. Use of Phototherapy in Children. Pediatr Dermatol. 2017 Mar. 34 (2):150-155. [Medline].

Fernández-Guarino M, Aboin-Gonzalez S, Ciudad Blanco C, Velázquez Tarjuelo D, Lázaro Ochayta P. Treatment of adult diffuse pityriasis lichenoides chronica with narrowband ultraviolet B: experience and literature review. Clin Exp Dermatol. 2017 Apr. 42 (3):303-305. [Medline].

Takahashi K, Atsumi M. Pityriasis lichenoides chronica resolving after tonsillectomy. Br J Dermatol. 1993 Sep. 129 (3):353-4. [Medline].

Noda S, Asano Y, Sato S. Pityriasis lichenoides et varioliformis acuta exacerbated after tonsillectomy. Eur J Dermatol. 2011 Jul-Aug. 21 (4):648-9. [Medline].

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Panse I, Bourrat E, Rybojad M, Morel P. Photochemotherapy for pityriasis lichenoides: 3 cases. Ann Dermatol Venereol. 2004. 131(2):201-3. [Medline].

Pavlotsky F, Baum S, Barzilai A, Shpiro D, Trau H. UVB therapy of pityriasis lichenoides–our experience with 29 patients. J Eur Acad Dermatol Venereol. 2006 May. 20(5):542-7. [Medline].

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Brazzelli V, Carugno A, Rivetti N, Cananzi R, Barruscotti S, Borroni G. Narrowband UVB phototherapy for pediatric generalized pityriasis lichenoides. Photodermatol Photoimmunol Photomed. 2013 Dec. 29(6):330-3. [Medline].

Lazaridou E, Fotiadou C, Tsorova C, et al. Resistant pityriasis lichenoides et varioliformis acuta in a 3-year-old boy: successful treatment with methotrexate. Int J Dermatol. 2010 Feb. 49(2):215-7. [Medline].

Griffith-Bauer K, Leitenberger SL, Krol A. Febrile Ulceronecrotic Mucha-Habermann Disease: Two Cases with Excellent Response to Methotrexate. Pediatr Dermatol. 2015 Nov. 32 (6):e307-8. [Medline].

Lis-Święty A, Michalska-Bańkowska A, Zielonka-Kucharzewska A, Pypłacz-Gumprecht A. Successful therapy of cyclosporin A in pityriasis lichenoides et varioliformis acuta preceded by hand, foot and mouth disease. Antivir Ther. 2015 Dec 15. [Medline].

Mark Tye Haeberle, MD Assistant Clinical Faculty, Division of Dermatology, University of Louisville School of Medicine

Mark Tye Haeberle, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Dermatologic Surgery, American Society of Dermatopathology, International Society of Dermatopathology

Disclosure: Received income in an amount equal to or greater than $250 from: UpToDate.

Jeffrey P Callen, MD Professor of Medicine (Dermatology), Chief, Division of Dermatology, University of Louisville School of Medicine

Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: Lilly; Amgen <br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Allergen; Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Daniel S Loo, MD Associate Professor of Dermatology, Residency Program Director, Department of Dermatology, Tufts Medical Center

Daniel S Loo, MD is a member of the following medical societies: American Academy of Dermatology, Association of Professors of Dermatology

Disclosure: Nothing to disclose.

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Peter A Klein, MD Associate Professor, Department of Dermatology, University Hospital, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

Gregory J Raugi, MD, PhD Professor, Department of Internal Medicine, Division of Dermatology, University of Washington at Seattle School of Medicine; Chief, Dermatology Section, Primary and Specialty Care Service, Veterans Administration Medical Center of Seattle

Gregory J Raugi, MD, PhD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Peter A Klein, MD  Associate Professor, Department of Dermatology, University Hospital, State University of New York at Stony Brook

Disclosure: Nothing to disclose.

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