Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Case Report
2010:76:2;172-175
doi: 10.4103/0378-6323.60572
PMID: 20228548

Pityriasis lichenoides with ulceronecrosis and hyperthermia: A rare variant of pityriasis lichenoides et varioliformis acuta

Sarvjit Kaur Virdi, Amrinder Jit Kanwar, Uma Nahar Saikia
 Departments of Dermatology, Pathology, PGIMER, Sector 12, Chandigarh - 160 012, India

Correspondence Address:
Sarvjit Kaur Virdi
#1090 Phase 3B2, Mohali, Sector 60, Punjab - 160 059
India
How to cite this article:
Virdi SK, Kanwar AJ, Saikia UN. Pityriasis lichenoides with ulceronecrosis and hyperthermia: A rare variant of pityriasis lichenoides et varioliformis acuta. Indian J Dermatol Venereol Leprol 2010;76:172-175
Copyright: (C)2010 Indian Journal of Dermatology, Venereology, and Leprology

Abstract

Pityriasis lichenoides with ulceronecrosis and hyperthermia (PLUH) is a severe variant of pityriasis lichenoides et varioliformis acuta that is characterized by high fever and papulo-necrotic skin lesions. We report the case of a 49-year-old male with typical features of PLUH along with an unusual manifestation of extensive skin necrosis including involvement of the intertriginous regions. Systemic administration of corticosteroids and antibiotics did not help to control the disease and the patient succumbed to death due to its fulminant nature.
Keywords: Pityriasis lichenoides, ulceronecrosis, hyperthermia

Introduction

Pityriasis lichenoides (PL) is an uncommon, acquired spectrum of skin conditions that poses various challenges to patients as well as clinicians. It is a difficult and debatable disorder to diagnose, categorize and treat. Besides these inherent obstacles, PL merits awareness because of its potential to progress to cutaneous lymphoma[1] or an ulceronecrotic presentation, both of which carry a significant risk of mortality. The scope of PL presentations is delineated along a continuum of multiple variants including pityriasis lichenoides et varioliformis acuta (PLEVA), pityriasis lichenoides chronica (PLC), and febrile ulceronecrotic Mucha-Habermann disease (FUMHD), also known as pityriasis lichenoides with ulceronecrosis and hyperthermia (PLUH). We report a 49-year-old male patient with fulminant course of pityriasis lichenoides with ulceronecrosis and hyperthermia with ultimately fatal outcome in a short course of time.

Case Report

A 49-year-old male, milk vendor by occupation, presented with erythematous papules and crusted plaques all over the body and ulcerations in the oro-genital tract of 15 days duration. The lesions started as multiple, erythematous, papulo-plaques on the upper limbs (involving axillae, palms and soles), trunk, lower limbs and face including nasal cavity and upper eyelids. They progressed in number and size to form large ulcerated plaques with thick crusting. Simultaneously, ulcerations appeared in the oral cavity and on the genitalia. The disease severity incapacitated him from his routine activities.

The illness was associated with high grade fever. There was no history of drug intake or sore throat prior to the onset of illness. There were no associated complaints of any joint pains, dysuria, respiratory or abdominal problem. There was no history of any extramarital contact. There was no similar illness in the family. He was not a known diabetic or hypertensive.

On general physical examination, he was average built, nourished and febrile (1010 F) with tachycardia at the time of examination. There were two to three non-tender, discrete, mobile superficial inguinal lymph nodes on right side. Systemic examination did not reveal any finding.

On detailed cutaneous examination, around 25-30% of body surface area was involved with bilateral and symmetrical distribution of the lesions on upper limbs, trunk and lower limbs [Figure - 1]. The lesions were seen as discrete and coalescent erythematous and edematous papules. Most of the lesions showed pustulation and hemorrhagic crusting. These lesions enlarged to form punched-out ulcers with well-defined margins varying in size from 1-10 cms with necrotic tissue on the base [Figure - 2]. Ulcerations with hemorrhagic crusting were present at the margins of the nose as well as on the upper and lower lids. Oral mucosa showed multiple superficial and deep ulcers at angles of the lips, tongue and buccal mucosa. Genital mucosa showed multiple ulcers around 1 cm in size, well-defined, non-tender and non-indurated on the prepuce and glans penis with associated phimosis. Palms and soles showed multiple well-defined erythematous maculopapular and pustular lesions. Nails were discolored with longitudinal ridging. Hair was normal. There was no enlargement of the peripheral nerves.

Based on the clinical presentation, the differential diagnosis of erythema nodosum leprosum (ENL) necroticans, malignant syphilis, ecthyma gangrenosum, Wegeners granulomatosis and ulceronecrotic variety of PLEVA were kept. On laboratory investigation, the haemogram showed leucocytosis (total leucocyte count of 14.3X109/L) with normal differential count and peripheral blood film picture. Other parameters such as erythrocyte sedimentation rate, C-reactive protein, random blood sugar, routine urine examination, renal function tests were found to be normal. Liver function tests showed raised enzyme levels (ALT 109 IU, AST 81.23 IU, alkaline phosphatase 347 U/L). VDRL, TPHA, viral markers (hepatitis B and C) and HIV were non-reactive/ negative. There were no giant or acantholytic cells from oral cavity. Gram stain from the lesions on the body showed pus cells and no microorganisms.

Slit-smear examination did not show any AFB. Blood, urine and throat cultures were negative. Both ANA and ANCA (against both proteinase 3 and myeloperoxidase) were negative. Chest X-ray, ECG and ultrasound abdomen were normal. Skin biopsy from the ulcer [Figure - 3] showed necrotic epidermis with moderately dense inflammatory infiltrate in the dermis. Skin biopsy from the raised erythematous and oedematous papules [Figure - 4] showed moderate exocytosis and spongiosis in the epidermis. Dermis showed dense inflammatory infiltrate composed mainly of lymphocytes around the blood vessels accompanying edema and infiltration of the vessels with inflammatory infiltrate at many sites indicating small vessel vasculitis suggestive of PLEVA. Direct imunofluorescence study demonstrated deposition of fibrinogen on deep dermal vessel wall.

On the basis of the clinico-pathological correlation, the final diagnosis of ulceronerotic variant of Pityriasis lichenoides et varioliformis acuta was made. The patient was treated with antibiotics and high dose of oral corticosteroids as well as symptomatic treatment. This was followed by only mild to moderate improvement. The patient reported in follow-up with exacerbation of the disease. On re-admission, approximately six weeks later, new lesions continued to develop, intravenous antibiotics and pulse therapy was instituted and nursing care augmented. But the patient succumbed to death secondary to acute respiratory distress syndrome and multisystem failure.

Discussion

Pityriasis lichenoides with ulceronecrosis and hyperthermia (PLUH) represents a fulminant and potentially lethal variant of pityriasis lichenoides et varioliformis acuta (PLEVA). In 1916 Mucha and in 1925 Habermann reported an acute form of pityriasis lichenoides characterized by the abrupt onset of papulovesicular eruptions and described it as pityriasis lichenoides et varioliformis acuta (PLEVA) or Mucha-Habermann disease. Later, in 1966, Degos reported a rare febrile ulceronecrotic variant of PLEVA. PLEVA and PLUH occur most commonly in children, adolescents and young adults. The cutaneous lesions in PLEVA are usually asymptomatic, but may be pruritic and may heal with scarring. These patients rarely have systemic signs. PLUH on the other hand, often starts out as classic PLEVA but goes on to develop widespread ulceronecrotic lesions and is associated with a high mortality rate particularly in adults and immunocompromised patients. Till date only 31 cases of PLUH have been reported in English literature.[2]

Although the exact etiology for PLUH is unknown, several reports have suggested the possibility of hypersensitivity to an infectious agent. The elevation of microbe-specific antibody titers, deposition of immune complexes in dermal vessels, familial outbreaks and associated constitutional symptoms have been offered as potential evidence for infectious causality. Auster et al. reported adenovirus isolated from urine to be the possible etiologic agent in PLUH and interstitial pneumonitis in their patient.[3] A patient described by Hoghton et al.[4] had lymphocytic myocarditis consistent with viral causation; De Cuyper et al.[5] have described a patient who had respiratory infection before the abrupt onset of disease. Puddu et al.[6] had positive blood cultures for Staphylococcus aureus and Pseudomonas aeruginosa. They also detected circulating IgM immune complex and deposition of C3 in dermal vessels. Many different infectious agents have been implicated in pathogenesis, but there has been no consistent finding so far.[7] The reported etiologic pathogens include EBV, HIV, parvovirus B19, adenovirus, Staphylococcus aureus and group A Streptococcus on throat cultures, Mycoplasma pneumoniae and Toxoplasma gondii.[8] Transition of PLEVA to PLUH has been associated with increase levels of TNF-alpha in one patient.[9]

Another postulated mechanism for PLEVA′s pathogenesis is lymphocytic proliferation. Lopez-Estebaranz et al, however, implied that PLUH represents an inflammatory disorder rather than a T-cell lymphoproliferative process as they did not detect abnormalities in T-cell receptor gene analysis of DNA from skin lesion and peripheral blood.[10]

Our patient had extensive skin and mucosal involvement characterized by great number of large, coalescent ulcerations. However, no infective agent was found from the skin, throat, blood or urine samples. In the biopsy specimen, lymphocytic infiltrate within and around the vessel wall was seen. Direct immunofluorescence study demonstrated deposition of fibrinogen on deep dermal vessel wall. Results of slit smear examination, serologic tests for syphilis, antinuclear and anti-DNA antibodies and antineutrophil cytoplasmic antibodies against both proteinase 3 and myeloperoxidase were negative. Immunohistochemical studies and TNF-alpha levels could not be performed due to lack of facilities.

Management of PLUH is controversial and a considerable number of therapies have been tried. These include systemic corticosteroids, antibiotics, acyclovir, dapsone, methotrexate, psoralen plus ultraviolet A, TNF-alpha inhibitors (such as infliximab and etanercept). A combination of these agents has been tried in some patients. In our patient, systemic coticosteroids in dosages greater than 1 g/kg per day, along with oral and intravenous antibiotics, were used. However, despite the best efforts, no improvement was seen. The patient was then administered dexamethasone pulse therapy due to rapid flare up of the lesions. Despite corticosteroid therapy and supportive therapy, the fulminating course of the disease with multisystem failure led to death of the patient.

References
1.
Cozzio A, Hafner J, Kempf W, Häffner A, Palmedo G, Michaelis S, et al. Febrile ulceronecrotic Mucha-Habermann disease with clonality: a cutaneous T-cell lymphoma entity? J Am Acad Dermatol 2004;51:1014-7.
[Google Scholar]
2.
Aytekin S, Balci G, Duzgun OY. Febrile ulceronecrotic Mucha-Habermann disease: a case report and a review of the literature. Dermatol Online J 2005;11:31.
[Google Scholar]
3.
Auster BI, Santa Cruz DJ, Eisen AZ. Febrile ulceronecrotic Mucha-Habermann's disease with interstitial pneumonitis. J Cutan Pathol 1979;6:66-76.
[Google Scholar]
4.
Hoghton MA, Ellis JP, Hayes MJ. Febrile ulcernecrotic Mucha-Habermann disease a fatality. J R Soc Med 1989;82:500-1.
[Google Scholar]
5.
De Cuyper C, Hindryckx P, Deroo N. Febrile ulcernecrotic pityriasis lichenoides et varioliformis acuta. Dermatology 1994;189:50-3.
[Google Scholar]
6.
Puddu P, Cianchini G, Colonna L, Girardelli CR, Ferranti G, De Pita O. Febrile ulceronecrotic Mucha-Habermann's disease with fatal outcome. Int J Dermatol 1997;36:691-4.
[Google Scholar]
7.
English JC 3rd, Collins M, Bryant-Bruce C. Pityriasis lichenoides et varioliformis acuta and group-A beta hemolytic streptococcal infection. Int J Dermatol 1996;192:277-9.
[Google Scholar]
8.
Klein PA, Jones EC, Nelson JL, Clark RA. Infectious causes of Pityriasis lichenoides: a case of fulminant infectious mononucleosis. J Am Acad Dermatol 2003;49:S151-3.
[Google Scholar]
9.
Tsianakas A, Hoeger PH. Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha-Habermann disease is associated with elevated serum tumour necrosis factor-alpha. Br J Dermatol 2005;152:794-9.
[Google Scholar]
10.
López-Estebaranz JL, Vanaclocha F, Gil R, García B, Iglesias L. Febrile ulceronecrotic Mucha-Habermann's disease. J Am Acad Dermatol 1993;29:903-6.
[Google Scholar]

Fulltext Views
490

PDF downloads
132
Show Sections