Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
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
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
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 - 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
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
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
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Case Report
doi: 10.4103/0378-6323.45222
PMID: 19172033

Generalized morphea, lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male

Sarvjit Kaur Virdi, Amrinderjit Singh Kanwar
 Department of Dermatology, PGIMER, Sector 12, Chandigarh - 160 012, India

Correspondence Address:
Sarvjit Kaur Virdi
Department of Dermatology, PGIMER, Sector 12, Chandigarh - 160 012
How to cite this article:
Virdi SK, Kanwar AS. Generalized morphea, lichen sclerosis et atrophicus associated with oral submucosal fibrosis in an adult male. Indian J Dermatol Venereol Leprol 2009;75:56-59
Copyright: (C)2009 Indian Journal of Dermatology, Venereology, and Leprology


Generalized morphea is a disease characterized by wide-spread sclerosis of the skin. A 39-year-old man presented with history of multiple pigmented and bound-down plaques on the body along with mucosal involvement. Dermatological examination showed multiple indurated and sclerosed plaques with follicular plugging in few of them and gross thickened eroded and glazed tongue. The constellation of these findings with histopathological correlation led us to diagnosis of this spectrum of cutaneous involvement. The coexistence of localized morphea with lichen sclerosis et atrophicus has been reported earlier but existence of these entities with submucosal fibrosis in a same patient is documented here and is the first of its kind.
Keywords: Generalized morphea, Lichen sclerosis et atrophicus, Oral submucous fibrosis


Generalized morphea is a disorder of unknown etiology. The onset of disease is insidious with development of plaques resembling those of localized morphea. These plaques are commonly much larger than those seen in localized disease, being many centimetres in diameter. The coexistence of morphea and lichen sclerosis et atrophicus (LSA) in the same patient suggests that these lesions represent a spectrum which may reflect similar etiologic events or closely related pathologic processes in these two diseases. [1] Oral submucous fibrosis (OSM) is a long-lasting disease of the oral mucosa that mainly appears to be caused by chronic exposure to the constituents of areca nut. [2] It is characterized by development of white and indurated changes of the cheeks, lips, palate, and tongue due to fibrosis leading to restriction of mouth opening. The involvement of oral mucosa with submucosal fibrosis is unknown in morphea or LSA. We describe a case with coexistence of these of skin lesions and concomitant mucosal involvement.

Case Report

A 39-year-old man presented with complaints of multiple pigmented and bound-down plaques on the skin of 5-years duration, followed by thickening of tongue with erosions of 3-years duration and ulceration of hands with associated deformities of 9-months duration. The lesions initially started as asymptomatic plaques over arms, followed by similar lesions on trunk, lower extremities, hands, and the face. He also noted development of white-colored patches in few of these lesions and progressive tightening of affected skin. There was thickening of tongue and difficulty in protrusion of tongue with few erosions. There was gross ulceration over the dorsa of hands and contracture deformities of all the fingers that prevented him to perform his normal daily activities. He did not complain of any bluish discoloration of fingers, dyspnea, dysphagia, weight loss, or any other systemic complaint. He was a chronic alcoholic and a smoker for last 18 years. There was no history of any betel nut chewing at any time, though he attributed the changes of the oral cavity to an amalgam dental filling that was removed within a few months of start of the disease. Despite removal, the process of thickening of tongue continued. There was no similar complaint in any other family member.

He was moderately built and nourished and general physical as well as systemic examination did not reveal any findings. On cutaneous examination, there were multiple, well-defined, large skin colored to hyperpigmented, indurated, and sclerosed plaques distributed all over the body [Figure - 1]. On the face, there was a single large plaque involving the left eye with subsequent ectropion [Figure - 2]. The facial expressions were preserved. Another large plaque extended from nape of the neck to occipital region leading to scarring alopecia [Figure - 3]. There was involvement of pinnae with similar changes. In addition, few plaques exhibited hyperpigmented margins, adherent dark scales, follicular plugging, and depigmentation [Figure - 4]. The dorsal surface of both hands showed ulceration on the medial aspect with dirty unhealthy crusted granulation tissue [Figure - 5]. The skin of hands was adherent and there was fixed flexion deformities of all the fingers. The nails were thickened and yellowish. There were no ragged cuticles or digital pitted scars.

Oral cavity examination showed thickened and glazed tongue with erosions at few places [Figure - 6]. There was difficulty in opening of the mouth. He was unable to touch the hard palate with tip of his tongue as well as there was inability to protrude the tongue. There was fibrosis of both soft palate and tongue. There were few similar depigmented lesions over shaft and glans penis. There was no difficulty in retracting the prepuce but the shaft of penis was turned unidirectionally.

Investigations revealed Hb of 13 gm/dL, TLC of 6700/mm 3 with normal differential and platelet count. The urinalysis and serum biochemistry were normal. Tests for rheumatoid factor, antinuclear antibody, AntiHCV, and HbsAg were negative. Spirometry and barium swallow were normal. Abdominal ultrasound showed fatty liver changes. X-ray of wrist and metacarpophalangeal and interphalangeal joints showed periarticular erosions, osteopenia, and flexion deformity. Skin biopsy was done from four regions: (1) biopsy from forearm showed thinned out epidermis with flattened rete ridges. Dermis had mild to moderate perivascular lymphoplasmacytic cell infiltrate; in addition, dermis was completely collagenized with hyaline deposition describing changes of morphea; (2) biopsy from lesion with follicular plugging showed hyperkeratotic epidermis with flattened rete ridges. Dermis was densely sclerosed with mild perivascular inflammatory infiltrate. The adenexal structures were normally placed in the deeper dermis. This was consistent with lichen sclerosis et atrophicus; (3) biopsy from tongue showed no epidermal changes and mild chronic inflammatory infiltrate in subepithelium, that too in addition was heavily collagenized describing changes of submucosal fibrosis; and (4) ulcer edge biopsy was composed of sheets of acute inflammatory cells, fibrin, plasma, few dermatophytes, and bacterial colonies. The infiltrate was concentrated more around blood vessels with endothelial swelling but there were no changes of fibrinoid necrosis or malignancy.

After detailed history, examination, and investigations, final diagnosis of generalized morphea with LSA and submucosal fibrosis was made and the patient was administered dexamethasone pulse therapy in a dose of 140 mg daily for three days along with antibiotic course and supportive treatment. On his first follow-up visit, there was mild subjective improvement in his tightness of the skin and he is on further treatment with pulse therapy.


Scleroderma is a group of chronic autoimmune diseases with skin thickening as the hallmark of the disease. Traditionally, the term "scleroderma" encompasses two groups: localized scleroderma and systemic sclerosis. Generalized morphea and LSA are now classified under localized sclerosis by Peterson et al , due to histological similarities between two and the frequent occurrence of LSA with the other morphea subtypes. [3] Generalized scleroderma is characterized by wide-spread sclerosis of the skin in which plaques become confluent and affect more than two body sites. Sometimes, it may develop as an extension of localized morphea or be associated with lesions of LSA. [4] Both these disorders have female preponderance and mainly affect children and young adults.

The etiology of these diseases is still unclear and their relationship is obscure. Vascular damage and an increased level of adhesion molecules and cytokines are seen in early lesions of morphea. There is increased expression of type 1 collagen in morphea. Collagen synthesis is increased in both LSA and morphea, with increased noncollagenous protein glycosaminoglycan in morphea. [5] Coexistence of these entities have been described initially by Uitto et al , in 10 patients in the year 1980. Thereafter, there are only sporadic case reports in the literature. [6],[7]

OSF is a chronic debilitating and a premalignant condition of the oral cavity. The pathogenesis of the disease is not well established. Epidemiological evidences strongly indicate the association of the betel quid (BQ) habit and OSF. Various findings indicate the disease to be a consequence of disturbances in the homeostatic equilibrium between synthesis and degradation of extracellular matrix (ECM), wherein collagen forms a major component. Transforming growth factor-beta (TGF-beta) is a potent stimulator of production and deposition of the ECM. [8] Various studies have taken into account the role of chewing or smoking tobacco in development of OSF. It was inferred that neither chewing nor smoking tobacco increased the risk of developing OSF. [9] On the other hand, it was also found that it was frequency of chewing and not the total duration that was directly correlated to OSF. [10] In addition to tobacco use, intake of specific/unknown nutrients may have a role in the development of oral precancerous lesions. [11]

Our patient had extensive fibrosis limited to skin and mucosal surfaces. Over a period of five years the disease progressed rapidly without any systemic involvement supporting the clinical impression that morphea is a benign, self-limited disease with progression to systemic form being a rare occurrence. However, morphea can be very disabling and in a study by Peterson et al , conducted in Olmsted County, disability was observed in 11% of individuals affected with morphea. [12] The extension of the lesions of LSA over the dorsal and palmar aspect of the hands was associated with formation of severe contractures and ulceration leading to disability. This could be due to acute inflammatory process causing fibrosis of the affected site. Various other causes of ulcers such as vasculitis and malignancy were excluded histopathologically. Radiological changes like periarticular erosions, osteopenia, and flexion deformities have been reported with localized or generalized morphea as seen in our patient.

Our patient was a chronic smoker and an alcoholic. The development of OSM in him may be an independent occurrence although the basic pathology in all these diseases is related to excess collagen deposition, but the involvement of oral mucosa has not been reported with either generalized morphea or LSA. There was no history of BQ chewing at any time but there was history of dental amalgam filling prior to onset of disease in the oral cavity. But even subsequent to its removal, the changes of OSM continued and there are no reports to suggest dental filling as its cause. On the contrary, there are a few studies in literature to indicate intake of alcohol or tobacco smoking or even other unknown dietary factors as the inciting factors. A similar case of localized morphea with OSM has been described earlier but the changes occurred secondary to BQ habit. [13]

This patient has many unusual presentations that are difficult to explain despite good history and examination and serves worth reporting. Therefore to conclude, morphea and LSA may be associated in a same patient and even transitional forms may occur; LSA is now categorized as subepidermal morphea; and OSM may have other causes apart from BQ chewing.

Uitto J, Santa Cruz DJ, Bauer EA, Eisen AZ. Morphea and lichen sclerosus et atrophicus: Clinical and histopathologic studies in patients with combined features. J Am Acad Dermatol 1980;3:271-9.
[Google Scholar]
Canniff JP, Harvey W. The aetiology of submucous fibrosis: The stimulation of collagen synthesis by extracts of areca nut. Int J Oral Surg 1981;10:163-7.
[Google Scholar]
Paterson LS, Nelson AM, Su WP. Classification of morphoea (localized scleroderma). Mayo Clin Proc 1995;70:1068-76.
[Google Scholar]
Patterson JA, Ackerman AB. Lichen sclerosus et atrophicus is not related to morphoea: A clnical and histologic study of twenty-four patients in whom botn conditions were reputed simultaneously. Am J Dermatopathol 1984;6:323-35.
[Google Scholar]
Griffiths MR, Priestley GC. A comparison of morphoea and lichen sclerosus et atrophicus in vitro : The effects of para-aminobenzoate on skin fibroblasts . Acta Dermatol Venereol 1992;7215-8.
[Google Scholar]
Tremaine R, Adam JE, Orizaga M. Morphea coexisting with lichen sclerosus et atrophicus. Int J Dermatol 1990;29:486-9.
[Google Scholar]
Shono S, Imura M, Ota M, Osaku A, Shinomiya S, Toda K.. Lichen sclerosus et atrophicus, morphoea and coexistence of both diseases. Histological studies using lectins. Arch Dermatol 1991;127:1352-6.
[Google Scholar]
Rajalalitha P, Vali S. Molecular pathogenesis of oral submucous fibrosis: A collagen metabolic disorder. J Oral Pathol Med 2005;34:321-8.
[Google Scholar]
Yang YH, Lien YC, Ho PS, Chen CH, Chang JS, Cheng TC, et al . The effects of chewing areca/betel quid with and without cigarette smoking on oral submucous fibrosis and oral mucosal lesions. Oral Dis 2005;11:88-94.
[Google Scholar]
Shah N, Sharma PP. Role of chewing and smoking habits in the etiology of oral submucous fibrosis (OSF): A case-control study. J Oral Pathol Med 1998;27:475-9.
[Google Scholar]
Gupta PC, Hebert JR, Bhonsle RB, Sinor PN, Mehta H, Mehta FS. Dietary factors in oral leukoplakia and submucous fibrosis in a population-based case control study in Gujarat, India. Oral Dis 1998;4:200-6.
[Google Scholar]
Peterson LS, Nelson AM, Su WP, Mason T, O'Fallon WM, Gabriel SE. The epidemiology of morphoea (localized scleroderma) in Olmsted County 1960-1993. J Rheum 1997;24:73-80.
[Google Scholar]
Ahmed A, Aryad M. Localized morphoea associated with oral submucous fibrosis. J Coll Physicians Surg Pak 2006;16:141-2.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections