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   Abstract
   Introduction
   Case Report
   Discussion
   References
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CASE REPORT
Year : 1995  |  Volume : 61  |  Issue : 2  |  Page : 113-115

Generalised morphoea with lichen sclerosus et atrophicus and unusual bone changes




Correspondence Address:
PVS Prasad


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Source of Support: None, Conflict of Interest: None


PMID: 20952909

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  Abstract 

A 26-year-old male patient presented with multiple plaques on the limbs and trunk suggestive of morphoea. He also exhibited multiple, small, atrophic, hypopigmented macules on the left side of the trunk, the histopathology of which was consistent with lichen sclerosus et atrophicus (LSA). The patient developed large ulcers on the left leg and foot, and contractures with flexion deformity of the left ring and little fingers. This combination of generalised morphoea with LSA and unusual osteolytic bone changes is uncommon.


Keywords: Morphoea, Lichen sclerosus et atrophicus


How to cite this article:
Prasad P, Padmavathy L, Sethurajan S, Kumar P. Generalised morphoea with lichen sclerosus et atrophicus and unusual bone changes. Indian J Dermatol Venereol Leprol 1995;61:113-5

How to cite this URL:
Prasad P, Padmavathy L, Sethurajan S, Kumar P. Generalised morphoea with lichen sclerosus et atrophicus and unusual bone changes. Indian J Dermatol Venereol Leprol [serial online] 1995 [cited 2019 Oct 14];61:113-5. Available from: http://www.ijdvl.com/text.asp?1995/61/2/113/4162



  Introduction Top


Generalised morphoea is characterised by widespread sclerosis of an idiopathic nature. Large plaques are commonly seen on the trunk and upper thighs, but other areas may also be involved.[1] Localised or generalised scleroderma may coexist with LSA, which presents as hypopigmented macules and atrophic areas.[2],[3] Various osseous lesions have been described in morphoea. They include radiological abnormalities in the spine or ribs, shortened ulna, contracted pelvis or deformities of the feet and toes.[4]

There is no universally accepted or proven therapy for scleroderma. Favorable responses have been reported with azathioprine, and potassium para-amino benzoate. Topical or intralesional steroids, antimalarials and Vitamin E have also been reported to be effective.


  Case Report Top


A 26-year-old male tailor was admitted with the history of generalised skin lesions and joint stiffness for 3 years, multiple leg ulcers for 2 years, and drooping of the fingers of the left hand for 6 months.

On examination, there were multiple, well-defined, large, dull, erythematous, indurated and sclerosed plaques over the limbs and the left side of the trunk. The right axilla, abdomen and groin showed plaques with central clearing and a well-defined periphery with scaling. The plaque on the left hand involved both the palmar and the dorsal aspects and was indurated. There were hypopigmented, atrophic macules exhibiting Koebner's phenomenon on the face, the left side of trunk and the limbs [Figure - 1]. The anterior aspect of the left leg and dorsum of the left foot showed large nonhealing ulcers.

Investigations revealed a Hb of 8 gm%, TC of 10,300/mm[3] with a normal differential count, and a ESR of 140 mm/hour, The urinalysis was normal, The FBS was 63 mg%, PPBS 70 mg%, urea 21 mg%, serum calcium 10.2 I.U., serum phosphorus 5.3 I.U. and serum alkaline phosphatase 21 I. U. The LE cell phenomenon was negative on three occasions and the VDRL was nonreactive. Tests for rheumatoid factor, antinuclear antibodies and serum immunoglobulin levels could not be done due to lack of facilities. Pus cultured from the ulcers revealed Pseudomonas aeruginosa sensitive to gentamycin and colistin. A skin biopsy from the hypopigmented macule revealed features suggestive of LSA [Figure - 2]. The histopathology

of one of the hyperpigmented plaques was consistent with the diagnosis of morphoea. Biopsy of the ulcer revealed on acute inflammatory cell infiltrate. An X-ray of the left hand showed osteolytic lesions on the proximal phalanx of the left ring and little fingers [Figure - 3]. A skeletal survey was otherwise normal.

The patient was treated with chloroquine, 250 mg b.i.d., which was continued for 6 months at tapering doses. The ulcer healed with appropriate antibiotic therapy. Physiotherapy was given for the flexion deformity to prevent further contractures. After 6 months of treatment, the patient was subjectively better. The colour of the plaques was returning to normal and their size and induration were less.


  Discussion Top


Morphoea may be occasionally associated with LSA. The coexistence of LSA and morphoea has been reported in 10 patients by Uitto et al.[6] The histologic appearance of guttate lesions may vary from LSA to morphoea. This suggests that the aetiopathogenesis may be similar in both.[7] In our case the presence of LSA was confirmed by histopathological studies. This case presents a transitional form between plaque-like morphoea, generalised morphoea and LSA.

Many types of radiological abnormalities have been reported in localised or generalised scleroderma. Our patient exhibited osteolytic lesions on the proximal phalanges of the left ring and little fingers which led to the flexion deformity. These lesions, which are rare, are probably due to the presence of cutaneous changes. In addition, our patient also had chronic ulcers on the leg and foot which healed with appropriate antibiotic treatment. Other causes of leg ulceration, such as mycotic infection, malignancy, organic vascular diseases, necrobiosis lipoidica, were ruled out and the response of the ulcer to antibacterials points to a primary bacterial cause.

 
  References Top

1.Rowell NR, Goodfield MJD. The connective tissue diseases. In: Textbook of dermatology (Champion RH, Burton JL, Ebling FJG, eds). 5th edn. Oxford: Blackwell Scientific Publications, 1992;2225-35.  Back to cited text no. 1    
2.Ackernman AB, Patterson JAR. Lichen sclerosus et atrophicus is not related to morphea: A clinical study of 24 patients. Am J Dermatopathol 1984;6:323-35.  Back to cited text no. 2    
3.Connelly MG, Winklemann RK. Coexistence of lichen sclerosus, morphea and lichen planus. J Am Acad Dermatol 1985;12:844-45.  Back to cited text no. 3    
4.Christianson HB, Dorsey ES, O'Leary PA, et al. Localised scleroderma, a clinical study of 235 cases. Arch Dermatol 1956;74:581-89.  Back to cited text no. 4    
5.Vincent F. Localised scleroderma. Med Clin N Am 1989;1143-55.  Back to cited text no. 5    
6.Uitto J, Eisen AZ, Bauer EA. Morphea and lichen sclerosus et atrophicus. J Am Acad Dermatol 1980;3:271-3.  Back to cited text no. 6    
7.Eisen AZ, Uitto J, Bauer EA. Scleroderma. In; Dermatology in general medicine (Fitzpatrick TB, Eisen AZ, Klaus W, et al, eds). 3rd end. New York: McGraw-Hill, 1987;1841-2.  Back to cited text no. 7    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4]



 

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