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 Table of Contents  
CASE REPORT
Year : 2021  |  Volume : 8  |  Issue : 1  |  Page : 48-51

Erythematous linear patch on lower limb complicated by lymphatic filariasis: a diagnostic dilemma


Department of Dermatology, AIIMS, Gorakhpur, U.P., India

Date of Submission17-Jul-2020
Date of Decision21-Oct-2020
Date of Acceptance02-Nov-2020
Date of Web Publication07-Apr-2021

Correspondence Address:
Sushantika S
Senior Resident, Department of Dermatology, AIIMS, near Kunraghat, Gorakhpur-273008
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/Pigmentinternational.Pigmentinternational_

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  Abstract 


Asymptomatic erythematous patches are mostly congenital vascular malformations that may be a part of syndromes like Sturge-Weber or Klippel Trenaunay syndrome. Acquired erythematous lesions are difficult to diagnose with differentials ranging from acquired port-wine stain to early morphea. We are reporting a case of a middle aged woman suffering from early morphea hardly distinguishable from Port-wine stain that was further complicated by her acquiring lymphatic filariasis in the same leg.

Keywords: Early morphea, erythematous patch, port-wine stain


How to cite this article:
Gupta SK, Sushantika. Erythematous linear patch on lower limb complicated by lymphatic filariasis: a diagnostic dilemma. Pigment Int 2021;8:48-51

How to cite this URL:
Gupta SK, Sushantika. Erythematous linear patch on lower limb complicated by lymphatic filariasis: a diagnostic dilemma. Pigment Int [serial online] 2021 [cited 2021 Aug 2];8:48-51. Available from: https://www.pigmentinternational.com/text.asp?2021/8/1/48/313134




  Introduction Top


Asymptomatic erythematous patches are mostly congenital vascular malformations that may be a part of syndromes like Sturge-Weber or Klippel Trenaunay syndrome. Acquired erythematous lesions are difficult to diagnose with differentials ranging from acquired port-wine stain (PWS) to early morphea.

We are reporting a case of a middle-aged woman suffering from early morphea hardly distinguishable from PWS that was further complicated by her acquiring lymphatic filariasis in the same leg.


  Case report Top


A 45-year-old woman presented with asymptomatic pink to red-colored patch over left lower limb since 3 years. The lesion was gradually progressing in size and the patient started developing pain in the same leg for about 3 months at the time of presentation. There was no history of any neurological complaints or trauma or any specific long-term drug intake preceding the development of the lesion.

On examination, the patch was extending from ankle to thigh, mostly in posterolateral region [Figure 1],[Figure 2]. The lesion was neither blanchable nor tender and no abnormal pulsations could be palpated. According to the patient, it was extending upward. There was no family history of a similar lesion. No hypertrophy of limb or any other vascular anomaly was present. The skin over lesion was somewhat tight on palpation.
Figure 1 Erythematous patch on left lower limb of the patient

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Figure 2 The erythematous patch extending towards thigh on left leg

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We kept a differential diagnosis of acquired port wine stain and early inflammatory morphea. The histopathological examination of the lesion revealed epidermis with mild hyperkeratosis.

There was some interstitial lymphoplasmacytic inflammatory infiltrates among slightly thickened collagen bundle in dermis and subcutaneous fat extending toward eccrine coils.

[Figure 3],[Figure 4]. There was no significant atrophy of appendages or presence of dilated capillaries suggesting vascular malformation.
Figure 3 Mild hyperkeratosis of epidermis with some interstitial lymphoplasmacytic inflammatory infiltrates among slightly thickened collagen bundle in dermis

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Figure 4 Inflammatory infiltrates around appendages in dermis

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Color Doppler of the limb did not reveal any abnormality of the underlying arteries and veins. Her routine investigations were within normal limits.

The diagnosis was not confirmed after all these efforts, but keeping all the clinicopathological findings in consideration and going through literature, we considered early inflammatory morphea to be the closest diagnosis and started treatment for the same. She was prescribed hydroxychloroquine 200 mg twice a day with topical Calcineurin inhibitor once a day. After taking treatment for 1 month, she had relief in pain and edema of lower leg. The treatment was further continued for two more months with addition of Methotrexate 10 mg once weekly. After 3 months of treatment, she again noticed recurrence of pain with edema of left lower leg. There was no history of fever, but she did experience fatigue and lethargy with constant pain in leg. On examination, pitting edema was present near ankle and her inguinal lymph nodes were significantly enlarged. She was advised thick smear preparation of blood for microfilaria identification, complete blood cell count and antifilarial antibody card test for filarial antibody detection. Microfilaria were not identified on thick smear but antifilarial card test was positive and eosinophilia was marked in blood cell count (>500/mm3).

On suspicion basis for filariasis, she was started on Diethylcarbamazine 100 mg TDS for 3 weeks after which she had significant improvement in pain and edema of leg. The erythematous patch was persistent, but her constitutional symptoms improved significantly. She was again shifted on therapy for early morphea.


  Discussion Top


Morphea is a type of localized scleroderma with an incidence of around 0.3 to 3 cases per 100,000 inhabitants/year.[1] The most widely used classification for localized scleroderma is the Mayo Clinic Classification [Table 1].[2]
Table 1 Mayo Classification for localised scleroderma

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It is proposed that vascular damage (endothelial cellular damage) is the preliminary step in the pathomechanism of morphea and systemic sclerosis. Triggers like infection, hypoxia, trauma, radiation, reactive oxygen species, and antiendothelial cell autoantibodies contribute to vascular injury and further activation of T and B lymphocytes and mononuclear cells, secretion of proinflammatory mediators and growth factors, endothelial cell apoptosis, and fibroblast activation, which leads to vascular and tissue remodeling with fibrosis in morphea.[3],[4]

It is characterized by varying degrees of sclerosis, fibrosis and atrophy in the skin and subcutaneous tissues, sometimes extending deeply into muscle, bone, and brain.

Early morphea can present in many forms like hyperpigmented or hypopigmented patch without any associated complaints and may remain stable for a long time (6 months to 3 years) with a distinct change in color later on. Early stages of morphea are sometimes difficult to recognize, and histology may not be helpful in early cases because there is overlap, leading to misdiagnosis. Clinicopathological correlation is of paramount importance in such cases. Morphea should be considered if perineural inflammation is seen in histopathology.

There are just a handful of cases where early morphea was a close differential for acquired PWS or Fegeler’s syndrome that was first described by Traub in 1939[5] and Fegeler in 1949[6] [Table 2]. Fegeler’s syndrome mostly occur post trauma (30.5%), estrogenic impregnation (16.5%), and more rarely, medication, facial neuralgia, acoustic neuroma, shingles, glaucoma, and exposure to the heat or cold.[7]In many instances, early morphea is diagnosed as PWS and laser therapy is initiated for its management, but after months and years the lesion shows a change in color and histopathology of morphea is apparent. Nihjawan et al. recommended, in patients with acquired PWS, delaying pulsed dye laser (PDL) treatment until a diagnosis of early morphea can be excluded[8].
Table 2 Some of the case reports from literature where early morphea mimicked Port-wine stain and was treated accordingly

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The interesting point about this case was that on one hand her primary diagnosis was difficult to ascertain, filariasis complicated her condition after starting therapy that again complicated the management options of the patient.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Peterson LS, Nelson AM, Su WP et al. The epidemiology of morphea (localized scleroderma) in Olmstead Country 1960-1993. J Rheumatol 1997;24:73-80.  Back to cited text no. 1
    
2.
Peterson LS, Nelson AM, Su WP. Classification of morphea (Localized scleroderma). Mayo Clin Proc 1995;70:1068-76.  Back to cited text no. 2
    
3.
Sgonc R, Gruschwitz MS, Dietrich H, Recheis H, Gershwin ME, Wick G. Endothelial cell apoptosis is a primary pathogenetic event underlying skin lesions in avian and human scleroderma. J Clin Invest 1996;98:785-92.  Back to cited text no. 3
    
4.
Kahari VM, Sandberg M, Kalimo H, Vuorio T, Vuorio E. Identification of fibroblasts responsible for increased collagen production in localized scleroderma by in situ hybridization. J Invest Dermatol 1988;90:664-70.  Back to cited text no. 4
    
5.
Traub EF. Naevus flammeus appearing at the age of twenty three. Arch Dermatol 1939;39:752  Back to cited text no. 5
    
6.
Fegeler F. Naevus flammeus im Trigeminusgebiet nach Trauma im Rachmen eines Posttraumatisch-vegetativen syndrome. Arch Dermatol Syphilol 1949;188:416-22.  Back to cited text no. 6
    
7.
Millán-Cayetano JF, del Boz J, García-Montero P, de Troya-Martín M. Acquired port-wine stain (Fegeler syndrome): a report of 3 cases. Actas Dermo Sifiliograficas 2017;108. http:// dx.doi.org/10.1016/j.annder.2013.01.436  Back to cited text no. 7
    
8.
Nijhawan RI, Bard S, Blyumin M, Smidt AC, Chamlin SL, Connelly EA. Early localized morphea mimicking an acquired port-wine stain. J Am Acad Dermatol 2011;64:779-82, http://dx.doi.org/10.1016/j.jaad.2009.10.017.  Back to cited text no. 8
    


    Figures

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

  [Table 1], [Table 2]



 

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