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CORRESPONDENCE
Year : 2022  |  Volume : 67  |  Issue : 4  |  Page : 446-448
Morphea profunda masquerading as prurigo nodularis: An uncommon presentation


From the Department of Dermatology, SVS Medical College Mahbubnagar, Telangana, India

Date of Web Publication2-Nov-2022

Correspondence Address:
Angoori G Rao
From the Department of Dermatology, SVS Medical College Mahbubnagar, Telangana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.ijd_211_22

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How to cite this article:
Rao AG, Naresh M, Gupta S, Santhoshi G, Nandita K. Morphea profunda masquerading as prurigo nodularis: An uncommon presentation. Indian J Dermatol 2022;67:446-8

How to cite this URL:
Rao AG, Naresh M, Gupta S, Santhoshi G, Nandita K. Morphea profunda masquerading as prurigo nodularis: An uncommon presentation. Indian J Dermatol [serial online] 2022 [cited 2022 Nov 26];67:446-8. Available from: https://www.e-ijd.org/text.asp?2022/67/4/446/360320




Sir,

Morphea is a connective tissue disorder characterized by sclerosis of the skin. Morphologically, there are five types of morphea, namely, plaque, bullous, linear, generalized and deep. Morphea profunda is a rare variant of deep morphea, most commonly presents with solitary or extensive indurated or atrophic plaque with hyperpigmentation.[1]

A 45-year-old lady came to the dermatology department with complaints of multiple black-coloured patches over the legs for the last 10 months, associated with itching. Examination revealed multiple ill to well-defined hyperpigmented patches and plaques, distributed bilaterally symmetrical over the legs extending from below the knee to the dorsal surface of feet anteriorly [Figure 1], and just above the heels posteriorly [Figure 2]. The size of the smallest plaque was 2 × 1 cm, and the largest was 8 × 6 cm. The plaques were indurated and non-tender. The skin was not bound down, and there was no atrophy. Dorsalis pedis artery was palpable and equal on both sides. She was provisionally diagnosed with prurigo nodularis. Erythema induratum of Bazin was considered in the differential diagnosis. Routine haematological and biochemical investigations were unremarkable. Chest x-ray and ultrasonography of abdomen revealed a normal study. Mantoux test was negative. Serology for antinuclear antibodies, anti-double-stranded DNA, anti-ss DNA antibodies, anti-topoisomerase antibodies, anti-histone antibodies, rheumatoid factor, anti-Borrelia burgdoferi antibodies and human immunodeficiency virus (HIV) antibodies was non-reactive. Colour Doppler study for arterial and venous systems revealed a normal study. Sputum for acid-fast bacilli was negative. GeneXpert Mycobacterium Tuberculosis/Rifampicin assay for the detection of DNA of Mycobacterium tuberculosis complex was negative. Biopsy from a plaque on the right leg revealed the features of morphea profunda. However, not convinced with the biopsy report as the histopathological features did not correlate with the clinical diagnosis, a repeat biopsy was done from a plaque on the left leg. Repeat biopsy also confirmed the diagnosis of morphea profunda, which revealed basket weave lamellar orthokeratotic epidermis, and dermis showed haphazardly arranged collagen bundles, and trabeculae dividing the subcutaneous fat, were thickened, and showed pale, wavy collagen bundles. Blood vessel walls showed thickening with narrowing of their lumen with perivascular lymphocytes, plasma cells and histiocytes [Figure 3], [Figure 4], [Figure 5]. She was advised tab prednisolone 20 mg daily and tab methotrexate 7.5 mg weekly, and is under follow-up.
Figure 1: Multiple ill to well-defined hyperpigmented patches and plaques over both the legs extending from below the knee to the dorsal surface of feet anteriorly

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Figure 2: Ill to well-defined hyperpigmented patches and plaques over both the legs extending from below the knee to just above the heels posteriorly

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Figure 3: Basket weave lamellar orthokeratosis in the epidermis. Dermis shows haphazardly arranged collagen bundles and trabeculae dividing the subcutaneous fat. Blood vessel walls showed thickening with narrowing of their lumen with perivascular lymphocytes, plasma cells, and histiocytes (H&E stain, ×10)

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Figure 4: Dermis shows haphazardly arranged collagen bundles and thickened blood vessel walls with narrowing of their lumen and perivascular lymphocytes, plasma cells, and histiocytes. (H&E stain, ×40)

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Figure 5: Invasion of subcutaneous tissue by collagen and the trabeculae dividing subcutaneous fat are thickened (H&E stain, ×100)

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The aetiology of morphea is not known, and the pathogenesis has not yet been elucidated; however, three major factors are known to play a pivotal role in the pathogenesis of morphea, which include vascular changes, activated T cells, and altered connective tissue production by fibroblasts.[2] Trauma, medication, vaccination, malignancies, radiation, familial predisposition, borrelia infection, and helicobactor pylori infection may trigger the disease onset. However, there were no triggering factors attributable in the index case.

Morphea profunda is mostly seen in middle-aged patients with an equal gender distribution. It manifests with hyperpigmented, shiny, smooth atrophic patches with the skin bound down to the underlying structures. Even though the patches and plaques were hyperpigmented, but not atrophic, and skin was not bound down in the index case. Interestingly, the patches and plaques were distributed bilaterally symmetrical in the index case, which is rarely encountered in morphea profunda.

Antinuclear antibodies, anti-histone antibodies, and anti-ss DNA antibodies are known to be associated with a severe type of morphea, and these patients are at increased risk of muscle and joint morbidity.

Interestingly, atypical presentations of morphea profunda have been reported in the literature [Table 1],[3],[4],[5],[6] which include vesiculobullous lesions, lymphangiectasis, muscle weakness and atrophy.
Table 1: Published case reports of morphea profunda with varied presentations and associations

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There is no specific treatment for morphea profunda, and various drugs used in the treatment of morphea include systemic steroids, methotrexate, cyclosporine, D-penicillamine, mycophenolate mofetil, tocilizumab, ruxolitinib, infliximab, phototherapy and extracorporeal photochemotherapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Su WP, Person JR. Morphea profunda. A new concept and a histopathologic study of 23 cases. Am J Dermatopathol 1981;3:251-60.  Back to cited text no. 1
    
2.
Röcken M, Ghoreschi K. Morphea and lichen sclerosis. In: Blognia JR, editor. Dermatology. Philadelphia, USA:Mosby/Elsevier; 2003.  Back to cited text no. 2
    
3.
Dhabal A, Chandra A, Khan S, Barua JK. Localized morphea profunda: An atypical presentation. Indian J Case Reports 2020;6:478-80.  Back to cited text no. 3
    
4.
Budamakuntla L, Malvankar D. Extensive morphea profunda with autoantibodies and benign tumors: A rare case report. Indian Dermatol Online J 2012;3:208-10.  Back to cited text no. 4
[PUBMED]  [Full text]  
5.
Voermans NC, Pillen S, de Jong EM, Creemers MCW, M. Lammens K, Van Alfen N. Morphea profunda presenting as a neuromuscular mimic. J Clin Neur Musc Dis 2008;9:407-14.  Back to cited text no. 5
    
6.
Tuffanelli DL. Letter: Lymphangiectasis due to scleroderma. Arch Dermatol 1975;111:1216.  Back to cited text no. 6
    


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