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Linear greyish-brown indurated plaque over the neck
Corresponding author: Dr. Siddhartha Dash, Department of Dermatology, Srirama Chandra Bhanja Medical College and Hospital, Mangalabag, Cuttack, India. siddharth101990@gmail.com
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Received: ,
Accepted: ,
How to cite this article: Samal A, Dash S, Mohanty J, Mishra P. Linear greyish-black indurated plaque over the neck. Indian J Dermatol Venereol Leprol. 2026;92:122-4. doi: 10.25259/IJDVL_1558_2024
A 32-year-old woman presented with a solitary, asymptomatic, gradually enlarging, greyish-brown, raised, firm lesion extending from behind the left ear to the front of the neck for the past 18 months. There was no history of trauma, injectables or exposure to radiation in the affected areas. There was no history of photosensitivity, joint pain, or oral ulceration. Personal, family history and general examination were unremarkable. On cutaneous examination, an ill- to well-defined, firm to hard, greyish-brown, indurated plaque of size 12 x 2.5 cm was present over the neck extending from behind the left ear to the anterior aspect of the neck, not limiting the range of motion [Figure 1]. The upper 3 cm of the plaque showed depigmentation and atrophy. The rest of the cutaneous and systemic examination were unremarkable. Skin biopsy was done for histopathological examination.
- Ill- to well-defined, firm to hard, greyish-brown, indurated plaque present over the neck extending from behind the left ear to the anterior aspect of the neck.
Question
What is your diagnosis?
Answer
Diagnosis: Keloidal morphea
Investigations and follow-up
The differential diagnoses considered were keloidal morphea, keloid, linear dermatomyofibroma, zosteriform lymphangitis carcinomatosa, sclerodermic lupus erythematosus panniculitis (SLEP), and keloidal blastomycosis. All the biochemical, haematological, and radiological parameters were within normal limits. The antinuclear antibody test was negative. Histopathological examination revealed a rectangle-shaped appearance under scanner view [Figure 2a]. Higher magnification revealed normal epidermis, thickened collagen bundles arranged in a linear pattern along with superficial and deep perivascular and perieccrine lymphoplasmacytic infiltration and diminished adnexal structures [Figure 2b]. Verheoff’s Van Gieson stain showed a marked increase in coarse and fragmented elastin fibres [Figure 2c]. Immunohistochemistry for vimentin stain was only focally positive [Figure 2d]. A final diagnosis of keloidal morphea was made based on clinico-pathological findings. The patient was started on oral methotrexate (0.3 mg/kg/week) along with intravenous methyl prednisolone pulse therapy (1000 mg for 3 days). There was considerable improvement after three pulse therapies given one month apart.
- Histopathology showing a rectangle-shaped appearance under scanner view (Haematoxylin and eosin, 40x).
- Higher magnification revealed normal epidermis, pandermal sclerosis of collagen, diminished adnexal structures with minimal perivascular chronic inflammatory infiltrates (Haematoxylin and eosin, 100x).
- Verheoff’s Van Gieson (VVG) stain showed a marked increase in coarse and fragmented elastin fibres (Verhoeff Van Geison, 40x).
- Immunohistochemistry for vimentin stain was only focally positive (Immunohistochemistry (IHC), 100x).
Discussion
Keloidal morphea is a rare variant of scleroderma, mostly affecting young, middle-aged women of African origin.1 It can occur in association with diffuse systemic sclerosis or localised scleroderma.2 It presents as keloid-like nodules or plaques, which are irregularly shaped and firm at palpation. Keloidal morphea was first described in 1854 by Thomas Addison as an ‘untrue keloid’.3 Although the pathogenesis is unknown, it is thought to be due to an excessive fibrosing reaction ascribed to collagen formation. The literature suggests a combined mechanism with a dermal inflammatory process of sclerosis resulting in keloidal lesions. High levels of tenascin and transforming growth factor-beta (TGF-β) cytokines have been seen in these lesions.4 Histologically, they are characterised by horizontally arranged eosinophilic collagen fibres and spindled fibroblasts. The differential diagnosis of keloidal morphea includes keloid, SLEP, lobomycosis, and dermatomyofibroma. Keloids can be differentiated by the absence of elastic fibres. Lack of lobular lymphocytic panniculitis differentiates it from SLEP. Absence of histiocytic infiltrate, multinucleated giant cells, and fungal cells excludes lobomycosis. Dermatomyofibroma can be ruled out by the absence of spindle-shaped tumour cells arranged in intersecting fascicles that run parallel to the skin surface. In dermatomyofibroma, adnexal structures are preserved, and elastic fibres are often thickened and increased. The cells are positive for vimentin and often for smooth muscle actin, but are focally positive for CD34, factor XIIIa, and desmin.5,6 The treatment options of keloidal morphoea include oral, topical, or intralesional corticosteroids, topical tacrolimus, topical or oral vitamin D analogues, imiquimod, psoralen and ultraviolet A (PUVA), methotrexate, cyclosporine, azathioprine, and methyl prednisolone pulse.1 Ntiri et al. has identified a total of 61 reported cases in the literature so far.7 Our case portrays that keloidal morphea is an important diagnostic consideration in patients who present with linear, pigmented, indurated plaques over the neck. An early diagnosis aids in halting the disease process, thereby preventing contractures, cosmetic disfigurement, and evaluating for any associated systemic sclerosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
Use of artificial intelligence (AI)-assisted technology for manuscript preparation
The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript and no images were manipulated using AI.
References
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