|Year : 2021 | Volume
| Issue : 1 | Page : 104-106
A case of lichen planus pigmentosus in blaschkoid pattern in a 10-year-old female
Tulika Rai, Ayushi Bohara, Prasanna Kumar Jha
Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India
|Date of Submission||20-Oct-2019|
|Date of Decision||16-Feb-2020|
|Date of Acceptance||15-Apr-2020|
|Date of Web Publication||19-Feb-2021|
Prasanna Kumar Jha
Department of Dermatology and Venereology, Institute of Medical Sciences, Banaras Hindu University, Sir Sunderlal Hospital, Varanasi - 221 005, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
We describe a rare case of lichen planus pigmentosus (LPP) involving a 10-year-old female child with the involvement of the anterolateral aspect of the left thigh following the lines of Blaschko. LPP is a rare pigmented variant of lichen planus. Lines of Blaschko are lines of normal cell development. Histopathology showed melanophages in the papillary dermis with perivascular lymphocytic infiltrate and focal vacuolar change in basal layer consistent with a diagnosis of LPP.
Keywords: Lichen planus pigmentosus, lines of Blaschko, LPP
|How to cite this article:|
Rai T, Bohara A, Jha PK. A case of lichen planus pigmentosus in blaschkoid pattern in a 10-year-old female. Clin Dermatol Rev 2021;5:104-6
| Introduction|| |
Lichen planus pigmentosus (LPP) is a rare pigmented variant of lichen planus (LP). Lines of Blaschko are lines of normal cell development. It was first reported from India in 1974 by Bhutani et al. who also coined the term. LP distributed along Blaschko lines was first described by Taniguchi et al. in 1993 as “Blaschkonian LP”. The pattern of pigmentation is mostly diffuse or reticular, rarely blotchy, or perifollicular. This can be presented as rare variants such as linear or Blashkoid, zosteriform, and inverse pattern.
| Case Report|| |
A 10-year-old female child, presented in the outpatient clinic of dermatology department with asymptomatic and progressive hyperpigmented lesions for 3 years. On examination, irregular, discrete to confluent, brown-to-black hyperpigmented, nonatrophic, noncontinuous, and nonscaly macules were present which first appeared over the anterolateral aspect of the left upper thigh and gradually extended downward over the lateral side of the thigh and upwards toward left iliac crest [Figure 1]. There was no history of preceding erythema, scaling, or other lesions. Mucous membranes, scalp, and nails were spared. No history of any drug intake, topical application, sun exposure, contact with chemicals, perfumes, or plants or trauma. There was no history of systemic disease and no relevant family history. Systemic examination and routine laboratory tests were within the normal limits. Histopathology revealed sparse superficial perivascular lymphocytic infiltrate with numerous melanophages within the papillary dermis. The papillary dermis was slightly thickened and showed dense inflammation. Overlying epidermis showed the focal vacuolar change in the basal layer. The epidermis was flattened at places [Figure 2]. On the basis of clinical features and supporting histopathological findings, the diagnosis of LPP in a Blaschkoid distribution was made. The patient was given low dose prednisolone (0.5 mg/kg/day) along with mometasone 0.1%, after initial unresponsiveness to topical steroids. She was lost to follow-up.
|Figure 2: Histopathology revealed sparse superficial perivascular lymphocytic infiltrate with numerous melanophages within the papillary dermis. The papillary dermis was slightly thickened and showed densec inflammation. Overlying epidermis showed the focal vacuolar change in the basal layer. The epidermis was flattened at places (H&Ex10)|
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| Discussion|| |
LPP is a rare pigmentary variant of LP . Onset usually occurs in the third or fourth decade with slight female preponderance. It principally affects sun-exposed areas such as the face, trunk, and upper extremities as well as flexural areas, such as the axillary, inguinal, and submammary regions with face and neck being most frequent sites. It may involve the mucosa but spares palms, soles, and nails.,, It usually manifests in the chronic and persistent course. Usually are asymptomatic, but pruritus reported in 30% of patients. Although exact etiology remains unknown, it may represent a lichenoid reaction to unknown agents such as viral infections, vaccinations, trauma, ultraviolet exposure, topically applied mustard oil which contains a potential photosensitizer allyl thiocyanate, amla oil, cosmetic agents such as kumkumand hair dyes.,, Pathogenesis also remains unknown; however, cell-mediated immune response with CD8+ T-lymphocytes along with mediators such as interferon-gamma, tumor necrosis factor-alpha, interleukin 6, and lymphocyte function-associated antigen 1 has some role. There are three rare variants of LPP, which include linear or Blashkoid, zosteriform, and inverse variant. Zosteriform LP appears with a metameric pattern in relation to segmental cutaneous nerves and occurs along with dermatomal distribution. Lesions of the Blaschkoid pattern appear in the shape of “V” with open arms in dorsal raquis, in the form of horizontal “S” in the anterior trunk, spiral in the scalp and in longitudinal shapes in limbs. Blaschkoid pattern suggests the development of LPP may be determined during embryogenesis. The genetic mosaicism in an acquired Blaschko-linear inflammatory dermatosis could be responsible for the cutaneous antigenic mosaicism that may induce a mosaic T-cell response. LPP inversus occurs in covered intertriginous locations such as groins and axillae and mostly affects white-skinned persons. LPP has been reported to be associated with scarring alopecia and circulating antinuclear antibodies, frontal fibrosing alopecia, acrokeratosis of Bazex and head-–and-neck carcinoma (paraneoplastic LPP), hepatitis C infection, and nephrotic syndrome. There have been various case reports of LPP. In 2004, Hong et al. were the first to describe LPP, which presented in a linear pattern on the leg and the arm. Seo et al. later reported a case of linear LPP, which involved a 60-year-old male patient with a history suggestive of gastric cancer. He developed unilaterally on his neck and chin. Few cases have been reported since then.
Differential diagnosis is LP, erythema dyschromicum perstans/Ashy dermatoses, nevoid hypermelanosis, incontinentia pigmenti, lichen striatus, riehl's melanosis, drelated melanoses, postinflammatory hyperpigmentation. Classical LP differentiated by shiny, purplish papules and plaques, and also mucosa, scalp, and nails are frequently involved, which are spared in LPP. Although the histopathological findings are similar, melanin incontinence is more prominent in LPP than in classic LP. LP lesions may rarely occur concomitantly with LPP. However, in LPP, pruritus, the hallmark of LP is usually absent., Lichen striatus almost always has preceding inflammatory papules or a scaly eruption, which lasts for 4 months–4 years and a perivascular and peri-adnexal inflammatory cell infiltration. Linear and whorled nevoid hyper-melanosis usually present within a few weeks of birth, and it is associated with congenital anomalies. The histology shows basal pigmentation without pigment incontinence. Ashy dermatosis characterized by ash colored polycyclic macules which are not restricted to sun-exposed areas and have elevated erythematous border. In ashy dermatosis and postinflammatory hyperpigmentation, the histopathology shows slightly smoothed epidermis with a scanty perivascular lymphocytic infiltration., Other uncommon differentials are a macular variety of discoid lupus erythematosus (scarring, atrophy, erythema, dyspigmentation, typical holoprosencephaly (histopathology), absence of basal layer degeneration and melanin incontinence); the linear variety of fixed drug eruption (typical drug history, especially trimethoprim, temporal association with drug intake, distinctive histopathology), and focal dermal elastolysis (yellowish skin nodules and histopathology showing degeneration of elastic tissue).
Currently, no specific treatment is available. As melanophages are present in the papillary dermis, the disease is usually resistant or shows only a mild response to topical treatments. Multiple oral and topical drugs have been tried including 10% aqueous solution of dimethyl sulfoxide, topical steroids, keratolytics, tacrolimus, Vitamin A, hydroquinone with/without retinoic acid, azelaic acid, kojic acid, and glycolic acid, griseofulvin, prednisone, retinoids, and chloroquine,,,, and recently laser treatment, but none of these showed consistent results.
| Conclusion|| |
We present this case because of its rarity and atypical presentation and should be considered in the differential diagnosis of macular hyperpigmentation.
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]