|Year : 2018 | Volume
| Issue : 1 | Page : 73-75
Blaschkolinear lichen nitidus: A rare presentation
Rajeshwari Dabas, Manasa Shettisara Janney, Sandeep Arora, Radhakrishnan Subramaniyan
Department of Dermatology, Venereology and Leprosy, Command Hospital Air Force Bangalore, Bengaluru, Karnataka, India
|Date of Web Publication||9-Jul-2018|
Dr Manasa Shettisara Janney
Department of Dermatology, Venereology and Leprosy, Command Hospital Air Force Bangalore, Old Airport Road, Bengaluru - 560 007, Karnataka
Source of Support: None, Conflict of Interest: None
Lichen nitidus is an uncommon, chronic, usually asymptomatic papulosquamous dermatosis of unknown etiology, originally described by Felix Pinkus, a dermatopathologist in 1901. It generally presents as multiple, tiny, discrete, flesh-colored, dome-shaped papules most commonly on the extremities, genitalia, chest, and abdomen. We hereby report an uncommon and atypical presentation of “Blaschkolinear lichen nitidus” involving the trunk. A 15-year-old boy presented with a 1-year history of gradually progressive, multiple, small, raised lesions on the left side of the trunk. On examination, there were multiple, skin-colored to erythematous, discrete to confluent papules on the left side of the trunk in a curvilinear fashion. Dermoscopy, histopathology, and immunohistochemistry supported the clinical diagnosis of Blaschkolinear lichen nitidus. The patient was treated with topical steroid with good response.
Keywords: Blaschkolinear, immunohistochemistry, lichen nitidus, papulosquamous dermatosis
|How to cite this article:|
Dabas R, Janney MS, Arora S, Subramaniyan R. Blaschkolinear lichen nitidus: A rare presentation. J Mar Med Soc 2018;20:73-5
| Introduction|| |
Lichen nitidus is an uncommon papulosquamous dermatosis with an unrecognized etiology and pathogenesis. The association of lichen nitidus with lichen planus is controversial. Immunohistochemical (IHC) studies to characterize dermal lymphocytic infiltrate and to link the two dermatoses are inconclusive.,
We hereby report a rare case of unilateral Blaschkolinear lichen nitidus with atypical distribution. Very few cases of blaschkolinear lichen nitidus have been reported in the existing literature.,
| Case Report|| |
A 15-year-old male with no known comorbidities presented with the complaints of multiple nonpruritic, gradually progressive, small, raised lesions on the left side of the trunk of 1-year duration. Lesions initially appeared on the abdomen and gradually progressed toward the back in a curvilinear fashion. There was no history of any other skin, nail, or mucosal lesions or any systemic symptoms. Dermatological examination revealed multiple, tiny, discrete to confluent, skin-colored to erythematous, flat-topped papules of 1–5 mm size over the left side of abdomen extending posteriorly in an “S” pattern along the Blaschko's lines with no signs of koebnerization [Figure 1]. The dermoscopic evaluation showed circular, pale structureless areas with a peripheral rim of delicate pigment network and serpentine vessels. Histopathological examination revealed thinning of epidermis with degenerative changes in basal cell layer and irregular deep invagination of rete ridges. Papillary dermis showed dense mixed inflammatory infiltrate hugging the epidermis consisting predominantly of lymphohistiocytic cells. Rete ridges were seen bending downward and inward and seemed to clutch the infiltrate in a claw-like manner [Figure 2]. On IHC examination, the infiltrate was positive for CD45, CD3, CD20, CD68, CD4, and CD1a [Figure 3].
|Figure 1: Left: Linear blaschkoid papules of lichen nitidus, pretreatment with evident flat topped morphology (inset) and right: resolution on treatment|
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|Figure 2: Upper left: Photomicrograph showing thinning of epidermis with typical appearance of rete ridges of “claw clutching the ball” H and E × 10; upper right: dense mixed inflammatory infiltrate hugging the epidermis consisting predominantly of lympho-histiocytic cells; lower left: Dermoscopic – nonpolarized appearance of flat topped papules × 60; lower right: Dermoscopic- polarized appearance of circular, pale structure less areas with a peripheral rim of delicate pigment network and serpentine vessels|
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|Figure 3: Immunohistochemistry revealing positive antigen status for CD4 and CD3, 68, 1a, S100, CD8 (inset) (×40)|
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Based on the clinicopathological examination, a diagnosis of Blaschkolinear lichen nitidus was made, and the patient was started on topical 0.05% clobetasol propionate cream. Lesions regressed in 4 weeks, and the patient is under follow-up.
| Discussion|| |
Lichen nitidus is an uncommon papulosquamous dermatosis of unknown etiology with an incidence of 0.034% in Afro–Americans. Diagnosis is mostly clinical with the classic presentation of tiny, discrete, pinhead-sized, flat-topped papules over extremities, trunk, and genitalia. However, in rare instances, there can be varied morphology, distribution, and associations necessitating histopathological examination for accurate diagnosis. Atypical presentations of lichen nitidus reported until date include linear, vesicular, purpuric, actinic, hemorrhagic, perforating, follicular, periappendageal, generalized, and palmoplantar variants. Although a few case reports have revealed its association with lichen planus, erythema nodosum, segmental vitiligo, lichen spinulosus, psoriasis, atopic dermatitis, neurofibromatosis type 1, juvenile myelomonocytic leukemia, Niemann–Pick disease, congenital megacolon, Crohn's disease, juvenile chronic arthritis, and Down's syndrome, the exact pathogenesis is obscure.,,
Lichen nitidus is considered as a variant of lichen planus by some, but this belief is much debated. IHC study to resolve this uncertainty by Smoller and Flynn revealed mixed cellular infiltrate characterized by macrophages and a helper T-cell response with few HECA-452+ cells in lichen nitidus in contrast to lichen planus wherein majority of lymphocytes were CD4+/HECA-452+ suggesting dissimilar pathogenesis. IHC study in our case was positive for CD45, the leukocyte common antigen. It further revealed CD3 (+++) and CD4 positive T helper phenotype with some CD20 (+) positive B cells in the papillary dermis hugging epidermis. Few cells were positive for CD68 and CD1a indicating the presence of macrophage/histiocyte and Langerhans cells, respectively, similar to another study by Nakamizo et al. The IHC findings are similar to the observations made by Smoller et al. on lichen nitidus and signify the mixed nature of inflammatory infiltrate in lichen nitidus vis-à -vis lichen planus. Furthermore, the digression of the type of infiltrate in our study as against the CD4+ and CD8+ T cell infiltrate in lichen planus reported in earlier studies supports the theory of dissimilar pathogenesis in these two conditions.
Lichen nitidus is generally asymptomatic, and in two-thirds of patients, it resolves spontaneously in a few months to 1 year. Treatment is instituted to address the cosmetic concerns of the patient. Various therapies such as systemic retinoids, enoxaparin sodium, itraconazole, cyclosporine, topical steroids, topical calcineurin inhibitors, and phototherapy have been used successfully in lichen nitidus. Resolution of lesions is reported as early as 4 weeks to 5 months with topical steroids and systemic retinoids, respectively.
The lines of Blaschko are “a system of lines on the human skin which the linear naevi and dermatoses follow.” It was first described in 1901 by Alfred Blaschko. These lines represent the developmental growth pattern within the skin and do not correspond to the local nervous, vascular or lymphatic structures. The embryological basis of the distribution of these lines is not clear and is thought to be due to “mosaicism” where two or more genetically distinct cell populations are present in an individual derived from a single zygote.
Some of the dermatoses that follow the Blaschko's lines include lichen striatus, linear psoriasis, linear lichen planus, linear porokeratosis, linear Darier's disease, linear mucinosis, mycosis fungoides, linear cutaneous lupus erythematosus, extragenital lichen sclerosus, etc. Blaschkolinear lichen nitidus is rare and infrequently reported.
Blaschkolinear pattern, distinctive IHC markers, early and complete response to topical steroid in the index case is noteworthy.
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
Conflicts of interest
There are no conflicts of interest.
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