10.5826/dpc.1304a232
text/xml
2160-9381
3218
en
Isil Karaarslan
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
acitretin
Keratosis Lichenoides Chronica (Nekam Disease): Dermoscopic and in Vivo Reflectance Confocal Microscopy Findings
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2023-10-31
2023-02-17
2023-10-31
2024-01-15
e2023232
Dermatol Pract Concept
Isil Karaarslan
2023/10/31
10.5826/dpc.1304a232
e2023232
Department of Dermatology, Medical Faculty, Ege University, Izmir, Turkey
2160-9381
acitretin
en
e2023232
Levine A, Markowitz O. Introduction to reflectance confocal microscopy and its use in clinical practice. JAAD Case Rep. 2018;4(10):1014-1023. doi:10.1016/j.jdcr.2018.09.019
Keratosis Lichenoides Chronica (Nekam Disease): Dermoscopic and in Vivo Reflectance Confocal Microscopy Findings
https://dpcj.org/index.php/dpc/article/download/3218/2393
  Keratosis Lichenoides Chronica (Nekam’s Disease):
    Dermoscopic and in Vivo Reflectance Confocal
                 Microscopy Findings
                           Ayris Ozturk1, Ayda Acar1, Banu Yaman2, Isil Karaarslan1


1 Department of Dermatology, Medical Faculty, Ege University, Izmir, Turkey
2 Department of Pathology, Medical Faculty, Ege University, Izmir, Turkey



Key words: dermoscopy, confocal microscopy, keratosis, lichenoid eruptions, acitretin
Citation: Ozturk A, Acar A, Yaman B, Karaarslan I. Keratosis Lichenoides Chronica (Nekam’s Disease): Dermoscopic and in Vivo
Reflectance Confocal Microscopy Findings. Dermatol Pract Concept. 2023;13(4):e2023232. DOI: https://doi.org/10.5826/dpc.1304a232
Accepted: April 7, 2023; Published: October 2023
Copyright: ©2023 Ozturk et al. This is an open-access article distributed under the terms of the Creative Commons
Attribution-NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted
noncommercial use, distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Ayris Ozturk, MD, Department of Dermatology, Medical Faculty, Ege University, 35100, Bornova, Izmir, Turkey.
Mobile: +90 531 9751441, E-mail: ayrisyanmaz@gmail.com




Introduction                                                             erythroderma (Figure 1). For the management of idiopathic
                                                                         pulmonary fibrosis, he was prescribed 100 mg of azathio-
Keratosis lichenoides chronica (KLC), also known as Nekam                prine and 4 mg of prednisolone. There was no family history
disease, is a rare chronic inflammatory skin disorder charac-            of similar eruption, nor any skin disorders diagnosed.
terized by asymptomatic hyperkeratotic papules in a linear or                 A physical examination revealed erythroderma and
reticular pattern on the trunk and extremities, along with a             generalized pink-brown infiltrated papules with reticu-
­facial rash resembling seborrheic dermatitis and oral ulcers [1].       lar coalescence on the face, trunk, and extremities. Oral
Histopathology reveals lichenoid dermatitis features, and the            ­mucosa and nails were unaffected. Dermoscopy displayed
disease follows a chronic, progressive course with challeng-             globular Wickham striae, dotted and linear vessels, diffuse
ing treatment [2]. This report presents a KLC case, highlight-           brown-gray granules, and perifollicular granules and dots
ing dermoscopy and reflectance confocal microscopy (RCM)                 (­Figure 2B) [3].
findings.                                                                     RCM demonstrated a regular honeycomb pattern on the
                                                                         epidermal level, dense inflammatory infiltration featuring

Case Presentation                                                        dendritic cells, and single-cell necrosis on basal layers. The
                                                                         irregular dermoepidermal junction (DEJ) and upper dermis
A 39-year-old man presented with 3 years of reticular                    displayed a sheet-like cell distribution, including scattered
­lichenoid lesions on the trunk and extremities, along with              small bright round spots correlating lymphocytes, dendritic




Research Letter | Dermatol Pract Concept. 2023;13(4):e2023232                                                                        1
           Figure 1. (A, B) Generalized lichenoid papules exhibiting a reticular coalescence resulting in erythroderma. (C, D)
           A lichenoid infiltration with parakeratosis, basal vacuolar degeneration, lymphoid exocytosis, and dermal inflam-
           matory cell infiltrations with melanophages on histopathology (H&E x100, x200).




cells, single or aggregated plump bright triangular inflam-           Prednisolone dosage was gradually reduced while increasing
matory cells corresponding to melanophages, satellite ne-             acitretin therapy to 35 mg daily. The patient displayed a re-
crosis, vacuolization, and dilated vessels in the upper dermis        markable response to acitretin therapy, with no recurrence in
­(Figure 2, C and D).                                                 two years, continuing acitretin as part of ongoing treatment.
    Histopathology revealed hypergranulosis, epidermal
basal necrotic keratinocytes, and basal lymphoid infiltration
                                                                      Conclusion
(Figure 1, C and D). Superficial dermis exhibited a dense
band of mononuclear cell infiltration with some melano-               Dermoscopy and RCM are established in vivo diagnostic
phages and dilated vessels. Immunofluorescence results were           techniques with notable significance, obviating the require-
negative, while immunohistochemistry showed positivity                ment for histopathologic examination in lesion diagnosis.
for CD3, CD8, CD7, and CD4. Laboratory evaluation was                 The epidermis, DEJ, and upper dermal morphological fea-
unremarkable.                                                         tures can be analyzed using RCM [4]. RCM findings of KLC
    The patient was diagnosed with Nekam disease with                 have not been described in the literature.
clinicopathologic correlation. Treatment involved systemic                The RCM findings of the patient correlated with the
prednisolone at 0.5 mg/kg combined with acitretin at 10 mg.           histopathologic examination. Bright round spots at the DEJ




2                                                                          Research Letter | Dermatol Pract Concept. 2023;13(4):e2023232
             Figure 2. (A) Reticular erythematous lesions on the left forearm (dermoscopy and RCM imaging area). (B) Thick
             whitish lines, dotted and linear vessels, and brown-gray irregular and perifollicular granules and dots on dermos-
             copy. (C) RCM image revealing a sheet-like distribution of cells at the irregular DEJ, featuring scattered small, bright,
             round spots corresponding to lymphocytes (blue arrows), dendritic cells (orange arrows), and single or aggregated,
             plump, bright, triangular inflammatory cells correlating with melanophages (yellow arrows). (D) An RCM image
             showing sheets of inflammatory cells in the upper dermis and obscuring the DEJ, containing small, bright, round
             spots corresponding to lymphocytes (blue arrows), satellite necrosis (necrotic keratinocyte adjacent to a lymphocyte,
             red asterisk), dendritic cells (orange arrows), plump, bright inflammatory cells correlating with melanophages (yel-
             low arrows), vacuolization (green arrows), and dilated vessels (white asterisk).




were associated with dense inflammatory infiltration in-                   References
volving dendritic cells. In certain areas, single-cell necrosis
characteristic of Civatte bodies in lichen was observed. Ir-                1. Pistoni F, Peroni A, Colato C, Schena D, Girolomoni G. Kerato-
                                                                               sis lichenoides chronica: Case-based review of treatment options.
regularities at the DEJ and dilated vessels were associated
                                                                               J Dermatol Treat. 2016;27(4):383-388. doi:10.3109/09546634.
with interface dermatitis. The plump-bright cells present
                                                                               2015.1115818
at the DEJ and in the superficial dermis were indicative of                 2. Böer A. Keratosis Lichenoides Chronica: Proposal of a Con-
melanophages.                                                                  cept: Am J Dermatopathol. 2006;28(3):260-275. doi:10.1097
   This case report illustrates the dermoscopy and RCM                         /00000372-200606000-00052
features of KLC with histopathological correlation, em-                     3. Nandini AS, Zacharias M. A study of dermoscopic patterns of
                                                                               Wickham’s striae in lichen planus. Int J Res Dermatol. 2022;8(2):
phasizing their diagnostic value. Combined with clinical
                                                                               206-11.doi:10.18203/issn.2455-4529.IntJResDermatol20220379
findings, RCM and dermoscopy features can enhance KLC
                                                                            4. Levine A, Markowitz O. Introduction to reflectance confocal
diagnosis.                                                                     microscopy and its use in clinical practice. JAAD Case Rep.
                                                                               2018;4(10):1014-1023. doi:10.1016/j.jdcr.2018.09.019




Research Letter | Dermatol Pract Concept. 2023;13(4):e2023232                                                                                 3
