10.5826/dpc.1303a154
text/xml
2160-9381
2953
en
Kabir Sardana
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
cyclophosphamide
Cyclophosphamide in a Recalcitrant Case of Nekam’s Disease
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2023-07-31
2022-11-06
2023-07-31
2023-08-24
e2023154
Dermatol Pract Concept
Kabir Sardana
2023/07/31
10.5826/dpc.1303a154
e2023154
Department of Dermatology Venereology and Leprosy, Atal Bihari Vajpayee Institute of Medical Sciences and Dr Ram Manohar Lohia Hospital, New Delhi, India
2160-9381
cyclophosphamide
en
e2023154
Mayer J, Krejcí M, Doubek M, et al. Pulse cyclophosphamide for corticosteroid-refractory graft-versus-host disease. Bone Marrow Transplant 2005;35:699-705. PMID: 15696180 DOI: 10.1038/sj.bmt.1704829.
Cyclophosphamide in a Recalcitrant Case of Nekam’s Disease
https://dpcj.org/index.php/dpc/article/download/2953/2218
              Cyclophosphamide in a Recalcitrant Case
                        of Nekam’s Disease
                         Aishwarya Muddebihal1, Ananta Khurana1, Kabir Sardana1


1 Department of Dermatology Venereology and Leprosy, Atal Bihari Vajpayee Institute of Medical Sciences and Dr Ram Manohar Lohia
  Hospital, New Delhi, India



Key words: Nekam’s disease, recalcitrant, cyclophosphamide
Citation: Muddebihal A, Khurana A, Sardana K. Cyclophosphamide in a recalcitrant case of Nekam’s disease.
Dermatol Pract Concept. 2023;13(3):e2023154. DOI: https://doi.org/10.5826/dpc.1303a154
Accepted: January 2, 2023; Published: July 2023
Copyright: ©2023 Muddebihal 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: Dr. Ananta Khurana, Professor, Department of Dermatology, Venereology and Leprosy, Atal Bihari Vajpayee
Institute of Medical Sciences and Dr Ram Manohar Lohia Hospital, New Delhi, -110001. Phone no: +91-9212370467
E-mail: drananta2014@gmail.com
Ethics Statement: The patient in this manuscript has given written informed consent for publication of case details and clinical
photographs.




Introduction                                                             gradually progressed. She also complained of burning sen-
                                                                         sation on eating. Cutaneous examination revealed multiple,
Keratosis Lichenoides Chronica, also called as Nekam’s dis-              linear, hyperkeratotic violaceous plaques over flexor aspect
ease, is a rare acquired muco-cutaneous inflammatory dis-                of upper limbs, dorsum of hands, trunk, abdomen, buttocks,
order affecting adults and rarely children. It is characterized          and lower limbs. (Figure 1A) She also had rosacea-like le-
clinically by asymptomatic violaceous papules and plaques                sions on the face (Figure 1B) and multiple aphthous ulcers
arranged in linear and reticular fashion and histologically by           over bilateral buccal mucosa (Figure 1C), the combination
interface dermatitis [1]. It may be associated with features             of features suggesting Nekam’s disease.
like rosacea, seborrheic dermatitis like rash (75%), recur-                   Histopathology revealed parakeratotic hyperkeratosis
rent oral aphthous ulcer (50%), palmoplantar keratoderma                 covering acanthotic and focally flattened epidermis and
(40%) and nail dystrophy (30%) [2]. We report a rare con-                band-like lymphocytic infiltrate admixed with plasma cells
dition in its classical form and a sustained remission with              abutting the basal layer which showed vacuolization con-
cyclophosphamide.                                                        firming the clinical diagnosis.
                                                                              Her routine blood investigations were normal. The

Case Presentation                                                        patient had received methotrexate (7.5-15 mg for 3 months)
                                                                         and acitretin (25 mg once daily for 1 month) previously with
A 25-year-old female, presented with 5-years history of mild             no clinical improvement. She was subsequently administered
itchy lesions which had started during pregnancy, and had                pulse intravenous methylprednisolone 500 mg for 3 days



Research Letter | Dermatol Pract Concept. 2023;13(3):e2023154                                                                      1
    Figure 1. (A) Erythematous to violaceous papules and plaques in reticular fashion over both upper limbs. (B) Rosacea like lesions on
    face. (C) Aphthous ulcer over right buccal mucosa. (D) Resolution of cutaneous lesions with post inflammatory hyperpigmentation
    after five months of cyclophosphamide.




which was deferred later due to ECG changes. She was then               Conclusions
treated with cyclophosphamide 100mg once daily (OD)
which was tapered to 50 mg OD after a month, followed                   Nekam’s disease and other lichenoid disorders are charac-
by subsequent tapering to 50 mg alternate days. Progressive             terized by an interface inflammatory cell infiltrate largely
improvement occurred during this with resolution of muco-               consisting of lymphocytes and plasma cells. At a molecular
sal lesions and all cutaneous lesions with post inflammatory            level CD4+ Th1 cells and CD8+ T lymphocytes, and Natural
hyperpigmentation (Figure 1D) at the end of five months, at             killer cells mediate the damage to basal cells in this group
which point cyclophosphamide was stopped. The patient’s                 of disorders [3]. Phototherapy, retinoids, methotrexate, dap-
blood counts and urine analysis were closely monitored                  sone, steroids, antimalarials, ciclosporin, efalizumab, have
throughout the course of treatment and demonstrated no                  all been tried with variable efficacy in Nekam’s disease [4].
abnormalities. Four months later she had a mild recurrence              Cyclophosphamide is a potent immunosuppressive agent
which responded to cyclophosphamide 50mg given 3 days                   which has demonstrated efficacy in treating resistant der-
a week for a month. The patient has since been off systemic             matoses including recalcitrant lichen planus and cutaneous
treatment with occasional minor localized recurrences man-              lupus, dermatomyositis, PLEVA, refractory graft versus host
ageable with topical agents alone.                                      disease, etc; all characterized by an interface infiltrate rich in




2                                                                           Research Letter | Dermatol Pract Concept. 2023;13(3):e2023154
lymphocytes. This is as lymphocytes are exquisitely sensitive    3. Tziotzios C, Lee JYW, Brier T, et al. Lichen planus and li-
to the drug owing to lack of detoxifying enzyme aldehyde            chenoid dermatoses: Clinical overview and molecular basis.
                                                                    J Am Acad Dermatol. 2018;79(5):789-804. DOI: 10.1016/j.jaad
dehydrogenase [5,6]. The reported case thus exemplifies the
                                                                    .2018.02.010. PMID: 30318136.
drug use in yet another typically difficult to treat dermato-
                                                                 4. Nomura T, Toichi E, Miyachi Y, Kabashima K. A Mild Case
logic condition, and adds to the armamentarium to deal with         of Adult-Onset Keratosis Lichenoides Chronica Successfully
this recalcitrant disorder.                                         Treated with Narrow-Band UVB Monotherapy. Case Rep Der-
                                                                    matol. 2012;4(3):238-241. DOI: 10.1159/000345277. PMID:
                                                                    23185159. PMCID: PMC3506084.
References                                                       5. Deakin CT, Campanilho-Marques R, Simou S, et al. Efficacy and
                                                                    Safety of Cyclophosphamide Treatment in Severe Juvenile Der-
 1. Aruna C, Ramamurthy DV, Neelima T, Bandaru H. Nekam’s
                                                                    matomyositis Shown by Marginal Structural Modeling. Arthritis
    disease. Indian Dermatol Online J. 2016;7(6):520-522. DOI:
                                                                    Rheumatol. 2018;70(5):785-793. DOI: 10.1002/art.40418.
    10.4103/2229-5178.193923. PMID: 27990390. PMCID:
                                                                    PMID: 29342499. PMCID: PMC5947636.
    PMC5134169.
                                                                 6. Mayer J, Krejcí M, Doubek M, et al. Pulse cyclophosphamide for
 2. Taberner R, Puig L, Fernández-Figueras T, Alomar A. Ker-
                                                                    corticosteroid-refractory graft-versus-host disease. Bone Marrow
    atosis lichenoides chronica. J Eur Acad Dermatol Venereol.
                                                                    Transplant. 2005;35(7):699-705. DOI: 10.1038/sj.bmt.1704829.
    2001;15(1):84-85. DOI: 10.1046/j.1468-3083.2001.00178-7.x.
                                                                    PMID: 15696180.
    PMID: 11451338.




Research Letter | Dermatol Pract Concept. 2023;13(3):e2023154                                                                     3
