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Year : 2022  |  Volume : 67  |  Issue : 5  |  Page : 603-606
Childhood keratosis lichenoides chronica: Treated successfully with acitretin and NBUVB

Department of Dermatology, Venereology and Leprology, Pt B. D. Sharma University of Health Sciences, Rohtak, Haryana, India

Date of Web Publication29-Dec-2022

Correspondence Address:
Priyadarshini Sahu
Department of Dermatology, Venereology and Leprology, Pt B. D. Sharma University of Health Sciences, Rohtak, Haryana
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_123_22

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How to cite this article:
Sahu P, Dayal S, Sachdeva M, Chakraborty D. Childhood keratosis lichenoides chronica: Treated successfully with acitretin and NBUVB. Indian J Dermatol 2022;67:603-6

How to cite this URL:
Sahu P, Dayal S, Sachdeva M, Chakraborty D. Childhood keratosis lichenoides chronica: Treated successfully with acitretin and NBUVB. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 25];67:603-6. Available from:


An 8-year-old female presented with itchy, scaly, and crusted skin lesions all over the body for 1 year [Figure 1]a, [Figure 1]b, [Figure 1]c, [Figure 1]d. History of improvement during summer, after exposure to sunlight, was present. On examination, she had multiple erythematous to lichenoid papules coalescing to form plaque in the bilaterally symmetrical reticular pattern on the trunk and both extremities. Verrucous plaques were present on the buttocks and extremities. There was hypertrophy of the periungual areas of fingers and toes, conjunctival hyperemia, and oral erosions. Routine investigations were within normal limits. Skin biopsy was advised and was consistent with the features of Keratosis lichenoides chronica (KLC) [Figure 1]d.
Figure 1: (a) Erythematous to violaceous hyperkeratotic papules and plaques arranged in a bilaterally symmetrical reticular pattern abdomen, bilateral hands and legs; (b) on the back and buttocks; (c) Erosions on oral mucosae; (d) Histopathological examination shows epidermal atrophy, hyperkeratosis, pigment incontinence and a band of lymphocytic infiltrate in the dermis [Hematoxylin and eosin stain (H&E); 40×]

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The patient was started on NBUVB thrice weekly at an initial dose of 50 mJ/cm2 with a 10% increment of the previous dose on each subsequent visit. The patient did not achieve complete improvement, even after 1 month of NBUVB. Thus, in addition to NBUVB, the patient was started on acitretin (1 mg/kg/day). After 2 months of this combination, flattening of lesions with total healing in some areas and fading of the erythema occurred [Figure 2]. Initially, the patient was given thrice weekly for 6 months, then twice weekly for 4 months, and then weekly for the next 2 months. A total of 112 treatment sessions of Narrow band ultraviolet B (NBUVB) were given. There was complete clearance of lesions with no side effects and no recurrence during the 6 months follow-up period.
Figure 2: (a and b): Clearance of the lesions from the abdomen after oral acitretin plus NBUVB phototherapy

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KLC is a rare keratinization disorder, typically affecting adults 20–50 years of age. The disease is very rare with approximately 26 reported cases of pediatric-onset KLC till date and few from India. The cases of pediatric KLC are summarized in [Table 1].[1],[2],[3] Few cases of familial KLC are due to a germline mutation in NLR family, pyrin domain containing 1 (NLRP1). NLRP1 is an inflammasome sensor gene, which activates inflammatory cytokines such as keratinocyte growth factor, interleukin-1, and tumor necrosis factor-alpha, resulting in epidermal hyperplasia and keratosis.[4] Mucosal involvement, ocular manifestations, palmoplantar keratoderma, and nail dystrophy are reported in 20–30% of patients.[5] Along with the cutaneous lesions, ocular and oral mucosal involvement was also seen in our patient. The differential diagnosis of KLC includes lichen planus, lichen planopilaris, pityriasis rubra pilaris, mycosis fungoides, and drug eruptions.
Table 1: Review of literature on pediatric onset KLC

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Cases of regression during the summer or spontaneous healing have also been reported. Our case had shown dramatic progression over time. Good results were seen in patients treated with systemic retinoids, psoralen with ultraviolet A and NBUVB.[2],[6] There are recent reports of successful use of photochemotherapy and acitretin in treating adolescent KLC patients, who had pediatric-onset KLC.[2] In our case, NBUVB might have exerted its therapeutic effect through antiproliferative and immunosuppressive mechanisms in the treatment of KLC.[4] Oral acitretin has keratolytic, antiproliferative, and anti-inflammatory effects in KLC. However, prolonged use of acitretin in children is limited by its side effects. Further, NBUVB may enhance the anti-inflammatory effect of oral acitretin and minimize the side effects by reducing the dose of both retinoids and phototherapy. Thus, we conclude that combining NBUVB with oral acitretin augments the efficacy of each other and can be used in difficult-to-treat conditions of KLC. On a detailed literature search, successful treatment of NBUVB and oral acitretin has not been reported so far, although acitretin with PUVA is a well-documented treatment for KLC.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Nasiri S, Rakhshan A, Zahedi K, Dadkhahfar S, Gheisari M. Childhood-onset keratosis lichenoides chronica: A case report. Clin Case Rep 2021;9:1462–5.  Back to cited text no. 1
Julien B, Pierre K, El-Haber C, Samer G, Fatima G, Hala M, et al. Two cases of paediatric keratosis lichenoides chronica with review of the clinical and histopathological features of pediatric versus adult presentation and treatment with acitretin. J Clin Exp Dermatol Res 2019;10:476.  Back to cited text no. 2
Nasimi M, Azizpour A, Lajevardi V, Nikoo A, Etesami I. A case of pediatric keratosis lichenoides chronica with unusual presentation of severe oral erosions. JAAD Case Rep 2018;4:267-9.  Back to cited text no. 3
Zhong FL, Mamaï O, Sborgi L, Boussofara L, Hopkins R, Robinson K, et al. Germline NLRP1 mutations cause skin inflammatory and cancer susceptibility syndromes via inflammasome activation. Cell 2016;167:187-202.  Back to cited text no. 4
Pistoni F, Peroni A, Colato C, Schena D, Girolomoni G. Keratosis lichenoides chronica: Case-based review of treatment options. J Dermatolog Treat 2016;27:383-8.  Back to cited text no. 5
Demirci E, Boyvat A, Arica IE, Kocyigit P, Ozdemir E, Heper AO. Keratosis lichenoides chronica: Market response to PUVA in combination with acitretin. Acta Derm Venereol 2005;85:552-3.  Back to cited text no. 6
Patrizi A, Neri I, Passarini B, Varotti C. Keratosis lichenoides chronica: a pediatric case. Dermatology. 1995;191:264-7.  Back to cited text no. 7


  [Figure 1], [Figure 2]

  [Table 1]


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