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LETTER TO EDITOR
Year : 2022 | Volume
: 23
| Issue : 3 | Page : 256-257
Treatment of Classic Juvenile Pityriasis Rubra Pilaris with Oral Isotretinoin
Pooja Vilhekar, Kirti Jangid, Swagata Tambe
Department of Dermatology, Seth V.C. Gandhi and M.A Vora Municipal General Hospital, Mumbai, Maharashtra, India
Correspondence Address:
Kirti Jangid
Department of Dermatology, Seth V.C. Gandhi and M.A Vora Municipal General Hospital, M G Road, Ghatkopar East, Mumbai - 400 077, Maharashtra
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/ijpd.ijpd_165_21
Sir,
Pityriasis rubra pilaris (PRP) is a group of papulosquamous disorders. Juvenile PRP is rare with a prevalence of approximately 1:5000–1:50,000. We report a case of classical juvenile PRP in a 9-year-old female child successfully treated with oral isotretinoin.
A 9-year-old female presented with a 7-day history of diffuse scaling with erythema over the face and erythematous scaly plaques on the palms and soles. Lesions progressed over a period of 10 days to form pruritic well-demarcated scaly plaques involving her elbows, knees, back [Figure 1] with islands of sparing, follicular erythematous papules across her trunk with evidence of koebnerization, and diffuse scaling on the scalp.
Figure 1: Pretreatment images of the patient. Diffuse scaling and erythema of the face (a), hyperkeratotic scaly plaques on palms (b), well-demarcated scaly plaques involving the right elbow and bilateral knees (c and d)There was no history of fever, drug intake, photosensitivity, and atopic diathesis. Her past medical, family history, and review of systems were noncontributory.
The skin biopsy revealed compact hyperkeratosis with alternate parakeratosis and orthokeratosis overlying hypogranulosis and hypergranulosis with dermis showing dilated blood vessels [Figure 2]. Based on the clinical and histopathological findings, a diagnosis of juvenile classic PRP was established.
Figure 2: Histopathological images of skin biopsy stained by hematoxylin and eosin showing compact hyperkeratosis with alternate parakeratosis and orthokeratosis overlying hypogranulosis and hypergranulosis with dermis showing dilated blood vessels (a: H and E, ×100 and b: H and E, ×400)The patient was managed initially with topical desonide lotion, emollients, and hydroxyzine syrup for a period of 2 weeks with no significant response and rapid progression of the disease. Considering the risk of impending erythroderma, isotretinoin was started at 0.5 mg/kg for a period of 2 months until all lesions cleared [Figure 3] thereafter reduced to 0.25 mg/kg for 1 month following which patient has been on topical emollients. Oral isotretinoin was considered over methotrexate as most studies mention systemic retinoids as first-line therapy, and acitretin was nonaffordable. She was investigated with complete blood count, blood sugars and fasting lipids at the onset of treatment and repeated monthly until Isotretinoin discontinuation. The patient has been on monthly follow-up without any evidence of recurrence for the past 6 months.
Figure 3: Posttreatment images of the patient. Resolved scaling and erythema of the face (a), trunk with clearance of lesions and postinflammatory hypopigmentation (b), resolved hyperkeratosis of palms (c), elbows showing lesion clearance with postinflammatory hypopigmentation (d) Discussion 
PRP is a group of rare inflammatory papulosquamous disorders characterized by scaly well-demarcated salmon-colored erythematous plaques. It can progress to erythroderma with islands of sparing.[1],[2]
Griffith classified PRP into Type I (classic adult), Type II (atypical adult), Type III (classic juvenile), Type IV (circumscribed juvenile), Type V (atypical juvenile), and Type VI (human immunodeficiency virus-associated).[3]
Management of juvenile PRP is challenging. Most literature mentions the first line of treatment as topical emollients, topical corticosteroids, keratolytic, and topical Vitamin D3 analogs. Systemic therapy comprising retinoids, methotrexate, antiretroviral therapy in HIV-associated types, phototherapy, photochemotherapy, extracorporeal photopheresis, and biological immunosuppressants such as secukinumab and ustekinumab have shown benefits. Systemic retinoids are considered first-line systemic agents in moderate-to-severe disease with recommended dosages of 1 mg/kg daily isotretinoin or 0.5 mg/kg daily acitretin with marked improvement in 3 to 6 months. Laboratory evaluation includes complete blood count with differential, blood sugars, and fasting lipids to be repeated monthly for the first 2 months and every 3 monthly thereafter. The most common side effects are dry skin and mucous membranes. Less commonly encountered are hyperlipidemia, transaminase elevations, and visual changes. Rarely, retinoids could induce hyperostosis and premature epiphyseal closure, especially in prepubertal patients, treated with high doses for long durations.[4],[5] The prognosis for this skin disorder is variable and unpredictable.
We report successful treatment of juvenile classic PRP with isotretinoin 0.5 mg/kg daily for 2 months with gradual tapering thereafter. Our patient's lesions started improving within the first 4–5 weeks, and there was complete clearance of all lesions in 8 weeks.
Declaration of patient consent
The authors certify that they have obtained all appropriate consent forms, duly signed by the parent(s)/guardian(s) of the patient. In the form, the parent(s)/guardian(s) has/have given his/her/their consent for the images and other clinical information of their child to be reported in the journal. The parents understand that the names and initials of their child/children will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References
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