Indian Journal of Dermatology
: 2020  |  Volume : 65  |  Issue : 6  |  Page : 542--543

Flexural circumscribed juvenile pityriasis rubra pilaris: An unusual presentation

Md Zeeshan, Abhijeet K Jha, Noorussaba Arfeen, RK P Chaudhary 
 Department of Skin and VD, Patna Medical College and Hospital, Patna, India

Correspondence Address:
Md Zeeshan
Department of Skin and VD, Patna Medical College and Hospital, Patna

How to cite this article:
Zeeshan M, Jha AK, Arfeen N, P Chaudhary R K. Flexural circumscribed juvenile pityriasis rubra pilaris: An unusual presentation.Indian J Dermatol 2020;65:542-543

How to cite this URL:
Zeeshan M, Jha AK, Arfeen N, P Chaudhary R K. Flexural circumscribed juvenile pityriasis rubra pilaris: An unusual presentation. Indian J Dermatol [serial online] 2020 [cited 2022 May 22 ];65:542-543
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Pityriasis rubra pilaris (PRP) is a chronic papulosquamous disorder of unknown etiology characterized by branny desquamation, palmoplantar keratoderma, and follicular hyperkeratosis.[1]

Circumscribed juvenile type PRP (type IV) occurs in prepubertal children or young adults and is characterized by well-defined scaly plaques on elbows, knees, and palmoplantar keratoderma. There is paucity of literature on circumscribed juvenile type PRP with flexural involvement. We report a case of circumscribed juvenile type PRP who had flexural presentation.

A 13-year-old boy presented with pruritic scaly follicular papules, which coalesced to form plaques in axillae, flexural aspect of elbows and knees, groins, and ankles along with hyperkeratosis of palms and soles since last 4 years. The patient first noticed thickening of the palms and soles along with occasional painful fissuring and winter exaggeration. Then, multiple pea-sized lesions developed on palms and soles, which gradually spread to involve the dorsa of hands and feet. The lesions were accompanied by depigmentation. Within the next 2 years, pea-sized lesions spread to involve the flexures of elbows and knees. The child was born to nonconsanguineous parents and there was no history of similar disease in the family. On examination, the patient was of normal physical and mental development. There was no evidence of any associated systemic disease. On cutaneous examination, diffuse bilateral symmetrical, brownish scaly keratotic plaques were present on axillae, flexures of elbows and knees, groins, and ankles along with depigmentation of hands and feet [Figure 1] and [Figure 2]. Examination of the hair and nails was normal. Routine laboratory tests, serum zinc level, and serum thyroid level were all within normal range.{Figure 1}{Figure 2}

Histological examination of a skin biopsy taken from an erythematous area on the axilla showed alternating zones of parakeratosis and orthokeratosis in both vertical and horizontal direction with hypergranulosis, broad rete ridges, thick suprapapillary plates, lymphocytic perivascular infiltrate in the dermis, and follicular plugging with perifollicular parakeratosis [Figure 3]a, [Figure 3]b.{Figure 3}

Both the clinical and histological pictures were compatible with PRP and the patient was commenced on acitretin (0.5 mg/kg) along with topical keratolytics (10% urea cream) and emollients.

PRP was first described in 1835 by Tarral.[2] It is defined as a group of chronic disorders characterized by reddish orange plaques with pityriasiform scaling showing follicular keratoses, palmoplantar keratoderma, and sometimes, erythroderma.[3]

Based on clinical characteristics, age of onset, behavior, and prognosis, PRP was initially classified by Griffiths in 1980 into five categories.[4] Type IV is circumscribed juvenile onset PRP, the most common type of PRP in children, with well-defined involvement, frequently affecting the knees and elbows. In these sites, perifollicular papules with central keratotic plugs coalesce to form well demarcated, scaly, erythematous plaques. A waxy, orange red, diffuse, palmoplantar keratoderma is also commonly observed. Our patient also had well-demarcated localized keratotic plaque on the axilla, elbows, groin, knees, and ankles but had flexural distribution along with palmoplantar keratoderma.

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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.

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1Griffiths WAD. Pityriasis rubra pilaris - An historical approach: Clinical features. Clin Exp Dermatol 1976;1:37-50.
2Tarral C. General psoriasis desquamation from the parts covered by hair. In: Rayer P, editor. A Theoretical and Practical Treatise on the Diseases of the Skin. 2nd ed. London: Bailliere; 1835. p. 6489.
3Sehgal VN, Srivastava G, Dogra S. Adult onset pityriasis rubra pilaris. Indian J Dermatol Venereol Leprol 2008;74:311-21.
4Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol 1980;5:105-12.