|Year : 2015 | Volume
| Issue : 2 | Page : 124-126
HIV-associated Pityriasis rubra pilaris in a 15-year-old girl
Ezejiofor Ogochukwu Ifeanyi, Ozor Glagys Angela, Okpala Chibuzo Ifeanyi, Enechukwu Nkechi Anne, Nwankwo Henry Madu
Department of Medicine, Dermatology Unit, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra, Nigeria
|Date of Web Publication||9-Jun-2015|
Dr. Ezejiofor Ogochukwu Ifeanyi
Department of Medicine, Dermatology Unit, Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra
Pityriasis rubra pilaris (PRP) is a chronic skin disease characterized by small follicular papules, disseminated yellowish-pink scaling patches and occasionally palmoplantar hyperkeratosis. Both the childhood and adult types are classified in different existing variants, and there is a recent addition of the human immunodeficiency virus (HIV)-associated disease. It is a rare dermatological condition, and its coexistence with HIV is also very rare, thus the need to report this case.
Keywords: Antiretroviral therapy, pityriasis rubra pilaris, retroviral infection
|How to cite this article:|
Ifeanyi EO, Angela OG, Ifeanyi OC, Anne EN, Madu NH. HIV-associated Pityriasis rubra pilaris in a 15-year-old girl. Trop J Med Res 2015;18:124-6
| Introduction|| |
Pityriasis rubra pilaris (PRP) is a papulosquamous disorder of unknown etiology that often progresses to erythroderma and causes a disabling keratoderma of palms and soles.
The disease was originally classified by Griffiths  into five groups based on clinical appearance, age of onset, and prognosis. Recently, a sixth group was proposed by Miralles et al.  to accomodate those associated with human immunodeficiency virus (HIV) which appear to differ from the classic forms.
The typical features of PRP include follicular hyperkeratosis and reddish-orange, scaling dermatitis with islands of normal skin.
Histopathological examination reveals hyperkeratosis, alternating parakeratosis, and orthokeratosis, both in the vertical and horizontal plane of the stratum corneum and a mild superficial perivascular lymphocytic infiltrate.
No single therapy is universally effective, and some cases do not respond to multiple therapies.  The most successful treatment options are retinoids, photochemotherapy (PUVA), and antimetabolites (methotrexate). 
This is the first case of PRP associated with HIV seen in our dermatology clinic, and due to the paucity of reports on this subject matter, especially in this part of the world, we decided to report this case to stress this association.
| Case Report|| |
A 15-year-old secondary school female student presented to us with a 2-year history of pruritic scaly skin rashes, which started from the scalp and later spread to her face, trunk, and extremities. There was associated thickening of the palm and sole, thereby limiting the use of her hand in writing and other activities. These rashes did not get worse with exposure to sunlight.
She was diagnosed with HIV about 1 year before her presentation to the clinic and was placed on highly active antiretroviral therapy (HAART). This had no effect on the lesions, which were also treated by several other unidentified creams. There was no family history of similar lesions. Neither any associated nodular facial lesions (acne conglobata) nor any lesion on the groin or axilla (hidradenitis suppurativa). Physical examination showed scaly circumscribed and confluent reddish-orange plaques on the scalp, trunk, and extremities with islands of normal skin [Figure 1], [Figure 2] and [Figure 3].
|Figure 2: Fillicular hyperkeratosis with scales and Island of normal skin|
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There were also follicular papular rashes both within and at the periphery of the patches, with diffuse thickening of the palms and the soles. Nails were thickened with subungual hyperkeratosis. There was also patchy alopecia of the scalp. No mucosal lesion was found. Examination of respiratory, cardiovascular, and central nervous system were essentially normal and there were no abnormal abdominal findings.
Skin biopsy showed alternating orthokeratosis, parakeratosis, acanthosis, broad and short rete ridges, and preserved granular layer in keeping with PRP [Figure 4]. The HIV screening was confirmed positive. The liver function test (LFT), serum E/Ur/Cr, and the complete blood count were essentially normal.
|Figure 4: Histology of skin section showing hyperkeratosis, acanthosis,and parakeratosis|
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| Discussion|| |
There is no single diagnostic laboratory or genetic marker for the diagnosis of PRP.
Clayton et al.  have emphasized the importance of clinical and histopathological correlation in establishing the diagnosis of PRP.
Our patient had both clinical and histopathological features suggestive of PRP, which prompted the diagnosis. PRP has been associated with several autoimmune diseases such as autoimmune thyroiditis, dermatomyositis, myasthenia gravis, and vitiligo.  There is also an existing case report of a 38-year-old woman with classic adult PRP Type I and systemic sclerosis. 
PRP has also appeared simultaneously with subacute cutaneous lupus erythematosus (SCLE), isolated immunoglobulin (Ig) A deficiency, hypogammaglobinemia, celiac sprue, atopy, diabetes mellitus, underlying malignancy, and therapy with agents known to disturb lymphoid function.  Our patient had no features suggesting any of these diseases.
Association of HIV with PRP is a recent finding. By 2000, there were at least 14 reported cases.  In the documented cases, the onset of PRP occurred shortly after or at the same time when the patients tested positive for HIV infection.  None developed PRP before HIV infection leading to suggestions that HIV plays an important pathogenic role. The patients' responsiveness to treatment with zidovudine or triple therapy and relapse when treatment was stopped gave further support to the hypothesis. 
This PRP Type VI is characterized by the presence of HIV infection, usually without evidence of immunosupression, a poor prognosis, a poor response to etretinate, and variable association with lesions of acne conglobata, hidradenitis suppurativa, and lichen spinulosus.  These lesions have also been reported in HIV-infected patients without PRP, prompting Resnick et al.  to hypothesize that the constellation of these features represents a genuine HIV-associated follicular syndrome, as they have not all been described in the same patient without HIV. Our patient had no feature suggesting the above mentioned lesions.
Misery et al.  went one step further and suggested that HIV serology should be included in routine laboratory test in adult patients with PRP, as it had been reported as the first sign of HIV infection.
The success of zidovudine in treating PRP Type VI prompted Griffith to use it for treating three cases of HIV-negative PRP Type I.  The result was disappointing. This suggests that the effect of zidovudine on HIV itself appears more important than any effect on PRP.
Documented cases of PRP in patients with HIV have led to a renewed interest in possible underlying immune mechanisms of the disease. It has been hypothesized that the pathogenesis of PRP may be related to abnormal immune response to antigenic triggers, in this case, the HIV itself. Other hypothesized pathogenic mechanisms include: Vitamin A deficiency, deficiency of retinol-binding protein and genetic factor, but all these have remained inconclusive.
| Conclusion|| |
We have reported this case to emphasize once more the association of PRP with HIV infection, which has been reported in the past and prompted the introduction of PRP Type VI in the previous Griffith's classification of five groups. This association is, however, uncommon but it is important enough to warrant screening of all PRP patients for HIV, as this condition can be a very early manifestation of HIV.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]