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Abstract

  1. Top of page
  2. Abstract
  3. CONCLUSIONS
  4. REFERENCES

A 45-year-old previously healthy man presented with minimally itchy spiny papular lesions of 3 years' duration and discharging nodular cystic lesions for the past 2 years. Initially, lesions appeared on his ears, followed by the gradual appearance of similar lesions over his face, back, and extremities. The lesions were not associated with photosensitivity. Over the years, the patient continued to have similar lesions without any significant response to various topical medications and oral antibiotics. There was no history of fever or any other systemic complaints and the patient denied any other significant medical problems in the past. Cutaneous examination revealed multiple, grouped, spiny papular lesions coalescing to form plaques at places over the ears, extremities, and trunk (Figure 1). Elongated, horny, follicular spires were noted on the top of the papular lesions. Multiple discharging nodular lesions with crusting were seen predominantly over the chest, abdomen, and back (Figure 2), and closed comedonal lesions were noted on the face and trunk. Multiple orange-brown scaly plaques were seen over the extensor aspect of the patient's thighs (Figure 1, inset). A few nails showed wedge-shaped thickening without subungual hyperkeratosis or other nail changes. Palms, soles, and oral mucosa were normal. Considering the atypical cutaneous findings, the clinical possibility of pityriasis rubra pilaris (human immunodeficiency virus [HIV]-associated type 6 PRP) was considered. Hematoxylin and eosin stain of skin biopsy specimens taken from the spiny papular and plaque lesions revealed marked hyperkeratosis with alternating orthokeratosis and parakeratosis with follicular keratotic plugging (Figure 3; Figure 3, inset). Irregular broad acanthosis was also detected in the epidermis. Dermis showed moderate perivascular lymphomononuclear inflammatory infiltrate. Thus, the skin biopsy was consistent with PRP. HIV serology by enzyme-linked immunosorbent assay (ELISA) with 3 different kits was found to be positive for HIV-1. The patient denied any risk factors associated with acquiring HIV infection, and laboratory values for complete blood cell count, routine serum biochemical profile, and lipid profile were normal. The patient was prescribed oral isotretinoin and asked to come for follow-up for CD4 T-cell count, but did not return.

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Figure 1. Spiny follicular papules with hyperkeratosis spires emerging from top over the ear. Closed comedones can be appreciated on the face. (Inset) Psoriasiform plaques with spiny follicular papules over left thigh.

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Figure 2. Nodules with discharge and crust over back.

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Figure 3. Hyperkeratosis with parakeratosis, irregular broad acanthosis, and follicular keratotic plugging at places (hematoxylineosin stain, original magnification ×10). (Inset) Parakeratotic plugging with perivascular lymphomononuclear infiltrate (hematoxylin-eosin stain ×40).

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Pityriasis rubra pilaris (PRP) is a papulosquamous disorder of uncertain etiology comprised of a spectrum of clinical subtypes. In 1980,1 researchers classified it into 5 types based on the type of skin lesions and patient age. A new subtype, human immunodeficiency virus (HIV)-associated PRP (type 6 PRP), was added in 1995.2 During the past 15 years, 12 cases2–10 of type 6 PRP have been reported from various parts of the world.

Several psoriasiform disorders have been reported to occur more frequently and/or with increased severity in patients with HIV infection,11 including psoriasis, seborrheic dermatitis, Reiter disease, and acquired ichthyosis. Type 6 PRP is characterized by nodulocyctic acne lesions, lichen spinulosus-like lesions, resistance to therapy, and poor prognosis. Prominent follicular plugging with formation of spicules, an unusual finding in classic adult-onset PRP, is a common observation in HIV-associated PRP. The erythematous desquamating papules tend to become confluent and the patient frequently becomes erythrodermic. Islands of uninvolved skin can be seen, but unlike classic adult PRP, follicular components (plugs and spicules) seldom disappear.9 In addition to PRP, isolated follicular manifestations (eg, lichen spinulosus12 and acne conglobata5) have also been reported in HIV-infected patients, leading researchers to describe these patients as suffering from a spectrum of a unique and distinctive disease, an HIV-associated follicular disorder.13 They all manifest generalized follicular disease, including nodulocystic inflammatory folliculitis, prominent keratotic spires, and possibly generalized papulosquamous findings indistinguishable from PRP. This disease can be designated by the wider term HIV-associated follicular syndrome13 or HIV-associated follicular occlusion triad.10 The striking nodulocystic folliculitis sets this disorder apart from classic PRP.

The cases of HIV-associated PRP reported to date suggest that PRP may be unmasked or precipitated in a genetically susceptible individual7 or otherwise be associated with HIV infection. The relation of HIV infection and onset of PRP may be coincidental or it may be correlated. That only 13 patients2–10 (Table I) including the index case have been reported in the English literature to date gives credence to a coincidental etiology; however, low prevalence of PRP in general may be the cause of such rarity.

Table Table.  Review of Cases of PRP in Association With HIV Infection Reported in English Literature
Patient No.AuthorsAge/SexSexual PreferenceHIV Diagnosed Before or After Eruption of Skin LesionsCD4 T-Cell Count (Cells/mm3)Cutaneous LesionsTreatment GivenSubsequent Status
1 and 2Blauvelt et al328/MBisexualAfter368PlaquesAZTNo improvement
 PPKUVB + etretinateSlight improvement
2Blauvelt etal328/MHomosexualBefore (2 mo)311AC, LS, nail involvement, plaques, palmoplantar scalingIsotertinoinSide effects
 Etretinate + PUVA + UVBNA
 AZT + trimethoprim-sulphamethoxazole + etretinateSlight improvement
3Auffret et al428/FNABefore (2 y)714AC, LS, PPK, HS, plaques, nail involvementIsotretinoinMinimal improvement
 AZTSide effects
 EtretinateLack of efficacy
 Died of pulmonary infection and shock
4Resnick et al5 (not labeled as PRP)45/MNAAfter510AC, LSMinocycline + prednisolone (1)Limited improvement
 1 + isotretinoin (2)Exacerbation followed by relapse
 2 + AZT50% improvement
5Menni et al64/MNABefore (since birth)1140PPK, keratotic scaly papulesNACleared spontaneously
6Martin et al729/MHomosexualBefore (3 mo)>500AC, acuminate follicular papulesMinocyclineNo improvement
 IsotretinoinNo improvement
 Etretinate + UVBComplete clearing
7Martin et al740/MBisexualNANAAC, PPK, follicular hyper keratotic plaquesIsotretinoinSome improvement
 EtretinateConsiderable improvement
8Martin et al727/MHomosexualBefore (6 mo)699Plaques, PPKEtretinatePartial clearing
 AZTSignificant improvement
9Sanchez- Regana etal835/MNAAfter86PPK, keratotic acuminate follicular papules, plaquesAZTCompletely cleared
 AZT + etretinateSide effect of etretinate
10Miralles et al232/MHomosexualAfter597AC, PPK, nail changes, follicular keratotic papules, plaquesAZT + etretinateDied due to acute renal failure, Candida septicemia, disseminated intravascular coagulation
11Gonzalez- Lopez etal934/FNABefore (3 years)309AC, follicular papules with plugs and hyper keratotic spiculesAZT + lamivudine + saquinavirComplete clearance maintained even at 18 mo of treatment
12Blasdale et al1024/MHomosexualBefore (15 months)600AC, PPK, HS, plaques, nail involvementIsotretinoin + indinavir + stavudin + lamivudineSome improvement initially, cleared while not on therapy, ultimately died of renal failure
13Present case45/MNAAfterNAAC, LS, plaques, nail involvementIsotretinoinLost to follow-up
Abbreviations: AC, acne conglobata; AZT, zidovudine; F, female; HIV, human immunodeficiency virus; HS, hidradenitis suppurativa; LS, lichen spinulosus; M, male; NA, not available; PPK, palmoplantar keratoderma; PRP, pityriasis rubra pilaris; PUVA, psoralen ultraviolet A; UVB, ultraviolet B.

The exact pathogenesis of type 6 PRP has not been elucidated. Some researchers14 have suggested that follicular inflammation caused by infection of the hair bulge region by HIV was responsible for the evolution of PRP in these patients. Immunosuppression does not appear to be significantly associated, as all but 1 patient had more than 300 cells/mm3 CD4 T-cell count in the peripheral blood. In almost equal proportions of patients, only later was HIV suspected to have played a role in PRP, based on either atypical cutaneous findings with PRP and subsequent HIV identification or diagnosis of PRP early (2 months to 3 years) after positive HIV results.

In the majority of reported cases, PRP lesions improved following initiation of HIV treatment. In 1 patient, highly active antiretroviral therapy with zidovudine, lamivudine, and saquinavir resulted in significant improvement in skin lesions.9 This result suggests that HIV infection per se may be significantly responsible for PRP lesion development. Two patients died of septicemia, even though they received antiretroviral therapy.2,4

While classic PRP affects both sexes equally, most patients with PRP in association with HIV were either homosexual or bisexual men (when sexual preference was known); only 2 patients were women. It is difficult to draw inference from the small number of reported cases; however, given the causal role of human herpes virus-8 (HHV-8) in Kaposi's sarcoma in homosexual men with acquired immunodeficiency syndrome, further research is needed on the relationship of HHV-8 with PRP in HIV-infected patients.

Many therapeutic modalities have been employed in the treatment of HIV-associated PRP (Table I). None of the treatment options have been found to be universally effective. At least 2 patients have had spontaneous clearance of lesions without treatment.6,10 Oral retinoids, which are very promising in treating classic PRP, do not seem to be effective in HIV-associated PRP.

CONCLUSIONS

  1. Top of page
  2. Abstract
  3. CONCLUSIONS
  4. REFERENCES

Although type 6 PRP is now an accepted diagnosis in standard dermatology textbooks,15 its differentiation from and relation to HIV-associated follicular syndrome need to be explored in detail as more such cases are reported in the future. A notable proportion of patients described to date have had no systemic complaints prior to the PRP diagnosis, and HIV was suspected only on the basis of atypical cutaneous manifestations of PRP. Thus, a middle-aged patient who presents with the classic triad of features of nodulocystic acne, lichen spinulosus, and psoriasiform lesions or isolated nodulocystic acne or lichen spinulosus-like lesions should be tested to detect early HIV infection.

REFERENCES

  1. Top of page
  2. Abstract
  3. CONCLUSIONS
  4. REFERENCES
  • 1
    Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol. 1980;5:105112.
  • 2
    Miralles ES, Nunez M, De Las Heras ME, et al. Pityriasis rubra pilaris and human immunodeficiency virus infection. Br J Dermatol. 1995;133:990993.
  • 3
    Blauvelt A, Nahass GT, Pardo RJ, et al. Pityriasis rubra pilaris and HIV infection. J Am Acad Dermatol. 1991;24:703705.
  • 4
    Auffret N, Quint L, Domart P, et al. Pityriasis rubra pilaris in a patient with human immunodeficiency virus infection. J Am Acad Dermatol. 1992;27:260261.
  • 5
    Resnick SD, Murrell DF, Woosley JT. Pityriasis rubra pilaris, acne conglobata, and elongated follicular spines: an HIV-associated follicular syndrome? J Am Acad Dermatol. 1993;29 (2, pt 1):283.
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  • 7
    Martin AG, Weaver CC, Cockerell CJ, et al. Pityriasis rubra pilaris in the setting of HIV infection: clinical behaviour and association with explosive cystic acne. Br J Dermatol. 1992;126:617620.
  • 8
    Sanchez-Regana M, Fuentes CG, Creus L, et al. Pityriasis rubra pilaris and HIV infection: a part of the spectrum of HIV-associated follicular syndrome. Br J Dermatol. 1995;133:818819.
  • 9
    Gonzalez-Lopez A, Velasco E, Pozo T, et al. HIV-associated pityriasis rubra pilaris responsive to triple antiretroviral therapy. Br J Dermatol. 1999;140:931934.
  • 10
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    Sadick NS, McNutt NS, Kaplan MH. Papulosquamous dermatoses of AIDS. J Am Acad Dermatol. 1990;22:12701277.
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    Misery I, Faure M, Claidy A. Pityriasis rubra pilaris and human immunodeficiency virus infection—type 6 pityriasis rubra pilaris? Br J Dermatol. 1996;135:10081009.
  • 15
    Judge MR, McLean WH, Munro CS. Disorders of keratinization. In: BurnsT, BreathnachS, CoxN, GriffithsC, eds. Rook's Textbook of Dermatology. 7th ed. Oxford, England: Blackwell Science; 2004:34.