Rapidly Progressive Erythroderma Caused by Pityriasis Rubra Pilaris Dustin Portela DO*, Dana Burandt BS**, Steve Grekin DO*** *Dermatology Resident, Oakwood Southshore Medical Center, **OMS III Michigan State University, *** Program Director Dermatology Residency Oakwood Southshore Medical Center Abstract Treatment We present the case of a 50-year-old male who developed rapidly Clinical differential diagnosis included erythrodermic psoriasis, pityriasis The Griffiths’ classification scheme describes six different types of PRP, progressive erythroderma as a complication of pityriasis rubra pilaris rubra pilaris, and drug induced phototoxicity. differing in clinical presentation, lesion distribution, course, and duration (PRP), requiring hospital admission. The initial eruption developed (Table 1). The majority of patients are Type I, “classic adult,” with There is no universally effective treatment for PRP and some cases may even demonstrate resistance to both systemic and topical therapies6. A lack following a sunburn. Following hospital discharge the patient has Initial laboratory evaluation was within normal limits and included generalized distribution and a cephalocaudal progression. In addition to the experienced a protracted course of erythroderma, which was treated with cosmetic and functional implications of the tight scales of the scalp and of thorough research comparing current treatment options exists due to the complete blood count with differential, comprehensive metabolic panel, rarity of the condition. Traditionally, retinoids, methotrexate, or cyclosporine and acitretin as well as topical corticosteroids. We briefly and urinalysis. face, the waxy, thickened skin of the soles and palms can crack resulting in review the various classifications of PRP as well as potential treatment painful fissures. The onset is acute and 80% resolve within a three-year cyclosporine are used as systemic therapy. Topical emollients, options and prognosis. period3. corticosteroids, and keratolytics supplement the oral treatment. Biologic Two 4mm punch biopsies were obtained and revealed elongation of rete medications against psoriasis, such as tumor necrosis factor (TNF) ridges, hyperkeratosis and confluent parakeratosis. There was a mild Table 1 antagonists, may have value in treating PRP, given the histological and superficial perivascular lymphocytic and neutrophilic infiltrate as well as clinical similarities between the two diseases1,4,7. Introduction the presence of extravasated red blood cells. PAS stain was negative for Clinical Name % of Features fungi and colloidal iron stain was negative for mucinosis. A diagnosis of Type PRP Erythroderma is a generalized redness to the skin with or without scaling. pityriasis rubra pilaris was rendered. Patients It can be a manifestation of many common primary disorders of the skin I Classic adult 55% Generalized distribution, cephalic to including psoriasis, atopic dermatitis, or drug reactions; or less common At the initial visit, the patient was started on triamcinolone 0.1% cream caudal spread, red- orange plaques disorders such as cutaneous lymphoma or pityriasis rubra pilaris. Potential and instructed to follow up in two days to review his biopsy results. Initial with “islands of sparing”, Perifollicular complications of erythroderma include peripheral edema, hypothermia, follow-up revealed progressing erythroderma in a cephalic to caudal keratotic papules, waxy palmoplantar electrolyte imbalance, and high output heart failure. Prompt identification direction with islands of spared skin as well as more extensive keratoderma of the underlying disorder and treatment of erythroderma can prevent hyperkeratosis of the palms and soles with fissuring and marked edema. At many complications and potentially be life saving. this time, he was started on oral cyclosporine and acitretin. Despite these II Atypical adult 5% Generalized distribution, areas of medications the erythroderma progressed and he developed 3+ pitting eczematous dermatitis with Conclusion edema of the lower extremities. He was admitted to the hospital for fluid ichthyosiform scale on legs, and electrolyte management. During the hospitalization, his laboratory keratoderma with coarse lamellated Pityriasis rubra pilaris is an uncommon chronic skin condition, which can abnormalities included a mild hypoalbuminemia and hypoproteinemia. Case Presentation scale, occasional alopecia potentially lead to erythroderma. The majority of PRP patients will Following hospital discharge, the patient’s dose of cyclosporine had been present as adults with a generalized eruption beginning on the head and progressively tapered, and the dose of acitretin had been increased. The III Circumscribed 25% Focal distribution, elbows and knees neck, which then generalizes in a caudal direction. Unique features A 50-year-old Caucasian male presented with a three-day history of mildly patient remained erythrodermic, but had experienced much less scaling, include “islands of sparing” and a waxy palmoplantar keratoderma. pruritic erythematous papules and patches progressing from his head to his and the fissuring to his palms and soles had resolved. He continued to juvenille show erythema & follicular papules, prepubertal onset Although the etiology is unknown most patients presenting with classic chest and upper arms after experiencing a sunburn during work. He also experience moderate pruritus and difficulty in body temperature symptoms will experience resolution within three years. There are no complained of redness to his hands and feet. The rash began two months regulation. universally successful treatments and the patient approach must be earlier as a single, red, scaly patch on his scalp, which appeared after a IV Classic 10% Generalized distribution with clinical individualized. mushroom hunting excursion. He had treated the patch with a mid potency Discussion juvenille findings similar to Type I, onset in first topical corticosteroid prescribed by his primary physician with a 2 years of life or in adolescence presumptive diagnosis of psoriasis. The patient had a family history significant for psoriasis, but no other skin disorders. His past medical Pityriasis rubra pilaris (PRP) is an uncommon, chronic skin condition of V Atypical 5% Generalized distribution with follicular history was significant for hypertension, controlled with atenolol. A review unknown etiology. It is characterized by hyperkeratotic follicular papules juvenille hyperkeratosis and erythema, of systems was negative for constitutional symptoms at his initial and palmoplantar keratoderma. The coalescence of papules bordered by scleroderma-like presentation. uninvolved skin creates the appearance of “islands of sparing” between changes of hands salmon-colored, scaling plaques. Progression to erythroderma is a potential and feet, onset in first few years of life complication. Physical examination revealed a well appearing male with brightly References erythematous, hyperkeratotic, follicular-based papules and scaly patches VI HIV- Generalized distribution with findings PRP affects approximately 2.5 per million of the population and does not coalescing on the scalp, face, chest, and upper extremities (Figure 1). associated similar to Type I, can occur in 1. Petrof G, Almaani N, Archer CB, Griffiths, WAD, Smith CH. A differ based on race or gender. There is a bimodal distribution for age of Examination of his hands and feet revealed erythema and hyperkeratosis of follicular association with acne conglobata and systematic review of the literature on the treatment of pityriasis rubra onset, including childhood for familial cases and the fifth or sixth decade the palms and soles (Figure 2). syndrome hidradenitis suppurativa in HIV- pilaris type 1 with TNF-antagonists. J European Acad Derm Venerology for acquired cases1,2. infected individuals 2013; 27:131-135 2. Griffiths WA. Pityriasis Rubra Pilaris – An Historical Approach. 2. Griffith’s classification scheme of pityriasis rubra pilaris. Adapted from Clinical features. Clin Exp Dermatol. 1976; 1:37–50. Bolognia, 3rd Ed. Fig. 9.9 p 164 3. Griffiths WAD. Pityriasis Rubra Pilaris. Clin Exp Dermatol. 1980; 5:105-12 4. Klein A, Landthaler M, Karrer S. Pityriasis Rubra Pilaris: A Review of While the pathogenesis of PRP remains uncertain, abnormal vitamin A Diagnosis and Treatment. Am J Clin Dermatol. 2010; 11:157-70 metabolism, specifically a deficiency of retinol binding protein, and human 5. Fuchs-Telem D, Sarig O, Van Steensel M et al. Familial Pityriasis immunodeficiency virus (HIV) have been studied as possible causes. Rubra Pilaris Is Caused by Mutations in CARD14. Am J Hum Genet. Autoimmune diseases, sunburn, infections, and malignancies are linked as 2012; 91:163-70. 4 trigger factors; however, most cases occur without an inciting event . 6. Müller H, Gattringer C, Zelger B et al. Infliximab Monotherapy as First- line Treatment for Adult-onset Pityriasis Rubra Pilaris: Case Report and The familial type of PRP, Type V, follows an autosomal dominant mode of Review of the Literature on Biologic Therapy. J Am Acad Dermatol. 2008 inheritance, early age of onset, incomplete penetrance, and variable Nov; 59(5): S65-70. expression. In a recent study, Fuchs-Telem et. al. showed that mutations in 7. Ivanova K, Itin P, Haeusermann P. Pityriasis Rubra Pilaris: Treatment CARD14, which regulates inflammatory processes through nuclear factor with Biologics - A New Promising Therapy? Dermatol. 2012; 224(2):120-5 kappa B (NF-kB) and is strongly expressed in the skin, cause familial 8. Wood GS, Reizner G. Dermatology. 3rd ed. Elsevier Saunders; 2012. 5 PRP . Chapter 9, Other Papulosquamous Disorders; p. 157-69 Figure 1 RESEARCH POSTER PRESENTATION DESIGN © 2015 Figure 2 www.PosterPresentations.com.