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André D. Généreux, MD; David A. Wetter, MD Widespread erythematous Internal Medicine Residency, Abbott-Northwestern Hospital, Minneapolis, Minn skin eruption (Dr. Généreux); Department of Dermatology, College of Medicine, Mayo Clinic, The patient presented with a salmon-colored rash Rochester, Minn (Dr. Wetter) from head to toe. The distinctive clinical presentation [email protected] and a biopsy pointed to an uncommon diagnosis. DEPARTMENT EDITOR

Richard P. Usatine, MD University of Texas Health Science Center at San Antonio

The authors reported no potential A 48-year-old woman sought care for a The patient’s palms were waxy and ery- conflict of interest relevant to this widespread pruritic skin eruption that began thematous and her feet had hyperkeratosis. A article. on her upper back and spread to her arms, complete blood count, comprehensive meta- lower trunk, and lower legs. She’d had the bolic panel, and lipid panel were normal. A rash for approximately 2 months and didn’t skin biopsy demonstrated psoriasiform der- have any systemic symptoms. A course of matitis with alternating areas of orthokeratosis prednisone prior to her presentation failed to and parakeratosis (the presence of keratino- improve the rash. She denied a personal or cyte nuclei within the stratum corneum where family history of rheumatologic or dermato- nuclei typically aren’t found). logic disease and reported no new medica- tions or exposures. On physical exam, she was afebrile and her vital signs were normal. The rash had red- ● WHAT IS YOUR DIAGNOSIS? to-salmon–colored scaling patches with dis- crete and coalescing follicular papules. There ● HOW WOULD YOU TREAT THIS were prominent islands of sparing (FIGURE 1). PATIENT?

FIGURE 1 A cephalocaudal rash with prominent islands of sparing

IMAGES COURTESY OF: MAYO CLINIC DEPARTMENT OF DERMATOLOGY, A B ROCHESTER, MINN

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TABLE Types of rubra pilaris1,4

Type Population Typical course

I Adult Spontaneous remission within 3 years

II Adult Chronic course

III Pediatric Spontaneous remission within 1 year

IV Pediatric Relapsing and remitting

V Pediatric Chronic course

VI HIV-infected Variable course

HIV, human immunodeficiency virus.

Diagnosis: tions, and malignancy. The course and prog- The patient was given a diagnosis of pityria- nosis of the erythroderma varies with the sis rubra pilaris (PRP) based on her distinc- underlying condition causing it.6 tive clinical presentation. This included the z is a common cause of exfo- In rare cases, presence of prominent islands of sparing, the liative dermatitis in adults. Erythroderma may pityriasis rubra red-to-salmon scaling patches with follicular occur in patients with underlying psoriasis pilaris has been papules, the waxy of her palms, and after discontinuing, or rapidly tapering, sys- associated the cephalocaudal progression of her rash. temic corticosteroids.7 Because PRP is a papu- with internal The patient’s skin biopsy findings (in particu- losquamous eruption, it is often confused with malignancy and lar, the alternating orthokeratosis/parakerato- psoriasis.1,3 human immuno- sis) were also supportive of the diagnosis and z Dermatitis. Several subtypes of deficiency virus helpful to exclude other potential causes of dermatitis can be associated with eryth- infection. erythroderma (described below). roderma. These include atopic, seborrheic, al- PRP most often affects middle-aged lergic contact, airborne, and photosensitivity individuals with an equal sex distribution. dermatitis. The etiology and pathogenesis of PRP are z Drug eruptions. Numerous pharma- not well understood. In rare cases, it has cologic agents have been associated with the been associated with internal malignancy development of widespread drug-induced and human immunodeficiency virus (HIV) skin eruptions. These eruptions include the infection.1,2 PRP may stem from a com- severe reaction of toxic epidermal necrolysis, bination of a dysfunction in vitamin A which always involves sloughing of skin. metabolism, genetic factors, and immune dys- z Malignancy. Both cutaneous T-cell regulation.3 Six types of PRP have been identi- lymphoma (including mycosis fungoi- fied; they differ in the way they present and the des) and internal malignancies can lead to populations affected TABLE( ).1,4 erythroderma.6,8,9 z PRP has several distinguishing fea- PRP can be confused tures from other causes of erythroderma. The with other causes of erythroderma natural course of classic adult PRP (type 1) is PRP can cause erythroderma (also known variable, but is typically self-resolving within as exfoliative dermatitis), which is the term 3 years of onset. Clinical findings include red- applied to an erythematous eruption with to-salmon–colored follicular hyperkeratosis scaling that covers ≥90% of the body’s surface that forms papules or plaques with scales. area. Akhyani et al found that PRP is respon- There are often prominent islands of central sible for approximately 8% of all erythro- sparing. Palmoplantar keratoderma is also dermas;5 the other causes of erythroderma commonly observed. The disease typically are manifestations of numerous conditions, spreads in a cephalocaudal fashion and may including psoriasis, dermatitis, drug erup- progress to generalized erythroderma.1

182 THE JOURNAL OF FAMILY PRACTICE | MARCH 2017 | VOL 66, NO 3 Treatment includes oral retinoids z Our patient was treated with oral acitre- In the initial evaluation of most cases of eryth- tin 70 mg/d. At a 3-month follow-up visit, her roderma, it is important to perform a skin skin showed signs of partial improvement. The biopsy (a 4-mm punch is often best) with a patient was lost to follow-up. JFP request for a rush reading to avoid missing a possibly severe and life-threatening diagnosis. CORRESPONDENCE Skin biopsy is often not diagnostic, but may André D. Généreux, MD, Department of Internal Medicine, Abbott-Northwestern Hospital, 800 East 28th Street, Minne- show alternating parakeratosis and orthokera- apolis, MN 55407-3799; [email protected]. tosis (as in this case). Careful correlation of the histopathologic findings with the clinical pre- sentation is what usually leads to the diagno- References 1. Klein A, Landthaler M, Karrer S. Pityriasis rubra pilaris: a review sis. Obtaining 2 punch biopsies may be helpful of diagnosis and treatment. Am J Clin Dermatol. 2010;11:157- if there are multiple morphologies present or if 170. 2. González-López A, Velasco E, Pozo T, et al. HIV-associated pity- is suspected. If the patient riasis rubra pilaris responsive to triple antiretroviral therapy. Br is not physiologically stable, hospitalization is J Dermatol. 1999;140:931-934. 3. Bruch-Gerharz D, Ruzicka T. Chapter 24. Pityriasis rubra pi- warranted. laris. In: Goldsmith LA, Katz SI, Gilchrest BA, et al, eds. Fitz- patrick’s Dermatology in General Medicine. 8th ed. New York, Oral retinoids (eg, acitretin) are the first- NY:McGraw-Hill;2012. line treatment for PRP. PRP is a rare disease, 4. Sehgal VN, Srivastava G. (Juvenile) Pityriasis rubra pilaris. Int J so the best treatment data available include Dermatol. 2006;45:438-446. 5. Akhyani M, Ghodsi ZS, Toosi S, et al. Erythroderma: a clinical studies involving small case series. Other study of 97 cases. BMC Dermatol. 2005;5:5. Cases of classic treatments include methotrexate and pho- 6. Sehgal VN, Srivastava G, Sardana K. Erythroderma/exfoliative adult (type 1) dermatitis: a synopsis. Int J Dermatol. 2004;43:39-47. totherapy, but results are mixed and patient- 7. Rosenbach M, Hsu S, Korman NJ, et al; National Psoriasis Foun- pityriasis rubra dependent.1,3 In fact, some patients have dation Medical Board. Treatment of erythrodermic psoriasis: pilaris typically from the medical board of the National Psoriasis Foundation. J experienced flare-ups when treated with pho- Am Acad Dermatol. 2010;62:655-662. self-resolve totherapy; therefore, it is not a commonly used 8. Chong VH, Lim CC. Erythroderma as the first manifestation of colon cancer. South Med J. 2009;102:334-335. within 3 years treatment for PRP. 9. Ge W, Teng BW, Yu DC, et al. Dermatosis as the initial presenta- of onset. Tumor necrosis factor (TNF)-alpha tion of gastric cancer: two cases. Chin J Cancer Res. 2014;26:632- 638. inhibitors, including infliximab, adalimumab, 10. Garcovich S, Di Giampetruzzi AR, Antonelli G, et al. Treatment and etanercept, have been used increasingly of refractory adult-onset pityriasis rubra pilaris with TNF-al- pha antagonists: a case series. J Eur Acad Dermatol Venereol. with varying degrees of success.10-12 TNF-alpha 2010;24:881-884. inhibitors have a relatively good safety profile 11. Walling HW, Swick BL. Pityriasis rubra pilaris responding rap- idly to adalimumab. Arch Dermatol. 2009;145:99-101. and should be considered in refractory cases. 12. Eastham AB, Femia AN, Qureshi A, et al. Treatment options for If there are associated conditions, such as HIV, pityriasis rubra pilaris including biologic agents: a retrospec- tive analysis from an academic medical center. JAMA Dermatol. treating these may also result in remission.2 2014;150:92-94.

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