Psoriasis and Other Papulosquamous Skin Disorders



Fig. 4.1
Plaque psoriasis on the torso. Note the Koebnerization along the patient’s surgical scar



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Fig. 4.2
Guttate psoriasis


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Fig. 4.3
Diaper psoriasis. Note the psoriatic lesions outside the diaper area (Courtesy of Magdalene Dohil)




Management Strategies


Topical medications are the mainstay of treatment, with phototherapy and systemic agents utilized for more recalcitrant, diffuse disease. There is a significant risk of rebound of psoriasis with systemic corticosteroids and these should generally be avoided, particularly with pustular and erythrodermic types. Anthralin, coal tar, and keratolytics are best used for thicker plaques, in conjunction with topical corticosteroids. Salicylic acid is indicated only for children older than age 6 years. Topical treatments are recommended in conjunction with second- and third-line therapies, including narrowband-ultraviolet B (nbUVB) and other forms of phototherapy, methotrexate, and other systemic medications. No treatment is without side effects, and the severity of disease and co-morbidities should be weighed against the risks with each form of treatment.


Investigations Recommended




























For diagnosis

 Most often a clinical diagnosis, but may consider biopsy if unable to distinguish from other papulosquamous disorders, especially pityriasis rubra pilaris

 Streptococcal cultures and anti-streptolysin O (ASO) titer for guttate psoriasis

 Complete blood count (CBC), complete metabolic panel (CMP) for severe generalized pustular disease

For treatment

 For co-morbidities: consider measuring body mass index, blood pressure periodically. If overweight or obese, consider fasting glucose, fasting lipids, and alanine transaminase (ALT)

 Methotrexate: pregnancy test (for women of child-bearing potential), CBC, CMP, tuberculosis testing at baseline, monitor CBC and hepatic panel during treatment with periodic repeat tuberculosis testing

 Retinoids: pregnancy test (for women of child-bearing potential), CBC, AST, ALT, fasting lipids at baseline and during treatment

 Cyclosporine: CBC, CMP, magnesium (Mg), uric acid, blood pressure at baseline and during treatment, tuberculosis testing at baseline with periodic repeat testing

 TNF inhibitors: CBC, CMP, tuberculosis testing, consider hepatitis profile and HIV testing in at-risk groups before initiation, periodic re-assessment during therapy

Biopsy may be useful to differentiate psoriasis from other papulosquamous disorders. Histologically, psoriasis shows parakeratosis and orthokeratosis, epidermal hyperplasia with uniform elongation of the rete ridges, loss of the granular cell layer, and formation of spongiform pustules and parakeratotic microabscesses. The capillary vessels within the superficial dermis are slightly dilated and may have associated neutrophils and lymphocytes. If a positive streptococcal culture is obtained, antibiotics should be initiated to adequately treat streptococcus infection, although the benefits for psoriasis lesions are inconsistent. In overweight or obese patients, clinicians should consider screening for known co-morbidities such as fatty liver disease, diabetes, and hyperlipidemia, but no consensus regarding screening exists at present.


Table 4.1
First line therapies
























Low-, mid-, or high-potency topical corticosteroids BID

B

Calcipotriene (Dovonex) ointment BID

A

Calcitriol (Vectical) ointment BID

B

Tacrolimus (Protopic) 0.03 %/0.1 %a ointment BID

C

Pimecrolimus (Elidel) 1 % cream daily

E

Calcipotriene plus betamethasone propionate daily

B


aStrength used in the published literature

The potency of the topical corticosteroid chosen should be based upon disease severity and location. High-potency steroids are helpful for more severe disease on the palms and soles, with low-potency steroids and caution regarding quantity appropriate for less severe disease on the face or flexures. Vitamin D analogs and topical calcineurin inhibitors are common adjunct therapies, and may even be monotherapy at sites at risk for corticosteroid side effects, such as the face or flexural areas. Combination calcipotriene and betamethasone propionate (Taclonex) was proven safe and effective in an open-label phase II trial, and is the first medication approved by the Food and Drug Administration (FDA) specifically for the treatment of pediatric psoriasis [3].


Table. 4.2
Second line therapies





















Goeckerman regimen (daily application of crude coal tar ointment with UVB phototherapy)

D

Dithralin cream (0.01–4 %) cream via short contact, gradually increasing dosage

B

Tazarotene (Tazorac) 0.05 %/0.1 % cream or gela daily

E

nbUVB for an average of 3 months

C

Methotrexate (typical dose 0.2–0.4 mg/kg/week; reported doses from 0.14 to 0.7 mg/kg/week)

D


aFormulation used in the published literature

Goeckerman treatment is time intensive and is not as commonly used today, limited by odor, risk of folliculitis, staining of skin/clothing, and possible mutagenicity. Anthralin creams can be used as monotherapy or with keratolytics via short contact therapy, though skin irritation is frequently reported. Topical retinoids have been shown to be effective in adults, but there is limited evidence in children [4]. Several retrospective studies have shown nbUVB to be effective in children with widespread or recalcitrant disease, especially when used together with topical medications. It is the preferred type of phototherapy due to lower risk of burning and malignancy, although broadband UVB and UVA are other options [1]. Methotrexate is the most commonly used systemic medication for psoriasis worldwide due to cost and availability [4]. Despite a lack of prospective trials in children, several case studies show significant benefit [5].


Table 4.3
Third line therapies

















































Psoralen plus UVA (PUVA)

D

Excimer laser

D

Oral retinoids

 Acitretin (Soriatane) 0.5–1 mg/kg/day

D

 Isotretinoin (Accutane) 0.75 mg/kg/day

E

 Etretinate (where available) 0.5–1.25 mg/kg/day

D

Cyclosporine 1.5–5 mg/kg/day

D

TNF inhibitors

 Etanercept (Enbrel) 0.8 mg/kg/week, max 50 mg

A

 Infliximab (Remicade) 3.3–5 mg/kg at weeks 0, 2, 6, then every 8 weeks

E

 Adalimumab (Humira) 24 mg/m2, max 40 mg, every 2 weeks

E

Ustekinumab (Stelara) 45 mg at weeks 0 and 4, then every 12 weeks

A

Colchicine (Colcrys) 0.25 mg TID or 0.5 mg BID

E

Fumaric acids (Fumaderm, Europe only)

E

Dapsone 1 mg/kg/day

E

PUVA can be effective in children who have failed other forms of phototherapy, but it should be used with caution given a higher risk of burning and malignancy. Excimer laser may be even more effective for localized thick lesions in children than in adults [6]. Acitretin can augment therapy with topical or nbUVB treatments. Oral retinoids are particularly beneficial in pustular and erythrodermic psoriasis [7, 8]. Cyclosporine may be beneficial, but due to nephrotoxicity risks, should not be used for more than 1–2 years. It additionally should not be used with nbUVB due to elevated risk of malignancy. TNF inhibitors are effective for moderate to severe disease not responding to topicals or phototherapy, but may have the potential for severe infections. Results from randomized controlled trials in adolescents have shown positive effects compared to placebo for both etanercept and ustekinumab [9, 10]. There are also isolated case reports of successful treatment with colchicine, fumaric acid, and dapsone.



Reactive Arthritis



Clinical Features


Reactive arthritis (ReA, formerly known as Reiter’s syndrome) is a seronegative spondyloarthropathy characterized by the classic triad of urethritis, conjunctivitis, and arthritis. Although more commonly seen in adult males, many cases have been reported in children. ReA usually arises several weeks after a sexually transmitted infection (endemic) or diarrheal illness (epidemic). Epidemic cases are far more common in the pediatric population. HLA antigen haplotype-B27 (HLA-B27)-positive individuals make up 60–80 % of patients, and they have more frequent skin findings. Involvement of the heart, kidneys, and CNS is rare in ReA. Chronic disease occurs less often in children.

Diagnosis is based on the clinical picture, which may be difficult as only one-third of patients present with the classic triad. Symptoms may be separated by years, although fever and vague constitutional symptoms may provide clues to the diagnosis. Several specific mucocutaneous manifestations, when present, may be diagnostically helpful. Keratoderma blenorrhagicum is the most “classic” (albeit rare) cutaneous finding, appearing as psoriasiform and hyperkeratotic papules, pustules, and plaques most commonly on the palms and soles. Circinate balanitis/vulvitis is more common and is rarely seen outside of ReA, with well-demarcated erosions (females and circumcised males) or hyperkeratotic plaques (circumcised males) on the genitalia. Ocular and urogenital symptoms are common, while painless oral and psoriatic nail changes may occur.


Management Strategies


Most studies of ReA have focused on treating the arthritis, not mucocutaneous findings. Management is multidisciplinary, centered on treating the triggering infection and managing symptoms. No consensus exists regarding the benefit of antibiotics, but positive serologies should be treated. For patients in whom a urogenital infection is found, sexual partners also require treatment. NSAIDs are the mainstay of initial arthritis treatment, with immune-modifying therapies used for recalcitrant disease. Further discussion regarding treatment of arthritis and other non-cutaneous symptoms is beyond the scope of this chapter. Treatment of mucocutaneous lesions can largely be achieved with topical therapy, although systemic treatments may be helpful in recalcitrant or severe disease.


Investigations Recommended




































For diagnosis

 Consider biopsy

 Blood, urine, and stool cultures

 Synovial fluid culture, white blood cell count

 Serologic tests: CBC, ESR, CRP, rheumatoid factor, ANA

 Urinalysis

 Ophthalmologic exam

 Consider HIV RNA PCR

 Consider tuberculin skin prick test or Quantiferon gold

For treatment

 HLA-B27 testing

 Sulfasalazine: CMP, CBC at baseline and during treatment

 Methotrexate: pregnancy test (for women of child-bearing potential), CBC, CMP, tuberculosis testing at baseline, monitor CBC and hepatic panel during treatment with periodic repeat tuberculosis testing

 Cyclosporine: CBC, CMP, Mg, uric acid, blood pressure at baseline and during treatment, tuberculosis testing at baseline with periodic repeat testing

Septic arthritis is always in the differential for mono- or oligoarthritis, and should be ruled out with joint aspiration and blood cultures. Serologies for common ReA pathogens, such as Chlamydia trachomatis. Neisseria gonorrhea, Mycoplasma genitalium, Yersinia, Salmonella, Shigella, and Campylobacter jejuni, should be assessed. HIV testing is warranted when disease onset is severe and sudden. ESR and CRP may be elevated, while rheumatoid factor and ANA must be negative. HLA-B27 typing assists in prognosis, as positive patients have a greater risk of chronic disease. If risk factors for tuberculosis (TB) are present, diagnostic testing should be performed, as there are a few case reports of TB-associated ReA.


Table 4.4
First line therapies





















Low-, mid-, or high-potency topical corticosteroids plus salicylic acid

D

Topical salicylic acid and hydrocortisone plus aspirin

E

Tacrolimus (Protopic) 0.03 %/0.1 %a ointment

Eb

Tazarotene (Tazorac) 0.05 %/0.1 %a cream or ointmenta daily

Eb

Calcipotriene (Dovonex) cream daily

Eb


aStrength and formulation used in the published literature

bBased on adult data (used due to lack of pediatric data)

There has been success with topical steroids ranging from class VII to class I. Salicylic acid aids in the penetration of corticosteroids. One combination of topical salicylic acid and hydrocortisone plus oral aspirin (concentrations/dosages not given) resulted in complete remission in a 6-year-old girl, with clearing of skin lesions within 3 weeks [11]. Topical tacrolimus, calcipotriene, and tazarotene have been effective in treating skin disease for several adults already on systemic therapy [12, 13]


Table 4.5
Second line therapies















Sulfasalazine (Azulfidine) 500 mg daily, increased to 2,000 mg daily

Aa

Methotrexate between 10 and 50 mg/week

E

Dapsone 50 mg daily plus tacrolimus (Protopic) 0.1 % ointment

Ea


aBased on adult data

Sulfasalazine can induce remission if started within 3 months of onset [14]. Methotrexate is particularly effective for persistent skin symptoms [15]. One adult patient with circinate balanitis recalcitrant to class I topical corticosteroids responded within weeks to dapsone plus tacrolimus ointment, but was unable to wean off either therapy [16].


Table 4.6
Third line therapies




























Oral retinoids

 Acitretin (Soriatane) 0.3–0.75 mg/kd/day

Ea

 Etretinate (where available) 0.5–1 mg/kg/day

Ea

Cyclosporine 0.5 mg/kg/day slowly tapered

Ea

TNF inhibitors

 Infliximab (Remicade)

Ea

 Etanercept (Enbrel)

E (Ca)

 Adalimumab (Humira)

Ea


aBased on adult data

Acitretin and etretinate are especially effective and safe in HIV-positive patients based on adult studies [15]. Cyclosporine treatment was successful in one patient who failed etretinate [17]. Anti-TNF therapies have also shown benefit, particularly when used in combination with other systemic treatments like methotrexate [18].


Pityriasis Rubra Pilaris



Clinical Features


Pityriasis rubra pilaris (PRP) is a papulosquamous disorder characterized by well-demarcated orange-red plaques, follicular keratosis, and palmoplantar involvement, often with “islands of sparing” within lesions (Fig. 4.4). Other findings include furfuraceous scaling of the scalp, yellow-brown discoloration and thickening of the nails, subungual hyperkeratosis, and splinter hemorrhages [19]. Ectropion and erythroderma can be serious complications. Unlike the adult form, pediatric PRP may show a predilection for males over females (3:2). Acute illness or trauma may precipitate the disease. PRP is a clinical diagnosis that may be supported by histopathology that shows acantholysis, an acanthotic epidermis with alternating orthokeratosis and parakeratosis in both vertical and horizontal directions, and follicular plugging.

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Fig. 4.4
“Islands of sparing” seen in classic (type III) pityriasis rubra pilaris

Griffiths’ classification is most commonly used to describe the different forms of PRP. Types I and II encompass adult disease. A description of the different types affecting children can be found in Table 4.7. Type III is the most common type in children, and is identical to type I in adults. Types IV (Fig. 4.5) and V are less common forms of the disease, and do not always fit the classic description of PRP. Type VI was not part of Griffiths’ original classification, but has been proposed to encompass cases associated with HIV infection.

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Fig. 4.5
Type IV, or circumscribed, pityriasis rubra pilaris (Courtesy of Dr. Sheila Fallon Friedlander)



Table 4.7
Types of pityriasis rubra pilaris found in children




























Type

Description

Characteristics

III

Classic juvenile type

Keratotic papules progress in a cephalocaudal pattern and may coalesce into plaques

IV

Circumscribed juvenile type

Keratotic papules and plaques confined to the elbows and knees (Fig. 4.4)

V

Atypical (familial) juvenile type

Ichthyosiform characteristics and more prominent erythema

VI

HIV-associated type

Often characterized by filiform keratosis with comorbid acne conglobata


Management Strategies


No randomized, controlled trials exist in either the pediatric or adult literature evaluating treatment options for PRP and thus, recommendations are based on retrospective reviews and case reports. Initial treatment includes topical corticosteroids, keratolytics, and retinoids. If control cannot be achieved with topical therapies, oral retinoids are the preferred treatment. Immunomodulating drugs are an option if retinoids fail or have intolerable side effects. Timing of phototherapy initiation is unclear due to mixed results. Pruritus can be lessened with oral antihistamines or, in refractory cases, topical capsaicin has been used. Spontaneous resolution is possible, even likely, in type III patients within 1–3 years. For HIV-associated disease, highly active retroviral medications are required [20].


Investigations Recommended
























For diagnosis

 Usually a clinical diagnosis, but may biopsy to confirm or distinguish from psoriasis and other disorders (may need more than one sample to note definitive characteristics)

 HIV testing

For treatment

 Retinoids: pregnancy test (for women of child-bearing potential), CBC, AST, ALT, fasting lipids at baseline and during treatment

 TNF inhibitors: CBC, CMP, tuberculosis testing, consider hepatitis profile and HIV testing in at-risk groups before initiation, periodic re-assessment during therapy

 Methotrexate: pregnancy test (for women of child-bearing potential), CBC, CMP, tuberculosis testing at baseline, monitor CBC and hepatic panel during treatment with periodic repeat tuberculosis testing

 Cyclosporine: CBC, CMP, Mg, uric acid, blood pressure at baseline and during treatment, tuberculosis testing at baseline with periodic repeat testing

As PRP can be associated with HIV, all patients with risk factors or other symptoms should undergo HIV testing. Classic histology showed alternating ortho- and parakeratosis, hypergranulosis, and a predominantly lymphocytic sparse superficial perivascular infiltrate. Laboratory evaluation is only required for systemic therapies and varies by drug (oral retinoids and TNF inhibitors shown here).


Table 4.8
First line therapies


















Low-, mid-, or high-potency topical corticosteroids

D

Tretinoin (Retin-A) 0.01 %/0.025 %/0.04 %/0.05 %a/0.1 % creama or gel daily

E

Tazarotene (Tazorac) 0.05 %/0.1 %a cream or gela daily

E

Pimecrolimus (Elidel) 1 % cream BID

Eb


aStrength and formulation reported in literature

bBased on adult data

Initial treatment for PRP should focus on topical therapies. Several case reports have shown complete response to topical corticosteroids, ranging from low to high potency [21]. Single case reports of different topical retinoids have also shown efficacy [22, 23]. One adult case report demonstrated clearance with pimecrolimus cream [24].


Table 4.9
Second line therapies



































Oral retinoids

 Isotretinoin (Accutane) 1–2 mg/kg/day for 6 months (average)

D

 Etretinate (where available) 0.5–1 mg/kg/day for 3–6 months

E

 Acitretin (Soriatane) 0.3–0.75 mg/kg/day

E

 Alitretinoin 30 mg daily for 22 weeks

Da

Vitamin A, at least 20,000 units daily

E

Calcipotriene (Dovonex) ointment

E

nbUVB with oral retinoids

E

PUVA

E

Goeckerman treatment

E


aBased on adult data

Systemic therapies should be considered after failure of topical treatment or for more severe or diffuse disease, with topical therapies often continued as adjuncts. Retinoids are the mainstay of systemic treatment, with isotretinoin being the most commonly used in children [25]. Treatment with vitamin A or topical calcipotriene has shown benefit in single case reports [26, 27], but other reports contradict these findings. Phototherapy may be beneficial but may also aggravate PRP [28]. It should be reserved for cases refractory to retinoids and topical therapy. Goeckerman treatment, while effective in a few case reports, is not as commonly used.


Table 4.10
Third line therapies























TNF inhibitors

 Etanercept (Enbrel) 50 mg twice weekly with slow taper

E

 Infliximab (Remicade) 5 mg/kg/dose at weeks 0, 2, 6, and every 8 weeks after

E

Methotrexate

E

Cyclosporine 3 mg/kg/day with slow taper

E

Fumaric acids (Fumaderm, Europe only), start at 30 mg daily, maximum of 720 mg daily

E

Immunomodulating drugs show great promise for recalcitrant disease. There are a few case reports and one retrospective review of children receiving TNF inhibitors without significant side effects [29]. Numerous reports show no benefit with methotrexate, although one case report at an unspecified “low dose” was successful. Cyclosporine was effective in a single pediatric patient [30]. Fumaric acid treatment is currently only available in Europe [28].


Pityriasis Lichenoides



Clinical Features


Pityriasis lichenoides (PL) is an uncommon, acquired dermatosis encompassing a spectrum of disease states, with pityriasis lichenoids et varioliformis acuta (PLEVA) at one end and pityriasis lichenoides chronica (PLC) at the other. PL has a slight male predominance. Acute versus chronic describes the development of lesions, and not necessarily the disease course [31]. Overlap between PLC and PLEVA is common within the same patient. Etiology is unknown, although numerous viral triggers have been proposed and several viruses cultured from affected patients. Currently, PL is felt to lie at the benign end of a spectrum of clonal T-cell disorders. There are a few reports of PL progressing to cutaneous T-cell lymphoma, although some cases may have initially been misdiagnosed as being more benign. Differentiation of PLEVA from lymphomatoid papulosis is important (see section “Lymphomatoid Papulosis”).

Diagnosis can be made clinically, but histology may be helpful for confirmation. Pruritus occurs in half of patients, while skin tenderness is rare. PLEVA begins as an acute, generalized eruption of papules that develop necrosis and form hemorrhagic crusts, with the potential for varioliform scars (Fig. 4.6). Mild constitutional symptoms may exist. A rare and severe subtype, febrile ulceronectrotic Mucha-Habermann disease (FUMHD), shows rapid and coalescent necrosis of skin lesions, severe systemic symptoms, and potential mucous membrane involvement. Fatalities have only been reported in adults. PLC consists of small, scaly, red to brown, polymorphic papules and plaques (Fig. 4.7). The lesions may occur in crops or continuously over time, leaving post-inflammatory dyspigmentation.

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Fig. 4.6
Characteristic lesions of PLEVA with some varioliform scarring (Courtesy of Dr. Lawrence Eichenfield)


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Fig. 4.7
Pityriasis lichenoides chronica


Management Strategies


No randomized, controlled trials have evaluated PL treatment options, with recommendations based on retrospective reviews and case reports. PLEVA usually runs a self-limited course, whereas PLC often relapses and remits over years. However, chronic disease and spontaneous remission are seen across the spectrum of disease, making treatment efficacy difficult to assess. Patients younger than 4 years of age may have a higher rate of relapse. Pediatric patients tend to have a longer disease course compared to adults, and residual dyspigmentation is more common [32].

Although mid- to high-potency topical corticosteroids and antihistamines may help pruritus, they do not alter the disease course. Phototherapy and oral antibiotics are the two mainstays for initial treatment. Refractory disease may be treated with methotrexate or the addition of acitretin. Slow tapering to prevent relapse is critical for all treatment plans.


Investigations Recommended




















For diagnosis

 Consider biopsy

 FUMHD: blood cultures, imaging to rule out other etiologies, CBC, CRP, ESR, CMP, LDH

For treatment

 Consider streptococcal swab and serologies for Toxoplasma gondii, EBV, HIV, CMV, VZV

 Methotrexate: pregnancy test (for women of child-bearing potential), CBC, CMP, tuberculosis testing at baseline, monitor CBC and hepatic panel during treatment with periodic repeat tuberculosis testing

No consensus exists regarding the use of laboratory testing for diagnosis or follow-up of PL. The exception is FUMHD, as these patients are critically ill with multi-organ damage and secondary infections requiring close management. In other types, an infectious workup is obtained only if warranted by the history. If workup is positive, treatment should be directed accordingly.


Table 4.11
First line therapies























Phototherapy (narrowband or broadband UVB)

D

Oral antibiotics

 Erythromycin 30–60 mg/kg/day (max 2 g/day) divided in 3–4 doses, at least 3 months

D

 Azithromycin (Zithromaz) Z-pack dosing or 500 mg × 3 days, every other week

D

 Tetracycline 2 g/day

D

FUMHD: Methotrexate 15–20 mg/week plus high dose IV corticosteroids 40–60 mg/kg/day minimum (dosing in young children not established)

E

Phototherapy, usually nbUVB, is first-line treatment for PL [33, 34]. Number of treatments and total dose varies widely, with disagreement regarding maintenance. One retrospective study showed no benefit to concomitant systemic medications [34]. Oral antibiotics are first-line therapies as well, especially when phototherapy is not feasible. Traditionally, oral erythromycin is prescribed, but azithromycin is a newer option [35, 36]. Tetracyclines are reserved for patients older than 8 years of age. First-line treatment for FUMHD should include early initiation of high-dose corticosteroids and methotrexate, along with inpatient monitoring, aggressive wound management and debridement, and supportive care [37].


Table 4.12
Second line therapies















Methotrexate 5–7.5 mg/week

D

PUVA ± acitretin (Soriatane)

D

Tacrolimus (Protopic) 0.03 % ointment BID, or 0.1 % ointment BID plus azithromycin

E

Methotrexate is second-line treatment for patients with unresponsive, severe disease. According to one study, 20 % of patients responded to PUVA alone, and addition of acitretin may provide additional benefit [38]. Topical tacrolimus, both as monotherapy and with azithromycin, showed promise in a few individual case reports [36].


Table 4.13
Third line therapies





























Bromelain

Ea

FUMHD:

 Cyclophosphamide (Cytoxan), 1,000 mg/m2 monthly

E

 Infliximab (Remicade)

Ea

 Cyclosporine

Ea

 PUVA

Ea

 Prednisone plus nbUVB

Ea

 IVIG

Ea


aBased on adult data

Bromelain is a supplement that should only be considered in patients who refuse traditional medical therapy [39]. One case report of a child with FUMHD and CNS vasculitis responded to the addition of IV cyclophosphamide [40]. Individual adult case reports of several other treatments for FUMHD show varying success.


Lymphomatoid Papulosis



Clinical Features


Lymphomatoid papulosis (LyP) is a lymphoproliferative disease of CD30-positive T-cells, and is rarely seen in the pediatric population. It exists on a spectrum of clonal T-cell disorders, with malignant histologic characteristics but usually a benign clinical course. It may co-occur with lesions of PL or anaplastic large-cell lymphoma (ALCL). The disease displays three classic histologic subtypes, with two more recently proposed (see Table 4.14) [41]. Type A is the most common, with type B being uncommon and types C-E very rare. These subtypes all appear the same clinically, and have not been shown to affect disease course in pediatric patients. There are conflicting reports of CD4 versus CD8 predominance in children [42, 43]. While LyP lesions themselves are benign, they can be associated with lymphoma and hematologic malignancies. Although the risk is far lower than in adults with the disease, a few cases of lymphoma have been reported in affected children decades after onset, necessitating lifelong follow-up. There is a lower incidence of T-cell clonality in pediatric patients, which may account for the better prognosis.


Table 4.14
Histologic subtypes of lymphomatoid papulosis
































Type

Histology

Histologic mimic

A

Large, pleomorphic, atypical CD30-positive (Reed-Sternberg) cells with a wedge-shaped mixed inflammatory infiltrate. Eosinophilia in up to 40 %

Hodgkin’s disease

B

Small, CD30-negative lymphocytes with cerebriform nuclei in an epidermotropic infiltrate

Plaque-stage mycosis fungoides

C

Monomorphous, atypical large CD30-positive cells in sheets, with fewer other inflammatory cells making up the infiltrate. No epidermotropism

CD30-positive large cell lymphoma

D

Epidermotropic infiltrate of both atypical CD8-positive and atypical CD30-positive lymphocytes

Primary cutaneous aggressive epidermotropic CD8-positive cytotoxic T-cell lymphoma

E

Angioinvasive CD30-positive infiltrates, often with medium-sized, atypical CD8-positive lymphocytes

Extranodal natural killer/T-cell lymphomas

Classic LyP lesions consist of erythematous papules and nodules, sometimes with central ulceration. Patients suffer from cyclic eruptions that last anywhere from 2 to 8 weeks if untreated, and multiple locations are often involved. They may report a preceding viral illness. The disease can be confused with PLEVA, but LyP lesions are usually fewer and larger, with the average age of onset between 7 and 9 years. Lesions may be pruritic or tender, and often leave behind scars. The tongue may rarely be involved. LyP can be difficult to differentiate from insect bites, especially when eosinophilia is present on histology. Singular lesions can also be mistaken for primary cutaneous ALCL.


Management Strategies


If lesions are limited in number, it is reasonable not to treat while maintaining close follow-up. More numerous lesions or those in cosmetically sensitive areas should be treated due to risk of scarring. In these cases, first line treatments in children include topical corticosteroids and judiciously increasing sun exposure. For more severe, symptomatic, or widespread cases, methotrexate and phototherapy are the best options. Recurrences are common no matter the treatment employed, although rare cases of complete resolution have been reported. Oral corticosteroids are not beneficial.

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Jul 13, 2017 | Posted by in Dermatology | Comments Off on Psoriasis and Other Papulosquamous Skin Disorders
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