Ghai Essential Pediatrics8th

Treatment

It is important to counsel the parents and the child about the chronic nature of the disease and the likelihood of relapses. Several options are available for treatment depending on thetype and extent of disease (Table25.12).

Table 25.12: Treatment of psoriasis

*PUVA: Psoralen and ultraviolet A (UVA). Two hours after ingestion of 0.6 mg/kg of 8-methoxypsoralen, and after applicationofanemollient onthelesions,thepatient is exposed to gradually increasing doses of UVA, provided either from an artificial source or from su.nlight. The therapy should not be used in children <6 yr of age

Lichen Planus

It is an acute or chronic dermatosis involving skin,mucous membranesandnails.Theetiologyisunknown.Alichenoid eruption is seen after intake of drugs like chloroquin and as a manifestation of graft vs. host disease.

The lesions are pruritic, polygonal, violaceous and flat topped papules (Fig. 25.37) with white streaks (Wickham striae)onthesurface.Theyareseenonwrists,aroundankles andmay appear at sites of trauma (Koebner phenomenon). Mucosal involvement is seen in 25% patients in form of

Fig. 25.37: Lichen planus: Plane polygonal, violaceous papules

reticulate lacey pattern on buccal mucosa, tongue and gingivaorsuperficialerosionsontongueandbuccalmucosa. Annular lesions are seen on genitalia. Rarely scarring alopecia is present; nail changes are infrequent in children. The therapy of lichen planus is outlined in Table 25.13.

herpes viruses (HHV-7, HHV-6). The illness, usually between 10 and 35 yr, begins with a 'herald patch' in 80% cases. Lesion is characteristically oval, wrinkled with a collarette of scales at the periphery. This is followed by multipleoval to round smaller scaly secondary eruptions. Their arrangement is characteristic lesions run downwards and outwards from the spine (Christmas tree appearance) alonglinesofcleavage.Theconditionresolvesspontaneously withi.n 2-10 weeks. No treatment is usually required. Oral antihistamines, calamine lotion and topical steroids may be used to decrease itchi.ng. Exposure to sunlight makes the lesions resolve more quickly. Recalcitrant lesions may be treated with ultraviolet light.

Pemphigus Vulgaris

This isthe mostcommonvariantofpemphigus,accounting for over 80% cases. The condition is characterized by IgG antibodies against desmogleins 3 and 1, which are cell-to­ cell adhesion molecules. Patients show flaccid bullae on normal looking skin, whichruptureearly to form crusted erosions. The usual sites are scalp, face, flexures andtrunk. Oral lesions might antedate skin lesions in 50% of patients and eventually 80-90% of patients develop oral lesions. Mucosa! lesions are characteristically painful erosionswith ragged margins.

The therapy of pemphigus vulgaris is supportive, including maintaining water and electrolyte balance and controlling systemic infections. Treatment with corti­ costeroids, either as daily dose or monthly bolus is recommended. Occasionally therapy with azathioprine, methotrexate and cyclophosphamide is required.

Chronic Bullous Disease of Childhood

This blistering disorder is characterized by linear deposition of IgA in the basement membrane. The condition is seen in children less than 5 yr of age with slight female preponderance. The lesions are itchy, tense bullae on an erythematous base. New lesions appears around previous lesions resulting in a 'string of pearl' appearance. The lesions are usually grouped around the orifices (perioral, perinasal,perigenital or perianal). They are also frequently seen on lower abdomen, buttocks, knees and elbows. The oral mucosa is involved in one­ half cases. The illnessresolves within 2 yr of onset in most cases. Patients with milddisease are treatedwith dapsone (1-2 mg/kg), while those with extensive disease required combined therapy with dapsone and oral corticosteroids.

DISORDERS OF PIGMENTATION

Vitiligo

It affectsboth sexes equally andpeak incidence isbetween 10 and 30 yr. A positive family history is present in 2030% patients. Vitiligo is associatedwithotherautoimmune disorders, e.g. thyroid disorders and alopecia areata, suggesting an autoimmune etiology.

The lesions are characterized by depigmented (chalky white or pale white) macules with sharp scalloped margins, which might coalesce to form geographical patterns. Lesional hair may be depigmented (leukotrichia). Lesions may be present anywhere on the body, but areas prone to trauma are most susceptible. The lesion may be focal (;?:1 macules at a single site), segmental (unilateral, dermatomal usually along distribution of mandibular division of the facial nerve), acrofacial (periorificial and acral) (Fig. 25.38) and universalis (extensive, generalized due to confluence of patches).

The course isslowlyprogressive, thoughcan sometimes progress rapidly. Spontaneous pigmentation is seen in 10% of patients. Segmental vitiligo has a stable course. Patients with vitiligo should be examined for cutaneous

associations (alopeciaareata, atopicdermatitis), endocrine disorders (diabetes mellitus, Addison disease, hypo­ parathyroidism and thyroid disorders) and pernicious anemia.

Treatment

Predictors of poor prognosis include long-standing disease, leukotrichia and lesions on resistant areas (bony prominences, nonhairy, nonfleshy areas and mucosae). The patient and family should be reassured. Sunscreens and cosmetic cover up may be needed. The treatment of vitiligo is shown in Table 25.14.

Table 25.14: Treatment of vitiligo

Localized disease

Intolerance to PUVA Oral steroids

*PUVA/PUVA sol: Psoralen and ultraviolet A (UVA) or sunlight; not to be used in children <6 yr of age

In refractory butstablepatientsof nonsegmentalvitiligo and stable segmental vitiligo surgical techniques like punch grafting, split skin grafting, blister grafting and melanocytes transfer can be tried.

Freckles and Lentigines

Both are characterized by presence of discrete hyper­ pigmented macules. Lesions of freckles are seen in red haired, very fair children. Lentigines show no such predilection. Lesions of freckles are seen in light exposed parts of body (face, V of neck and dorsolateral aspect of forearms) with conspicuous absence on covered skin. Lentigines do not show predilection and may be seen on mucosae (Peutz-Jeghers syndrome). Freckles (Fig. 25.39) are lighter, with less delineated edges and show variegationincolor including darkening onsun exposure. Lentigines are darker, sharply defined and do not darken on sun exposure. Lentigines may be a cutaneous marker of multisystem syndromes.

DRUG ERUPTIONS

Drug eruptions are adverse events that occur after systemic or topical administration of a drug (Table 25.15).

Diagnosis

Fig. 25.39: Freckles: Discrete hyperpigmented macules with variegation in color on the face of a fair child

after any length of treatment, some drugs are more suspect and the most recent introduction the most likely cause. The role of drug provocation test is controversial but may be needed to find the culprit drug in patients on multiple drugs as well as to find safe alternative drugs.

Treatment

Withdrawal of drug is most effective approach but is not easy as the child may be taking several drugs or the suspected drug may be difficult to withdraw. A chemically unrelated substitute may not be available.

Symptomatic therapy is provided using antihistaminics. Therapy for anaphylactic reactors might be required. In

patients with Stevens-Johnson syndrome-toxic epider­ mal necrolyses complex, treatment includes maintenance of fluid-electrolyte balance and wound care. The role of systemic steroids is controversial. Intravenous immuno­ globulins and cyclosporine have also been used success­ fully.

INFECTIONS----

Skin can be infected with bacteria, virus, and fungi.

Pyodermos

Based on morphology and extent of infections, pyodermas are classified as shown in Table 25.16.

The causative organisms include S. aureus or S. pyogenes or both. Predisposing factors underlying include skin disease (scabies, atopic dermatitis, pediculosis), poor hygiene, and systemic diseases (diabetes, immune deficiencies). Clinical features of different types of pyodermas are discussed in Tables 25.17 to 25.19.

Table 25.16: Classification of pyodermas

Table 25.17: Clinical features of follicular pyodermas

Fig. 25.40: Folliculitis: Erythematous follicular papule, often surmounted by pustules

Treatment

General measures for treatment include local hygiene, rest and limb elevation in case of spreading pyodermas and NSAIDs, if pain and constitutional symptoms are present. Topical antibiotics like mupirocin, sodium fusidate and nadifloxacin are used for localized lesions. Patients with extensive spreading lesions, in presence of constitutional symptoms require therapy with systemic antibiotics.

StaphylococcalScaldedSkin Syndrome

The condition is mediated by hematogenously spread exotoxin produced by S. aureus present in infected site distant from the involved skin (e.g. otitis media, pneumonitis)andrarelyinskin. Thedisorderusuallyaffects newborns and infants <2 yr of age. Erythema and tendernessis followed by superficial peeling ofskininthin

Fig. 25.41: Impetigo contagiosa: Honey colored crusted lesions around mouth

Fig. 25.46: Indeterminate leprosy· Ill-defined hypopigmented, hypoesthetic lesion on the face

Fig. 25.47: Borderline tuberculo1d: Wei I defined erythematous plaque with satellite lesions

Investigations

Slit skin smears. Slit skin smears, from the lesions and ear lobules, are stained with modified Ziehl-Neelsen method. The smears are usually negative in TT/BT/BB leprosy. The Lepromin test is generally not required for the diagnosis. On histologic examination, perineural granulomas (epitheloid cells in tuberculoid and foamy cells in lepromatous leprosy) are typical, but might not be seen in indeterminate, Bl and LL forms.

Treatment

The patient andparentsshould be reassured and counselled regarding treatment compliance and care of hands, feet and eyes. For the purpose of treatment, leprosy is classified into paucibacillary and multibacillary leprosy. Multidrug therapy is instituted based on number of lesions (Table 25.21). Therapy of lepra reactions is shown in Table 25.22.

Verruca (Warts)

Warts are caused by human papilloma virus, of which there are more than 100 types. They are transmitted by

Table 25.21: WHO recommendations for treatment of leprosy in children aged 10-15 yr

Doses in children: Rifampicin 10 mg/kg; clofazimine 1 mg/kg daily, 6 mg/kg monthly; dapsone 2 mg/kg

Table 25.22: Treatment of reactions in leprosy

NSAIDs nonsteroidal anti-inflammatory drugs are avoided *Thalidomide is a teratogenic agent and avoided in girls in the reproductive age

close contact and auto-inoculation. In children, nongenital warts are common, with an incidence of up to 10%. The clinical features of various types of warts are listed in Table 25.23.

Molluscum Contagiosum

Patients show multiple pearly white, dome shaped papu­ les with central umbilication (Fig. 25.50). Cheesy material can be expressed from the lesion. The lesions are seen on any part of the body. Widespreadlesions are seen in atopic dermatitis and immunocompromised patients. The condition is self limiting, and clears within an one-yr. Therapy comprises Wart paint or mechanical extirpation under cover of a topical anesthetic.

Herpes Simplex Virus (HSV) Infections

HSV infection can be asymptomatic and when sympto­ matic, the manifestations depend on whether the infection is primary or recurrent. Primary type 1 infection may present as acute gingivostomatitis with closed grouped vesicles on an edematous base which rupture to form polycyclic erosions (Fig. 25.51). There may be associated malaise, fever and lymphadenopathy. Patientswithherpes simplex labialis have a prodrome of burning and stinging followed by grouped vesicular lesions with background of slight erythema. These lesions may evolve into erosions

Fig. 25.48: Verrucavulgaris: Firm papuleswith hyperkeratotic, clefted surface

Fig. 25.50: Molluscum contagiosum: Pearly white dome shaped papules with central umbilication

with polycyclic margins, leaving area of depigmentation after healing (Fig. 25.52). No treatment is generally required, except in severe infection or immunocompro­ mised patients where treatment with oral acyclovir may be given for 5-7 days.

Dermatophytoses

Three genera of fungi cause dermatophytoses Tricho­ phyton, Epidermophyton and Microsporum. The infection is given different names depending on the site affected. Dermatophyte infection of skin is known as tinea corporis, of groin as tinea cruris, of hands as tinea manuum, of feet as tineapedis andofnailsastinea unguium. The classical lesion is an annular or arcuate plaque with a clear center and an active edge showing papulovesiculation and scaling.

Tineo Capitis

Fig. 25.51: Herpes gingivostomatitis: Closed grouped vesicles on an edematous base which coalesce to form polycyclic erosions

Noninflammatory or epidemic type. Caused by anthropophilic organisms and so is responsible for epidemics. It presents as a patch of alopecia with marked scaling at periphery (Fig. 25.53). Hairs break off easily and inflammation is minimal.

Inflammatory or kerion. Caused byzoophilicorganisms and so does not cause epidemics. It presents as a boggy swelling which drains pus from multiple openings (Fig. 25.54).Hairis easily pluckable without pain.Usually associated with occipital lymphadenopathy.

Favus. Caused by T. schoenleinii, presents as yellowish,foul smelling cup-shaped crusts with matting of hair.

Tineo Corporis

Shows classical features of tinea and is most frequent on exposed parts. Infection of face is common in children. The diagnosis is confirmed by the KOH test that shows fungal hyphae. Culture helps in identification of species and this is important in patients with tinea capitis.

The therapy of tinea capitis comprises griseofulvin (15 mg/kg/day of ultramicrosized formulation) for a durationof 8weeks. Terbinafine (5mg/kg/dayfor4 weeks) is effective in noninflammatory tinea capitis. Longer treatment (8weeks)is neededforkerion.Useof terbinafine in children is hindered by absence of liquid formulation and an unpleasant aftertaste of the tablet. Washing with ketoconazole shampoo helps to reduce transmission. Sharing of combs and head wear should be avoided.

Localized lesions of tinea corporis are managed by topical therapy (azoles available as clotrimazole,

Fig. 25.53: Tinea capitis: Area of nonscarring alopecia with minimal inflammation

miconazole or ketoconazole in lotion, gel and cream formulations). Widespread lesions require systemic antifungal therapy with terbinafine (2 weeks) or griseofulvin (for 4 weeks).

Candidiasis

Candida albicans, a normal commensal becomespathogenic in the presence ofpredisposing factors that include obesity, diabetes and immunocompromised states. Less frequently other species like C. glnbrata may be involved. In children, candidiasis presents as oral thrush, vulvovaginitis, intertrigo, candidal diaper dermatitis or paronychia.

Oral thrush presents as soft, creamy white to yellow, elevated plaques, that are easily wiped off to leave an erythematous, eroded or ulcerated surface. The lesions are seen on buccal mucosa, tongue, palate and gingiva.

Candida/ intertrigo presents as erythematous, moist, macerated lesions with a frayed irregular edge with satellite pustules, chiefly present in major skin folds, like axillae, groins and neck.

Candida/ diaper dermatitis is characterized by well defined weeping eroded lesions with scalloped border and a collar of overhanging scales and satellite pustules. The lesion begins in the perianal region, spreading to perineum, upper thighs, lower abdomen and lower back.

The KOH testshowsbudding yeasts and pseudohyphae and culture confirms the diagnosis. Predisposing factors should be addressed and the area should be kept dry. Topicaltherapywithimidazoles (clotrimazole, miconazole and ketoconazole) and nystatin creams for folds and lotions for oral mucosa. Systemic therapy with weekly fluconazole orpulseitraconazole isgivenfor patients with onychomycosis.

Pityriasis Versicolor

This condition is caused by commensal yeast, M. furfur. Adolescentspresent with scaly, perifollicular macules with variable pigmentation (hypopigmented, erythematous or hyperpigmented). The fine branny scales are accentuated by gentle abrasion with a glass slide. The lesions are frequently seen on upper torso (both anterior and posterior), neck and sometimes also on proximal part of upper extremities. KOH mount shows a mixture of short branched hyphae and spores (spaghetti and meatball appearance). Topical therapy with imidazoles (keto­ conazole 2%) for 3 consecutive days or selenium sulfide 2.5% lotion applied once a week for 4 weeks is sufficient in most cases. Systemic therapy with fluconazole is occasionally required.

DISEASES CAUSED BY ARTHROPODS

Scabies

Scabies is caused by Sarcoptes scabei var hominis and transmitted by close contact with infested humans.

Clinical features comprisethesymptoms of severe itching, more intense at night. The primary lesion is a burrow, a grey thread like serpentine line with a minute papule at the end; papules and papulovesicles may also be seen. Secondary lesions consist of pustules, eczematized lesions and nodules. Lesions are seen in webs of hands, on wrists, ulnar aspects of forearms, elbows, axillae, umbilical area, genitalia, feet and buttocks. Face is usually spared except in infants in whom scalp, palms and soles (Fig. 25.SSA) are also involved. Nodular lesions are seen on genitalia (Fig.25.SSB). Secondarystreptococcal infection may result in acute glomerulonephritis.

Treatment

All close contacts of the patient, even if asymptomatic, should be treated. Overzealous laundering of bed linen and clothes is not warranted. The topical scabicide should be applied all over body below the neck including on the free edge of nails, genitals, soles of feet after hydration of body with a bath. Scabicides available include:

Permethrin 5%. Overnight single application is treatment of choice beyond 2 months of age.

Crotamiton 10%: Two applications daily for 14 days, is recommended for infants less than 2 months.

Benzyl benzoate 25%. Three applications at 12 hourly intervals.

Ivermectin, single oral dose of 200 µg/kg body weight, in children older than 5 yr is the treatment of choice for epidemics (as in orphanages). Antibiotics are given, if secondary infection is present. Antihistamines are given for 1-2 weeks to reduce pruritus.

Pediculosis

Louse is an obligate ectoparasite and two species infest humans: Perliculosis '11111ia1111s (P. '11111101111s cnpitis, head louse and P. '111111n1111s corporis, body louse), and Pilthirns pubis (pubic louse)

Head louse infestation is transmitted by close contact and pubic louse infestation is acquired by children from infested parents. Head louse infestation is common in children while pubic louse infestation is infrequent but

when it occurs it also involves eyelashes and eye brows. The chief symptoms are severe pruritis involving

infested region. Though adultliceare difficult to find, nits (egg-capsules) are easily seen firmly cemented to hair on which they can be slided but not flicked off. The lesions may show secondary infection, eczematization and occipital lymphadenopathy.

All family members should be treated. The chief pediculocides are:

• Permethrin, l'X, lotion, single 10 min application to wet hair followed by rinsing. Repeat application after 7 days.

• Gamma benzene hexacloride, l % single over night

application to dry hair followed by rinsing. Second application used after 7 days.