Dual diagnosis and the skin. Update on dermatologic disorders in children and adults with dual diagnosis. Self-injurious behavior (SIB)

Transcription

1 Dual diagnosis and the skin Update on dermatologic disorders in children and adults with dual diagnosis Neuropsychological factors Julie V. Schaffer, MD Assistant Professor of Dermatology and Pediatrics Director of Pediatric Dermatology New York University School of Medicine Environmental factors Psychodermatology Complex interaction between the mind and the skin Neuro-immuno-cutaneous system Understanding the emotional and psychosocial context of skin disease is important for optimal management Koo & Lebwol Am Fam Phys 2001 Gupta & Gupta J Am Acad Dermatol 1996 Self-injurious behavior (SIB) Common, destructive behavior disorder in individuals with developmental disabilities Typically a specific action focused upon a particular body site e.g. picking an area on the arm, biting the hand Symons & Thompson J Intellect Disability Res

2 Psychosocial/emotional components of SIB Study of boys with fragile X syndrome (n=55) 58% displayed SIB Most often hand/finger biting, head hitting, skin picking/scratching, and hair pulling SIB tended to be associated with difficult task demands and changes in routine Suggests escape from aversive stimuli and difficulty regulating emotion Symons et al Am J Med Genet 2003 Pain and SIB Study of nonverbal children with severe developmental disability (n=101) 44% displayed SIB Similar pain expression in those with and without SIB Individuals without chronic pain (n=64) 48% displayed SIB SIB was more frequent and favored head & hands Individuals with chronic pain (n=37) 35% displayed SIB SIB was less frequent and favored sites of pain Breau et al J Pediatr 2003 Altered nociception and SIB Possible relationship to endogenous opioids High baseline levels Stimulation of release Normal Abnormal diurnal variation of substance P levels, higher cortisol levels Substance P Epidermal nerve fibers* with variable morphology, irregular spacing, and increased substance P SIB Mast cell Increased degranulated mast cells in the skin* *From non-injured body sites Symons et al Pain 2008 Symons et al Brain Behav Devel 2008 Opioids? Inflammation, pain/itch/other 2

3 Neurotic excoriations Pathologic skin picking Common sites Extensor extremities Scrotum, perianal area Clinical findings Weeping, crusted, or lichenified lesions Postinflammatory hypo- or hyperpigmentation Predisposing conditions Anxiety Depression OCD Walling et al Clin Exp Derm 2007 Lichen simplex chronicus Caused by habitual scratching/rubbing Vicious itch-scratch cycle Xerosis, irritation, & atopy are predisposing factors Linked to psychosocial stress, OCD In individuals with DD, may occur in the setting of repetitive behaviors Possible association with neuropathy Favors the occiput/neck, wrists/forearms, ankles, scrotum/vulva Solak et al Clin Exp Dermatol 2008 Lotti et al Dermatol Ther 2008 Prurigo nodularis Breaking the itch-scratch cycle Emollients Antipruritics (variable efficacy) Topical pramoxine Sedating antihistamines (esp. doxepin) Corticosteroids High-potency topical, under occlusion in the short term (e.g. flurandrenolide tape) Intralesional Exclude an underlying systemic cause e.g. CBC/differential, BUN/creatinine, LFTs, TFTs, ferritin Behavioral therapy 3

4 Daily bathing OK if Luke-warm water Minimal mild cleanser Basics of skin care Application of emollient immediately after bathing ( soak and smear ) Ointments & creams are more moisturizing than lotions If needed, first apply topical medications to appropriate areas, then emollient all over Dermatitis treatment plan Exacerbation Daily use of a topical corticosteroid High-level maintenance to usual sites Intermittent use of topical corticosteroid and/or topical calcineurin inhibitor Low-level maintenance to all skin Emollients Avoidance of triggers ± Bleach in bathwater Proper topical corticosteroid use Short-term daily use for flares (up to several weeks at a time) Long-term intermittent use (e.g. twice weekly or bursts of daily use for flares) x Long-term continuous use Dermatitis artefacta Patient deliberately produces lesions but denies having a role No conscious motive, but psychological gain Relatively high-functioning patients Self-inflicted injury is typically produced with something other than the fingernails Sharp instruments, cigarettes, chemicals Clinical findings Lesions with bizarre/angular shapes Visible, reachable sites Finore et al Pediatr Derm

5 Hair pulling / trichotillomania Riga-Fide disease / traumatic oral granuloma Ceyhan et al Clin Exp Dermatol 2009 Domingues-Cruz et al Pediatr Dermatol 2007 Zaenglein et al J Am Acad Dermatol 2002 Secondary infections: impetigo & ecthyma Staphylococcal folliculitis 5

6 Staphylococcal furunculosis Staphylococcus aureus colonization Outbreaks of skin infections reported in long-term care facilities and group homes for individuals with DD Increased recurrences in those with Down syndrome, related to impaired neutrophil chemotaxis Sites of MSSA/MRSA colonization Anterior nares, throat Skin folds, e.g. axillae and groin Sites of eczematous dermatitis Fomites, e.g. medical devices Buehelmann et al Infect Control Hosp Epidemiol 2008 Staphylococcus aureus decolonization Irritant diaper dermatitis Nasal Mupirocin?Retapamulin studies in progress Cutaneous Chlorhexidine- or triclosan-containing washes 0.005% sodium hypochlorite baths (1/4 cup household bleach in 1/2 standard tub) Simor et al Infect Dis Clin N Am

7 Incontinence and irritant dermatitis Treatment of irritant diaper dermatitis Spectrum of severe forms in patients of all ages Jacquet s erosive diaper dermatitis Perianal pseudoverrucous papules/nodules Granuloma glutale Robson et al J Am Acad Dermatol 2006 Markham et al Arch Dermatol 2000 Scrupulous hygiene with gentle cleansing Discontinue potential irritants (e.g. benzocaine, resorcinol) Assess for and treat candidal or bacterial superinfections Culture swab Therapy to calm acute flare Mild topical corticosteroid (e.g. HC 2.5% or desonide cream) Azole antifungal (e.g. ketoconazole or econazole cream) anti-inflammatory as well as antimicrobial Mix in hand and apply ~TID Avoid combo products with inappropriately strong corticosteroid Barrier, barrier, barrier Zinc oxide in petrolatum/lanolin base New zinc oxide + miconazole ointments Vusion, Triple Paste AF Apply thick coat with each diaper change Candidal diaper dermatitis Candidiasis: not just for little ones 7

8 Staphylococcal infection in the diaper area Angular cheilitis: irritant + candidal components Drooling Management of angular cheilitis Therapy for acute flare Topical corticosteroid (e.g. desonide ointment) + azole antifungal Assess for and treat associated thrush Barrier, barrier, barrier Frequent application of petrolatumbased ointment/lip balm Other assessments Culture swab of lips and nares Dental/orthodontic evaluation (e.g. for malocclusion) Additional irritant or allergen exposure Nutritional status Winter et al Spec Care Dentist 2008; Cheilitis: irritant + candidal in Down syndrome Predisposing factors Large, protruding scrotal tongue Thick, fissured, everted lips Open mouth posture drooling Angular (25%) and diffuse (25%) cheilitis Both associated with Candida (75%) Scully et al Br J Derm 2002 Asokan et al Indian J Dental Res

9 Skin conditions in Down syndrome: autoimmune disorders Alopecia areata (5-20%) Vitiligo (~5%) Skin conditions in Down syndrome: connective tissue disorders Elastosis perforans serpiginosa (~1%) Anetoderma Collagenomas Daneshpazhooh et al Pediatr Dermatol 2007 Madden et al Clin Exp Dermatol Daneshpazhooh et al Pediatr Dermatol 2007 Madden et al Clin Exp Dermatol 2007 Skin conditions in Down syndrome: adnexal disorders Folliculitis (30-40%) Keratosis pilaris (up to 25%) Multiple syringomas (10-30%) Milia-like calcinosis cutis (syringocentric) Skin conditions in Down syndrome: ichthyosiform and vascular Acquired ichthyosis Keratoderma Cutis marmorata Acrocyanosis Madden et al Clin Exp Dermatol 2007 Schepis et al Dermatology 2002 Madden et al Clin Exp Dermatol

10 For recalcitrant periorificial dermatitis. Consider a nutritional deficiency Inquire about dietary habits Full skin, genital, and oral examination Laboratory testing Zinc deficiency (acquired acrodermatitis enteropathica) Dermatitis: periorificial ± acral Diarrhea Alopecia Erythema with erosions, crusting, desquamation Sanchez et al J Cutan Pathol 2007 Niacin (B 3 ) deficiency (pellagra) Dermatitis Photodistribution: acral, face/neck (Peri)orificial: cheilitis, glossitis, perineal involvement Diarrhea Dementia Riboflavin (B 2 ) & pyridoxine (B 6 ) deficiencies Ocular Oral: cheilitis, glossitis, periorificial dermatitis Genital Cakmak et al Eur J Dermatol 2006; Sanchez et al J Cutan Pathol 2007 Friedli et al N Engl J Med

11 Dual diagnosis and the skin Neuropsychological factors Neuropsychological factors X Environmental factors 11