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Chapter 36: Acantholytic Disorders of the Skin

Varsha Parthasarathy; Junwen Deng; Jihad Alhariri

ACANTHOLYTIC DISORDERS OF THE SKIN

Therapeutic Approach

Acantholytic disorders of the skin are a heterogeneous group of disorders characterized by the loss of intercellular contacts between suprabasal keratinocytes, or acantholysis. The three major acantholytic disorders of the skin are Darier-White disease (DD), Hailey-Hailey disease (HHD), and Grover disease (GD). DD and HHD are autosomal dominant disorders, whereas GD is a sporadic disease of unknown etiology that is mostly seen in patients with sun-damaged skin. While clinical trial data are needed to determine disease-specific therapies, therapeutic strategies are aimed at reducing inflammation, relieving symptoms, and prevention of secondary infections. Treatment for localized inflammation should be with topical monotherapy or combination therapy with corticosteroids, antibiotics, calcineurin inhibitors, and retinoids. Treatment for generalized disease include systemic agents such as oral acitretin and isotretinoin. See Table 36-1.

Table 36-1Acantholytic Disorders of the Skin Treatment Table
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Table 36-1 Acantholytic Disorders of the Skin Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Emollients DD, HHD, GD Prevention of epidermal water loss and dehydration Urea-based: 30%-40% cream, lotion, or gel as needed; lactic acid-based: 10% cream as needed Burning, itching, stinging, redness, irritation None IV1
Zinc oxide HHD Anti-inflammatory, maintenance of skin microbiome 40% cream as needed Skin irritation None III2
Topical antibiotics GD, HHD Anti-microbial; treatment of and prevention of secondary infections 0.2% erythromycin ointment, 1% clindamycin ointment, 0.1% gentamicin ointment, mupirocin 2% ointment; all TID Local site irritation, erythema, urticaria None III,3 IV4
Topical corticosteroids DD, HHD, GD Inhibition of inflammatory transcription factors and prostaglandins, vasoconstriction Moderate to high potency: 0.1%-0.5% triamcinolone cream, 0.1% betamethasone valerate, 0.05% clobetasol ointment, 0.1% mometasone furoate cream; all BID Skin atrophy, telangiectasia, hypopigmentation None III5-7
Tacrolimus/Pimecrolimus DD, HHD Binding of FKBP-12, calcineurin/cytokine inhibition Tacrolimus 0.1% ointment BID/pimecrolimus 1% cream BID Skin irritation, burning sensation None III8-10
Topical retinoids DD, GD Topical retinoid, reduction of desquamation and keratinocyte proliferation 0.05%-0.1% isotretinoin gel QD; 0.1% tretinoin gel QD; 0.05% tazoretene gel QD; 0.1% adapalene gel QD Erythema, burning, skin irritation and dryness, risk of teratogenicity Screen for pregnancy in women of childbearing potential

Isotretinoin: IIb11

Tretinoin: III12

Tazarotene: III13

Adapalene: III14
5-Fluorouracil DD, HHD Pyrimidine analog, inhibition of DNA replication and repair 1%-5% cream 3 times weekly Burning, crusting, allergic contact dermatitis, erythema, hyperpigmentation, photosensitivity, leukocytosis None III15-16
Vitamin D analogs HHD, GD Localized modulation of keratinocytes and inflammatory mediators 3 mg/g calcitriol ointment BID; 0.005% calcipotriol ointment QD Tingling or burning of skin, erythema, skin rash None III17-19
Systemic Therapy
Oral doxycycline DD, HHD Inhibition of bacterial protein synthesis, reduction of inflammation 100 mg QD to BID Diarrhea, enamel staining, erythematous rash, skin photosensitivity Avoid in pregnancy and in children <8 y III20-21
Oral corticosteroids DD, GD Inhibition of ...

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