Dyshidrosiform bullous pemphigoidL12.0

Levine et al. 1979 (Archives of Dermatology).

Dyshidrosiform bullous pemphigoid is a rarely acrally localized variant of bullous pemphigoid. To date, < 100 patients have been reported.

M:w=1:1; the incidence of dyshidrosiform bullous pemphigoid has not yet been clarified. Retrospective studies on patients with bullous pemphigoid, in which attempts were made to determine the number of patients with dyshidrosiform bullous pemphigoid, show that the incidence of this variant of bullous pemphigoid is between 3.5% and 45% in relation to a total collective (Cohen PR 2021).

The pathogenesis of dyshidrosiform bullous pemphigoid is assumed to be identical to that of classic bullous pemphigoid. However, the preference of dyshidrosiform bullous pemphigoid for the acral localization, especially for palms and soles, has not yet been clarified.

Contact sensitization (e.g. to metal allergens) has been cited as an aggravating or triggering factor in cases of autoimmunological predisposition. For example, in a nickel-sensitized patient, a single oral nickel challenge led to new blisters of bullous pemphigoid within 24 hours, confirming the role of the metal as a trigger for the pathogenesis of her dyshidrosiform bullous pemphigoid. Drug reactions can also trigger dyshidrosiform bullous pemphigoid.

Patients with dyshidrosiform bullous pemphigoid were either adult patients or pediatric patients. Most adult patients (>80%) were >60 years old at the time of diagnosis.

Clinically, dyshidrosiform bullous pemphigoid manifests itself on the hands and feet, often preferentially on the palms of the hands or soles of the feet with recurrent or long-term persistent, often painful blisters or medium-sized and large blisters, often with hemorrhagic tinged contents (Chessa MA et al. 2020). The blisters on the palms of the hands and soles of the feet are often initially confused with dyshidrotic dermatitis. Simultaneous infestation of hands/feet together with other localizations is possible.

Possible blood eosinophilia

Western blot tests and enzyme immunoassays for autoantibodies against bullous pemphigoid antigen 1 (BPAg1) or bullous pemphigoid antigen 2 (BPAg2) are expected to be positive.

The histologic findings are identical to the findings in classic bullous pemphigoid. Observed are: subepidermal blisters with or without infiltrates of eosinophils in the dermis.

Immunofluorescence: Analogous to the classic bullous pemphigoid, linear deposits of complement component 3 (C3) and immunoglobulin G (IgG) are found along the dermoepidermal junction and the blister surface.

Proof of BmAk.

The morphology of palmar and plantar dyshidrosiform bullous pemphigoid lesions resembles vesicular (dyshidrotic) hand or foot dermatitis.

In addition to dyshidrosis or pompholyx, the clinical differential diagnosis includes allergic and irritant contact dermatitis

chronic bullous disease in childhood

Dermatophyte infection (bullous)

Epidermolysis bullosa aquisita

Erythema multiforme

Herpes gestationis

Impetigo (bullous)

Lichen planus (bullous)

linear IgA disease

scabies.

Systemic corticosteroids (50-100mg prednisolone equivalent/day)

Alternative: Dapsone (50-200mg/day)

Alternative: Oral antibiotics such as erythromycin or doxycycline

Immunosuppressants, primarily azathioprine, are used in combination with corticosteroids for the treatment of dyshidrosiform bullous pemphigoid.

Casusitis (Forschner A et al. 2005)

An 80-year-old woman presented with a spontaneously occurring bullous dermatosis limited to the soles of both feet and causing severe itching. She also suffered from Parkinson's disease and depression and had been treated with levodopa, benserazide and mirtazapine for several years.

Clinical examination revealed multiple tense and hemorrhagic bullae up to 3 cm in diameter on both soles of the feet and multiple confluent dyshidrotic vesicles. The rest of the skin, including the mucous membranes and palms, was normal.

The initial clinical diagnosis was podopompholyx, but histopathologic findings and direct immunofluorescence revealed a diagnosis of bullous pemphigoid with subepidermal blisters and linear deposits of C3 and immunoglobulin G (IgG).

Indirect immunofluorescence was positive, IgG autoantibodies bound to the epidermal site of salt-cracked skin, and circulating antibodies against bullous pemphigoid antigens 1 and 2 were found.

Based on this typical clinical picture, the diagnosis of dyshidrotic pemphigoid, a localized form of bullous pemphigoid, was made.

Therapy: Under systemic treatment with prednisolone, 40 mg/day, the skin healed completely within 2 weeks. Nothing is known about later recurrences.

1. Braun B et al. (2002) Bullous pemphigoid first manifestation under the picture of dyshidrosiform hand and foot eczema and prurigo nodularis Hautarzt 53:739-743.
2. Chessa MA et al. (2020) Multiple itchy blisters and painful erosions on the hands. J Dtsch Dermatol Ges 18: 634-637.
3. Cohen PR (2021) Dyshidrosiform bullous pemphigoid. Medicina (Kaunas) 57:398.
4. Dayal S et al. (2017) Dyshidrosiform pemphigoid localized on the hands in a child: a rare occurrence. An Bras Dermatol 92:714-716.
5. Forschner A et al. (2005) Localized pemphigoid on the soles of both feet. Int J Dermatol 44:312-314.
6. Nellore A et al. (2023) Bullous Pemphigoid Limited to the Hands and Feet: A Rare Case of an Infantile Disease Pattern Seen in an Elderly Patient. Cureus 15:e38853.