Dédée F. Murrell - Clinical Cases in Autoimmune Blistering Diseases

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Springer International Publishing Switzerland 2015

Dde F. Murrell (ed.) Clinical Cases in Autoimmune Blistering Diseases Clinical Cases in Dermatology 10.1007/978-3-319-10148-4_1

1. An Elderly Patient with a Generalized Pruritic Eruption

Stefanie Hfliger 1

(1)

Department of Dermatology, University Hospital Bern, Freiburgstrasse, 3010 Bern, Switzerland

Stefanie Hfliger

Email:

Luca Borradori (Corresponding author)

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A 86-year-old patient presented with generalized pruritic eruption of 3 month duration. The patient had a past history of type 2 insulin-dependent diabetes mellitus, dyslipidemia and arterial hypertension. On examination, the patient showed widespread excoriations, prurigo-like lesions, post-inflammatory hypopigmentations, with atrophic scarring distributed predominantly over his trunk, upper limbs, neck, and scalp (Fig. ). Light microscopy studies showed changes consistent with chronic prurigo. Direct immunofluorescence microscopy studies obtained from perilesional skin showed linear deposits of IgG and C3 along the epidermal basement membrane zone. By indirect IF microscopy using NaCl-separated normal human skin, there were circulating IgG autoantibodies binding the epidermal side of the split. The search of circulating anti-BP180 antibodies by ELISA was positive (41.7 U/ml; N: 9

Figure 1.1

( a ) Excoriations, postinflammatory hypopigmentations and atrophic scarring on trunk and arms; ( b ) Close-up view of excoriated lesions and scarring with isolated milia on arm; ( c ) Erosions, crusting and atrophic scarring on the scalp; ( d ) Erosion on buccal mucosa

What Is Your Diagnosis?

• Bullous pemphigoid (BP)
• Mucous membrane pemphigoid
• Brunsting-Perry pemphigoid

Discussion (1)

Diagnosis of BP is not always easy and straightforward. Manifestations of BP might resemble those of a variety of dermatoses, including drug reactions, contact dermatitis, prurigo, fixed urticaria, vasculitis, arthropod reaction and scabies (Table ].

Table 1.1

Clinical presentations of bullous pemphigoid

Chronic prurigo, prurigo nodularis-like features

Papular pemphigoid

Eczematous lesions

Erythema multiforme-like and Lyell-like pemphigoid

Lymphomatoid papulosis-like

Ecthyma-like

Palmo-plantar lesions (dysidrosiform pemphigoid)

Intertrigo (vegetating pemphigoid)

Vesicular pemphigoid

Erythrodermic pemphigoid

Brunsting-Perry form (variant of cicatricial pemphigoid with skin lesions)

Localized forms

Pretibial

Peristomal

Umbilical

Stump pemphigoid

On paralyzed body sites

On irradiated/traumatised body sites

Our patient presented with chronic excoriated lesions and post-inflammatory changes predominantly localized on the upper trunk and his head and as well as isolated lesions of the buccal mucosa. Immunopathological findings were consistent with the pemphigoid group of autoimmune bullous disorders. We favor the diagnosis of an unusual form of chronic prurigo-like BP [] with a peculiar extensive cutaneous involvement. In the absence of well recognized criteria, a conclusive classification of our case is not possible.

How Do You Manage This Patient?

• Topical corticosteroids
• Tetracyclines and nicotinamide
• Systemic steroids
• Systemic Steroids and immunsuppressants

Our BP patient was first treated with topical clobetasol propionate 0.05 % combined with doxyciclin, 200 mg daily and nicotinamide, 2 g daily. Since this regimen did not sufficiently control his skin disease, the patient was first given sulfasalazine and later oral prednisolone, 0.5 mg per kg body weight. The latter treatment resulted in control of the disease, but lead to a severe weight gain and a decompensation of his diabetes. Finally, topical steroids were initiated in combination with azathioprine 75 mg daily which lead to a complete remission of the skin disease.

Discussion (2)

BP has frequently a chronic evolution with remissions and relapses. It is associated with significant morbidity, such as severe itch, bullous and eroded lesions, and impetiginisation. The impact on the quality of life is significant [].

Prior starting a therapy in patients with bullous pemphigoid, the overall clinical context and the evidence about the available therapeutic intervention should be considered: (1) affected patients have usually an advanced age, older than 75 years of age, (2) they have frequently additional comorbidities, such as neurological or cardiovascular diseases (3) they show a significantly increased mortality patients during the first year of treatment. Finally, (4) so far, except for topical and systemic steroids, there are no studies which have validated the use of the other drugs, which have been commonly used in BP [].

The first line therapeutic option in localized, mild bullous pemphigoid consists of high potency topical corticosteroids based on their effectiveness demonstrated in two prospective controlled studies [].

Second-step and non-validated options include tetracyclines in combination with nicotinamide, topical calcineurin inhibitors and dapsone. Oral prednisone (0.5 mg/kg) can also be employed. In generalized disease, first-line treatment consist of either topical steroids applied to the entire body over 412 months [].

The optimal duration of treatment has not been defined. Based on clinical experience, we recommend an average treatment duration of 612 months, except in cases of steroid-resistance or steroid-dependence. The patients should be free of symptoms for 1 to 6 months under minimal therapy with oral prednisone (0.1 mg/kg/day), or clobetasol propionate (20 g/week), or immunosuppressants.

References

Daniel BS, Borradori L, Hall 3rd RP, Murrell DF. Evidence-based management of bullous pemphigoid. Dermatol Clin. 2011;29:61320. PubMed CrossRef

Hanno R, Foster DR, Bean SF. Brunsting-Perry cicatricial pemphigoid associated with bullous pemphigoid. J Am Acad Dermatol. 1980;3:4703. PubMed CrossRef

Joly P, Roujeau JC, Benichou J, Picard C, Dreno B, Delaporte E, Vaillant L, DIncan M, Plantin P, Bedane C, Young P, Bernard P, Bullous Diseases French Study Group. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med. 2002;346:3217. PubMed CrossRef

Joly P, Roujeau JC, Benichou J, Delaporte E, DIncan M, Dreno B, Bedane C, Sparsa A, Gorin I, Picard C, Tancrede-Bohin E, Sassolas B, Lok C, Guillaume JC, Doutre MS, Richard MA, Caux F, Prost C, Plantin P, Chosidow O, Pauwels C, Maillard H, Saiag P, Descamps V, Chevrant-Breton J, Dereure O, Hellot MF, Esteve E, Bernard P. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Investig Dermatol. 2009;129:16817. PubMed CrossRef

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