Linear IgA Bullous Dermatosis With Laryngeal Involvement

Presentation of a Case

We present the case of a 54-year-old patient diagnosed with pemphigus approximately 10 years ago due to blister-like skin lesions, with no mucosal involvement and with a predilection for extensor surfaces. The patient attended the ENT clinic for the first time 2 years ago for odynophagia and dysphagia of several weeks’ duration. Vesiculobullous lesions were observed throughout the pharyngeal mucosa on examination. We referred the patient to dermatology for further study due to a possible relationship with cutaneous pemphigus, and after skin biopsy and direct immunofluorescence a diagnosis of IgA linear bullous dermatosis was confirmed.

Dapsone 100 mg and prednisone 10 mg were prescribed as baseline treatment, combined in recent months with sulfasalazine 1 g/8 h.

Despite this, the patient was seen on numerous occasions in the emergency department for acute dyspnoea and stridor and treated with high-dose intravenous cortico-therapy and support measures.

On examination (Fig. 1), erosive blistering lesions, some haemorrhagic, were observed in the oropharynx, larynx and hypopharynx. The presence of erosive lesions in the nasal cavities was noteworthy, with significant dryness of the mucous membranes and formation of scabs. We also observed generalised atrophy of the turbinates and presence of intranasal synechiae.

Figure 1.

Vesiculobullous, erosive and haemorrhagic lesions, oedema and areas with fibrin deposits in the nasal mucosa and laryngopharyngeal area are observed. A) Oropharynx. B) Right nostril. C) Lingual face of epiglottis. D) Oedema and interarytenoid synechia, reduction of laryngeal vestibule.

The abovementioned lesions were seen at the level of the larynx, as well as some areas with fibrin deposit in the supraglottis, in the arytenoid region and pyriform sinuses. A moderate reduction of the laryngeal vestibule was also observed due to mucosal oedema and formation of synechia in the arytenoid region

To date, the patient has not required tracheotomy, although due to the progression of the lesions after each outbreak and the worsening of his baseline dyspnoea, it seems likely that it will be necessary in the future.

Discussion

IgA linear dermatosis is a chronic acquired bullous disease, recognised as an entity in its own right since 1979 by Jablonska and Chorzelski,¹ differentiating it from dermatitis herpetiformis and bullous pemphigoid. It has an annual incidence in Europe of .5 cases per million inhabitants.²

Its clinical presentation consists of the appearance of vesiculobullous lesions with a predilection for the extensor surfaces and with mucosal involvement in up to 70% of cases and particularly oral and conjunctival involvement in the head and neck, extension to the larynx is very rare.²

There are 2 age peaks. There is a child form in the first years of life, between 5 months and 6 years, which usually disappears after puberty and more frequently affects the face, genitals and thighs. There is another peak in adulthood, between the fourth and fifth decades of life, with a predilection for the face, trunk and limbs.

In most cases it is of idiopathic origin, although it has been associated with some drugs, such as vancomycin, NSAIDs, captopril and amiodarone.³ Gluten-sensitive enteropathy has been associated in 25% and 33% of cases, but in a milder form than that observed in 90% of patients with dermatitis herpetiformis.⁴

IgA autoantibodies target the proteins LAD-1 (120 kD) and LABD97 (97 kD), proteolytic fragments of glycoprotein BP 180 (type XVII collagen), located in the lamina lucida.⁵ Its clinical presentation is heterogeneous, usually in the form of tight blisters of varying size, although it can also manifest itself in the form of papules and clear or haemorrhagic vesicles. The lesions sit on skin of normal appearance or erythematous base, with a generally symmetrical distribution. They are usually located on the extensor surfaces of the limbs, the trunk, the buttocks and the face. The mucous membranes can be involved in up to 70% of cases, particularly with oral and conjunctival involvement, although on very rare occasions, as in this patient, the laryngeal mucosa may be affected, even compromising the upper airway.^2,3

Although there are numerous cases described with involvement of the oral mucosa,^6–8 we find few published studies of patients with IgA dermatitis with involvement of the oral mucosa.⁹

The histological study (Fig. 2) shows the presence of blisters in the subepidermal space that contain abundant neutrophils and a variable number of eosinophils, as well as non-specific inflammatory infiltration in the dermis. Less frequently, micro-abscesses of neutrophils are observed in the dermal papillae.¹⁰

Figure 2.

The patient’s skin biopsy. A) A subepidermal blister can be seen, with epidermis of normal appearance and unaltered keratinisation. The blister cavity contains fibrin with nuclear dust and neutrophils. The floor of the blister corresponds to the papillary dermis showing perivascular infiltration of lymphocytes and interstitial infiltration of neutrophils, with practically no eosinophils (haematoxylin-eosin stain ×4). B) Direct immunofluorescence in which a linear band of IgA is observed in the basement membrane (×400 magnification).

Direct immunofluorescence is the key to diagnosis, observing the linear deposit of IgA along the basement membrane, and enables a differential diagnosis with other bullous diseases. IgA autoantibodies target proteins LAD-1 (120 kD) and LABD97 (97 kD), proteolytic fragments of the glycoprotein BP 180 (type XVII collagen), at the level of the lamina lucida.²

In some cases, IgM and C3 deposits can also be detected along with IgA. Indirect immunofluorescence is positive in adults in only 30% of cases and at low titres, which increases to 75% in children.²

A differential diagnosis must be made with other dermatoses such as bullous or cicatricial pemphigoid, dermatitis herpetiformis, epidermolysis bullosa acquisita or even toxic epidermal necrolysis, among others

The treatment of choice for IgA linear dermatitis is dapsone or sulphonamides, and systemic corticosteroids can be combined. Other therapeutic options are immunosuppressants such as colchicine, mycophenolate mofetil or cyclosporine. In drug-induced cases, discontinuation is usually sufficient.

Conflict of Interests

The authors have no conflict of interests to declare.