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Saudi Journal of Kidney Diseases and Transplantation
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Year : 2020  |  Volume : 31  |  Issue : 2  |  Page : 553-555
Bullous lesions in a young adolescent postrenal transplant

Division of Pediatric Nephrology, Sheikh Khalifa Medical City, Abu Dhabi, United Arab Emirates

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Date of Submission22-May-2019
Date of Decision30-Jun-2019
Date of Acceptance02-Jul-2019
Date of Web Publication09-May-2020


Kidney transplant recipients frequently suffer from skin infections and malignancies. New dermatosis can appear after transplantation. Although children are maintained on varying degrees of chronic immunosuppression, there is still a possibility of autoimmune blistering skin conditions, which can pose a diagnostic challenge in terms of clinical presentation. Histopathology of skin lesions is very important which helps in correct diagnosis and prompt treatment. We report an extremely rare case of linear IgA dermatosis in a child who was postrenal transplant and treated successfully with dapsone and steroids.

How to cite this article:
Kumar G, AlHadhrami HK. Bullous lesions in a young adolescent postrenal transplant. Saudi J Kidney Dis Transpl 2020;31:553-5

How to cite this URL:
Kumar G, AlHadhrami HK. Bullous lesions in a young adolescent postrenal transplant. Saudi J Kidney Dis Transpl [serial online] 2020 [cited 2021 May 15];31:553-5. Available from: https://www.sjkdt.org/text.asp?2020/31/2/553/284037

   Introduction Top

Linear IgA bullous dermatosis (LABD) is an acquired subepidermal immunoglobulin- mediated vesiculobullous disease. The diagnosis requires a biopsy of the lesion. We report a child who was postrenal transplant and deve-loped this skin condition and treated successfully with dapsone and steroids.

   Case Report Top

Informed consent was obtained from the parents of the child for publication of the case as well as the picture of the child.

A 13-year-old male child, ESRD secondary to congenital nephrotic syndrome transplanted five years back (donor being father), presented to emergency with few bullous skin lesions on the face. He was otherwise comfortable and not sick looking. There was no history of any new medication intake or recent illness. The rash flared up in next two days and spread to the trunk and lower limbs. Oral mucosa, palms, and soles were spared. The rash was intensely pruritic and the bullae were different sized and tense [Figure 1]. Multiple bullae, vesicles, and erosions grouped in an annular pattern involving the trunk, extremities, face, and genitals were seen. Many lesions exhibited honey-colored crusts and excoriations. The characteristic“collarettes” of blisters formed as new lesions arose in the periphery of old lesions. There was no fever. Herpes simplex virus and varicella polymerase chain reaction were negative. Blood culture sent on admission was sterile. There was no previous history of any skin disease in child or family.
Figure 1: Skin Lesions of index child.

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The current immunosuppression included tac- rolimus/azathioprine/prednisolone. Laboratories done showed creatinine 77 pmol/L (baseline value for child). Full blood counts showed mild leukocytosis and inflammatory markers were slightly increased with a C-reactive protein 23 mg/L (normal <5).

Punch biopsy from upper arm was taken and is shown in the [Figure 2]. A punch biopsy from a lesion showed subepidermal blister (arrow) containing edema fluid and a mixture of mainly eosinophils and neutrophils and a few lymphocytes (star). There was superficial peri- vascular lymphocytic infiltrate admixed with eosinophils which are seen also interstitially. An immunofluorescence revealed linear IgA and C3 deposition along the dermoepidermal junction, confirming a diagnosis of LABD. There was no IgG, C5b-9, or fibrinogen deposition.
Figure 2: Histopathology of skin lesions.

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The child was treated with steroids and dapsone and showed good recovery of the lesions over the next four weeks. The child is currently doing well.

   Discussion Top

Postrenal transplantation, children may be at risk of infectious complications due to immu- nocompromised state. Many such infections such as herpes and varicella can present as skin rash and infections need to be ruled out. However, noninfectious skin rash may also occur and should be investigated properly to reach to a specific diagnosis. Rainey et al reported noninfectious diffuse vesiculobullous skin rash in a 41-year-old woman with pancreas/kidney transplant recipient where the skin rash was initially presumed to be infectious in origin and later diagnosed as Linear IgA dermatoses.[1] Our index child also developed skin lesions which were bullous. Further biopsy however confirmed the diagnosis of linear IgA dermatosis.

The bullous skin diseases are being divided into two categories based on whether the skin is affected within the epidermis (pemphigus vulgaris, pemphigus foliaceus, paraneoplastic pemphigus, and IgA pemphigus) or at the epidermal-dermal interphase (linear IgA dermatosis, bullous pemphigoid, and dermatitis herpetiformis). The differential diagnosis to be considered for autoimmune blistering skin disorders represents a rare group of auto- antibody-induced dermatosis against desmo- somal and hemidesmosomal molecules. The common age of onset for pemphigus and pemphigoid, as well as dermatitis herpeti- formis, encompasses the adult age, but all these disorders can be observed in neonates and/or during childhood. Bullous pemphigoid involves antibodies against proportion of desmosome at dermal-epidermal junction with immunofluorescence (linear deposition of IgG/C3).

LABD, also known as linear IgA dermatosis or linear IgA disease, is a subepidermal vesiculobullous disease, which can affect both adults and children. It is characterized by linear deposition of IgA along the basement membrane zone (BMZ), with the possible presence of circulating IgA anti-BMZ antibodies. The diagnosis of LABD requires three criteria:

  1. vesicular or bullous eruption, usually confined to the skin and rarely involving the mucous membranes
  2. subepidermal vesicle with a predominantly neutrophilic infiltrate on histology; and
  3. BMZ-specific IgA antibody deposited in a linear pattern without the presence of other immunoglobulins under direct immunofluorescence examination of perilesional skin.[2]

Usually idiopathic, LABD can be drug- induced. Various drugs have been associated with the drug-induced form of the disease, often in association with systemic vancomycin but also reported with phenytoin, amiodarone, captopril, and nonsteroidal anti-inflammatory drugs. The differential diagnosis of chronic bullous dermatoses of childhood includes bullous impetigo, dermatitis herpetiformis, and bullous pemphigoid.

The histological features are an overlap between linear IgA dermatosis versus bullous pemphigoid versus dermatitis herpetiformis. The presence of linear IgA positivity at the dermoepidermal junction is in favor of the linear IgA dermatosis over dermatitis herpeti- formis which show granular IgA deposition. The absence of IgG immune reactant staining is against the diagnosis of bullous pemphigoid.

The first line of treatment for linear IgA dermatosis includes sulfones (dapsone) ± low- dose systemic corticosteroids. Dapsone is the treatment of choice due to its anti-inflammatory and immune-modulatory effects. It is thought to inhibit lysosomal activity, myelo- peroxidase-mediated iodination, and adherence of neutrophils to the BMZ. There is a risk of hemolytic anemia in G6PD-deficient patients. The course of linear IgA dermatosis is variable and unpredictable as it may spontaneously remit in some cases and others it may last for years. Various therapeutic approaches have been used in nonresponder patients such as mycophenolate mofetil and rituximab.[3]

Bullous diseases are difficult to diagnose[1] and immunofluorescence studies are extremely important to distinguish between diseases such as classic LABD and bullous pemphigoid. Children postrenal transplant developing bullous lesions should have a skin biopsy of lesions to nail the diagnosis and treatment should be tailored specifically based on findings.

Conflict of interest: None declared.

   References Top

Rainey A, Auerbach J, Shah K. Non-infectious diffuse vesiculobullous rash in a pancreas and renal transplant recipient. Transpl Infect Dis 2018;20:e12990.  Back to cited text no. 1
Egan CA, Zone JJ. Linear IgA bullous dermatosis. Int J Dermatol 1999;38:818-27.  Back to cited text no. 2
Farley-Li J, Mancini AJ. Treatment of linear IgA bullous dermatosis of childhood with mycophenolate mofetil. Arch Dermatol 2003; 139:1121-4.  Back to cited text no. 3

Correspondence Address:
Gurinder Kumar
Division of Pediatric Nephrology, Sheikh Khalifa Medical City, Abu Dhabi
United Arab Emirates
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DOI: 10.4103/1319-2442.284037

PMID: 32394935

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