Superadded chromomycosis in recessive dystrophic epidermolysis bullosa: An easily misdiagnosed entity

Gaurav Dash; Swetalina Pradhan

doi:10.4103/ijpd.IJPD_47_20

CASE REPORT

Year : 2021 | Volume : 22 | Issue : 2 | Page : 145-147

Superadded chromomycosis in recessive dystrophic epidermolysis bullosa: An easily misdiagnosed entity

Gaurav Dash¹, Swetalina Pradhan²
¹ Assistant Professor, Hitech Medical College and Hospital, Bhubaneswar, Odisha, India
² Department of Dermatology, All India Institute of Medical Sciences, Patna, Bihar, India

Date of Submission	25-Mar-2020
Date of Decision	26-Apr-2020
Date of Acceptance	25-Nov-2020
Date of Web Publication	31-Mar-2021

Correspondence Address:
Swetalina Pradhan
Department of Dermatology, All India Institute of Medical Sciences, Patna, Bihar
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/ijpd.IJPD_47_20

Abstract

Recessive dystrophic epidermolysis bullosa (RDEB) (Hallopeau Siemens) is one of the most severe forms of epidermolysis bullosa (EB) with generalized vesiculation over the skin and mucous membrane resulting in extensive scarring with exuberant granulation tissue. Because of widespread erosions, patients are prone to secondary infection. We are reporting a 10-year-old male child having RDEB who presented with a nonhealing ulcer with verrucous growth over bilateral knees for 2 years which was considered as part of the disease by many dermatologists and was later diagnosed to be superadded chromoblastomycosis based on histopathology. We are highlighting this case to increase awareness among dermatologists regarding the occurrence of superadded deep fungal infections like chromoblastomycosis in patients of RDEB which is usually missed or misdiagnosed as part of the disease.

Keywords: Chromoblastomycosis, Hallopeau Siemens, recessive dystrophic epidermolysis bullosa, superadded deep fungal infection

How to cite this article:
Dash G, Pradhan S. Superadded chromomycosis in recessive dystrophic epidermolysis bullosa: An easily misdiagnosed entity. Indian J Paediatr Dermatol 2021;22:145-7

How to cite this URL:
Dash G, Pradhan S. Superadded chromomycosis in recessive dystrophic epidermolysis bullosa: An easily misdiagnosed entity. Indian J Paediatr Dermatol [serial online] 2021 [cited 2021 Apr 18];22:145-7. Available from: https://www.ijpd.in/text.asp?2021/22/2/145/312835

Introduction

Epidermolysis bullosa (EB) is a genetic disorder in which there is a separation of skin and mucosa from underlying connective tissue resulting in the formation of blisters spontaneously or after minor trauma.^[1] Recessive dystrophic EB (RDEB) (Hallopeau Siemens) is the severe form of EB in which blisters present all over the body on the skin and mucosae from birth resulting in extensive scarring with excessive granulation tissue.^[2] Because of active erosions, patients are susceptible to develop secondary infection. We report a 10-year-old boy having RDEB who presented with nonhealing ulcers with verrucous growth over bilateral knees for 2 years and was found to have superadded chromoblastomycosis.

Case Report

A 10-year-old boy born out of consanguineous parentage presented with recurrent erosions on the skin and oral mucosa, multiple scars all over the body since childhood, and nonhealing ulcers with verrucous growth over bilateral knees for 2 years. The parents were concerned about the growths over knees which were not healing compared to other erosions. It was diagnosed as dystrophic EB (DEB) by various dermatologists, and the patient was explained about the skin lesions and verrucous growth being the part of the same disease. On examination, there were erosions mostly over trauma prone areas, especially elbow, knee, and multiple healed scars with milia over the trunk and face. There was fusion of fingers and toes (pseudosyndactyly) giving rise to mitten deformities. Nails were absent. Ophthalmological examination showed bilateral corneal opacity. Oral mucosa examination revealed erosions with poor dental hygiene and decreased mouth opening because of contracture. Examination of knees showed verrucous growths on both the knees with multiple black dots on the surface [Figure 1]. on history and examination findings, a clinical diagnosis of Recessive dystrophic EB (RDEB) with superadded chromoblastomycosis was made. Routine investigations such as complete blood count, liver function tests, and kidney function tests were within normal limits. KOH mount from ulcer with granulation tissue was negative. However, a histopathology study from both the verrucous plaques revealed pseudoepitheliomatous epidermal hyperplasia with chronic granulomatous inflammation in the dermis and copper penny bodies at the center in high power consistent with chromoblastomycosis [Figure 2]a and [Figure 2]b. The patient was started on itraconazole 100 mg twice daily for chromoblastomycosis, and barrier dressing with paraffin gauze dressing (Jelonet^®) and topical antibiotics was done for the active erosions. The patient was counseled to avoid trauma and wear loose clothes and use thick gauze pads over bilateral elbows and thick gloves and socks in hands and feet. After 1 month of itraconazole therapy, there was complete improvement in verrucous growth over the right knee and around 60% improvement over the left knee [Figure 3]. Subsequently, the patient was lost to follow up.

Figure 1: (a) Verrucous growths over bilateral knees. (b) Multiple black dots on the surface

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Figure 2: (a) H and E stain, scanner view showing pseudoepitheliomatous epidermal hyperplasia with chronic granulomatous inflammation in the dermis. (b) H and E stain, ×400 magnification showing suppurative granulomatous reaction with copper penny bodies in the center (marked by black arrow)

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Figure 3: Complete improvement in verrucous growth over the right knee and around 60% improvement in growth over the left knee

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Discussion

Recessive dystrophic EB (RDEB) is a type of dystrophic EB characterized by vesicles all over the whole body rupturing to form erosions which heal with extensive scarring with exuberant granulation tissue.^[2],[3],[4] There is a fusion of digital web spaces due to scarring resulting in pseudosyndactyly.^[5],[6]Scarring in the oral mucosa causes ankyloglossia and microstomia which impairs food intake and results in poor dental hygiene and caries.^[7] Similarly, corneal erosions and scarring cause loss of vision. Due to the presence of widespread erosions on the body, these patients are prone to secondary infections with various Gram-positive organisms, particularly Staphylococcus aureus and streptococci, as well as Gram-negatives such as Pseudomonas aeruginosa and Proteus, and Candida species.^[8]

Therapy of RDEB aims to prevent blister formation and aggressive treatment of secondary infection, thus enabling wound healing. Prevention of blisters is attempted by the use of soft and loose-fitting clothes, padding the bony prominences, and avoidance of trauma, excess heat, and adhesives on the skin. Prevention of infection is achieved by changing dressings daily, applying topical antibiotics to lesions, and nonsticky dressings (Jelonet) to denuded areas.

Our patient presented with blisters, erosions, erosions with granulation tissue, extensive scarring, milia, contractures, mitten deformities of hands and feet, bilateral corneal opacity, anonychia, decreased mouth opening, oral erosions, and poor dental hygiene for which a diagnosis of Recessive dystrophic EB (RDEB) (generalized severe) was made clinically. Initially, persistent verrucous growth over the knee was misdiagnosed as a part of the disease course for 2 years but later was suspected to be chromoblastomycosis looking at the black dots on the surface and confirmed by histopathology.

Chromoblastomycosis is a chronic fungal infection of the skin and subcutaneous tissues caused by pigmented or dematiaceous fungi such as Fonsecaea pedrosoi and Cladosporium carrionii which are implanted into the skin by trauma.^[9] It can be easily identified by the blackish dots over granulation tissue which is characteristic of chromoblastomycosis. Useful diagnostic tests include KOH mount and histopathology. It is usually treated with itraconazole 200–400 mg/day or terbinafine 250–500 mg/day for a period of 6–12 months.^[9] Our patient responded to oral itraconazole. Patients of RDEB who usually present with erosions, scars, and ulcers with heaped up granulation tissue and deep fungal infections like chromoblastomycosis can be easily missed. We are reporting the case to increase awareness among dermatologists regarding the occurrence of superadded deep fungal infections like chromoblastomycosis in patients of RDEB having nonhealing verrucous growths not responding to conventional therapy and proper dressing.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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