hidden under a cauliflower like growth a case of cutaneous chromoblastomycosis and response to combination therapy

Hidden under a cauliflower-like growth: A case of cutaneous chromoblastomycosis and response to combination therapy a Department of Dermatology, King Fahad Central Hospital, Jizan, Saudi

Hidden under a cauliflower-like growth: A case of cutaneous chromoblastomycosis and response to combination therapy

a

Department of Dermatology, King Fahad Central Hospital, Jizan, Saudi Arabia

b Department of Pathology, King Fahad Central Hospital, Jizan, Saudi Arabia Received 28 September 2016; accepted 12 November 2016

Abstract

Chromoblastomycosis is a rare, chronic fungal infection of skin and subcutaneous tissue It is caused by several pigmented fungi com-monly seen in tropical and sub-tropical regions Here, we report a case of chronic cutaneous chromoblastomycosis in a middle aged man from the southern part of Jizan, Saudi Arabia, who presented to our derma clinic with verrucous cauliflower like growth on the right hand Later on, histopathological study showed pathognomonic characteristic brown colored spores (copper pennies or medlar bodies)

of the fungus within dermal abscess The patient was cured after a 6 month combination use of itraconazole and cryotherapy Review of literature showed this is the first cutaneous chromoblastomycosis reported from Saudi Arabia

Ó 2016 The Authors Production and hosting by Elsevier B.V on behalf of King Saud University This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/)

Keywords: Chromoblastomycosis; Copper pennies; Medlar bodies; Itraconazole; Cryotherapy

1 Introduction

Chromoblastomycosis is a chronic deep skin mycosis

caused by pigmented fungi that are implanted into skin

from the environment The most common etiologic agents

are Fonsecaea pedrosoi, Phialophora verrucosa and

Clado-phialophora carrioni (Ameen, 2009) All have low

viru-lence, produce similar clinical manifestations and can be

isolated from soil and plant debris The pigmented,

so-called, dematiaceous fungus (Fonsecaea pedrosoi) is the

etiologic agent in 90–96% of cases (Bonifaz et al., 2001)

The infection occurs sporadically in South America, Carib-bean region, Madagascar, Australia, and Japan It may also occur as an imported infection outside the usual ende-mic areas The disease is most frequent in male rural work-ers The infection usually follows implantation through tissue injury (Rubin et al., 1991) Following inoculation

by skin trauma, local infection with slowly growing, raised scaly plaques or warty cauliflower-like lesions develop (Minotto et al., 2001) The presented case displayed fea-tures of warty cauliflower like tumor on the right hand

2 Case report

A 68 year old Saudi man presented to our department with single well defined verrucous firm to hard longitudinal lesion on the right hand that had started 25 years back According to the patient, the lesion had enlarged slowly and he remembered no traumatic skin lesion except minor scratches from gardening many years back It began as a http://dx.doi.org/10.1016/j.jdds.2016.11.001

2352-2410/ Ó 2016 The Authors Production and hosting by Elsevier B.V on behalf of King Saud University.

This is an open access article under the CC BY-NC-ND license ( http://creativecommons.org/licenses/by-nc-nd/4.0/ ).

⇑ Corresponding author.

E-mail address: alattas101khalid@yahoo.com (K.M Al Attas).

Peer review under responsibility of King Saud University.

Production and hosting by Elsevier

Available online at www.sciencedirect.com

www.jdds.org

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small, round nodule, whitish-brown in color and hard in

consistency

Physical examination revealed erythematous,

hyperker-atotic, contiguous, indurated, longitudinal plaques with

warty, crusted areas with black dots Adjacent to these

pla-ques was a larger whitish area with scar-like appearance

along with few scattered nodular lesions (Fig 1) The

regio-nal lymph nodes were not palpable

Laboratory examination disclosed low hemoglobin (Hb

11 g/dl; normal 12–16) Liver function, renal function,

blood sugar and erythrocyte sedimentation rate were

nor-mal Serology for sexually transmitted diseases including

syphilis, human immunodeficiency virus, hepatitis B, C

was negative Microbiological study was inconclusive and

chest X-ray was normal

Biopsy specimens taken from the lesion showed

pseu-doepitheliomatous epidermal hyperplasia and suppurative

granulomatous inflammation in the dermis There were

epithelioid cells and foreign body giant cells, some with

engulfed brown color pigmented sclerotic bodies (copper

pennies or medlar bodies) Morphologically, round bodies

are sized about 7–10 microns; compatible with

chro-moblastomycosis (Figs 2 and 3)

Based on these findings, we established a definitive

diag-nosis of cutaneous chromoblastomycosis The patient was

started on itraconazole 100 mg twice daily along with

cryotherapy sessions separated by an interval of 1 month

Initially, topical antibiotic (fusidic acid) was also used to

treat the secondary bacterial infection Treatment with

itra-conazole was maintained for 6 months The lesions healed

leaving residual scarring and depigmentation in some areas

(Fig 4) No recurrence was observed during 12 month

follow-up peroid

3 Discussion

Chromoblastomycosis is endemic in tropical and

sub-tropical regions Cases from Saudi Arabia are rare and

often related to work with tropical woods and fields ( al-Hedaithy et al., 1988) Our patient remembered no trau-matic skin lesion except minor scratches from gardening many years back He had no travel history outside Saudi Arabia The lesion started 25 years back as a small, round

Figure 1 Showing firm, hyperkeratotic, contiguous plaques with black

dots and crusted areas on a erythematous base Adjacent to these plaques,

larger whitish areas with scar-like appearance along with few scattered

nodular lesions are seen.

Figure 2 Showing thick walled brown color, pigmented round fungal spores(medlar bodies or copper pennies) within giant cells of granuloma and mixed suppurative and granulomatous dermal inflammation.

Figure 3 Showing budding of fungal elements in GMS staining.

Figure 4 Showing cured lesions with some depigmentation and mild residual fibrosis.

2 M.K Ahsan et al / Journal of Dermatology & Dermatologic Surgery xxx (2016) xxx–xxx

nodule, whitish-brown in color and hard in consistency In

the literature (Bonifaz et al., 2001; Ezughah et al., 2003) the

lower extremities are typically involved, but in our patient

the lesions was on the hand

Histopathology is pathognomonic for

chromoblastomy-cosis, but the identification of the causative fungal species

can only be obtained by culture Delay in diagnosis of

chromoblastomycosis up to several years is not unusual,

as in our patient Chromoblastomycosis can be

con-founded with squamous cell carcinoma (SCC), especially

if there is clinical absence of pigmentation and

histopathol-ogy shows pseudoepitheliomatous hyperplasia of the

epi-dermis (Minotto et al., 2001; Bonifaz et al., 2001) In our

case, though there was pseudoepitheliomatous hyperplasia

and history of long standing infection, no change to SCC

was found

It is difficult to treat chromoblastomycosis because of

differences in antifungal sensitivity patterns and responses

among the species isolated Furthermore, the disease itself

is refractory in nature particularly in more serious clinical

forms The different treatment modalities available have

not been compared in clinical settings Recurrence is

com-mon, so the recommendation is for long term treatments

ranging from 3 to 18 months (Queiroz-Telles et al., 2008)

Common complications include secondary bacterial

infec-tion with lymphadenitis and less frequently, the

develop-ment of SCC in lesions that have been present for a long

time (Minotto et al., 2001) The only complication in our

case was secondary impetiginization, which was resolved

with topical antibiotic

The best systemic antifungals seem to be itraconazole

and terbinafine because of their spectrum of action and

safety in long term regimens (Ameen, 2009) However,

tis-sue fibrosis secondary to long term infection can reduce

drug tissue level In our patient, itraconazole twice daily

was used for 6 months

Antifungal combined with cryotherapy has emerged as a

possible treatment option in recent years Cryotherapy is

associated with the best outcome of all the physical

modalities with a cure rate of 40.9% when used as

monotherapy (Castro et al., 2003) It appears to be more useful when combined with systemic antifungal to treat long standing cases Surgery may be the best choice in the early stage of disease but not in late and advanced case

In our patient, 6 cycles of cryotherapy (one cycle/-month) combined with itraconazole (100 mg twice daily) for 6 months achieved cure and caused minimal local effects like hypopigmentation and mild residual fibrosis Recent publications also support the use of combined ther-apies that is not amenable to surgical treatment

Finally, a higher grade of clinical suspicion and a wider differential diagnosis of the pigmented skin tumors would have been needed to initiate proper and adequate treatment Early starting of an appropriate combined treatments are crucial for management of chromoblastomycosis

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Bonifaz, A., Carrasco-Gerard, E., Saul, A., 2001 Chromoblastomycosis: clinical and mycologic experience of 51 cases Mycoses 44 (1–2), 1–7 [PubMed]

Castro, L.G., Pimentel, E.R., Lacaz, C.S., 2003 Treatment of chro-momycosis by cryosurgery with liquid nitrogen: 15 years’ experience Int J Dermatol 42, 408–412

Ezughah, F.I., Orpin, S., Finch, T.M., Colloby, P.S., 2003 Chromoblas-tomycosis imported from Malta Clin Exp Dermatol 28 (5), 486–487 (PubMed)

Minotto, D., Varejao Bernarti, C.D., Mallmann, L.F., Albano Edelweiss, M.I., Scrofemeker, M.A., 2001 Chromoblastomycosis: a review of 100 cases in the state of Rio Grande do Sul, Brazil J Am Acad Dermatol 44, 585–592

Queiroz-Telles, F., Esterre, P., Perez-Blanco, M., Bonifaz, A., 2008 Chromoblastomycosis: an overview of clinical manifestations, diagno-sis and treatment Med Mycol 47, 3–15

Rubin, H.A., Bruce, S., Rosen, T., McBride, M.E., 1991 Evidence for percutaneous inoculation as the mode of transmission for Chro-moblastomycosis J Am Acad Dermatol 25, 951–954

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