Epidermal Nevus with Epidermolytic Hyperkeratosis: A Case Report

 

*Fatima K,¹ Banu SG,² Kamal M³

 

1. *Dr. Kaniz Fatima, Resident, Phase-B, Department of Pathology, Bangabandhu Sheikh Mujib Medical University (BSMMU).joty2005.kfz@gmail.com
2. Sultana Gulshana Banu, Associate Professor, Department of Pathology, Bangabandhu Sheikh Mujib Medical University (BSMMU).
3. Mohammed Kamal, Professor, Department of Pathology, Bangabandhu Sheikh Mujib Medical University (BSMMU).

 

*Correspondence.

 

Abstract

Epidermal nevus has various histological patterns. Epidermal nevus having features of epidermolytic hyperkeratosis is a rare condition. This lesion is clinically indistinguishable from other epidermal nevi but shows characteristic histological features. Diagnosis of this entity is important management. Epidermolytic hyperkeratosis is an autosomal dominant disease. Offspring of these patients may have generalized epidermolytic hyperkeratosis. Here we present a case of 12 years old boy with Epidermal nevus with epidermolytic hyperkeratosis, which is a rare entity.

[Journal of Histopathology and Cytopathology, 2020 Jan; 4 (1):65-69]

 Keywords: Epidermal nevus, Epidermolytic hyperkeratosis, Generalized epidermolytic hyperkeratosis, Line of Blaschko.

 Introduction

Epidermal nevus comprises a heterogenous group of diseases. It is a congenital non-inflammatory cutaneous hamartoma. It may occur sporadically or as a part of several syndromes. The condition affects 1 in 1000 people in the world.¹ Histologically, epidermal nevi are composed of keratinocytes, apocrine glands, eccrine glands, sebaceous glands or other components of pilosebaceous unit. Epidermal nevi are traditionally asymptomatic. Small number of cases of epidermal nevi show histologic features of epidermolysis hyperkeratosis. Epidermolytic hyperkeratosis has been observed in variety of benign and malignant skin condition or hereditary disorders. Epidermal nevus with epidermolysis hyperkeratosis has a significant clinical importance. This patient carries the risk of parenting a child of gerneralizedepidermolytichyperkeratosis.² We report a case of epidermal nevus showing epidermolytic hyperkeratosis in a 12 year old boy for the rarity of the entity.

 Case report

A 12 year old boy of a non-consanguineous parentage, presented with non-pruritic, dark coloured elevated skin eruptions since birth. The lesion was first observed over the dorsum of right foot. After that lesions gradually appeared in front of leg and thigh, lower abdomen and flexor aspect of both forearm. Lesions were not related with any seasonal variation. Patient had a normal birth history and developmental milestones. Right sided extremities were more involved than left side.Examination revealed numerous hyperpigmented warty papules distributed in both extremities and lower abdomen (Figure. 1 and 2).  Hair, nails and oral mucosa were normal. Other system examination revealed no abnormality. No laboratory investigation was done.

Clinically it was diagnosised as Linear verrucous epidermal nevus. For histopathological examination 3 mm punch biopsy was taken from right foot. Histological examination revealed hyperkeratosis, acanthosis, papillomatosis, elongated rete ridges. The dermis revealed mild perivascular infiltration of chronic inflammatory cells.Some foci also revealed perinuclear vacuolization of the keratinocytes in spinous and granular layers, and increased number of keratohyalin granules in the stratum granulosum (Figure 3, 4 and 5).  So, histologically it was diagnosed as epidermal nevus with epidermolytic hyperkeratosis.

Discussion

Epidermal nevus is hamartoma of skin, occurs due to over growth of epidermis. It arises from embryonic ectoderm as a result of mosaic postzygotic mutations. Lesions are present at birth in about half of the patients or may develop early in childhood. Depending on the affected component of the epidermis epidermal nevus can be divided into two types: keratinocytic or non organoidand organoid type.³ Keratinocytic epidermal nevus is the most common type of epidermal nevus. It occurs due to overgrowth of keratinocytes. Different varients of keratinocytic epidermal nevus are seen, such as linear epidermal nevus, hard nevus of Unna, soft epidermal nevus and nevus verrucosus etc.¹ On the other hand organoid type shows predominantly another component of skin.⁴ Epidermal nevus occurs as a result of activated genetic mutation in FGFR-3, HRAS or PIK3CA genes. FOXN1 is highly expressed in these lesions.¹ Most common pattern of keratinocytic nevus is linear epidermal nevus. The lesions are verrucous, skin-coloured dirty gray or brown coloured papule, which coalesce to form serpiginious plaques. They follow the line of Blaschko. These lines are thought to be representative pathways of epidermal cell migration and proliferation during development of fetus.⁵

Linear epidermal nevus may be either localized or systematized. In localized type, only one linear lesion is present and lesion is confined to one side of the body. Common sites are head, trunk and extremities. In systematized type there are many parallel linear lesions are seen. They may be unilateral or bilateral.

Localized and more commonly systematized linear epidermal nevus may be associated with skeletal deformity and CNS deficiency.⁶ Rarely squamous cell carcinoma or basal cell carcinoma may arise in epidermal nevus.⁷

Epidermal nevus may occur as a part of epidermal nevus syndrome and may be associated with internal manifestation. These syndromes have characteristic cutaneous findings and at times relevantly specific internal findings.¹  The six different types of epidermal nevus syndromes are nevus sebaceous, CHILD (congenital hemidysplasia with ichthyosiform erythroderma and limb defects) syndrome, nevus comedonicus, Becker’s nevus, Proteus syndrome, phacomatosispigmentokeratotica.

Histologically epidermis of epidermal nevus is hyperplastic. There is variable hyperkeratosis, papillomatosis and acanthosis with elongation of rete ridges. Upto 62% of biopsy specimens have these pattern and these are called non-epidermolytic epidermal nevus. About 16% of epidermal nevi show features of epidermolytic hyperkeratosis. Other histologic patterns are psoriatic type, acrokeratosisverruciformis like type and a Darier’s disease like type.¹ Epidermolytic hyperkeratosis is more common in systematized type than localized type. This reaction pattern of skin was first described by Ackerman in 1970.⁷It occurs due to defective keratin genes (KRT-1 and KRT-2), which causes excessive and abnormal keratinization. The salient histologic features are- compact hyperkeratosis, perinuclear vacuolization of the cells in stratum malpighii, irregular cellular boundaries and increased numbers of large irregular keratohyaline granules. Epidermolytic hyperkeratosis is seen in other conditions, such as- bullous congenital icthyosiformerythroderma, icthyosisbullosa of Siemens, Vorner’spalmoplanterkeratoderma, melanocytic nevus, epidermolyticacanthoma, basal cell carcinoma and squamous cell carcinoma etc.

Main clinical differential diagnosis of epidermal nevus areepidermodysplasiaverruciformis, inflammatory linear verrucous epidermal nevus, linear psoriasis and lichenstriatus.Epidermodysplasiaverruciformis is a genetic disease characterized by HPV infection. This is usually associated with HPV 5 or 8, less commonly 3, 4, 5.¹ Histologically this lesion is characterized by acanthosis, hyperkeratosis, large cells with blue-gray cytoplasm, often with dysplastic change and irregular granular layer with rare perinuclear halo. Inflammatory linear verrucous epidermal nevus (ILVEN) is a type of epidermal nevus. These lesions are also distributed in linear pattern, but they are erythematous and itchy. Histologically ILVEN is characterized by some specific features, which are absent in epidermal nevus. These are- areas of depressed orthokeratosis with underlyinghypergranulosis, alternating areas of slightly raised parakeratosis with underlying hypogranulosis.⁸ In linear psoriasis, the lesions may be pink to red papules or silvery scales. Typical histologic findings are regular elongation of rete ridges, thin suprapaillary plates, hypo or agranulosis, Munro microabcess and spongiform pustules of Kogoj, which are absent in epidermal nevus. In lichen striatus, the lesions are erythematous papules arranged in linear pattern following lines of Blaschko. But histologically it differs from epidermal nevus by presence of vacuolar alteration of basal layer and band like lymphocytic infiltrate.⁷ Treatment modalities of epidermal nevus are topical cream, cryotherapy, laser and dermabrasion. But management is difficult, because the lesions  recur unless treatment extend into dermis.

Conclusion

Epidermal nevus with epidermolytic hyperkeratosis is a rare condition. As this is a mosaic genetic disorder of suprabasal keratin, it can be transmitted to offspring and produce generalized epidermolytic hyperkeratosis. So genetic counseling is essential for these patients.

References

1. James W, Berger T, Elston D, Neuhaus I. Andrew’s Diseases of the Skin. 12th ed. Philadelphia: ELSEVIER. 2016. pp 625-26
2. Guite Z, Pamei D, Gunto H, Das K. Epidermolytic hyperkeratosis in verrucous epidermal nevus. Journal of medical society. 2014; 28(1): 47-8.
3. Pollozhani N, Damevska K, Silvija D, Adjievska N, Gocev G. Epidermolytic hyperkeratosis: clue for diagnosis. Global Dermatology.  2017; 4(1): 1-3.
4. Ngan V (2003). All about the skin. Retrieved from DermNet NZ website.  https://www.dermanet.org.nz
5. Kouzak SS, Mendes MS, Costa IM. Cutaneous mosaicisms: concepts, patterns and classifications. An Bras Dermatol. 2013;88(4):507-517.
6. Edler D. in Lever’s Histopathology of the Skin. 10^th Philadelphia: LIPPINCOTT WILLIAMS & WILKINS; 2009. pp: 791-92
7. Das A, Podder I, Das A, Ghosh A, Shome K. Epidermolyticblaschkoidverrucous epidermal nevus: Report of two cases. Indian J DermatopatholDiagnDermatol. 2015; 2:46-8.
8. Meibodi NT, Nahidi Y, Jaridi Z. Epidermolytic hyperkeratosis in inflammatory linear verrucous epidermal nevus. Indian J Dermatol. 2011; 56:309-12.

 

Papillary Thyroid Carcinoma Arising within Mature Ovarian Teratoma: A Case Report

Sadaf A, ¹ Hossain MI,²  Sultana  N,³ Nasreen S,⁴ Rahman Z⁵

 

1. *Dr. Anika Sadaf, MD. (Pathology) Phase B, Resident, Department of Pathology, Chittagong Medical College, Chattogram, Bangladesh, 4203. anikasadaf261189@gmail.com.
2. Mohammad Ismail Hossain. Lecturer, Department of Pathology, Chittagong Medical College, Chattogram, Bangladesh, 4203.
3. Nahid Sultana. Senior Consultant, Obstetrics & Gynaecology. 250 Bedded General Hospital,Chandpur, Bangladesh, 3600.
4. Sayeeda Nasreen. Assistant Professor, Department of Pathology, Chittagong Medical College, Chattogram, Bangladesh, 4203.
5. Zillur Rahman. Professor, Department of Pathology, Chittagong Medical College, Chattogram, Bangladesh, 4203.

 *For correspondence

 Abstract

Mature cystic teratoma is the commonest ovarian germ cell tumor. Though malignant transformation is uncommon, papillary thyroid carcinoma has rarely been described as associated with ovarian teratomas. We report a case of a 34-years old multiparous woman who presented with acute abdominal pain and an ovarian mass. After salphingo-oophorectomy, the patient was diagnosed as papillary thyroid carcinoma that arose within a mature cystic ovarian teratoma. To our knowledge, this is the first reported case of papillary thyroid carcinoma arising within a mature ovarian teratoma in this tertiary health care center in Chattogram. We recommend long term follow up to see any metastatic possibility.

 [Journal of Histopathology and Cytopathology, 2020 Jan; 4 (1):60-64]

Keywords: Mature cystic teratoma, papillary thyroid carcinoma, struma ovarii.

Introduction

Among the ovarian germ cell tumors Mature Cystic Teratoma (MCT) is the most common and comprises 10–20% of all ovarian tumors. However, malignant transformation of MCT is not common and the incidence is 1–3%.¹ Squamous Cell Carcinoma (SCC) is the commonest type, found in 80% of cases.² Papillary thyroid carcinoma (PTC) within teratoma is one of the rarest types with ranges varying from 0.1% and 0.2%, and usually diagnosed postoperatively.³ The synchronous development of malignant struma ovarii and primary thyroid carcinoma is extremely rare, though a handful of cases were reported.⁴ So, presence of thyroid tissue in teratoma, should proceed with further work up to confirm the diagnosis and to explore the possibility of a malignant lesion in the mass- either primary or metastasis.⁵ Here we present a case of a patient with a PTC arising within a ovarian MCT.

Case Presentation
34-year-old multiparous woman who had abdominal pain, distention and irregular menstrual bleeding for approximately for 6 months, presented to the emergency service of Chittagong Medical College Hospital in May 2019 with the complaints of a sharp pain in lower abdomen, with accompanying vomitting. There was no previous medical or surgical history.

On abdominal examination, a tender mass adjacent to the left side of the umbilicus were detected. Manual examination of the vagina revealed tenderness and mass in left adnexal region. Paps smear was done with no abnormality detected. Haemogram and biochemical test results were normal except a hemoglobin level of 9.2 gm/dl. CA-125 was within normal limit and β-HCG was normal. Ultrasonography of the lower abdomen revealed a complex solid cystic mass measuring 82x62x55 mm, with mixed echotexture, compatible with dermoid cyst.

At laparotomy, a cystic mass of approximately 9x7cm size, with a white, smooth glistening surface, originating from the left ovary was observed. Opposite ovary was apparently normal and no adhesion or intra-abdominal deposit was observed. Left sided salphingo-oophorectomy was performed preserving the uterus and right ovary and sent to Department of Pathology, Chittagong Medical College, Chattogram for histopathological evaluation. On gross pathological examination, a cystic mass of 9 cm in diameter (fig-1) with 3cm fallopian tube were noted. On cross section, hair, sebum & fatty materials were come out and some thick greenish fluid was drained and some solid structure was observed on its wall (fig-2).

Microscopic examination revealed mature teratomatous component represented by skin with associated adnexal structures, muscles, fat, benign glands lined by mucin containing columnar epithelium (fig-3,4) and thyroid tissue, within the thyroid tissue foci papillary thyroid carcinoma (fig-5,6) was found. Lining cells had oval nuclei showing nuclear overlapping, grooving and intranuclear cytoplasmic inclusions. Follicles also contained amorphous eosinophilic thick colloid. The fallopian tube was unremarkable. Immunohistochemical (IHC) examination revealed positivity for TTF-1 (fig-8,9). With these findings, diagnosis of a “mature cystic teratoma with malignant transformation to papillary thyroid carcinoma” was made. Postoperatively plasma levels of T₃, T₄, TSH and thyroglobulin of the patient were normal. A normal parenchymal vasculature was identified by postoperative ultrasonography of the thyroid gland.

Discussion

Mature cystic teratomas are also known as dermoid cysts, because they are mostly cystic, skin & skin appendages are the most common structures. Thyroid tissue is present in 10% of the all cases.⁶ Teratomas containing more than 50% of thyroid tissues are called struma ovarii, often presented as monodermal teratoma.⁷ Malignant transformation of MCT is rare; however, several types of malignancy can develop from any one of three germ-cell layers. Squamous cell carcinoma, derived from ectoderm is the commonest type; less common malignancies include soft tissue sarcomas, adenocarcinomas, malignant melanomas, basal cell carcinomas, carcinoid tumors, and thyroid carcinomas.² Among thyroid carcinomas the most common histological type is the papillary carcinoma (44%), other types are follicular carcinoma (30%) and follicular variant of papillary carcinoma (26%).³

 The malignant change of an initially benign cystic teratoma is detected in patients between 40 and 60 years of age, older than its benign counterpart. Although the cancer occurs at any age, most patients are postmenopausal.¹ The tumor may present as pelvic discomfort, with a pelvic mass on abdominal imaging (USG, CT, MRI) or during laparotomy for any other reason. Preoperative definitive diagnosis of stroma ovarii or papillary thyroid carcinoma is not possible. The only possibility of preoperative diagnosis is by radioactive iodine scan (not done routinely).⁸ Various case reports have been published over the past few years regarding the histological diagnoses and treatment options. The diagnoses of thyroid carcinomas arising in teratomas should be made following the guidelines for diagnosing carcinomas in thyroid gland. Disease is treatable with good out come in most cases. Only 7% and 14% of patients with papillary carcinoma and typical follicular carcinoma, respectively died of disease. Due to rarity of disease no consensus on treatment has been made, however treatment options include oophorectomy, additional thyroidectomy, radioactive iodine and long term follow up with serum thyroglobulin measurement.⁹

In order to determine metastatic disease, in MCT cases undergoing malignant transformation, follow up of thyroglobulin (Tg) levels is recommended. The only source of circulating Tg is the thyroid tissue and ovarian teratomas containing thyroid tissue, which is a very rare condition. However, high Tg level in benign thyroid diseases hamper determination of it as a convenient tumour marker in MCT, who did not undergo thyroidectomy and who contain thyroid tissue with malignant transformation. On the other hand, the high levels of anti-thyroglobulin antibody (anti-Tg Ab) may cause Tg levels to be erroneously low. For this reason, the follow-up of Tg levels is favourable for patients, who underwent thyroidectomy only and for patients left with no or very little thyroid tissue. In order to evaluate Tg levels correctly, follow-up of Tg levels together with anti-Tg Ab levels is advisable as persisting high levels of anti-Tg Ab indicate a persistent disease.³ In our case, Plasma T₃, T₄, TSH and Tg level were normal and normal parenchymal vasculature was identified by ultrasonography of the thyroid gland. Anti-Tg Ab level can’t be performed due to patient’s refusal. Logani et al., 2001 was commented the absence of normal thyroid tissue and features of teratoma, in favour of a metastatic lesion originating from thyroid gland.¹⁰ In the presented case, histologic evidence of mature teratoma, normal thyroid tissue along foci of papillary thyroid carcinoma, and positive immunohistochemical stain for thyroid transcription factor-1(TTF-1) indicates primary thyroid carcinoma arising within MCT.

Conclusion

Whether further therapy with total thyroidectomy and radioiodine ablation may be beneficial is unknown. The rarity of MCT cases undergoing PTC transformation impedes the establishment of a protocol for treatment and follow-up. We recommend that a long-term follow-up of these cases is needed to know more about the prognosis and to see any local recurrence or metastasis.

References

1. Rim SY, Kim SM, Choi HS. Malignant transformation of ovarian mature cystic teratoma. Int J Gynecol Cancer. 2006; 16:140–44.
2. Pineyro MM, Pereda J, Schou P, Santos DL, Peña SDI, Caserta B,Pisabarro R. Papillary thyroid microcarcinoma arising within a mature ovarian teratoma: case report and review of the literature. Clinical Medicine Insights: Endocrinology and Diabetes. 2017; 10: 1–3.
3. Cokmez H,Gulbahar A, Yigit S, Aydin C. Oncocytic and tall columnar type papillary thyroid carcinoma arising on a mature cystic teratoma: A case report and literature review. J Pak Med Assoc. 2019; 69:116-19.
4. Tzelepis EG, Barengolts E, Garzon S, Shulan J, Eisenberg Y. Unusual case of malignant strumaovarii and cervical thyroid cancer preceded by ovarian teratoma: case report and review of the literature. Hindawi: Case Reports in Endocrinology. 2019 Mar 17; 1-7.
5. Yeasmin S. A case of papillary thyroid cancer and extraovarian pelvic Teratoma.Journal of the Endocrine Society. 2019; 3(1). available at:https://doi.org/10.1210/js.2019-SUN-603.
6. Bedir R,Yılmaz R. Coexistence of papillary thyroid cancer and hashimoto’sthyroiditis developing within an ovarian mature cystic teratoma. Journal of Mid-life Health. 2019 April 10; 10: 45-47.
7. ParulskaES, Pioch A, Chyrek EC, Wolinski K, Jurczyszyn DJ, Jedynska MJ, Majewski P, Zabel M,Ruchala M. The role of immunohistochemical examination in diagnosis of papillary thyroid cancer in strumaovarii. Folia Histochemica Et Cytobiologica. 2019;57(1):35–42.
8. Naeem M, Iqbal M, Imran MB,Tabassum R. Malignant strumaovarii: a rare case report. European Journal of Medical Case Reports. 2017; 2(1): 30-32.
9. Haider A, Hussain M, Hassan U, Loya A. Papillary thyroid carcinoma arising in ovarian teratomas: A report of three cases. Journal of Islamabad Medical & Dental College (JIMDC). 2015 Sep 27; 4(2): 88-90.
10. Logani S, Baloch ZW, Snyder PJ, Weinstein R, LiVolsi VA. Cystic ovarian metastasis from papillary thyroid carcinoma: A case report. Mary Ann Liebert, Inc. 2001; 11(11): 1073-1075.

 

Histopathological Diagnosis of Rhinofacial Entomophthoramycosis in a 16-Year-Old Girl: A Case Report

*Asaduzzaman,¹ Khandkar T,² Rahman DA³ 

 

1. *Dr. Asaduzzaman, Assistant Professor of Histopathology, Sheikh HasinaNational Institute of Burn and Plastic Surgery, Dhaka, Bangladesh. dr.asad37@gmail.com
2. Tahmina Khandkar, Assistant Registrar, Paediatric Nephrology, National Institute of Kidney Diseases and Urology, Shere-E-Bangla Nagar, Dhaka
3. DM Arifur Rahman, Assistant Professor, Department of Pathology, TMSS Medical College, Bogura

 *For correspondence

 Abstract

Rhinoentomophthoramycosis is an uncommon and severely disfiguring disease. It mainly involves the mucosa of the nares, nasal passages, nasal sinuses, nasopharynx, mouth and spreads to adjacent tissues causing disfigurement of face. Histopathological examinations and mycological cultures are the gold standard for confirmation of entomophthoramycosis. We report a case of a 16-year-old girl who presented with swelling and ulcer of face. Clinical presentation along with typical histopathologic findings were diagnostic in this case.

 [Journal of Histopathology and Cytopathology, 2020 Jan; 4 (1):55-59]

 Keywords: Entomophthoramycosis, zygomycosis, fungal infection of face, fungal granuloma, splendore-Hoeppli reaction

Introduction

Rhinoentomophthoramycosis is not so common in Bangladesh as well as other parts of the world. It is a grossly disfiguring disease. The medically important class zygomycetes are in two orders, the Mucorales and the Entomophthorales. Rhinofacialentomophthoralesmainly affects the mucosa of the nares, nasal passages, nasal sinuses, nasopharynx, mouth, and spreads to adjacent tissues causing disfigurement of the face. It occurs predominantly in immunocompetent individuals and live as saprophytes in soil and decaying plant matter.¹ Rhinofacialconidiobolomycosis affects the subcutaneous tissues of the face, especially the paranasal sinuses as well as the deeper organs.² We report the case of a teen-aged female who presented to us with facial swelling and ulcer and was diagnosed by histopathology.

 Case Report

An immunocompetent16-year-old girl from Chittagong presented to the outpatient department of Sheikh Hasina National Institute of Burn and Plastic Surgery with a one year history of progressive nasal and maxillofacial swelling. Swelling of face started from the nasal bridge and gradually spread into the left side of the face. For the facial swelling she had received multiple treatments, including glucocorticoids and antibiotics. But for the last one month she developed multiple ulcers with purulent discharge over the swelling. The physical examination reveals an ulcer over upper part of left cheek and swelling over bridge of nose. Adjacent area revealed erythema, edema, and tenderness over the nasal dorsum and forehead, extending to the soft tissue around left eyes (Fig. 1). Initially she has undergone biochemical and radiological investigations.

Laboratory investigations included a haemoglobin of 11.6 gm/dl, total leucocyte count 12.36×10⁹/L with a differential count within normal range, and platelet count was 400.1x 10⁹/L. Other biochemical tests were within normal limit. Serology for hepatitis B surface antigen and human immunodeficiency virus 1 and 2 were negative. MRI of face revealed diffuse soft tissue thickening involving the paranasal sinuses, skin and subcutaneous tissue in left side of face extending into the left zygomatoco-temporal region.  Direct naso-endoscopic examination revels left sided middle meatus. Septum and lateral wall of nose were congested. Middle meatus was adherent to the lateral wall of nose. At right side, crest of nose, middle meatus was distorted, nasal septum was absent/dehiscent on posterior part. There was no growth on nasopharynx. Biopsy specimens were obtained from multiple sites, including the forehead and the nose. The gross specimen consisted of two skin covered piece of tissue; largest one measured 4x3x1.5 cm and smaller one measures 1.5×0.8×0.5 cm. Skin surface showed multiple ulcers. The cut surface was solid and tan gray. Histopathological examination showed a chronic granulomatous inflammation (Fig. 2) with broad nonseptate branching hyphae surrounding amorphous eosinophilic substance, the Splendore-Hoeppli reaction (Fig. 3). Marked lympho-plasmacytic cell infiltrate with tissue eosinophilia and foreign body type of giant cells containing fungal elements were present. Periodic acid Schiff and Gomori-Methenamine-Silverstain highlighted the fungal elements and the surrounding amorphous eosinophilic material (Fig. 4 and 5). She was diagnosed as a case of Rhinofacialentomophthoramycosis. The patient was then on systemic antifungal therapy.

Discussion

Rhinofacialentomophthoramycosis is an uncommon fungal infection; it mostly occurs in the tropical and subtropical regions of different parts of the world. G. Bras reported the first case of a Jamaican native in 1965. It is predominantly a chronic mucocutaneous and subcutaneous infection. The name Entomophthorales was coined from the Greek word “Entomon” meaning insect implicating their pathogenic nature in insects. Formerly, the two orders, namely Mucorales and Entomophthorales, were classified in the phylum Zygomycota. Hibbett et al. suggested a comprehensive phylogenetic classification of the kingdom Fungi, and the phylum Zygomycota was eliminated as a result of polyphyletic characteristics.³ Therefore, the taxa belonging to Zygomycota were distributed among the phylum Glomeromycota and four subphyla of uncertain placement (incertaesedis). Entomophthorales and Mucorales as well as two other orders (Kickxellales and Zoopagales) were raised to the rank of subphyla and renamed as Entomophthoromycotina, Mucoromycotina, Kickxellomycotina,and Zoopagomycotina.⁴ Entomophthoromycotina encompasses twogenera that cause human infection, Basidiolus and Conidiolus.

Humans suffering from rhinoentomophthoromycosis get infected by the attachment of conidia of C. coronatus to nasal/sinusoidal mucosa. Initially, the disease presents like sinusitis.⁵ A nodule at the nostrils indicates expansion into the subcutaneous fat.⁶ The infection spreads within the subcutaneous fatty layers of the nasal bridge, eyelids, cheek, and upper lip. Swellings are firm, indolent, and, initially, often reddened and warm, while later they are often itchy.⁷ Mucosal swellings rarely affect laryngeal structures or cause dyspnoea. Ulcerations of skin or mucosa may occurs, as we found in our case. Skin-adherent structures, eye motility, and vision usually remain unaffected; and bones, vessels, muscle, and lymph nodes are rarely involved. The course of the disease is usually benign.⁸

The diagnosis is based on a combination of mycologic and histopathological tests, and clinical presentation.Histological examinations and mycological cultures are the gold standard for confirmation of entomophthoromycosis. Biopsy of skin lesions is preferred for diagnosis than pus, as the chances of positive identification with potassium hydroxide preparation and culture are better with tissue specimens.⁹ Entomophthoromycosis can be easily differentiated from other fungi by their characteristic hyphal morphology. The hyphae are broad, aseptate, or sparsely septate, with right-angle branching.¹⁰ The histological inflammatory reaction shows infiltration with lymphocytes, plasma cells, epithelioid cells, multinucleate giant cells, and histiocytes with an area of central necrosis that is surrounded by eosinophilic infiltration. This phenomenon is called Splendore–Hoeppli phenomenon.¹⁰ Our patient had all these typical features. PAS stain and GomoriMethenamine-Silver (GMS) stains are useful to demonstrate the fungal hyphae. Examination under fluorescent microscopy using fluorescent dye (Blankophor) wet mount preparation increases the sensitivity of diagnosis.¹¹ Definitive diagnosis requires culture, polymerase chain reaction testing, and immunohistochemistry.

Treatment for endomophthoromycosis ismedical and surgical. Systemic antifungal therapy and or surgical debridement is the primary choice in most cases. Several antifungal agents are used for the treatment of endomophthoromycosis such as itraconazole and amphotericin B.¹²

Conclusion

The entomophthoromycosis is a severe fungal disease that can affect both immunocompetent and immunocompromised individuals. Despite the clinical features, the disease requires biopsy for diagnosis, as histological examinations and mycological cultures are the gold standard for confirmation of entomophthoramycosis.This disease have a favorable prognosis if early treatments can be ensured.

 References

1. Manning RJ, Waters SD, Callaghan AA. Saprotrophy of Conidiobolus and Basidiobolus in leaf litter. Mycol Res. 2007; 111: 1437–1449.
2. Prabhu RM, Patel R. Mucormycosis and entomophthoramycosis: A review of the clinical manifestations, diagnosis and treatment. ClinMicrobiol Infect 2004;10 Suppl 1:31-47.
3. Hibbett DS, Binder M, Bischoff JF, Blackwell M, Cannon PF, Eriksson OE, et al. A higher-level phylogenetic classification of the fungi. Mycol Res 2007;111:509-47.
4. Kwon-Chung KJ. Taxonomy of fungi causing mucormycosis and entomophthoramycosis (zygomycosis) and nomenclature of the disease: Molecular mycologic perspectives. Clin Infect Dis 2012;54Suppl 1:S8-15.
5. Choon SE, Kang J, Neafie RC, Ragsdale B, Klassen-Fischer M, Carlson JA. Conidiobolomycosis in a young Malaysian woman showing chronic localized fibrosingleukocytoclasticvasculitis: a case report and meta-analysis focusing on clinicopathologic and therapeutic correlations with outcome. Am J Dermatopathol. 2012; 34: 511–522.
6. Choon SE, Kang J, Neafie RC, Ragsdale B, Klassen-Fischer M, Carlson JA. Conidiobolomycosis in a young Malaysian woman showing chronic localized fibrosingleukocytoclasticvasculitis: a case report and meta-analysis focusing on clinicopathologic and therapeutic correlations with outcome. Am J Dermatopathol. 2012; 34: 511–522.
7. Martinson FD. Chronic Phycomycosis of the Upper Respiratory Tract: RhinophycomycosisEntomophthorae. Am J Trop Med Hyg. 1971; 20: 449–455.
8. Ribes JA, Vanover-Sams CL, Baker DJ. Zygomycetes in Human Disease. ClinMicrobiol Infect. 2000; 13: 236–301.
9. Chowdhary A, Randhawa HS, Khan ZU, Ahmad S, Khanna G, Gupta R, et al.Rhinoentomophthoromycosis due to Conidioboluscoronatus. A case report and an overview of the disease in India. Med Mycol 2010;48:870-9.
10. El-Shabrawi MH, Arnaout H, Madkour L, Kamal NM. Entomophthoromycosis: A challenging emerging disease. Mycoses 2014;57Suppl 3:132-7.
11. Kumar Verma R, Shivaprakash MR, Shanker A, Panda NK. Subcutaneous zygomycosis of the cervicotemporal region: Due to Basidiobolusranarum. Med Mycol Case Rep 2012;1:59-62.
12. Prabhu RM, Patel R. Mucormycosis and entomophthoramycosis: A review of the clinical manifestations, diagnosis and treatment. ClinMicrobiol Infect 2004;10Suppl 1:31-47.