Indian Dermatology Online Journal

SKINDIA QUIZ
Year
: 2017  |  Volume : 8  |  Issue : 3  |  Page : 227--228

SkIndia Quiz 37: A persistent plaque in the pubic region


Lalit Kumar Gupta, Ranjana Beniwal, Ashok Kumar Khare, Asit Mittal, Sharad Mehta, Manisha Balai 
 Department of Dermatology, Venereology and Leprology, RNT Medical College, Udaipur, Rajasthan, India

Correspondence Address:
Lalit Kumar Gupta
Department of Dermatology, Venereology and Leprology, RNT Medical College, Udaipur - 313 001, Rajasthan
India




How to cite this article:
Gupta LK, Beniwal R, Khare AK, Mittal A, Mehta S, Balai M. SkIndia Quiz 37: A persistent plaque in the pubic region.Indian Dermatol Online J 2017;8:227-228


How to cite this URL:
Gupta LK, Beniwal R, Khare AK, Mittal A, Mehta S, Balai M. SkIndia Quiz 37: A persistent plaque in the pubic region. Indian Dermatol Online J [serial online] 2017 [cited 2022 Jan 25 ];8:227-228
Available from: https://www.idoj.in/text.asp?2017/8/3/227/202362


Full Text

A 70-year-old man presented with an itchy eczematous well-defined plaque of size 10 × 7 cm involving the pubic region, penis, and scrotum. It was eroded at places and covered with grayish-white scales and crusts [Figure 1]. The lesion had persisted almost unchanged for 15 years despite treatment. Skin biopsy stained with hematoxylin and eosin stain showed characteristic features [Figure 2]a and [Figure 2]b. Special stain with mucicarmine was positive [Figure 3]. Immunohistochemistry markers were positive for CK7 and negative for CK20 and CEA [Figure 4]. Whole body positron emission tomography scan did not reveal any abnormality.{Figure 1}{Figure 2}{Figure 3}{Figure 4}

 Question



What is your diagnosis?

Histology showed circumscribed collections of large cells with abundant pale-staining cytoplasm and pleomorphic nuclei in the epidermis [Figure 2]. Some of the cells had a “signet ring” appearance. Staining with mucicarmine was positive [Figure 3]. On immunohistochemistry [Figure 4], these cells were positive for cytokeratin 7(CK7) but negative for cytokeratin 20(CK20) and carcinoembryonic antigen (CEA).

 View Answer

 Answer



Extramammary Paget's disease.

 Discussion



Extramammary Paget's disease (EMPD) is a rare neoplasm of the apocrine gland bearing skin that presents as a slowly spreading, erythematous or leukoplakic, marginated plaque. The lesion can be eczematous, crusted, scaly, or occasionally ulcerated.[1] The hallmark of EMPD is its relentless progression despite topical therapy.

EMPD encompasses two different forms: Primary and secondary. The primary EMPD is initially limited to the epithelium and is not associated with distant adenocarcinoma. The secondary form is associated with an underlying adenocarcinoma from dermal adnexal glands or contiguous epithelium, usually of genitourinary or gastrointestinal tract.[2]

Diagnosis of EMPD rests on histological identification of Paget's cells that show strong cytoplasmic staining for Periodic acid-Schiff (PAS), mucicarmine and alcian blue. They also react positively with anti-cytokeratin (CAM 5.2), epithelial membrane antigen (EMA), cytokeratin 7 (CK7), CEA, and gross cystic disease fluid protein-15 (GCDFP-15).[3]

Wide surgical excision or Moh's micrographic surgery is the treatment of choice for EMPD. Topical treatment with imiquimod, bleomycin or 5-flurouracil, photodynamic therapy, radiotherapy, and laser therapy have been tried.[4] There is a need for long-term follow-up to exclude recurrence of disease or development of an associated malignancy.

Learning points

Any long-standing eczematous plaque in the apocrine gland–bearing areas that defies conventional treatment should arouse the suspicion of EMPDEarly recognition followed by biopsy and histological confirmation are crucial for timely surgical interventionThe patient should be investigated for underlying malignancies of the gastrointestinal and genitourinary tractHistology is quite characteristic and shows cells with abundant pale-staining cytoplasm and pleomorphic nuclei (signet ring cells).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Rao VR, Henry DH. Extramammary Paget's disease. Commun Oncol 2004;1:109-14.
2Isrow D, Oregel KZ, Cortes J, Gomez H, Milikowski C, Feun L, et al. Advanced extramammary Paget's disease of the groin, penis and scrotum. Clin Med Insights Oncol 2014;8:87-90.
3Chandawarkar RY, Ricchuti D, Amjad I, Marisco RE Jr, Wells MD. Extramammary Paget's disease of the perineum: Avoiding pitfalls in diagnosis and management. Can J Plast Surg 2003;11:205-8.
4Reddy IS, Swain M, Gowrishankar S, Murthy DB. Primary extramammary Paget's disease with extensive skeletal metastases. Indian J Dermatol Venereol Leprol 2012;78:89-92.