Photo Quiz

Multiple Vesicles and Bullae on the Scrotum

 


FREE PREVIEW. AAFP members and paid subscribers: Log in to get free access. All others: Purchase online access.


FREE PREVIEW. Purchase online access to read the full version of this article.

Am Fam Physician. 2013 May 1;87(9):645-646.

A 64-year-old man presented with lesions on the right side of his scrotum (Figure 1); they had been present for nearly 20 years. The patient reported no pain, oozing, or itching, and he had no history of trauma, injury, radiotherapy, surgery, or venereal infection.


Figure 1.

Examination revealed multiple f luid-filled, translucent vesicles and bullae. The lesions were intact and were not fragile. A shave biopsy was obtained.

Question

Based on the patient's history and physical examination findings, which one of the following is the most likely diagnosis?

A. Angiokeratomas of Fordyce.

B. Idiopathic scrotal calcinosis.

C. Leukemia cutis.

D. Lymphangioma circumscriptum.

E. Steatocystoma multiplex.

Discussion

The answer is D: lymphangioma circumscriptum. Shave biopsy showed slight acanthosis in the epidermis and multiple dilated lymphatic vessels in the papillary dermis (Figure 2).

View/Print Figure

Figure 2.

Shave biopsy of lymphangioma circumscriptum lesions showed slight acanthosis in the epidermis and multiple dilated lymphatic vessels in the papillary dermis.


Figure 2.

Shave biopsy of lymphangioma circumscriptum lesions showed slight acanthosis in the epidermis and multiple dilated lymphatic vessels in the papillary dermis.

Lymphangiomas can be classified into three main types: cavernous, circumscriptum (capillary), and cystic.1 Lymphangioma circumscriptum, the most common type, is the benign dilatation and malformation of inner lymphatic channels located in the deep dermal and subcutaneous tissues.1,2 The disease often affects the proximal portions of the extremities, but scrotal, vulvar, and mucosal lesions are rare. Lymphangioma circumscriptum is more common in women than in men. It can occur at any age, but usually appears at or soon after birth.1

The clinical presentation is characterized by thin-walled vesicles and bullae, which are filled with translucent lymphatic fluid. The lesions range from 1 to 5 mm in size. Histopathologic features include numerous cystic lymphatic spaces lined with a flattened endothelium in the dermis. There may be overlying hyperkeratosis, acanthosis, hemorrhage, and mononuclear inflammatory cells.1,3

Angiokeratomas of Fordyce are well-circumscribed, vascular lesions characterized by red or blue papules (i.e., dilated vessels). They are commonly associated with severe varicosity or varicocele. Patients often report bleeding after sexual intercourse or excoriation of the lesions.4

Idiopathic scrotal calcinosis is a rare, benign condition that causes hard, yellowish nodules. The dermal nodules consist of calcium deposits under a normal epidermis. They are usually asymptomatic, but may itch or produce discharge.5

Leukemia cutis is the infiltration of neoplastic leukocytes into the skin, causing clinically identifiable cutaneous lesions. It is associated with a poor prognosis. The lesions consist of papules, but mostly nodules that are usually asymptomatic and typically seen with the nodular morphology. Leukemia cutis can be accompanied by hepatosplenomegaly or a high serum leukocyte count. Histopathology shows diffuse, band-like or nodular infiltration of leukemic cells.6

Steatocystoma multiplex is characterized by multiple dermal cysts involving the pilosebaceous units. The dermal cysts may be localized or widespread, and asymptomatic or inflammatory. Histopathology shows well-encapsulated cysts with cyst walls.7

View/Print Table

Summary Table

ConditionCharacteristics

Angiokeratomas of Fordyce

Red or blue papules; bleeding after sexual intercourse or excoriation of the lesions; associated with severe varicosity or varicocele

Idiopathic scrotal calcinosis

Hard, yellowish dermal nodules consisting of calcium deposits under a normal epidermis; usually asymptomatic, but may itch or produce discharge

Leukemia cutis

Papules, but mostly nodules; accompanied by hepatosplenomegaly or a high serum leukocyte count; histopathology shows diffuse, band-like or nodular infiltration of leukemic cells

Lymphangioma circumscriptum

Thin-walled vesicles and bullae filled with translucent lymphatic fluid; histopathology shows numerous cystic lymphatic spaces lined with a flattened endothelium in the dermis

Steatocystoma multiplex

Multiple dermal cysts that are localized or widespread; asymptomatic or inflammatory; histopathology shows well-encapsulated cysts with cyst walls

Summary Table

ConditionCharacteristics

Angiokeratomas of Fordyce

Red or blue papules; bleeding after sexual intercourse or excoriation of the lesions; associated with severe varicosity or varicocele

Idiopathic scrotal calcinosis

Hard, yellowish dermal nodules consisting of calcium deposits under a normal epidermis; usually asymptomatic, but may itch or produce discharge

Leukemia cutis

Papules, but mostly nodules; accompanied by hepatosplenomegaly or a high serum leukocyte count; histopathology shows diffuse, band-like or nodular infiltration of leukemic cells

Lymphangioma circumscriptum

Thin-walled vesicles and bullae filled with translucent lymphatic fluid; histopathology shows numerous cystic lymphatic spaces lined with a flattened endothelium in the dermis

Steatocystoma multiplex

Multiple dermal cysts that are localized or widespread; asymptomatic or inflammatory; histopathology shows well-encapsulated cysts with cyst walls

Address correspondence to Engin Senel, MD, at enginsenel@enginsenel.com. Reprints are not available from the authors.

Author disclosure: No relevant financial affiliations.

REFERENCES

show all references

1. Patel GA, Schwartz RA. Cutaneous lymphangioma circumscriptum: frog spawn on the skin. Int J Dermatol. 2009;48(12):1290–1295....

2. Sims SM, McLean FW, Davis JD, Morgan LS, Wilkinson EJ. Vulvar lymphangioma circumscriptum: a report of 3 cases, 2 associated with vulvar carcinoma and 1 with hidradenitis suppurativa. J Low Genit Tract Dis. 2010;14(3):234–237.

3. Turan V, Ergenoglu M, Yeniel O, Ulukus M. Vulvar lymphangioma circumscriptum. Int J Gynaecol Obstet. 2009;107(3):256–257.

4. Trickett R, Dowd H. Angiokeratoma of the scrotum: a case of scrotal bleeding. Emerg Med J. 2006;23(10):e57.

5. Khallouk A, Yazami OE, Mellas S, Tazi MF, El Fassi J, Farih MH. Idiopathic scrotal calcinosis: a non-elucidated pathogenesis and its surgical treatment. Rev Urol. 2011;13(2):95–97.

6. Malbora B, Senel E, Avci Z, Ozbek N. Purpuric nodules and macules on the scalp of an 18-month-old boy [published correction appears in Skinmed. 2011;9(1):66]. Skinmed. 2010;8(5):305–306.

7. Senel E. Question: can you identify this condition? Steatocystoma multiplex. Can Fam Physician. 2010;56(7):667.

Contributing editor for Photo Quiz is John E. Delzell, Jr., MD, MSPH.

A collection of Photo Quizzes published in AFP is available at http://www.aafp.org/afp/photoquiz.

The editors of AFP welcome submissions for Photo Quiz. Guidelines for preparing and submitting a Photo Quiz manuscript can be found in the Authors' Guide at http://www.aafp.org/afp/photoquizinfo. To be considered for publication, submissions must meet these guidelines. E-mail submissions to afpphoto@aafp.org.



 

Copyright © 2013 by the American Academy of Family Physicians.
This content is owned by the AAFP. A person viewing it online may make one printout of the material and may use that printout only for his or her personal, non-commercial reference. This material may not otherwise be downloaded, copied, printed, stored, transmitted or reproduced in any medium, whether now known or later invented, except as authorized in writing by the AAFP. Contact afpserv@aafp.org for copyright questions and/or permission requests.

Want to use this article elsewhere? Get Permissions


More in AFP


Editor's Collections


Related Content


More in Pubmed

MOST RECENT ISSUE


Sep 15, 2016

Access the latest issue of American Family Physician

Read the Issue


Email Alerts

Don't miss a single issue. Sign up for the free AFP email table of contents.

Sign Up Now

Navigate this Article