Granulomatous Lymphangitis

Background

This is a rare disease characterized by persistence of scrotal swelling and presence of granulomatous inflammation. It may be associated with concomitant or subsequent development of Crohn’s disease.

OUTLINE

Epidemiology

Disease Associations

Pathogenesis

Laboratory/Radiologic/
Other Diagnostic Testing

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Special Stains/
Immunohistochemistry/
Electron Microscopy

Differential Diagnosis

Prognosis

Treatment

Commonly Used Terms

Internet Links

HISTOLOGICAL TYPES CHARACTERIZATION

General

VARIANTS

Granulomatous lymphangitis of the scrotum and penis Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation

Michael J. Murphy1, Barry Kogan2 and J. Andrew Carlson3 1

Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut, USA,2 Division of Urology, Albany Medical College, Albany, New York, USA,3 Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, New York, USA

Journal of Cutaneous Pathology 2001;28 (8), 419-424 Abstract quote

Background: Acquired lymphedema of the genitalia is a rare childhood presentation and is more common in elderly individuals secondary to pelvic/abdomenal malignancy or its therapy or worldwide due to filariasis.

Objective: Herein, we report a case of a healthy 11-year-old boy who presented with a 1-year history of chronic, asymptomatic scrotal and penile swelling.

Biopsy revealed edema, lymphangiectases and peri- and intralymphatic sarcoidal type granulomas. This histologic pattern of granulomatous lymphangitis is most commonly associated with orofacial granulomatosis (granulomatous cheilitis and Melkersson-Rosenthal syndrome) and Crohn’s disease. Treatment with topical steroids and physical support has resulted in marked improvement. No systemic disease (Crohn’s disease) is evident 1 year later. Literature review revealed 44 cases of genital lymphedema with non-infectious granulomas. The majority of these young patients had Crohn’s disease, frequently with anal involvement and a minority, both with and without Crohn’s disease, had orofacial granulomatosis.

Conclusions: Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Further clinical examination, additional laboratory studies and close follow-up for co-existing or subsequent development of Crohn’s disease should be performed. The overlap between granulomatous lymphangitis of the genitalia, Crohn’s disease and orofacial granulomatosis suggest that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn’s disease.

Henry JB. Clinical Diagnosis and Management by Laboratory Methods. Twentieth Edition. WB Saunders. 2001.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fourth Edition. Mosby 2001.

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Last Updated June 16, 2005

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Epidemiology
Disease Associations
Pathogenesis
Laboratory/Radiologic/ Other Diagnostic Testing
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Special Stains/ Immunohistochemistry/ Electron Microscopy
Differential Diagnosis
Prognosis
Treatment
Commonly Used Terms
Internet Links

HISTOLOGICAL TYPES	CHARACTERIZATION
General
VARIANTS
Granulomatous lymphangitis of the scrotum and penis Report of a case and review of the literature of genital swelling with sarcoidal granulomatous inflammation Michael J. Murphy1, Barry Kogan2 and J. Andrew Carlson3 1 Department of Dermatology, University of Connecticut Health Center, Farmington, Connecticut, USA,2 Division of Urology, Albany Medical College, Albany, New York, USA,3 Divisions of Dermatology and Dermatopathology, Albany Medical College, Albany, New York, USA	Journal of Cutaneous Pathology 2001;28 (8), 419-424 Abstract quote Background: Acquired lymphedema of the genitalia is a rare childhood presentation and is more common in elderly individuals secondary to pelvic/abdomenal malignancy or its therapy or worldwide due to filariasis. Objective: Herein, we report a case of a healthy 11-year-old boy who presented with a 1-year history of chronic, asymptomatic scrotal and penile swelling. Biopsy revealed edema, lymphangiectases and peri- and intralymphatic sarcoidal type granulomas. This histologic pattern of granulomatous lymphangitis is most commonly associated with orofacial granulomatosis (granulomatous cheilitis and Melkersson-Rosenthal syndrome) and Crohn’s disease. Treatment with topical steroids and physical support has resulted in marked improvement. No systemic disease (Crohn’s disease) is evident 1 year later. Literature review revealed 44 cases of genital lymphedema with non-infectious granulomas. The majority of these young patients had Crohn’s disease, frequently with anal involvement and a minority, both with and without Crohn’s disease, had orofacial granulomatosis. Conclusions: Granulomatous lymphangitis should be considered in the differential diagnosis of chronic idiopathic swelling of the genitalia, particularly in younger individuals. Further clinical examination, additional laboratory studies and close follow-up for co-existing or subsequent development of Crohn’s disease should be performed. The overlap between granulomatous lymphangitis of the genitalia, Crohn’s disease and orofacial granulomatosis suggest that granulomatous lymphangitis of the genitalia may represent a forme fruste of Crohn’s disease.