An 11-year-old boy presented with a history of a red swollen scaly penis, scrotum and anus for more than 1 year’s duration. His genitals are shown in Image 121. He denied any further symptoms and kept this ‘hidden’ from his parents. His family history was positive for psoriasis. Topical steroids and topical immune modulators were tried and had little to no effect. Due to the persistence of symptoms and the further development of oedema, a skin biopsy was performed which showed many granulomas. He was referred to a colleague for confirmation of the suspected diagnosis.
What is the diagnosis?
How was the referral and second biopsy helpful in making the diagnosis?https://s3-euw1-ap-pe-df-pch-content-public-u.s3.eu-west-1.amazonaws.com/9780429170423/236ca68c-f283-4df8-88b2-3b8ff49bccc9/content/fig121.jpg" xmlns:xlink="https://www.w3.org/1999/xlink"/>
This child has granulomatous lymphangitis (a variant of cutaneous Crohn’s Disease).
The child was referred to a gastroenterologist. An oesophagogastroduodenoscopy and a colonoscopy with biopsies were performed which revealed no evidence of active ileitis or colitis. However rectal biopsies revealed isolated microgranulomas. Laboratory studies showed high anti-Saccharomyces cerevisiae antibodies (ASCA). Tuberculin test and chest x-ray were normal. The gastroenterologist’s conclusion was metastatic (cutaneous) Crohn’s Disease (CD). The penile and scrotal swelling improved dramatically within a 9-month period of treatment. The child continued to feel well without any gastrointestinal symptoms related to CD. 1 In some cases, a rare condition such as CD may lead to concerns of child abuse. It is essential to recognize these diseases and refer to the appropriate specialist in order not to misdiagnose or delay required treatment.