Segmental Juvenile Xanthogranuloma

Abstract

A 5-month-old boy had erythematous nodules over the left side of his trunk with a segmental arrangement since birth. Histopathologic examination revealed sheets of foamy histiocytes infiltrating the dermis and subcutaneous fat, admixed with multinucleated giant cells and lymphocytes, making this an unusual case of juvenile xanthogranuloma appearing in a segmental distribution.

Multiple clustered dermatofibroma presenting in a segmental distribution

“…Our patient adds to the growing literature of benign tumours that can appear in a segmental distribution, including segmental cutaneous leiomyomatosis, segmental juvenile xanthogranulomas, segmental melanocytic naevi and segmental neurofibromatosis . In autosomal dominant conditions segmental manifestation has been reported in conditions like Hailey‐Hailey disease and neurofibromatosis .…”

Congenital‐type juvenile xanthogranuloma: A case series and literature review

“…The two most common complications of congenital JXG were ulceration and atrophic scarring. Large exophytic tumors appear to be most at risk of ulceration . Multiple cases of congenital JXG were also found to regress with atrophic scars and hyperpigmentation .…”

“…The most common clinical morphology was a firm, well-circumscribed tumor that increased in size over the first few months of life and had a yellow to erythematous color. Less common morphologies included exophytic tumors (6 cases), 3,6,10,15,25 agminated juvenile xanthogranulomas occurring on an erythematous patch or plaque (4 cases) 8,18,21,22 (Figure 4), large infiltrative plaques (5 cases) 11,17,19,24,28 (Figure 5), and subcutaneous masses without significant overlying skin change (4 cases). 2,9,13,26 Nine percent of the patients catalogued (3/33) had multiple cutaneous congenital lesions.…”

“…Large exophytic tumors appear to be most at risk of ulceration. 6,[13][14][15]19,22,23 Multiple cases of congenital JXG were also found to regress with atrophic scars and hyperpigmentation. 4,23,48 Lastly, infiltration into subcutaneous fat, fascia, and muscle was documented in nine cases using histopathology or imaging.…”

“…We excluded non‐English journal articles, cases without congenital onset, cases without histopathologic confirmation, and cases of systemic JXG without cutaneous congenital JXGs. In total, we identified 31 cases of congenital JXG involving the skin only and 16 cases of congenital JXG with systemic involvement …”