Coexistence of two types of clinical lesions in childhood-onset mastocytosis

Abstract

The vast majority of mastocytosis appear in childhood, urticaria pigmentosa (UP) and mastocytomas being the most common types. Terms such as "xanthelasmoid mastocytosis", "pseudoxanthomatous mastocytosis" or "nodular mastocytosis" have been introduced in the literature to describe the presence of yellowish papular or nodular lesions. We describe two children with cutaneous mastocytosis showing yellowish lesions in combination with other skin lesions. A 10-year-old girl presented with asymptomatic lesions in her vulva at birth, and developed brownish macules on her trunk years after. An eight-year-old boy presented with multiple yellowish papular lesions on his trunk, neck and limbs coexisting with a few clinically anetodermic lesions. No systemic involvement was found and the skin biopsy confirmed a cutaneous mastocytosis in both cases. The two patients are currently asymptomatic and are being periodically followed up. Mastocytoses may show a variety of clinical lesions, sometimes leading to misdiagnosis. Although there are previous reports, involvement of the mucosae and secondary anetoderma are not common findings in cutaneous mastocytoses. We consider that cutaneous manifestations of mastocytoses compose a clinical spectrum, thus explaining the coexistence of different clinical lesions and the development of uncommon presentations.

Combined central xanthomatous and peripheral macular skin lesions in an infant

“…These most commonly present as macules, papules, plaques, or nodules . Other unusual clinical presentations include telangiectatic and xanthelasmoid lesions . Three cases were reported as xanthomatous small yellow papules that initially involved the vulvar and groin regions and then spread to the rest of the body .…”

“…Other unusual clinical presentations include telangiectatic and xanthelasmoid lesions . Three cases were reported as xanthomatous small yellow papules that initially involved the vulvar and groin regions and then spread to the rest of the body . A combination of these lesions might occur in the same patient .…”

“…Three cases were reported as xanthomatous small yellow papules that initially involved the vulvar and groin regions and then spread to the rest of the body . A combination of these lesions might occur in the same patient . Coexistence of two or more different types in the same patient, particularly with distinct anatomic distribution, might impose diagnostic difficulties for pediatricians and dermatologists …”

“…A combination of these lesions might occur in the same patient . Coexistence of two or more different types in the same patient, particularly with distinct anatomic distribution, might impose diagnostic difficulties for pediatricians and dermatologists …”

“…The cells were focally and moderately positive for CD68, which also stained larger probably histiocytic cells. However, we used the CD68‐KP1 clone, which is less specific for histiocytes than CD68‐PGM1 or CD163, but it can be positive in mast cells . The cells were negative for CD2, MPO, CD1a, and S‐100 protein.…”