Papular Xanthoma Associated with Angiokeratoma of Fordyce: Considerations on the Nosography of This Rare Non-Langerhans Cell Histiocytoxanthomatosis

Abstract

Background: Papular xanthoma (PX) is a rare normolipidemic non-Langerhans cell histiocytoxanthomatosis affecting both children and adults. Objective: We describe an adult case of PX associated with angiokeratoma of Fordyce and review the literature in order to compare and discuss previous reports. Methods: We studied the clinical, histopathological, immunocytochemical and ultrastructural findings. Results: We report the findings of our case and compare our case with those described in the literature. Conclusions: Three clinical patterns of PX appeared to emerge in the review of the literature: a self-healing form, a persistent form and a progressive form. The progressive form of PX can be considered the same clinical entity that is also described as progressive nodular histiocytosis.

Disseminated asymptomatic yellowish papules on the face, trunk and limbs in a 3-year-old boy

“…[2,3] Ultrastructural study reveals no Birbeck granules or laminated bodies. The results of both immunostaining and ultrastructural study indicate foamy cells of non-Langerhans histiocytic origin.…”

“…[1] The diagnostic criteria in the literature include: (1) generalized asymptomatic yellowish papulonodular lesions that do not merge into plaques or involve flexural areas, (2) no visceral involvement, (3) normal lipid profile, (4) infiltrate of foamy cells with negative immunostaining for S-100 and CD1a, and (5) no primitive histiocytic phase or infiltrate of other inflammatory cells. [1,2] Initially, all cases of PX reported were adults and the lesions did not resolve spontaneously. In 1990, Caputo et al observed that the skin lesions in 10 children presented similar clinical and histopathologic features to those reported for adults, but with a self-healing course within 1-5 years, [3] where the healed lesions might leave transient hyperpigmentation and anetoderma-like scars.…”

“…In 1990, Caputo et al observed that the skin lesions in 10 children presented similar clinical and histopathologic features to those reported for adults, but with a self-healing course within 1-5 years, [3] where the healed lesions might leave transient hyperpigmentation and anetoderma-like scars. [2,3] Clinically, PX is manifested by asymptomatic, widespread yellowish 2-10 mm papulonodules on the head, face, extremities and trunk, with sparing of flexural areas, palms and soles, that do not show any tendency to merge into plaques. [1][2][3] The histological feature is a diffuse dermal infiltrate of xanthomatized histiocytes characterized by central nuclei with surrounding foamy cytoplasm with no other inflammatory cells such as lymphocytes, plasma cells or eosinophils.…”

“…[2,3] Clinically, PX is manifested by asymptomatic, widespread yellowish 2-10 mm papulonodules on the head, face, extremities and trunk, with sparing of flexural areas, palms and soles, that do not show any tendency to merge into plaques. [1][2][3] The histological feature is a diffuse dermal infiltrate of xanthomatized histiocytes characterized by central nuclei with surrounding foamy cytoplasm with no other inflammatory cells such as lymphocytes, plasma cells or eosinophils.…”

A Case of Xanthoma Disseminatum with Spontaneous Resolution over 10 Years: Review of the Literature on Long-Term Follow-Up

“…The lesions of XD may require differentiation from those of Langerhans cell histiocytosis, juvenile xanthogranuloma, papular xanthoma, generalized eruptive histiocytosis, progressive nodular histiocytosis, multicentric reticulohistiocytosis, and eruptive xanthoma [26,27]. Differential diagnoses of XD are listed in table 2.…”