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Progressive nodular histiocytosis (PNH) is a rare condition characterized by progressive eruption of multiple yellowish-brown papules and nodules on the skin and mucous membranes. We present the case of a 37-year-old Caucasian man with gradually increased appearance of nodular lesions on the forehead and right temple. These lesions were initially diagnosed as xanthomas and did not respond to intralesional injections of triamcinolone. Additional biopsy revealed an intense dermal infiltrate of foamy mononuclear epithelioid cells with a minor admixture of plasma cells, lymphocytes, and scattered multinucleated giant cells. On immunohistochemical staining, the lesional cells were positive for CD163 and CD68 and negative for CD1a, thus confirming a mononuclear-macrophage lineage. The clinical presentation and the histological impression lead to the diagnosis of PNH. This condition could be challenging, mimicking microscopically similar lesions of the non-Langerhans cell histiocytosis group. Although uncommon, PNH stands out due to its clinical and microscopic features and should be taken into consideration in the differential diagnosis of cutaneous histiocytoses.

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Infantile hemangioma can rarely have systemic involvement with grim consequences. We present a case of infantile hemangioma with extensive cutaneous and multiple internal organs components that was so unrelenting and led to early infant death. The patient was a nearly 3-week-old Caucasian girl born at 37 weeks by spontaneous vaginal delivery. The pregnancy was complicated by "toxemia." At birth, cutaneous hemangiomas were noted on the neonate. The lesions progressively enlarged, and she later was brought to the emergency department with progressive abdominal distention at 17 days of age. An abdominal CT scan showed hepatomegaly with hypervascular masses, and the presumptive diagnosis was diffuse infantile hepatic hemangioma. In the PICU, abdominal compartment syndrome developed, and the neonate was also noted to have severe hypothyroidism. She was managed with propranolol, high-dose levothyroxine, steroids, and emergency decompressive fasciotomy and other support measures. Despite that, DIC and multiorgan failure ensued, and she eventually died of the disease within 4 days. An autopsy examination showed a female infant with multiple well-circumscribed, dark purple, cutaneous lesions on the face, scalp, torso, and extremities; she had an enlarged liver (413 g; expected weight, 123 g) occupying almost the entire abdominal cavity and containing multiple large, dark red, soft lesions measuring up to several centimeters in diameter and displacing almost the entire liver; similar lesions were also present in both adrenal glands. Microscopically, all of these lesions consisted of irregular, well-formed anastomosing vascular channels lined by a single row of plump endothelial cells characteristic of infantile hemangioma, and immunohistochemically, the lesions were remarkable for strong CD34 and GLUT1 staining. Hepatic infantile hemangioma is perhaps the most dreadful component of infantile hemangioma, especially the so-called diffuse type as occurred in this case. It is often accompanied by abdominal compartment syndrome and severe hypothyroidism caused by type 3 iodothyronine deiodinase secretion by the tumor. For this special clinical scenario, the recommended clinical algorithm calls for steroids and extremely high dose of levothyroxine for management and preparation for organ transplantation in the face of continuous deterioration.