Abstract:

A case of the H syndrome was reported. An 18-year-old man presented with an 8-year history of progressive cutaneous sclerosis, skin hyperpigmentation, and hypertrichosis on the trunk and extremities. In addition, he suffered from short stature, hearing loss, cardiac anomaly, hepatosplenomegaly, and scrotal masses. Laboratory examination revealed hypogonadism. Cutaneous histopathologic examination showed hyperpigmentation of the basal layer with acanthosis, widespread fibrosis （moderate in dermis and severe in subcutis） and an infiltrate composed of histiocytes and plasma cells. A perivascular lymphocyte infiltrate was also observed throughout the dermis and subcutaneous fat tissue. These findings were consistent with the H syndrome. Differential diagnosis must be made with Winchester syndrome and polyneuropathy, organomegaly, endocrinopathy, M-protein and skin change （POEMS） syndrome.

Key words: POEMS syndrome