The treatment of sclerosing skin diseases [systemic sclerosis, localized scleroderma, lichen sclerosus et atrophicus, sclerodermoid graft-vs.-host disease, scleredema adultorum (Buschke), scleromyxedema and necrobiosis lipoidica] is difficult and remains a great challenge. Numerous treatments, some with potentially hazardous side effects, are currently used with only limited success. The introd...

Scleredema adultorum or Buschke's scleredema is a rare disorder that belongs to the group of mucinoses. Diffuse, sudden swelling, hardening and induration of the skin can occur in children and younger women as well as in older men. The dermis is thickened because of the increased collagen glycosylation, like that in diabetic stiff skin syndrome. The face is most often involved. In older persons...

Lichen myxedematosus, or papular mucinosis, is a skin disorder characterized by accumulation of mucin in the dermis. We present the case of a patient with HIV infection and lichen myxedematosus whose skin condition completely resolved with antiretroviral therapy.

Papular elastorrhexis is a rare cutaneous disorder that occurs predominantly during adolescence. The disorder is characterized by asymptomatic indurated white or flesh papules showing decreased and fragmented elastic fibers in the dermis. We herein report on a 12-year-old girl with multiple asymptomatic white, nonfollicular, firm papules scattered on the extremities and trunk. Histological exam...

Xanthomatous skin lesions and arthritis in children are not a common association. We present the case of a 3 year old girl who presented with xanthomatous lesions in the periungual region of both hands, around the nares and on her forehead, associated with significant arthritis that was clinically compatible with multicentric reticulohistiocytosis. However, pathology of the xanthomatous lesions...

Papular mucinosis or lichen myxedematosus is an idiopathic disorder characterized by papules, nodules or plaques caused by mucin deposition in the dermis, in the absence of thyroid disease. The present report describes an atypical case with exuberant lesions in the form of plaques, associated with subclinical hypothyroidism. In cutaneous mucinosis, there is an abnormal quantity of mucin in the ...

Systemic sclerosis (SSc) is characterized by vascular abnormalities, fi brosis, infl ammatory changes, and late stage tissue atrophy of the skin and several internal organs. In scleroderma-like disorders the distribution/characteristics of skin involvement is different. The skin involvement of the acral regions including digits is usually missing. Lack of Raynaud’s phenomenon, and scleroderma-s...