Biomaterial Database

[Scleroderma and porphyria cutanea tarda]. 1995

N Tsukazaki, and S Okada, and H Yoshida

Department of Dermatology, Nagasaki University School of Medicine.

Examination was made of sclerodermoid changes in porphyria cutanea tarda (PCT) and PCT in association with true scleroderma. Although sclerodermoid change in PCT is rare in Japan, it is important in the diagnosis of PCT when there are few other clinical signs characteristic of PCT. In the present case, slight improvement in cutaneous sclerosis was noted following treatment for PCT, but pulmonary changes somewhat intensified. The reason for the association of PCT with progressive systemic sclerosis (PSS) remains obscure. Elevated uroporphyrin due to PCT may possibly increase cutaneous sclerosis in PSS. A diagnosis of PCT should be considered for patients presenting scleroderma, since it is difficult to distinguish sclerodermoid changes from true scleroderma.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D012595	Scleroderma, Systemic	A chronic multi-system disorder of CONNECTIVE TISSUE. It is characterized by SCLEROSIS in the SKIN, the LUNGS, the HEART, the GASTROINTESTINAL TRACT, the KIDNEYS, and the MUSCULOSKELETAL SYSTEM. Other important features include diseased small BLOOD VESSELS and AUTOANTIBODIES. The disorder is named for its most prominent feature (hard skin), and classified into subsets by the extent of skin thickening: LIMITED SCLERODERMA and DIFFUSE SCLERODERMA.	Sclerosis, Systemic,Systemic Scleroderma,Systemic Sclerosis
D012867	Skin	The outer covering of the body that protects it from the environment. It is composed of the DERMIS and the EPIDERMIS.
D017119	Porphyria Cutanea Tarda	An autosomal dominant or acquired porphyria due to a deficiency of UROPORPHYRINOGEN DECARBOXYLASE in the LIVER. It is characterized by photosensitivity and cutaneous lesions with little or no neurologic symptoms. Type I is the acquired form and is strongly associated with liver diseases and hepatic toxicities caused by alcohol or estrogenic steroids. Type II is the familial form.