Relapsing Polychondritis – Skin Disorders

 Patients with relapsing polychondritis usually present with dramatically swollen, painful, red ears. Sometimes, the nose is involved in a similar manner. These skin changes reflect the presence of massive inflammatory changes in the underlying cartilage. Such changes occurring in the larynx, trachea, and bronchi may result in death. Ocular and articular abnormalities may also be present.

Reiter’s Syndrome

This disease occurs almost entirely in men. The cutaneous lesions of Reiter’s syndrome are essentially identical with those that occur in psoriasis. Pustular plaques on the palms and soles, together with small red scaling plaques on the glans penis, are characteristically present. Ocular inflammatory disease, sacroiliitis, and arthritis of the spine and larger peripheral joints complete the syndrome. Most patients with Reiter’s disease have an HLA haplotype that includes B27. Episodes of disease activity may be triggered by a variety of infectious etiologies.

Behcet’s Syndrome

Behcet’s syndrome is characterized by the presence of aphthous-like ulcers of the mouth and genitalia. These changes are usually accompanied by arthritis, uveitis, and a variety of neurologic changes. Thrombophlebitis, gastrointestinal involvement, erythema nodosum, and a peculiar pustular response to skin trauma (pathergy) are occasionally present.

Sarcoidosis

Sarcoidosis is characterized by the presence of noninfectious, noncaseating granulomas of many organs, including the skin. The most distinctive skin lesions consist of clusters of small, nonscaling, violaceous to skin-colored, dome-shaped papules. These papules are most often found on the face and neck. Coalescence of the papules to form annular plaques is frequently noted. Larger nodules and plaques are sometimes found on the trunk and extremities. Involvement of cutaneous scar tissue with sarcoidal granulomas is highly characteristic. Erythema nodosum of the lower legs frequently accompanies active lesions of sarcoid occurring in the lungs. Biopsy of the skin lesions (except those of erythema nodosum) reveals the characteristic granulomas and allows confirmation of diagnosis to be made on clinical examination. Recently, a mycobacterial origin for sarcoidosis has been postulated.

Acquired Immuno dificiency Syndrome (AIDS)

Several types of skin lesions are found in AIDS and, in fact, are often of help in making this diagnosis. The lesions of Kaposi sarcoma occur in about 25% of the patients. They consist of red to violaceous, smooth-surfaced papules and nodules. Lesions may be found anywhere on the body, but the most frequent distribution includes the face, arms, trunk, and mucous membranes. The lesions of AIDS-related Kaposi’s sarcoma differ from those of classical Kaposi’s sarcoma in that the former are smaller in size, are violaceous rather than blue-black, and are distributed on the upper half of the body instead of the legs. Less characteristic findings in AIDS include oral candidiasis, oral hairy leukoplakia seborrheic dermatitis of the face, minute pruritic erythematous papules and pustules of the trunk, and various other types of cutaneous opportunistic infections.

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By Robin Kumar Lim

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