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PubMed1986

The cutaneous mucinoses.

The cutaneous mucinoses are a group of connective tissue disorders characterized by the deposition of mucin, either focally or diffusely, in the interstices of the dermis. The diseases may be a primary (metabolic) or secondary (catabolic) process. Systemic abnormalities are seen with most of these disorders. This review discusses the primary mucinoses in which the predominant dermal mucin is hyaluronic acid. Current therapy and proposed mechanisms for the mucinoses are considered.

Age Factors↗
PubMed1987

Ichthyosiform sarcoidosis. Report of two cases and a review of the literature.

Although acquired ichthyosis has been associated with a number of systemic illnesses, an association with sarcoidosis has rarely been reported. We report two patients with acquired ichthyosis of the lower extremities whose diagnosis of cutaneous sarcoidosis was established by histologic examination. Systemic involvement in both patients included ocular and pulmonary disease. A diagnosis of sarcoidosis must be considered when a patient presents with acquired ichthyosis.

Adult↗
PubMed1988

Cutaneous infection with Mycobacterium avium-intracellulare scrofulaceum intermediate: a new pathogenic entity.

The atypical or environmental mycobacterial organisms constitute a large group of potential agents in the pathogenesis of cutaneous infection. The most commonly encountered mycobacterial infection after contact with water is Mycobacterium marinum. We report an unusual case of cutaneous mycobacterial infection caused by a newly described opportunistic mycobacteria, M. avium-intracellulare scrofulaceum intermediate. This case emphasizes the difficulty in distinguishing among the species M. avium-intracellulare, M. scrofulaceum, and M. gordonae.

Adult↗
PubMed1988

Adult celiac disease, small and medium vessel cutaneous necrotizing vasculitis, and T cell lymphoma.

Cutaneous necrotizing vasculitis of small- and medium-sized vessels developed in a patient with adult celiac disease who later was found to have visceral lymphoma with erythrophagocytosis. Immunologic and genetic probe studies showed the lymphoma to be of T cell differentiation. Celiac disease and T cell lymphoma have been associated previously with the development of cutaneous vasculitis.

Aged↗
PubMed1989

Cutis marmorata telangiectatica congenita: report of 22 cases.

Twenty-two cases of cutis marmorata telangiectatica congenita were evaluated during an 8-year period. All but two patients were examined in the first year of life; 14 of the 22 (64%) were female infants. Four patients had focal cutaneous atrophy associated with the reticulated vascular pattern, and eight had ulcerations of involved skin. Six (27%) had additional anomalies. Of these, three patients had a nevus flammeus, and one had congenital generalized fibromatosis and hemiatrophy. Two of the infants had glaucoma; one also had a facial nevus flammeus and the other had cutis marmorata telangiectatica congenita of the face. A congenital pigmented nevus and a localized venous malformation constituted the remaining associated defects. This disease is an uncommon cutaneous vascular anomaly that is most often solitary but occasionally may be associated with other developmental defects.

Child↗
PubMed1989

Congenital fascial dystrophy: stiff skin syndrome--a human counterpart of the tight-skin mouse.

Four patients are described with stony-hard induration of the skin and deeper tissues, most pronounced on the buttocks, thighs, and legs, and with limitation of joint mobility and contractures of the lower limbs. Two patients were siblings and one was the product of a consanguineous marriage. The disorder appears to be genetically determined, but the mode of inheritance has not been established. The disease was noticed in the patients' early infancy and was not progressive. Except for functional impairment of the lungs caused by an underdeveloped thorax that resulted from pressure of the thickened thoracic fascia, there was no involvement of the viscera or muscles and no immunologic abnormalities. The most important finding was markedly thickened fascia. This hereditary connective tissue disorder has all the characteristics of the tight-skin mouse.

Adult↗
PubMed1989

Lipophagic panniculitis of childhood.

The cases of three patients with an atrophic lipophagic granulomatous lipoatrophy are described and compared with 14 similar cases in the literature. In affected children erythematous lesions of the arms and legs usually occur, after which subcutaneous atrophy develops. Fever and edema are common. The histologic feature is a replacement of the fat lobule by lipophagic histiocytes and giant cells. No vasculitis or phlebitis is present. Previously, such cases have been called Weber-Christian disease or Rothmann-Makai syndrome, but they are best characterized by the distinctive clinical lesions, the course, and histologic findings. Corticosteroids apparently have been effective in some patients.

Arm↗
PubMed1989

Granulomatous eruption after BCG vaccine immunotherapy for malignant melanoma.

In a patient receiving intralesional bacille Calmette-Guérin vaccine injections for recurrent cutaneous metastases of malignant melanoma of the right leg, a striking eruption developed on that limb only. The eruption consisted of multiple 1 to 2 mm, reddish brown, flat-topped papules, with confluence into plaques. Histopathologic examination showed multiple tuberculoid granulomas within the papillary and reticular dermis; special stains and cultures were negative for mycobacteria.

BCG Vaccine↗
PubMed1989

Idiopathic erythermalgia: a congenital disorder.

Idiopathic erythermalgia during early childhood and adolescence is characterized by red, congested, burning pain of the lower extremities provoked by exercise or exposure to warmth. The clinical symptoms of idiopathic erythermalgia in a young woman and her mother are described. Histopathologic and immunofluorescence findings in biopsy specimens of affected areas of skin were consistent with a nonspecific inflammatory process. The condition was completely refractory to any treatment. Even the long-lasting relief of pain with one low dose of aspirin, which is a prerequisite for the diagnosis of thrombocytemic erythromelalgia, was lacking. Idiopathic erythermalgia appears to be a separate clinical entity and congenital disorder.

Adolescent↗
PubMed1989

Annular atrophic connective tissue panniculitis of the ankles.

A case of annular lipoatrophy of the ankles in a 4-year-old girl is presented. Histologically a lobular lymphohistiocytic panniculitis with masses of foam cells was present. This pattern resembles that seen in atrophic connective tissue panniculitis. The patient responded to treatment with prednisone and dapsone and now has residual lipoatrophy.

Ankle↗
PubMed1989

Chronic pruritic eruption in patients with acquired immunodeficiency syndrome associated with increased antibody titers to mosquito salivary gland antigens.

Five of seven patients with acquired immunodeficiency syndrome (AIDS) who had pruritus and a chronic, nonspecific-appearing skin eruption had increased antibody titers to antigens in the salivary glands of Aedes taeniorhynchus, a salt marsh mosquito common to South Florida. We hypothesize that the pruritus and skin lesions in patients with AIDS represent a form of chronic "recall" reaction. Increased antibody titers to mosquito salivary gland antigens may be a consequence of nonspecific B cell activation, a feature of AIDS.

Acquired Immunodeficiency Syndrome↗
PubMed1994

IgA anticardiolipin antibodies associated with Henoch-Schönlein purpura.

Henoch-Schönlein purpura is associated with the deposition of immune complexes containing IgA. The nature of the antigen in these immune complexes is uncertain but in some reported cases has included autoantigens such as IgA rheumatoid factor and IgA antineutrophil cytoplasmic antibody. We report the finding of an IgA class anticardiolipin antibody in a 51-year-old patient with Henoch-Schönlein purpura. A potential role for IgA autoantibodies in Henoch-Schönlein purpura needs to be further explored.

Antibodies, Anticardiolipin↗