On the relationship between subcorneal pustular dermatosis and pustular psoriasis.
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Biomedical subjects
Publications and source records attributed to H O Perry.
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"Adenoma sebaceum of Pringle" (ASP) is a misnomer. The tumor is not an adenoma and is not derived from sebaceous glands. The lesion is characterized by dermal fibrosis and associated vascular proliferation and dilatation. Changes in contiguous sebaceous glands and other adnexal structures are merely secondary. Thus, "angiofibroma" would be a more appropriate name. The histologic changes in ASP (and in related pathologic lesions) suggest that it is a hamartoma rather than a true neoplasm. However, the embryologic tissue of its origin is not definitively known.
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For many years, crude coal tar has been used for the treatment of psoriasis. The possible carcinogenic effect of crude coal tar and ultraviolet (UV) radiation (Goeckerman regimen), considered individually or in combination, has been of some concern to physicians. A 25-year follow-up study was completed on 280 patients with psoriasis who were hospitalized and treated with crude coal tar and UV radiation at the Mayo Clinic, Rochester, Minn, during the years 1950 through 1954. The results of this study suggest that the incidence of skin cancer is not appreciably increased above the expected incidence for the general population when patients are treated with coal tar ointments. It seems that the Goeckerman regimen (topical crude coal tar combined with UV radiation) can be used with minimal risk for skin cancer in the treatment of psoriasis.
Coal tar ointments have been used for many years in the treatment of chronic eczematoid dermatitis. The possible carcinogenic effect of coal tar ointment and ultraviolet light (Goeckerman regimen), considered individually or in combination, has been of concern to physicians. A 25-year follow-up study was completed on 426 patients with atopic dermatitis and neurodermatitis who were hospitalized and treated with coal tar ointments and ultraviolet light at the Mayo Clinic from 1950 through 1954. These results were compared with those from a similar study that examined patients with psoriasis who also were treated with coal tar ointments and ultraviolet light. The results of this study suggest that the incidence of skin cancer is not significantly increased above the expected incidence for selected populations of the United States. This study offers reassurance about the relatively small risk of skin cancer developing in patients with atopic dermatitis and neurodermatitis treated with coal tar ointments and ultraviolet light (Goeckerman regimen).
Livedo reticularis is a clinical pattern of cutaneous vascular involvement that can be seen in association with many diseases. The association of livedo reticularis and cerebrovascular accidents has been reported rarely in the past. We report on a 52-year-old woman with marked generalized livedo reticularis and cerebrovascular accidents who exhibited endarteritis obliterans without other pathologic changes on histologic examination of the skin. This constellation of findings suggest a primary vasculopathy with rather extensive involvement which merits our attention and further study.
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A beaded pattern of immunofluorescence (IF) occasionally occurs in routine FTA-ABS testing. This phenomenon strongly correlates with false-positive reactions in patients with SLE. We report on this IF finding in association with carcinoma of the colon.
Seven patients with blastomycosis-like pyoderma had skin lesions of four months' to six years' duration. The criteria for the diagnosis of blastomycosis-like pyoderma include the clinical presentation of large verrucous plaques with multiple pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically, and the growth of at least one pathogenic bacteria from the culture of a tissue-biopsy specimen. The differential diagnosis includes deep fungus infection (especially North American blastomycosis), bromoderma, pyoderma gangrenosum, mycobacterial infections, giant keratoacanthoma, and squamous cell carcinoma. Generally, the patients had one or more conditions that could have affected their systemic or local immunologic competence to infection. We believe that the clinical and histologic features in these cases of blastomycosis-like pyoderma were produced by an unusual, exaggerated, vegetating-tissue reaction to a primary or secondary bacterial infection.
Scleromyxedema is an uncommon cutaneous fibromucinous disease with a monoclonal protein, which has resisted a number of therapies. Eight cases followed up for as long as 12 years have provided an opportunity to observe the effects of melphalan treatment in this disease. The fibrohistiocytic and mucinous change of the skin in scleromyxedema and often the monoclonal protein can be controlled by low-dose chemotherapy. Although melphalan does not usually produce clinical toxic effects of importance, it is a myelotoxic drug and cytopenia is common; one patient died of acute myelomonocytic leukemia after ten years of successful therapy of the scleromyxedema, thus implying that long-term therapy may be dangerous by itself. These patients require close supervision. Leukocyte and platelet counts must be performed every three weeks, and the dosage of melphalan adjusted accordingly.
Three patients with porphyria cutanea tarda and lymphoma were seen at the Mayo Clinic. In one patient, the signs and symptoms of porphyria cutanea tarda and lymphoma occurred simultaneously, whereas in the other two, the porphyria was seen before the lymphoma occurred, three years before in one and one and a half years before in the other. Involvement of liver or marrow was not related to the development of porphyria cutanea tarda in any of the 3 patients. The porphyria cutanea tarda was not eased by radiotherapy or chemotherapy of the lymphoma, although it was probably less symptomatic after the patients were treated. While a relationship between porphyria cutanea tarda and lymphoma is speculative, confirmation will require a closer scrutiny of patients with these two diseases.
A 14-year-old boy with congenital poikiloderma had anhidrosis, palmoplantar-pitted keratoderma, traumatic bulla formation, and defective dentition, but no abnormalities of the hair, nails, or eyes. This patient was similar in some respects to others reported as having dermatopathia pigmentosa reticularis, the Franceschetti-Jadassohn syndrome, the Mendes da Costa syndrome, and acrokeratotic poikiloderma.
Generalized elastolysis is a rare and unique systemic disorder of connective tissue in which the elastic fibers suddenly become fragmented, disorganized and fewer in number with the resultant production of such entities as cutis laxa, emphysema, aortic aneurysms and bowel diverticula appearing in the organ system involved, that is, skin, lung, vasculature or gastrointestinal tract. Presented here are three cases that, illustrate the typical features of this condition. In addition, 14 more cases were retrieved from the literature and the information reviewed. Various etiologic factors relating to the synthesis and degradation of elastic tissue are discussed in light of recent findings in the biochemistry of connective tissue.
Two patients each with a two-year history of chronic ulcerative colitis developed, over a 24- to 48-hour period, painful pustules involving the skin and oral mucosa. The pustular eruption was associated with a severe exacerbation of the colitis. Culture of the lesions and of the blood failed to reveal an infectious cause for the eruption. Histologic study of a pustule in case 2 revealed suppurative folliculitis. The lesions subsided rapidly in case 1 when intravenous hydrocortisone and intravenous and intramuscular antibiotics were administered, and further resolution occurred after colectomy. A similar rapid resoultion of the pustules occurred in case 2 after initiation of treatment with intramuscular cortisone acetate and antibiotics and with colectomy. This pustular eruption may represent a variant of pyoderma gangrenosum.
Immunopathologic investigations were conducted on the sera and oral mucosal tissue specimens of 23 patients with cicatricial pemphigoid. A linear, continuous basement membrane zone pattern was noted in 83% of oral mucosal biopsy specimens studied. This pattern is indistinguishable from the pattern noted in immunofluorescence studies of bullous pemphigoid, herpes gestationis, and some cases of desquamative gingivitis. Complement studies provided data supportive of classical pathway activation in cicatricial pemphigoid tissue. Deposition of IgA with Factor B, properdin, and C3 raised the possibility of alternative pathway activation, a question requiring further study. Circulating antibasement membrane zone antibodies were noted in the sera of two patients with cicatricial pemphigoid.
Of 144 patients with bullous or cicatricial pemphigoid, nine with localized pemphigoid were seen at the Mayo Clinic between 1968 and 1975. In two patients the disease had become generalized before presentation, and in one it had evolved into bullous pemphigoid, the only case with positive indirect immunofluorescence. One additional patient showed mucosal lesions. Direct immunofluorescence was performed in five patients and showed linear deposition of C3 and fibrin but not immunoglobulins in three; in two patients immunofluorescence was negative. Localized pemphigoid can be divided into two types: that with scarring plaque-like lesions usually occurring on the head and neck predominantly in males, and that with localized bullous lesions usually occurring on the lower part of the legs of females.