Vitamin K-responsive coagulopathy in Henoch-Schölein purpura.

Henoch-Schönlein purpura is classically described as a systemic vasculitis without known platelet or clotting abnormality. A 15-year-old boy with Henoch-Schönlein purpura experienced a major hemorrhagic diathesis that was responsive to parenterally administered vitamin K. We believe that the gastrointestinal vasculitis prevented the absorption of available vitamin K necessary for the synthesis of clotting factors. Early treatment with parenteral vitamin K in similar cases should be considered.

Hereditary acrolabial telangiectasia. A report of familial blue lips, nails, and nipples.

We describe a mother and two daughters who had the following clinical manifestations: bluish discoloration of the vermillion ridge of the lips, nipple areolae, and nail beds; discrete telangiectasia of the chest, elbows, and dorsa of the hands; varicosities of the lower part of the legs; and (in the two daughters) migraine headaches. Routine histologic examination of tissue from the lips and elbows disclosed extensive, dilated, horizontal subpapillary telangiectases. Enzyme histochemical stains demonstrated activity of adenosine triphosphatase and leucine aminopeptidase around these dilated vessels. Alkaline phosphatase activity was strikingly absent from the dilated subpapillary vessels. By electron microscopy, these vessels were demonstrated to be postcapillary venules. We propose an autosomal dominant mode of inheritance.

The coexistence of psoriasis and lupus erythematosus. An analysis of 27 cases.

From 1950 through 1975, 27 patients at the Mayo Clinic, Rochester, Minn, had coexistent psoriasis and lupus erythematosus (LE). Of the 27 patients, ten had systemic LE (SLE), 13 had discoid LE, and four had drug-induced LE or an SLE-like syndrome. The onset of psoriasis preceded LE in ten patients and was concomitant with it in six. Photosensitivity was noted in 23 patients. In 20 patients, the lesions of psoriasis and LE remained clinically distinct. Morphologic overlap and clinical interaction occurred in seven patients in whom the disorders were severe and complicated by a third disease. Generally, this latter group had extensive psoriasis, prominent photosensitivity, and a poor response to therapy.

Resistant cutaneous infection caused by Mycobacterium chelonei.

Induration of the lower parts of the legs with abscess and ulcer formation occurred in a 60-year-old woman. Mycobacterium chelonei, a ubiquitous, saprophytic pathogen that uncommonly causes human disease, was cultured from biopsy material. Although spontaneous healing usually occurs in a few months with such infections, our patient's disease persisted for more than two years until control was achieved with minocycline hydrochloride.

Oral pemphigus vulgaris. A report of ten cases.

The conditions of ten patients were diagnosed as pemphigus vulgaris limited to the oral cavity. In eight patients the histopathologic findings were consistent with oral pemphigus. Direct immunofluorescence demonstrated intercellular substance (ICS) deposition of C3 either alone or in combination with IgG, IgA, IgM, C4, C1q, properdin, and factor B in all nine patients tested. Circulating ICS antibodies were detected initially in eight of the ten patients, nine of whom had positive titers at a later date, without subsequent development of cutaneous lesions. Physicians should be alerted to a diagnosis of pemphigus in patients who have chronic erosive disease confined to the oral cavity. Prompt diagnosis by means of routine histopathologic and immunofluorescence studies followed by early initiation of treatment are warranted.

Development of photosensitivity and an SLE-like syndrome in a patient with psoriasis.

An unusual case of photosensitive psoriasis and systemic lupus erythematosus-related syndrome was characterized by erythroderma, chronic urticaria, angioneurotic edema, intermittent low-grade fever, and polyarthralgias. Investigation revealed no measurable total hemolytic complement and markedly diminished levels of C4, C2, and C3. Microscopic examination of three skin biopsy sections of sun-exposed skin showed psoriasis. Skin biopsy sections of sun-exposed psoriatic plaques and of non-sun-exposed, uninvolved skin (which were stained with fluorescein-tagged anti-IgG, anti-IgM, anti-IgA, and anti-C3) showed granular deposits of IgM and C3 at the dermal-epidermal junction in the sun-exposed plaques, and IgM alone in a granular pattern at the dermal-epidermal junction in uninvolved skin. Antibodies to single-stranded but not double-stranded DNA were detected in the patient's serum. In addition, serum immune complex-like material was detected by sucrose density-gradient ultracentrifugation, standard anticomplementary assays, and radioimmunoassays using both C1q and monoclonal rheumatoid factor.

Mechanisms of sebum production and delivery in man.

Utilizing a solvent extraction system and colorimetric analysis, surface lipid levels were determined on the forehead. To understand the mechanisms involved in the sebaceous gland apparatus, repeated sampling at varying timed intervals were made. The data suggest that frequent surface samplings produce higher rates of lipid replacement. The increasing lipid replacement rates seen with sampling intervals of greater frequency do not follow a linear pattern. Also, lipid replacement rates show a gradual decline with time. This data suggest the existence of a multifunctional sebaceous apparatus involved in sebum production (a rate function), sebum storage (a volume function) and surface delivery (a rate function).

Association of islet cell carcinoma of the pancreas with subcutaneous fat necrosis.

A 57-year-old man with generalized, subcutaneous fat necrosis was found at autopsy to have islet cell carcinoma of the pancreas. The histologic diagnosis of islet cell carcinoma was confirmed by the finding by electron microscopy of characteristic intracytoplasmic granules within pancreatic neoplastic cells. Lipase levels were elevated in serum as well as in tissues in areas of subcutaneous fat necrosis. Enzyme histochemical stains for alkaline and acid phosphatase, leucine, aminopeptidase, succinic dehydrogenase, and indoxyl and nonspecific esterase were positive in the areas of subcutaneous fat necrosis. This is the first report of the association of islet cell carcinoma of the pancreas with generalized subcutaneous fat necrosis.

The sign of Leser-Trélat in a case of adenocarcinoma of the lung.

This is what we believe to be the first report of the sign of Leser-Trélat in association with occult adenocarcinoma of the lung. The sign of Leser-Trélat is proposed as a sign of possible occult malignancy, despite various suggestions to the contrary. Also, it is suggested that a tumor-produced humoral factor (eg, transforming growth factor-alpha [TGF-alpha]) could be responsible for both the acute eruption of the monomorphous seborrheic keratoses and the nearly concomitant development of acanthosis nigricans, which occurred in our case. The possible distinction between a hyperplastic and a neoplastic origin of various types of seborrheic keratosis is discussed in relation to this hypothetical humoral factor. In addition, we suggest a refinement of the definition of the sign of Leser-Trélat and discuss the use of "sign of Leser-Trélat" and "syndrome of Leser-Trélat" in relation to physical findings. All patients with the sign of Leser-Trélat should undergo a thorough evaluation for occult malignancy.

Minocycline-induced pigmentation at sites of cutaneous inflammation.

In four cases of minocycline hydrochloride-induced cutaneous pigmentation, blue-gray discoloration in sites of cutaneous inflammation was seen in all cases. An additional finding of generalized, brown hyperpigmentation with accentuation in sun-exposed areas was noted in one. Although all of the patients had used relatively high doses of medication, the variable duration of therapy before pigmentary changes and dearth of similar reports suggest an idiosyncratic response to this commonly used medication. Histochemical stains and electron microscopic studies suggest hemosiderin or a pigment with similar staining properties; a minocycline degradation product, however, cannot be discounted.

Neurological complications of cryosurgery.

Among the few serious complications of cryosurgery are neuropathies. Three cases are reported. Prevention of neuropathy from cryosurgery depends upon awareness of the superficiality of the course of certain peripheral nerves in certain anatomic sites and cautious practice or avoidance of cryosurgery in those sites.

Factitial cutaneous ulcers and nodules: the use of electron-probe microanalysis in diagnosis.

Two cases are reported in which persistent nodular or ulcerative skin lesions with granulomatous histology suggested possible injection of foreign material. Electron-probe microanalysis was used to determine the presence and nature of foreign materials present in the skin. This technic identified specific agents used as fillers in tablets, indicating the injection of pulverized tablet material. The patients were counseled regarding this evidence, and psychotherapy and drug rehabilitation were recommended in each instance. This technic provides a valuable tool for accurate identification of foreign substances in the skin.

Skin manifestations of pentazocine abuse.

Seventeen patients who had cutaneous complications following pentazocine injections are presented. The mean age was 50 years; total daily pentazocine dose ranged from 60 to 2,400 mg; evidence of psychiatric illness was present in 94%, and previous drug or alcohol abuse was noted in 65%. Fifty-three percent of our series of patients had a medical or paramedical background. Eighteen percent had diabetes mellitus, and 65% had a personal or family history (or both) of diabetes. A hypothesis is presented for this association. Characteristic histologic findings included fibrosis of the dermis and panniculus, with vascular alterations, fat necrosis with granulomatous inflammation, and vascular thrombosis with occasional endarteritis. We emphasize that medical and paramedical personnel and patients with a personal or family history of diabetes should be added to the group of patients considered to be at special risk for cutaneous complications of pentazocine injections.

The therapeutic response of urticarial vasculitis to indomethacin.

Ten patients with urticarial vasculitis, characterized clinically by persistent painful urticarial lesions, angioedema, recurrent arthralgia, abdominal pain, and low-grade fever, were selected for study. All patients had histologic evidence of leukocytoclastic vasculitis in the urticarial lesions. Results of direct immunofluorescence microscopy of urticarial lesions were positive in all nine of the patients tested. Treatment with indomethacin in dosages from 25 mg three times daily to 50 mg four times daily resulted in complete clearing of all disease manifestations in six of ten patients within 17 days and partial improvement in three. In eight of the ten patients, disease activity recurred within 48 hours after discontinuation of the use of indomethacin. Gastrointestinal irritation was the only side effect noted. Indomethacin is proposed as an effective mode of therapy in a disorder unresponsive to treatment with conventional medications for urticaria, including high-dose corticosteroids.

Cutaneous immunofluorescence in primary biliary cirrhosis.

Biopsy specimens of normal skin of 11 patients with primary biliary cirrhosis were studied by immunofluorescence for immunoglobulin and complement deposition. Of the 11 patients, seven had positive immunofluorescent staining, primarily in a granular pattern either at the basement membrane zone or around the blood vessels or in both locations. The principal immunoglobulin noted was IgM, which, in six of the seven patients, was seen at the basement membrane zone or around the vessels (or both). Deposition with C3, fibrin, IgA, and IgG was seen less frequently. In this series of patients, cutaneous lesions included discoid lupus erythematosus, lichen planus, vitiligo, chronic bullous lesions of the legs, and scleroderma. Non-organ-specific immunologic markers in this group included elevated levels of IgG, IgM, and IgA, antinuclear antibodies, anti-nDNA, rheumatoid factor, antimitochondrial antibodies, smooth-muscle antibodies, and circulating immune complexes. We wish to add cutaneous immunofluorescence as an additional immunologic marker for primary biliary cirrhosis.