Dermatomyositis with the features of inclusion body myositis associated with carcinoma of the bladder.

Inclusion body myositis (IBM) is a unique category of inflammatory myopathy. It is characterized histologically by the presence of muscle fibres with rimmed vacuoles and abnormal intracellular accumulations of proteins. We report here a 62-year-old patient with bladder carcinoma, where the signs of IBM overlapped with clinical features of dermatomyositis (DM). A combination of cutaneous changes typical for DM with histological and other features of IBM is exceedingly rare, and has not been previously addressed in dermatological literature. To the best of our knowledge this is also the first description of the association of DM/IBM with internal malignancy.

Chronic staphylococcal scalded skin syndrome.

Staphylococcal scalded skin syndrome (SSSS), not previously recorded as a chronic disease, persisted for 2 years in a 50-year-old woman with epilepsy and cerebellar ataxia. Lesions initially suggestive of erythema multiforme and toxic epidermal necrolysis evolved over 2 years into those typical for SSSS, with extensive erosions and subcorneal blisters, showing an epidermal split at the granular cell layer. Exfoliatin A-producing phage I-III Staphylococcus aureus, previously linked only to acute mild adult cases of SSSS, was cultured from purulent discharge in the patient's eyes, ears and open skin lesions. The roles of epilepsy and antiepileptic medications are discussed as possible predisposing factors.

Botulinum toxin therapy for palmar hyperhidrosis.

BACKGROUND: Severe palmar hyperhidrosis is a chronic disease, resistant to conventional therapy. Botulinum toxin inhibits sweat production by blocking release of acetylcholine from presynaptic membranes. OBJECTIVE: Our purpose was to evaluate the short- and long-term effectiveness of botulinum toxin therapy in treatment of palmar hyperhidrosis. METHODS: Four patients with severe palmar hyperhidrosis were treated with subepidermal injections of botulinum toxin. Fifty injections, 2 mouse units each, were used in each palm. Regional nerve blocks of the median and ulnar nerves were performed before the procedure. Patients were observed for 12 months after treatment. RESULTS: Botulinum toxin injections significantly reduced sweat production in the treated areas of the palms. Anhidrosis lasted for 12 months in one patient, 7 months in two patients, and 4 months in one patient. Mild weakness of the thumb lasting 3 weeks occurred in one patient. No other side effects were observed. CONCLUSION: Botulinum toxin provides an effective, safe, and long-lasting alternative therapeutic modality for treatment of severe palmar hyperhidrosis. Additional studies are needed for optimization of the technique.

The mysterious case of the premenstrual purple chin.

Recurrent episodes of a "purple chin" in an adolescent girl eluded definitive diagnosis for three years. The condition occurred premenstrually, but results of extensive tests failed to reveal any abnormal hormonal sensitivity. A diagnosis of factitial purpura was eventually established.

Detection of streptococcal class I M protein in psoriasis by confocal immunofluorescent microscopy.

Epidemiological evidence implicates Streptococcus pyogenes (group A) infection as a common triggering stimulus for psoriasis. Unequivocal demonstration of streptococcal antigens in psoriatic skin has been difficult due to cross-reactive antigens in both normal human tissue and group A streptococci, which complicate immunohistological analysis. In this study cryostat sections of involved psoriatic skin were stained with monoclonal antibody 111-15504 to group A streptococci. The epitope recognized by this antibody was found to be specific for group A streptococci and is associated with class I M protein. Streptococcal antigens were found in the dermal papillae and epidermis of psoriatic skin lesions of 20 out 38 patients. These findings indicate that specific S. pyogenes antigen, associated with class I M protein, is often present in psoriatic lesions. Such an antigen, originating from focal infection elsewhere could be responsible for T-lymphocyte inflammatory responses triggering the development of psoriatic lesions.

IgE bullous disease.

We report two patients with the typical picture of bullous pemphigoid who lacked two critical diagnostic immunopathological features of the disease, namely IgG or C3 bound to the epidermal basement membrane and circulating IgG antibodies directed against the basement membrane zone (BMZ). Both patients had dense infiltrates of eosinophils within their skin lesions, as well as markedly elevated serum IgE levels, while immunofluorescent studies with anti-IgE antibody revealed heavy IgE deposition on inflammatory cells within the dermis surrounding the bullae. These cells were confirmed to be eosinophils by means of specific staining with antibody to major basic protein (MBP). We speculate that this 'IgE bullous disease' resulted from IgE-mediated hypersensitivity induced by focal infection, both patients initially being helped by antibiotics. However, dramatic clearing of bullae was seen following surgical removal of a battery implant (Patient 1), and bilateral above-the-knee amputations of gangrenous legs (Patient 2).

Estrogen dermatitis.

BACKGROUND: Autoimmune progesterone dermatitis includes pruritus, urticaria, papulovesicular eruptions, and bullous erythema multiforme. Sensitivity to estrogen has not been described, although it was probably first recognized almost 50 years ago. OBJECTIVE: Our purpose was to assess sensitization to selected hormones in women with a significant premenstrual flare of skin lesions. METHODS: Intradermal skin tests to 18 hormones and control substances were performed in seven patients and four control subjects and read for immediate urticarial and delayed type reactions. Six other control subjects had only estrone and progesterone skin tests. The effect of the antiestrogen tamoxifen on the course of the dermatosis was studied. RESULTS: Seven women exhibiting severe premenstrual exacerbations of papulovesicular eruptions, urticaria, eczema, or generalized pruritus proved to have an unrecognized sensitivity to estrogen. Five patients had a positive delayed tuberculin-type skin test to estrogen. Two patients with generalized chronic urticaria had only an urticarial reaction to intradermal estrogens. Antiestrogen therapy with tamoxifen proved effective in all five patients, whereas elimination of oral estrogen therapy cured the other two patients. CONCLUSION: Women can become sensitized to their own estrogens; the major clue is worsening of the skin problem premenstrually. Positive intradermal skin tests to estrogens are diagnostic. Tamoxifen is a specific therapy. We have named this disorder estrogen dermatitis.

Cutaneous histiocytosis with Langerhans cell features induced by scabies: a case report.

An infant with biopsy-proven scabies developed nodular lesions. Histopathology revealed atypical histiocytes with Langerhans cell features. Within six months after treatment all skin lesions gradually disappeared. We suggest that the nodules in scabies can be due to Langerhans cell proliferation.

Koebner reaction in psoriasis due to secondary syphilis.

A patient with a two-year history of psoriasis showed a widespread eruption that was clinically and histologically similar to psoriasis. Results of blood tests revealed syphilitic infection, appearing to be secondary syphilis. The flare-up of guttate psoriasis was considered to represent a Koebner phenomenon due to secondary syphilis.