PAPULAR-PURPURIC GLOVES-AND-SOCKS SYNDROME

Background and Objective. Papular-purpuric gloves-and-socks syndrome (PPGSS) is a recently described dermatosis in which human parvovirus B19 (HPV B19) has been implicated as etiologic agent; however, it is suspected that PPGSS may be caused by various agents. This study was designed to survey the general characteristics of PPGSS and to determine the role of HPV B19 in its etiology. Methods. We analyzed data from 21 patients and examined serum samples from three new cases for various viruses. Results. The PPGSS displays a striking uniform clinical pattern. Histologic and immunofluorescence findings are nonspecific. Seroconversion of HPV B19 was reported in six cases and confirmed in two of our patients. In only one case was a possible causative role of Coxsackie virus B6 suggested consistently. Conclusions. The PPGSS represents a distinctive dermatosis and a manifestation of HPV B19 infection. Unlike erythema infectiosum, anti-HPV B19 antibodies seem to develop later after onset of the skin eruption and while viremia is still present.     

SWEET'S SYNDROME AND HYPOTHYROIDISM

Hypothyroidism and Sweet's syndrome occurring together were diagnosed in a 33 woman. This association is uncommon, but may be underdiagnosed and underreported.     

SENEAR—USHER SYNDROME AND INTERNAL MALIGNANCY

Summary.— Three cases of Senear—Usher syndrome are reported. All 3 had immunological evidence of co-existing pemphigus and lupus erythematosus. Two patients died of bronchial carcinoma. One surviving patient has probable metastatic neoplasia in the liver.     

HYPEREOSINOPHILIC SYNDROME WITH CUTANEOUS BLISTERS AND BOWEL NECROSIS

A 44 year old man presented with a bullous eruption associated with gross eosinophilia. Before effective treatment was instituted he developed abdominal pain and subsequently extensive bowel necrosis. The criteria for hypereosinophilic syndrome were met by persistent eosinophilia without an established cause, in association with multiple organ system involvement.     

STEVENS-JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS IN THAILAND

Background. Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are potentially life-threatening illnesses that have often been linked to drug exposure. Methods. We looked retrospectively for all cases of SJS and TEN that were admitted to Siriraj Hospital between 1981 and 1990 to determine the drug etiology. Results. Fifty-eight cases of SJS and 20 cases of TEN were identified. Eight patients initially had an SJS-like aspect, which subsequently evolved into TEN. A culpable drug was determined in 60 patients (77%). The mean time from first drug administration to onset of SJS or TEN was 6.8 ± 6.5 days (range, 1 to 28 days). A longer incubation period was observed with thiacetazone (10.5 ± 5.6 days), phenytoin (12 ± 8.5 days), and carbamazepine (11.3 ± 3.4 days). Conclusions. The culprit drugs included the following: antibiotics, 32 cases (penicillin, sulfonamides, tetracycline, erythromycin); anticonvulsants, nine (phenytoin, carbamazepine, barbiturates); antitubercular drugs, eight (thiacetazone); analgesics, four (acetylsalicylic acid, fenbufen); sulfonylurea, two; allopurinol, one; and others, four. The most frequent underlying diseases justifying the ingestion of one or more drugs in our patients were infections (52.7%), followed by pulmonary tuberculosis (10.8%), and by seizures (8.1%). The total mortality rate was 14%; 5% for SJS, and 40% for TEN. Mortality was not affected by the type of drug responsible.     

MULTIPLE HAMARTOMA SYNDROME WITH TRICHOEPITHELIOMAS AND BASAL CELL CARCINOMAS

A patient with multiple hamartomas developed severe disfiguring trichoepitheliomas on her face. Some years later she developed multiple basal cell carcinomas. The occurrence of trichoepitheliomas with multiple hamartoma syndrome has not previously been reported. This patient with trichoepitheliomas developed undoubted basal cell carcinomas without the other major features of basal cell naevus syndrome. There do, however, appear to be some similarities between basal cell naevus syndrome and multiple hamartoma syndrome and this issue is addressed.     

KERATITIS, ICHTHYOSIS AND DEAFNESS (KID SYNDROME)

A boy with ichthyosiform erythroderma also suffered from sensorineural deafness, superficial punctate keratitis and acanthosis nigricans. Histopathology revealed basket weave hyperkeratosis, hydropic degeneration of the basal layer, melanin incontinence and moderate mononuclear infiltrate. Typical histological changes were found in clinical lesions of acanthosis nigricans.