Linear IgA bullous dermatosis of childhood: a long-term study.

BACKGROUND: Linear IgA bullous dermatosis (LAD) of childhood is a rare acquired subepidermal blistering disease of young children. Most of the studies were reported from the USA and European countries. METHOD: Twelve cases of Thai patients diagnosed as LAD of childhood were analyzed concerning clinical, histopathological, immunopathological findings including treatment responses and courses compared with those of Caucasians. RESULT: The mean age of onset was 5.1 years. The areas of common involvement were the perioral region, lower abdomen, perineum, buttock, inner thighs and extremities. Histopathology in half of the cases showed features of dermatitis herpetiformis or bullous pemphigoid. All patients had positive linear IgA band at the basement membrane zone (BMZ) by direct immunofluorescence. Only one patient had positive circulating anti BMZ antibody at the titer of 1:10. Most patients responded well to dapsone. The mean duration before remission was 1.9 years. CONCLUSION: Our study in Thai patients with LAD of childhood produced data similar to previous studies carried out in the Caucasian nations.

Pityriasis rubra pilaris: a new proposed classification.

Clinical features of pityriasis rubra pilaris (PRP) were classified into four types based on a study of 168 cases. All patients, except one case of type IV, and 12 cases of type II, have salmon-coloured or erythematous thick plaques on the palms and soles, which extended beyond the dorsopalmar and plantar junctions. Twenty-one children and 11 adults presenting with such plaques were classified as type I PRP. Fifty-nine children and 27 adults presenting with circumscribed scaly erythematous patches on the elbows and knees were classified as type II. Twenty children and 16 adults with similar patches involving large areas of the trunk which were not generalized were classified as type III. Four children and 10 adults with exfoliative erythroderma associated with diffuse follicular plugging were classified as type IV. The diagnosis was confirmed by histopathology which showed follicular plugging and focal parakeratosis. However, this was not seen in type I. This new clinical classification facilitates recognition of this type of PRP despite the absence of follicular plugging. Two children, initially classified as type I, showed gradual progression to type II on follow-up, with two adults progressing to type III, and 1 adult to type IV. Similarly, two adults classified as type II, progressed to type III, and 1 adult into type IV. Associated findings were an adult of type II who developed localized pustular psoriasis and another adult who developed ovarian adenocarcinoma.

Treatment of pemphigus.

Ninety-eight cases of various types of pemphigus were treated between 1978-1987. Sixty-one cases were pemphigus vulgaris (PV), 22 cases were pemphigus foliaceus, generalized type (PFG) in which one case developed pemphigus vegetans, 11 cases were pemphigus foliaceus localized type (PFL), and four cases were pemphigus erythematosus (PE). Fifteen mild cases of PV and three mild PFG were treated with corticosteroid (prednisolone or prednisone) alone, and dapsone or cyclophosphamide (CP) were added as treatment failed in two cases of each. Dapsone alone was used effectively in three cases of mild PV. Eight cases of moderate and three cases of severe PV, as well as five cases of moderate PFG, failed to respond to corticosteroid alone but were cleared by the addition of CP. Thirty-two moderate cases of PV and PFG treated with a combination of corticosteroid 60 mg/day plus initial CP and 14 severe cases of PV and PFG treated with corticosteroid 120 mg/day plus initial CP, resulted in clearing skin lesions in 2 months. Azathioprine or chlorambucil were substituted in three cases who developed CP toxicity. Addition of gold sodiumthiomalate in six refractory cases when the above regimens failed, caused a complete remission in two and partial control in four. Higher dosage of prednisolone or prednisone more than 120 mg/day has never been used. Eleven cases of PFL and four cases of PE were treated with uneventfully good results. Intercellular antibody titers became negative within 4.67 months except in refractory cases, however, the treatment was continued for at least 3 years. Herpes simplex superimposed infection was more common than herpes zoster infection.(ABSTRACT TRUNCATED AT 250 WORDS)

Parapsoriasis and related conditions.

Classification of parapsoriases is revised into a simple practical table. Pityriasis lichenoides (guttate parapsoriasis) is not a type of parapsoriasis. The clinical features of small patch and large plaque parapsoriasis are described in detail. Six clinical varieties of large plaque parapsoriasis and three clinical varieties of exfoliative dermatitis including Sézary syndrome have been clearly recognized as distinctive categories. Histopathology is useful for the diagnosis of parapsoriasis and mycosis fungoides. Sézary cell count is not significant for the diagnosis of Sézary syndrome.

Sézary cell count in exfoliative dermatitis.

Sézary cells were present in a number of less than 1000/mm3 in 52 of 78 cases of various benign diffuse dermatoses and mycosis fungoides. The count was higher than 1000/mm3 in two cases of Sézary syndrome in which the histopathology was that of mycosis fungoides and in six cases of exfoliative erythroderma of undetermined etiology with the histopathology of nonspecific chronic dermatitis. One of these six cases developed palmoplantar keratoderma, but abnormal cells were not present in the tissue. The diagnosis of Sézary syndrome in these cases of exfoliative erythroderma could not be established, even though the Sézary cell count was more than 1000/mm3, because the abnormal cells were absent in the skin biopsy specimen. It is concluded that the number of Sézary cells present is not significant for the diagnosis of Sézary syndrome. Our cases with the diagnosis of Sézary syndrome are an erythrodermic variant of mycosis fungoides rather than a progressive conversion from exfoliative erythroderma.

Juvenile blistering diseases: the problems of diagnosis and treatment.

Correct clinical diagnosis in cases of chronic, relapsing, non-hereditary, blistering diseases in childhood could not be made without the aid of histopathology and immunofluorescence, since the morphology and the distribution of the lesions of bullous pemphigoid (BP), linear IgA bullous dermatosis (LAD) and dermatitis herpetiformis (DH) may be similar. Histopathology was helpful in about half of the cases. The results of immunopathology were very useful for the final diagnosis. Of twenty-one cases of juvenile blistering diseases, two cases which showed IgG on direct test with circulating antibodies were BP; three cases with deposition of IgG but without circulating antibodies were probably BP; three cases were either BP or LAD (IgG and IgA on direct test without circulating antibodies); nine cases were definite LAD (linear IgA only); one case which showed granular IgA in the dermal papillae and linear IgA was DH; and the last three cases were probably LAD and BP with non-immunoreactant deposits. Regardless of the diagnosis, dapsone and co-trimoxazole controlled eight cases and could be discontinued without relapse, while the other six cases were maintained on dapsone therapy alone. Prednisolone, when added in cases of poor response to either dapsone or co-trimoxazole, caused remission and was discontinued in three out of the five cases. Two cases were lost to follow up.

Pustular acne.

Pustules from 92 new cases of pustular acne and gram-negative folliculitis were cultured in aerobic medium for superimposed bacterial infection. During the treatment of 1,561 new patients with papular acne with tetracycline and topical clindamycin and antibacterial soaps, an additional 10 developed pustules. The majority showed in vitro resistance to ampicillin. The effective treatment was co-trimoxazole and topical gentamicin. A new classification of acne with practical therapeutic use is needed.