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The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus.

BACKGROUND: Cutaneous neonatal lupus erythematosus (NLE) is an uncommon disease described mainly through isolated case reports. OBJECTIVE: Our purpose was to examine the cutaneous spectrum, clinical associations, and course of disease in babies with anti-Ro-positive NLE. METHODS: This is a retrospective case series evaluation of newborns with anti-Ro-positive NLE seen at a single ambulatory care university center over a 20-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years. RESULTS: Four boys and 14 girls were included in our evaluation. Distribution of skin lesions in 18 babies was as follows: face, 17; periorbital "owl-eye" or "eye mask" facial rash, 14; scalp, 15; arms and legs, 13; trunk and groin, 6. Crusted lesions were predominant in 3. Photosensitivity was seen in 12, and features of cutis marmorata telangiectasia congenita were observed in 4. In 17 neonatal lupus was not suspected until the dermatology consultation. Noncutaneous manifestations included thrombocytopenia in 4, cholestatic hepatitis in 3, and congenital heart block in 3. Four patients had residual telangiectasia that persisted for 3 or more years but eventually cleared in 2 patients. Three babies had dyspigmentation that spontaneously cleared within 22 months. None had atrophy or scarring. CONCLUSION: Periorbital, scalp, and extremity lesions are common in cutaneous NLE. Crusted lesions predominated in male infants. In children selected by cutaneous involvement, thrombocytopenia and hepatic disease were present as frequently as cardiac disease and occurred more frequently in male babies with crusted skin lesions. Children with cutaneous NLE should be evaluated for hematologic and hepatic as well as cardiac involvement.

Antibodies, Antinuclear↗

4 cases of photocontact dermatitis due to ketoprofen.

We report 4 cases of photocontact dermatitis due to ketoprofen, a non-steroidal anti-inflammatory drug derived from propionic acid. We carried out a skin biopsy to examine the amount of ketoprofen in the eruptive skin. We investigated the cross-reactions between tiaprofenic acid, suprofen and ketoprofen by patch and photopatch testing. In case no. 1, 17 days after the discontinuance of Mohrus poultice (containing ketoprofen as an active ingredient), we detected ketoprofen 312.5 ng/g in the area of skin where the poultice was applied. All 4 cases reacted positively to the causative medicaments containing ketoprofen and ketoprofen 1% pet. 3 out of 4 cases reacted positively to tiaprofenic acid 1% pet. Only 1 case out of the ketoprofen and tiaprofenic acid positive cases reacted positively to suprofen 1% pet. Vehicles of patients' medicaments were negative in all 4 cases. We suspected that the key structure of the cross-reaction between ketoprofen and tiaprofenic acid and suprofen was the benzoyl radical.

Adult↗

Disseminated superficial actinic porokeratosis. Analysis of an affected family.

A family is analysed in which disseminated superficial actinic porokeratosis (DSAP) occurred in five members. All three children of one generation are affected. Clinical, histopathological and genetic aspects of DSAP are discussed. Disseminated superficial actinic porokeratosis appears to be a clinical variant of porokeratosis of Mibelli. Since DSAP is a genodermatosis inherited as an autosomal dominant it can occur in any geographical location; however excess sunlight can definitely exacerbate the condition.

Adult↗

Contact allergy to corticosteroids: the results of a two-centre study.

We report a comparative study of the patch-test results obtained with a corticosteroid series, added to the standard series, in two centres, one in Belgium and the other in the Netherlands. The frequencies of positive reactions to the corticosteroids differed considerably between the two centres, and we suggest several reasons for this.

Adrenal Cortex Hormones↗

Regional dermatoses in the African race. Papular lesions on the face.

Many diseases are peculiar to the black race and most practicing physicians in Africa have limited access to histopathologists, therefore, diagnosis is largely clinical. A retrospective study of 10,000 consecutive patients seen at the skin clinic of the Lagos University Teaching Hospital, Nigeria, was conducted with the objective of identifying the notable dermatoses affecting various parts of the body. The face and the shins were observed to have a very broad spectrum of dermatoses. Useful clinical descriptions of these dermatoses are highlighted in a three part paper to help practicing physicians.

Adult↗

[Hyperkeratosis follicularis et parafollicularis in cutem penetrans (Kyrle's disease). Description of a case].

A case of hyperkeratosis follicularis et parafollicularis in cutem penetrans Kyrle's disease in a 41-year-old woman is described. It was a recurrence of a lesion surgically removed and originally diagnosed as invasive squamous cell carcinoma. Kyrle's disease is a rare dermatosis and should not be confused with neoplastic conditions i.e. squamous cell carcinoma. Pathologists should be aware of such conditions in order to avoid misdiagnosis, which can imply over treatment for the patient.

Adult↗

[Comparative testing of Kamillosan cream and steroidal (0.25% hydrocortisone, 0.75% fluocortin butyl ester) and non-steroidal (5% bufexamac) dermatologic agents in maintenance therapy of eczematous diseases].

We report on 161 patients suffering from inflammatory dermatoses on hands, forearms, and lower legs who had been initially treated with 0.1% difluocortolone valerate. During the maintenance therapy carried out over a period of 3 to 4 weeks, we tested the efficacy of Kamillosan cream vs. 0.25% hydrocortisone, 0.75% fluocortin butyl ester, and 5% bufexamac in a bilateral comparative study. For the indications tested Kamillosan cream showed more or less equieffective therapeutic results as compared to 0.25% hydrocortisone. It is superior, however, to the non-steroidal anti-inflammatory agent 5% bufexamac as well as to 0.75% fluocortin butyl ester, a further glucocorticoid. With regard to neurodermitis, Kamillosan cream not only shows the same therapeutic effect as 0.25% hydrocortisone but is even of marked superiority towards other reference products.

Adolescent↗

Neutrophilic lobular (pustular) panniculitis associated with rheumatoid arthritis: a case report and review of the literature.

Rheumatoid nodules, which affect the subcutis around joints, are the most frequent specific cutaneous lesions of rheumatoid arthritis (RA). Panniculitis is a rarely reported and nonspecific complication of RA. We report a 42-year-old woman with seropositive RA who presented with a 2-month history of lower leg panniculitis. Biopsy of a leg nodule showed a lobular neutrophilic infiltrate with lipophages and central basophilic necrosis. In addition, focal changes of lipomembranous fat necrosis indicative of ischemic damage were identified at the margins of the lobular infiltrate. Neutrophilic lobular panniculitis is commonly detected in panniculitis secondary to bacterial infections, pancreatitis, and factitial causes. However, this pattern of panniculitis has also been reported in some cases of erythema nodosum-like lesions found in Behçet disease or bowel bypass syndrome and in rare cases of seropositive RA. These reported histologic findings fall into the spectrum of neutrophilic vascular reactions described by Jorizzo and Daniels for RA-associated dermatoses. In view of these findings. RA and related neutrophilic dermatoses (e.g., Behçet disease) should be included in the differential diagnosis of neutrophilic lobular panniculitis.

Adult↗

Acute pustulosis of the legs in diverticulitis with sigmoid stenosis: an overlap between bowel-associated dermatosis-arthritis syndrome and pustular pyoderma gangrenosum.

BACKGROUND: Bowel-associated dermatosis-arthritis syndrome denotes the occurrence of diarrhoea with arthritis and skin lesions related to bowel disease with or without bowel bypass. In this condition, the histological finding of cutaneous aseptic neutrophilic cell infiltrate is non-specific and common to a wide spectrum of neutrophilic dermatoses, including pyoderma gangrenosum. OBSERVATION: We describe a 78-year-old woman with fever, abdominal discomfort and arthralgias, who developed grouped pustular lesions on her shins with histologically spongiform pustule formation. Aetiological assessment disclosed diverticular disease with sigmoid stenosis. CONCLUSION: Although clinical and histological features in our case fit the diagnosis of bowel-associated dermatosis-arthritis syndrome, they may also correspond to a pustular variant of pyoderma gangrenosum. Our observation raises the question of the nosological classification of bowel-associated dermatosis-arthritis syndrome within the spectrum of neutrophilic diseases.

Acute Disease↗

Recalcitrant unilateral infection associated with congenital leg hypertrophy cleared by control of hyperhidrosis.

A man with congenital hypertrophy of the left leg experienced concomitant fungal and gram-negative bacterial infection of the foot on the same side. Oral antibiotic therapy failed, compromised by a post-surgical short bowel. Despite two hospitalizations for intensive care with intravenous antibiotic therapy, the infection did not clear until his plantar hyperhidrosis on the affected side was eliminated by the use of topical aluminum chloride.

Administration, Topical↗