Cutaneous neonatal lupus erythematosus

Neonatal lupus erythematosus (NLE) is caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies. The most common manifestations are cutaneous lupus lesions, third-degree heart block, and/or hematologic cytopenias. The disease is a transient process, although complete heart block, once established, is permanent. A Caucasian eight-month child presented with a one-month history of cutaneous lesions on the face. Physical cutaneous examination revealed Erythematous annular plaques with central atrophy Confluent in periorbital area

The remainder of the cutaneous examination was not remarkable.

Laboratory studies revealed positive ANA and anti-Ro/anti-La. Blood count, liver enzymes and cardiologic examination were normal, including echocardiography. Maternal serum was also positive for antinuclear antibodies and anti-SSA/Ro at the time we suspected the diagnosis of NLE in the child, but she was not diagnosed with any connective disease. Cutaneous neonatal lupus erythematosus diagnosis was made based on the characteristic skin changes, laboratory findings and maternal history. Photoprotection was prescribed and within a few months the cutaneous lupus lesions resolved spontaneously and the serum antibodies disappeared. NLE is an uncommon autoimmune disease caused by transplacental passage of maternal anti-Ro and/or anti-La autoantibodies,. Several studies described an increased prevalence of HLA-DR3 in mothers of children affected by NLE. The most common clinical findings include cutaneous lesions and third-degree heart block. [3] Hepatic abnormalities or hematologic cytopenias are less frequently reported. Most often, only one of these organ systems is affected, but involvement of two or more is also possible.

The diagnosis of cutaneous NLE is made with the clinical examination and maternal history, with verification of the presence of maternal anti-Ro autoantibodies. Neonatal lupus disease process is transient and cutaneous lesions resolve completely within weeks or few months. Otherwise, complete heart block, once established, is permanent. [4] This is a common and severe manifestation of neonatal lupus and the majority of cases require pacemaker implantation. There are studies that indicate that treatment of anti-Ro-positive mothers with corticosteroid early in pregnancy may prevent heart block

Eruptive pruritic papular porokeratosis

A 63-year-old Chinese man was referred for a papulosquamous eruption affecting his buttocks, groin, and forearms, which had been recurring over the past 13 years. He gave the history of repeated acute episodes of intense pruritus accompanied by the appearance of new lesions. There were no known triggers for the exacerbations, he was not known to suffer from any chronic illnesses, and he was not on any concurrent medications. Physical examination of the buttocks revealed multiple erythematous scaly annular plaques with ridged edges forming collarettes of scales, interspersed with older annular hyperpigmented macules. Similar clinical findings were also noted on examination of his forearms.

Oblique parakeratotic tiers (i.e. cornoid lamellae) arising within keratin-filled invaginations a. b. c. d. Intracorneal neutrophils not present. Irregular epidermal hyperplasia Absent granular layer and dyskeratotic cells. Superficial perivascular lymphocytic infiltrate was seen in the upper dermis

EPPP was first described by Kanzaki et al as a variant of disseminated superficial porokeratosis (DSP). The latter is classically an asymptomatic dermatosis. EPPP differs from DSP in this respect, as it clinically presents with repeated flares of intense pruritus together with the appearance of new lesions, as with our patient. Histological features are identical in both conditions

EPPP is a rare variant of porokeratosis with only around 10 cases previously reported in the English literature. Etretinate was used in a patient resulting in remission after one year. response to therapy is variable.
[5]

In general, however,