We describe a 7-year-old boy with dermatitis herpetiformis (DH) diagnosed on clinical and histologic evidence, negative direct immunofluorescence (DIF) findings for junctional IgA deposits in uninvolved skin, positive IgA endomysial and gliadin antibodies, and jejunal biopsy revealing a gluten-sensitive enteropathy. Treatment with dapsone led to the disappearance of cutaneous lesions and pruritus within 48 hours. Demonstration of IgA immune deposits in the dermal papillae has been the only acceptable criterion for the diagnosis of dermatitis herpetiformis. However, considering several reports in the literature of DH with a negative DIF and our own case, we believe that in the absence of the characteristic DIF pattern, one needs the combination of clinical, histologic, and immunologic data to support the diagnosis of DH. We also discuss recent developments in the diagnosis of DH.

Dyskeratosis congenital is reported in two siblings. They presented with the classic triad of mucocutaneous features: leukoplakia of the tongue, dystrophic nails, and a widespread reticulate pigmentation on the neck and upper chest. A genetic analysis was performed and a new missense mutation S356P, hemizygous, was identified in the DKC1 gene in both patients. Acitretin was started at a low-dose in both patients, resulting in clinical improvement and important, positive psychosocial effects.

We report a 4-year-old Caucasian girl with congenital skin lesions consisting of hyperpigmented macules that were bilateral and roughly symmetrical, covered with terminal hair and located on the scapular regions, shoulders, and arms. A cutaneous biopsy specimen showed the histologic pattern of Becker nevus. We discuss the classification of this case in the context of the published literature. CASE REPORTA 4-year-old Caucasian girl was born after a normal pregnancy and delivery under medical surveillance without complications. Her parents were in the third decade of life, nonconsanguineous, and had no significant health problems. During pregnancy the mother took no drugs except for supplements of iron and folic acid prescribed by her doctor. There were no relevant familial cases of cutaneous abnormalities.This child was observed in our dermatology department for the first time at the age of 4 years for congenital skin lesions on the scapular regions, shoulders, and arms consisting of bilateral and roughly symmetrical hyperpigmented macules with well-delimited, regular borders, covered with terminal hair (Figs. 1 and 2). There was also a small satellite lesion on the left mammary region. These skin lesions had always been asymptomatic and without evolution since her birth. Mechanical stimulation of these areas did not induce piloerection or any modification on inspection or palpation, observed under tangential lighting. Cutaneous biopsy specimen findings (Fig. 3) were suggestive of Becker nevus and the Masson-Fontana stain confirmed increased pigmentation of the basal layer.The child was otherwise normal, with weight and height in the 50th percentile. No chest or limb asymmetries or any other musculoskeletal defects were found, and her lumbosacral spine films were normal.

There have been several reports of linear IgA dermatosis (LAD) associated with drug exposure and lymphoproliferative malignancy, but trauma and burns have been suggested only in patients with bullous pemphigoid. We present a case of burn-induced LAD in a 48-year-old caucasian male presenting with a recent history of blistering eruption on the periphery of a cicatricial area caused by boiling methyl alcohol. Clinically, he presented a widespread bullous eruption. The direct immunofluorescence examination of a perilesional biopsy revealed an intense homogeneous linear pattern of IgA deposition consistent with the diagnosis of LAD. The patient responded to therapy with systemic steroids.

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