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.
The paradoxical adverse effects of tumor necrosis factor-alpha (TNF-alpha)
antagonists have been described frequently as a result of the widespread use of these
drugs. Among the TNF-alpha blocking agents, few reports exist relating the use of
adalimumab in cutaneous sarcoidosis, although all of them show good results. More
recently, sarcoidosis onsets have been reported with various TNF-alpha inhibitors.
The current case is, to our knowledge, the first to describe the exacerbation of
cutaneous lesions of sarcoidosis treated with adalimumab.
Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases
and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We
present the case of a 72-year-old man with multiple myeloma in complete remission
since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was
consistent with plasmacytoma. The patient was treated with melphalan, prednisolone
and radiotherapy. Despite optimal therapeutic response of the lesions, the disease
progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous
metastatic plasmocytomas were the first sign of progression of the disease.
Multiple autoimmune syndrome is a rare condition, described by Humbert and Dupond in
1988. It is defined by the association of at least 3 autoimmune diseases in the same
patient. Vitiligo is the most common skin condition in this syndrome. This article
presents the case of a 31-year-old male with vitiligo, alopecia areata, Crohn's
disease, psoriasis vulgaris and oral lichen planus. The rarity of this case is
highlighted by the coexistence of four autoimmune skin diseases in association with
Crohn's disease, never described in the literature.
This case illustrates a rare cause of leg ulcers and documents that IVIg may be an effective adjuvant treatment in the management of selected patients with antiphospholipid syndrome when conventional strategies using subcutaneous heparin and low-dose aspirin are insufficient.
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