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Myelodysplastic Syndrome Presenting as Generalized Granulomatous Dermatitis
Granulomatous dermatitides such as granuloma annulare, sarcoidosis, interstitial granulomatous dermatitis, necrobiosis lipoidica, and rheumatoid nodules are characterized by a dermal inflammatory infiltrate composed largely of histiocytes. In this case report, Balin et al describe an illustrative case of generalized granulomatous dermatitis as the heralding symptom of an underlying myelodysplastic syndrome (MDS) that progressed to acute leukemia, suggesting that clinicians should evaluate patients with unexplained granulomatous skin eruptions for MDS.
Congenital Epidermolysis Bullosa Acquisita
Epidermolysis bullosa acquisita (EBA) is a rare, chronic, autoimmune bullous dermatosis caused by autoantibodies against type VII collagen. In this case report, Abrams et al describe a neonate with transient neonatal EBA due to the passive transfer of maternal autoantibodies. The infant's mother had been diagnosed as having EBA 1 year before the infant was born. She had discontinued immunosuppressive therapy during her first trimester of pregnancy. Her daughter was born with multiple superficial and deep erosions on the face, chest, abdomen, and extremities, and histopathologic analysis, immunofluorescence studies, and enzyme-linked immunosorbent assay (ELISA) confirmed the diagnosis. With the expectation of spontaneous tapering in circulating antibody levels, immunosuppressive therapies were withheld in favor of supportive treatments. At age 2 months, all erosions were healed.
Remission in Dermatitis Herpetiformis
Dermatitis herpetiformis (DH) is an intensely pruritic autoimmune blistering disease associated with celiac disease. Treatment generally involves sulfone or sulfapyridine therapy and a strict restriction of gluten from the diet. Although other autoimmune blistering diseases may go into remission, the long-term prognosis for DH remains unclear. In this retrospective cohort study, Paek et al demonstrate that 12% of patients with DH went into remission. Factors associated with DH remission included age of onset older than 39 years. These data suggest that clinicians should continually reevaluate the need for medical therapy and a gluten-free diet for their patients with well-controlled DH.
Generalized Acquired Cutis Laxa Associated With Multiple Myeloma With Biphenotypic IgG-λ and IgA-κ Gammopathy Following Treatment of a Nodal Plasmacytoma
Cutis laxa (CL) is a rare dermatosis resulting in loose, wrinkled, redundant skin secondary to defects in dermal elastic tissue. Acquired CL (ACL) has a later onset and more generalized involvement than the inherited form. In this case report, New and Callen describe a 48-year-old man who developed ACL and granuloma annulare–like lesions as a paraneoplastic manifestation of early multiple myeloma years after being treated for a lymph node plasmacytoma. The cutaneous findings prompted further systemic investigation leading to the diagnosis of his early myeloma, suggesting that evaluation for the presence of a gammopathy with immunofixation electrophoresis should be considered in the setting of ACL.
Usefulness of BP230 and BP180-NC16a Enzyme-Linked Immunosorbent Assays (ELISAs) in the Initial Diagnosis of Bullous Pemphigoid
Bullous pemphigoid (BP) is the most common subepidermal autoimmune blistering disorder. Autoantibodies predominantly recognize BP230 and BP180, 2 structural components of the hemidesmosomal complex. Serum autoantibodies can be detected by indirect immunofluorescence (IIF) techniques, but these methods are difficult to standardize. Several ELISAs have been developed. In this large retrospective study, Charneux et al find no relationship between a positive BP230 score and disease extent at diagnosis but demonstrate that serum anti–basement membrane zone autoantibodies are more frequently detected on IIF with a positive BP230 score. The BP180 ELISA result was associated with disease extent at diagnosis and the mean number of blisters. The BP230 ELISA should not be systematically performed but reserved for cases with atypical features or in typical BP with negative BP180 ELISA findings.
This Month in Archives of Dermatology. Arch Dermatol. 2011;147(3):278. doi:10.1001/archdermatol.2011.34
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