Marsha L. Chaffins

Treatment of pemphigus and linear IgA dermatosis with nicotinamide and tetracycline: A review of 13 cases

Pemphigus usually requires long-term therapy with oral corticosteroids, which can cause significant morbidity. 1, 2 Several immunomodulating drugs, such as cyclophosphamide, azathioprine, and gold have proved beneficial as steroid-sparing agents.3, 4 However, these agents also have limited long-term utility because of their potential to induce renal and hepatic dysfunction and bone marrow suppression. Nicotinamide in combination with tetracycline has been reported to be effective for bullous pemphigoid (BP) and linear IgA bullous dermatosis (LABD),S,6 This regimen has the advantage of lower toxicity compared with corticosteroids and immunosuppressant regimens. We have treated 11 cases of pemphigus and two cases of LABD with nicotinamide and tetracycline and report our experience,

Solitary sclerotic fibroma of skin: a possible link with pleomorphic fibroma with immunophenotypic expression for O13 (CD99) and CD34

Background:  Solitary sclerotic fibroma (SF) presents as a well circumscribed dermal nodule, composed of sparse spindle cells with alternating wavy collagen fibers arranged in a storiform pattern. The histogenesis and nature of this histologically distinct lesion are uncertain. Whether this peculiar tumor represents a true hamartoma or a degenerating end of various fibrous lesions such as pleomorphic fibroma (PF), dermatofibroma, or angiofibroma is still controversial. High proliferating index of spindle cells in SF argues against the possibility of being a degenerating end product of another lesion.

Syringolymphoid hyperplasia with alopecia: two case reports and review of the literature

Syringolymphoid hyperplasia with alopecia is an uncommon, but histopathologically distinct, skin disorder that has been reported to occur with and possibly represent a syringotropic variant of cutaneous T-cell lymphoma. We report 2 patients with syringolymphoid hyperplasia with alopecia. Both had CD4-positive infiltrates; 1 also demonstrated loss of CD7. One patient had evidence of T-cell clonality by gene rearrangement studies, but neither had histologic evidence of cutaneous T-cell lymphoma. Because the natural progression of syringolymphoid hyperplasia with alopecia remains to be fully explained, close follow-up of patients is advised.

Immunohistochemical staining of palisading basal cells in Bowen's disease and basal involvement in actinic keratosis: contrasting staining patterns suggest different cells of origin.

Actinic keratosis (AK) and Bowens disease (BD) are common patterns of in situ squamous cell carcinoma of the epidermis. In AK, atypical keratinocytes proliferate in the lower portion of the epidermis including the basal layer. In contrast, BD features atypical squamous cells in all portions of the epidermis but initially leaves basal cells in palisades along the basement membrane. To characterize immunohistochemically keratocyte proliferation in AK and Palisading Basal Cells (PBC) in BD, we stained microarray samples of 45 AK and 25 BD with Molecular Immunology Borstel (MIB-1). Subsequent immunostaining of full mounted sections examined 11 BD, 7 AK, and 4 examples of psoriasis for MIB-1 (as a proliferative marker) and p53 (as a cell cycle regulatory marker). AK stained for MIB-1 and p53 antibodies only in lower portion of epidermis and included the basal layer. BD with typical PBCs stained positive for both markers throughout the epidermis, except for the basal layer. Psoriatic biopsies stained positively for the 2 markers only in the basal and parabasal layers. Normal epidermis adjacent to the lesions in AK and BD biopsies stained sparsely in the basal layers. The correlation of different histologic patterns of epidermal involvement with different immunohistochemical patterns of stains argues for different cells of origin for BD versus AK. Lack of expression of proliferative antigens in palisading basal cells in BD provides evidence that PBCs are not the cell of origin for BD. Conversely in AK, expression of MIB-1 and p53 in basal cells argues that these cells play a role in histogenesis of AK.

Vascular patterns in mature hypertrophic burn scars treated with fractional CO2 laser.

Fractional CO2 laser has recently emerged as a promising therapeutic modality to improve the texture and appearance of burn scars. An issue in many burn scars is persistent erythema, which traditionally has been treated with vascular lasers. Interestingly, fractional CO2 lasers have been shown to improve the appearance of burn scars, including erythema, but no mechanism has been proposed for this change.

Calciphylaxis: A cause of necrotic ulcers in renal failure

Background: Calciphylaxis is a rare and life-threatening condition occurring in patients with end-stage renal disease on dialysis. The diagnosis is frequently delayed or missed and therapy is often unsuccessful. Objective: The clinical and pathological components of calciphylaxis are emphasized in a patient that may facilitate early diagnosis and initiation of therapy. Results: Early recognition of the clinical signs and symptoms of calciphylaxis is crucial. The tissue diagnosis requires large elliptical biopsies because histological findings are segmental and often missed. Conclusion: Early diagnosis and intervention in calciphylaxis is critical. Parathyroidectomy should be considered in all cases.

Manual microdissection technique in a case of subcutaneous panniculitis‐like T‐cell lymphoma: a case report and review

Demonstration of T‐cell receptor gene monoclonality often plays an important role in the diagnosis of T‐cell lymphoma. When a test to detect monoclonality is performed on whole tissue sections, the presence of a reactive lymphocyte population may reduce sensitivity. This may be especially true for early or borderline cases of lymphoma. Microdissection techniques may be utilized to more readily identify a clonal population of lymphocytes. Subcutaneous panniculitis‐like T‐cell lymphoma represents a cutaneous lymphoid neoplasm whose clinical course may vary from an indolent, waxing and waning course to an aggressive course resulting in death. We report the first case of a microdissection technique used to facilitate diagnosing a case of subcutaneous panniculitis‐like T‐cell lympoma.

Histopathologic characteristics of common inflammatory skin disorders

Histopathology is integral to the accurate diagnosis of skin diseases. Nonneoplastic “inflammatory” skin diseases manifest relatively few histologic patterns that may be a source of confusion for the histopathologist. It is important to know the histopathologic reaction patterns that these diseases exhibit and to have a sound, fundamental approach that can be used to arrive at accurate, specific diagnoses. In this article, the histopathologic findings of the most commonly encountered inflammatory diseases of the skin are reviewed, with special emphasis on pattern recognition and clinical correlation.

Laser-Induced Neocollagenesis in Focal Dermal Hypoplasia Associated With Goltz Syndrome in a Girl

Importance Current models of Goltz syndrome cannot estimate the overall neocollagenesis and marked shift in collagen types after ablative fractional laser resurfacing (AFR) within treated areas of focal dermal hypoplasia (FDH). Objectives To clinically improve FDH by using AFR to characterize the specific ratio of collagen types associated with observed clinical changes. Design, Setting, and Participants This case report of a girl with Goltz syndrome used extensive laboratory evaluation and multiple observers blinded to the patient’s clinical status. Serial samples of clinically unaffected skin constituted internal control specimens, with clinical and histologic evaluations performed as part of a multicenter investigation. The analysis tested the hypothesis that thermal microtrauma caused by AFR created a unique environment that activated latent genes, inducing neocollagenesis and allowing the patient to adaptively produce the collagen subtype that was specifically deficient at baseline. Interventions Two AFR treatments were administered within an area of FDH. Histologic comparison of the pretreatment and posttreatment skin was performed using serial internal controls. Main Outcomes and Measures Histologic changes, including Herovici collagen staining to differentiate between types I and III collagen, within a treated area of mosaically affected FDH compared with clinically unaffected skin. Results This female patient presented in the second decade of life with self-described red, itchy skin within a large plaque of FDH on her left posterior thigh and calf. After AFR, skin tightening and symptomatic relief were reported. Histologic findings demonstrated objective thickening of the dermal collagen. A marked shift in collagen predominance from type III (fetal/early wound) to type I (adult/mature) was observed. Conclusions and Relevance Although further study is needed, this report shows promising results and raises important questions about gene expression and the epigenetics of Goltz syndrome–associated mutations and the local effects of AFR. Coupled with more rigorous investigation, this novel technique may help reveal molecular workarounds permitting innovative therapies that take advantage of the subtly different collagens that exist within the skin.

Melanoma Gene Expression Markers for Surveillance of Epidermolysis Bullosa Nevi Malignant Transformation

Discussion | Pseudotumor cerebri is characterized by symptoms of increased intracranial pressure: headache, visual disturbances (blurry vision, diplopia), nausea and vomiting, and stiff neck.1 It is commonly idiopathic, primarily affecting women of childbearing age who are overweight. Less frequently, it can occur in association with other disease conditions (usually endocrinologic abnormalities such as polycystic ovarian syndrome).2 Despite many anecdotal reports of drug-associated PTC, there are 4 primary drug classes that are considered by experts to have a true association with PTC1,3: (1) tetracyclines, including doxycycline and minocycline; (2) oral retinoids; (3) recombinant growth hormone; and (4) corticosteroids (following withdrawal). Cross-reactivity between these drug classes in patients with drug-associated PTC has not been reported. Symptoms usually regress completely after discontinuation of the drug therapy.1,4 The pathophysiological mechanisms of drug-associated PTC are not fully understood. It has been postulated that tetracyclines may reduce cerebrospinal fluid outflow at the arachnoid villi, while oral retinoids (through excess retinols and retinol-binding protein) may be directly toxic to arachnoid villi function.5,6 We report safe use of isotretinoin in 3 patients with histories of drug-associated PTC. When acne warrants isotretinoin treatment, a patient history of PTC should not preclude its use if careful surveillance for PTC recurrence can be conducted. Baseline evaluation by ophthalmology and/or neurology services should be considered.