Leprosy as a Diagnostic Challenge in the United States.

A 63-year-old woman from Central Florida presented to an outside clinic with a 2-year history of a progressive, asymptomatic cutaneous eruption and arthralgias. Her past medical history was significant for reported seronegative rheumatoid arthritis, for which adalimumab, methotrexate, and low-dose prednisone therapy were initiated 5 years prior. The skin eruption occurred shortly after a 4-week hospitalization during which these medications were withheld. At her initial outside evaluation, a biopsy was performed and interpreted as subacute cutaneous lupus erythematosus (SCLE). She was treated with hydroxychloroquine without improvement. A repeat biopsy was reported as consistent with interstitial granulomatous dermatitis (IGD). There was no improvement with potent topical corticosteroids.

Diagnostic utility of plasmacytoid dendritic cells in dermatopathology.

Differentiating cutaneous diseases that mimic each other clinically and histopathologically can at times be a challenging task for the dermatopathologist. At the same time, differentiation of entities with overlapping features may be crucial for patient management. Although not seen in normal skin, plasmacytoid dendritic cells usually infiltrate the skin in several infectious, inflammatory/autoimmune and neoplastic entities. Plasmacytoid dendritic cells can be identified in tissue using specific markers such as CD123 and/or blood-derived dendritic cell antigen-2. Plasmacytoid dendritic cells are the most potent producers of type I interferons and their activity may therefore be assessed indirectly in tissue using human myxovirus resistance protein A, a surrogate marker for type I interferon production. In recent years, accumulating evidence has established the utility of evaluating for specific plasmacytoid dendritic cell-related parameters (plasmacytoid dendritic cell content, distribution and clustering and/ or human myxovirus resistance protein A expression) as a diagnostic tool in differentiating cutaneous diseases with overlapping features such as the alopecias, lupus and its mimics, and neoplastic entities. In this review, we provide an update on the current evidence on this topic and on the contexts where this can be a useful adjunct to reach the histopathological diagnosis.

"PRE-COLUMBIAN MOULAGES". HUACOS, MUMMIES AND PHOTOGRAPHS IN THE INTERNATIONAL CONTROVERSY OVER PRECOLUMBIAN DISEASES, 1894-1910.

By the late nineteenth century an international controversy arose referred to the probable existence of certain diseases such as leprosy, syphilis and lupus in pre-Columbian America. Led by the American physician Albert Sidney Ashmead (1850-1911), it brought together scholars from Europe and the Americas. In this context, certain types of Peruvian archaeological pottery and "mummies", along with series of photographs illustrating the effects of these diseases in contemporary patients, met a prominent role as comparative evidence. In this article we analyze how this type of collections were used as evidence in the debates about pathologies of the past, an issue that from a historical standpoint have received considerably little attention.

Thalidomide and analogues: potential for immunomodulation of inflammatory and neoplastic dermatologic disorders.

Thalidomide and analogues are a class of immunomodulatory drugs or IMiDS. Thalidomide was initially approved by the U.S. Food and Drug Administation for treatment of erythema nodosum in leprosy and is now approved for multiple myeloma as well. A second generation IMiD, lenalidomide, is also approved for multiple myeloma and refractory myelodysplastic syndrome. Discovery of this class of drugs has been serendipitous and empirical, as the drug targets have been unknown. In this review, the authors integrate recent identification of drug targets of IMiDS, which include the inducible form of nitric oxide synthase (iNOS), Rho GTPase and caspase-1, with the developments in the understanding of the molecular biology of human inflammatory, infectious and neoplastic skin disorders. Because thalidomide reemerged through leprosy, the original disease classified by the T cell, the authors have also emphasized advances in the understanding of T-cell subsets in human skin disorders.

Lupoid cutaneous leishmaniasis: a report of 16 cases.

BACKGROUND: Lupoid cutaneous leishmaniasis (CL) is known as the chronic form of CL. However, keeping its clinical presentation in view, there is a need to revisit this form of disease. AIMS: To redefine/describe lupoid CL in view of clinical features. METHODS: It was a case series seen in Muzaffarabad (Pakistan) from Jan 2006 to May 2008. All patients clinically suggestive and consistent with laboratory diagnosis of CL were registered. Patients of all age groups and either sex having cutaneous lesions resembling lupus vulgaris or lupus erythematosus on the face or elsewhere were included in the study. Those having chronic fluctuating/relapsing course or scarring within the lesions were also included. Various demographic features of the patients and clinical patterns were recorded. Descriptive statistics were used for analysis. RESULTS: Of 254 registered patients of CL, 16 (6.3%) were diagnosed as lupoid CL. None of the patients had scarred lesions. Age ranged from 38 to 75 (55 + 15.11) years and duration of lesions varied from 4 to 32 (14.25 + 07.59) weeks. All patients had lesions over the face. Thirteen (81.25%) had a large solitary plaque extending over the nose and a large part of the cheeks and three (18.75%) had multiple lesions. Lesions were central/nasal in two (12.5%), unilateral/asymmetrical in four (25%) and bilateral/symmetrical in 10 (62.5%). Morphological patterns included erythematous/infiltrated (7), psoriasiform (6), ulcerated/crusted (2) and Discoid lupus erythematosus (DLE)[G1] like (1). CONCLUSION: Lupoid CL is not strictly a chronic form of disease, which presents on the face from the very onset and shows no scarring or recurrence.

Thalidomide: an experience in therapeutic outcome and adverse reactions.

BACKGROUND: The US FDA-approved thalidomide for the treatment of chronic recurrent/severe erythema nodosum leprosum. Thalidomide is also useful in many other inflammatory dermatological conditions where patients have exhausted other treatment options. METHODS: The beneficial and adverse clinical effects of thalidomide were studied in 25 patients suffering from different inflammatory dermatological conditions that were poorly controlled with conventional therapies. RESULTS: Thalidomide was found to be effective in various inflammatory dermatological diseases other than chronic recurrent erythema nodosum leprosum such as Behçet's disease, disseminated and hypertrophic discoid lupus erythematosus, erosive lichen planus, discoid lupus erythematosus-lichen planus overlap, recurrent aphthous stomatitis and prurigo nodularis. Deep vein thrombosis due to thalidomide occurred in 20% of these patients and appears to be a significant side effect. CONCLUSION: Thalidomide appears promising in a number of inflammatory dermatological conditions and will probably find new usages in future. The treating physicians need to be wary of the thrombo-embolic complications due to thalidomide especially when glucocorticoids or other chemotherapeutic agents such as doxorubicin, gemcitabine, 5-fluorouracil or dexamethasone-cyclophosphamide pulse therapy are being used concomitantly, and in patients of metastatic renal carcinoma, myelodysplastic syndrome or multiple myeloma receiving thalidomide/chemotherapy. Antiphospholipid or anticardiolipin antibodies appear to be other possible risk factors for this complication.

Cutaneous neonatal lupus erythematosus with unusual features.

A three month-old boy was brought by his mother with complaints of multiple reddish lesions on his trunk and face since birth. The patient had erythematous annular plaques with scaling on his extremities, palms and soles with periorbital erythema and edema giving the characteristic "eye mask" or "owl's eye" appearance. His mother did not have history of any illness. Hemogram, liver and renal function tests were within normal limits. A skin biopsy was suggestive of subacute cutaneous lupus erythematosus. Immunological work-up was positive for antinuclear antibodies (ANA) (1:40) with anti-Ro titers of 3.4 and 3.47 (>1.1 = clinically significant titre) in the mother and child respectively, although negative for anti-La antibodies. The child's electrocardiogram and 2D echocardiography were normal. We are presenting a case of anti-Ro-positive cutaneous lupus erythematosus with an uncommon skin manifestation.