Diya F. Mutasim

Paraneoplastic pemphigus. An autoimmune mucocutaneous disease associated with neoplasia.

Paraneoplastic pemphigus is a newly recognized disease that occurs in some patients with lymphoproliferative neoplasms and occasionally, solid tumors. Patients present with an acute illness of the mucosa and skin that shares clinical and histologic features with erythema multiforme, toxic epidermal necrolysis, and pemphigus vulgaris. These patients have antibodies against a complex of epithelial proteins that are present in desmosomes and hemidesmosomes. The course is usually fatal, except in some patients who undergo total resection of their neoplasm.

Skin manifestations of inflammatory bowel disease

Ulcerative colitis (UC) is an inflammatory disorder of the colon that is associated with several extraintestinal manifestations in multiple organs. Several mucous membrane and skin disorders occur in patients with UC. These disorders are not unique to UC and often occur secondary to other causes or in the absence of an apparent cause. One or more such disorders may occur together in association with UC. Mucous membrane and skin disorders may antedate, occur with, or postdate the onset of UC. The dermatologist plays an important role in suspecting the diagnosis of UC that presents with associated mucous membrane or skin disorders. This review covers the clinical presentation, differential diagnosis, workup, and management of selected mucocutaneous manifestations in UC.

Chronic eyelid lymphedema and acne rosacea: Report of two cases

OBJECTIVE The authors describe the clinical findings and surgical treatment of two patients affected by chronic eyelid lymphedema associated with facial acne rosacea. DESIGN Two interventional case reports. METHODS AND INTERVENTION: The clinical diagnosis of acne rosacea was based on the physical examination and confirmed by the histopathologic findings obtained from biopsy of the involved tissue. Surgical treatment was required to address the disfiguring chronic eyelid lymphedema and to correct the resultant mechanical lower eyelid ectropion in both patients. RESULTS Surgical debulking of the affected soft tissue resulted in very satisfactory cosmetic and functional improvement in both patients. CONCLUSIONS To our knowledge, this is the first series of cases of chronic eyelid lymphedema secondary to acne rosacea reported in the ophthalmic literature. Six similar cases have been described previously in the dermatologic literature; all of which had been treated medically without satisfactory results. Surgical debulking of the involved eyelids should be considered in patients affected by persistent symptomatic rosacea lymphedema.

A practical guide for serologic evaluation of autoimmune connective tissue diseases

UNLABELLED Serologic testing is important in the evaluation of patients with autoimmune connective tissue diseases (CTD). There are many techniques. Each of the tests has different sensitivity and specificity with varying diagnostic value. These serologic tests detect antibodies to numerous cellular components. The diagnostic significance and specificity of each antibody vary. Choosing the appropriate test and understanding its clinical utility is an important aspect in the diagnostic evaluation of patients with CTD. (J Am Acad Dermatol 2000;42:159-74.) LEARNING OBJECTIVE At the conclusion of this learning activity, participants should be familiar with the various serologic tests for CTD, should understand the associations of specific antibodies with individual CTD, and should identify the factors that influence the predictive value of these serologic tests.

Melanocytic Nevi of the Auricular Region Histologic Characteristics and Diagnostic Difficulties

We examined auricular melanocytic nevi to evaluate their architectural and cytologic features.A retrospective analysis of 21 auricular melanocytic nevi was conducted during 3 years. The nevi were evaluated for cytologic atypia, architectural disorder, location of epidermal nests, pagetoid spread, growth pattern (symmetry versus asymmetry), demarcation of lateral borders, and host response (lymphocytic infiltrate and/or lamellar fibroplasia).Eleven cases (52.4%) measured at least 6 mm in greatest dimension and 6 cases (28.6%) were asymmetric. Fourteen cases (66.7%) were ill demarcated. Pagetoid spread was present in 12 cases (57.1%). Ten cases (47.6%) showed moderate to severe cytologic atypia. Nucleoli were prominent in 9 cases (42.8%). None of the cases showed mitoses or apoptotic melanocytes.Auricular melanocytic nevi, like those occurring in the acral, flexural, and genital areas, may exhibit some histologic features commonly found in melanomas. Careful histologic interpretation of these lesions is recommended.

A case of intraepidermal Merkel cell carcinoma within squamous cell carcinoma in-situ: Merkel cell carcinoma in-situ?

We report a case of a 79-year-old Caucasian male who presented with a wrist lesion of combined intraepidermal Merkel cell carcinoma and squamous cell carcinoma in-situ. The two tumors were tightly admixed and distinct, and both were without any dermal or invasive components. No features of transition between the two tumors were seen. We suggest the term Merkel cell carcinoma in situ for tumors that demonstrate exclusive intraepidermal proliferation of neuroendocrine cells.

Herpes simplex virus infection masquerading as condyloma acuminata in a patient with HIV disease

Summary Verrucosa lesions in patients with human immunodeficiency virus (HIV) disease may be caused by viruses till her than the human papilloma virus. We describe a 32‐year‐old HIV‐positive black man who presented with a verrucosa lesion of the interlineal cleft that clinically resembled condyloma acuminata or verrucosa carcinoma. Histopathological examination revealed the changes f herpes virus infection, and culture of the tissue confirmed the presence of herpes simplex virus. Human papillomavirus was not detected by in situ hybridization or the polymerase chain reaction. Significant regression of the lesion was seen after 6 weeks of treatment with oral acyclovir.

Autoimmune bullous dermatoses in the elderly: an update on pathophysiology, diagnosis and management.

Elderly individuals are susceptible to autoimmune bullous dermatoses (ABDs), which may be associated with high morbidity and mortality. ABDs result from an autoimmune response to components of the basement membrane zone at the dermal-epidermal junction or desmosomes.Bullous pemphigoid results from autoimmunity to hemidesmosomal proteins present in the basement membrane of stratified squamous epithelia. Patients present with tense blisters in flexural areas of the skin. Mild disease may be treated with potent topical corticosteroids, while extensive disease usually requires systemic corticosteroids or systemic immunosuppressive agents such as azathioprine. Mucosal pemphigoid affects one or more mucous membranes that are lined by stratified squamous epithelia. The two most commonly involved sites are the eye and the oral cavity. Lesions frequently result in scar formation that may cause blindness. Patients with severe disease or ocular involvement require aggressive therapy with corticosteroids and cyclophosphamide.Epidermolysis bullosa acquisita results from autoimmunity to type VII collagen in the anchoring fibrils of the basement membrane. Lesions may either arise on an inflammatory base or be non-inflammatory and result primarily from trauma. Treatment options include corticosteroids, dapsone, ciclosporin, methotrexate and plasmapheresis/immunoapheresis.Paraneoplastic pemphigus results from autoimmunity to multiple desmosomal antigens. The disorder is associated with neoplasms, especially leukaemia, lymphoma and thymoma. Patients present with stomatitis and polymorphous skin eruption. The disease may respond to successful treatment of the underlying neoplasm or may require immunosuppressive therapy.

Cutaneous arteritis presenting with hyperpigmented macules: macular arteritis

Macular arteritis is a novel form of cutaneous arteritis in which the primary lesion is a hyperpigmented macule. Traditional stigmata of cutaneous vasculitis such as palpable purpura and erythematous nodules are not present. The disease is asymptomatic and appears to follow an indolent course. Systemic involvement has not been observed.

Patch granuloma annulare : Clinicopathologic study of 6 patients

BACKGROUND Granuloma annulare is a common skin disorder that usually presents with smooth papules arranged as annular plaques. Variants, such as disseminated, subcutaneous, and perforating granuloma annulare, have been described. OBJECTIVE The purpose of this study is to describe the clinical and histologic features of a distinct patch form of granuloma annulare. METHODS The clinical and histologic features of 6 patients with patch granuloma annulare were evaluated. RESULTS Six women 27 to 72 years of age had patches on the extremities. Two patients also had a lesion on the trunk. Only one patient had annular patches. Histologic examination showed an interstitial infiltrate of lymphocytes and histiocytes with diffuse necrobiosis. CONCLUSION Patch granuloma annulare is a distinct variant with rather subtle clinical and histologic features. A high index of suspicion both clinically and histologically aids in making the diagnosis.