Lynne Morrison

Bullous and cicatricial pemphigoid

Bullous pemphigoid (BP) and cicatricial pemphigoid are blistering mucocutaneous diseases characterized by detachment of the overlying epithelium from its stroma. IgG and complement components are deposited in all affected tissue at the level of blister formation--through the lamina lucida of the epithelium. The primary antibody response is of the IgG 4 subclass, and the antigens recognized by these autoantibodies have been shown to be 230 kD and 180 kD transmembrane proteins unique to the hemidesmosome of stratified squamous epithelial cells. Although it is suspected that these antigens are important in cell-substrate adhesion, this has not been proven. Stanley et al. have recently defined the sequence of a portion of the C terminal end of the 230 kD antigen and Diaz et al. have isolated a cDNA encoding for the 180 kD antigen. Structural data regarding these antigens should prove critical to definition of their presumed function. Therefore, BP is felt to be an autoimmune disease where the cutaneous lesions may solely be a consequence of binding of these antihemidesmosome autoantibodies to the specific antigen, but definitive proof of this assumption is incomplete.

Direct immunofluorescence microscopy in the diagnosis of autoimmune bullous dermatoses

Immunofluorescence (IF) studies are an important part of the laboratory evaluation of immunologically mediated bullous dermatoses and have become standard procedures for accurately diagnosing patients with these disorders.1,2 In general there are three main parameters that should be considered in establishing a diagnosis in this group of diseases: (1) the clinical findings, (2) the histologic features, and (3) the direct and indirect IF studies. Correlation of these three parameters allows the most accurate diagnosis of the immunobullous diseases, and lack of any one of these elements decreases the chance of arriving at a correct diagnosis, which is the cornerstone of optimal therapy. Although IF studies are only one part of the evaluation, they remain an essential part of the laboratory workup in the immunobullous disorders. In deciding when to consider obtaining IF studies, it is helpful to remember that immunobullous diseases can have a variety of presentations. In addition to the more expected findings of bullae, which are characteristically tense in the subepidermal blistering diseases and flaccid in pemphigus, these patients can also present with cutaneous or mucosal erosions alone if all blisters are broken, which is fairly common in the pemphigus group of diseases. Additionally, pruritus without lesions or a purely urticarial eruption for an extended period may be the presenting findings, particularly in bullous pemphigoid. Immunobullous disorders can have exclusively mucosal findings, showing simply oral erythema or desquamative gingivitis, or may have multiple mucous membranes involved with erythema and/or erosions. Individuals with persistent inflammatory mucous membrane disease often will benefit from immunofluorescent studies, since such a presentation can occur in pemphigus, pemphigoid, lichen planus, or lupus erythematosus.3 The persistent nature of the lesions in these disorders helps clinically distinguish them from the typically recurrent nature of oral erosions occurring in aphthae and erythema multiforme. This review summarizes the key IF findings of the major immunobullous diseases and follows an approach based on patterns of IF findings associated with various diseases. While some IF findings are characteristic or diagnostic, others have a differential diagnosis and require thoughtful clinical-pathologic correlation or further testing to establish a correct diagnosis. This approach should help provide a basis for understanding the significance of IF findings and improve meaningful correlation with clinical and histologic findings. A summary of the typical direct IF findings in autoimmune bullous dermatoses is found in Table 1.

Persistence of an allergen in hair after glyceryl monothioglycolate—containing permanent wave solutions

Glyceryl monothioglycolate, a reducing agent used in permanent waving solutions, is a recognized cause of allergic contact dermatitis. Because of an unexplained persistence of dermatitis in beauty shop clients after exposure to permanent wave solutions containing glyceryl monothioglycolate, we looked for the presence of this or a cross-reacting substance in permanent-waved hair as a possible source of continued exposure to the allergen. Seventeen subjects sensitive to glyceryl monothioglycolate underwent patch testing with hair samples collected before and at various times after a permanent wave containing glyceryl monothioglycolate. None reacted to hair that had not received a permanent, while 7 of 17 showed positive reactions to the permanent-waved hair. Positive reactions were seen in hair collected as long as 3 months after the permanent. This suggests that a glyceryl monothioglycolate-related allergen is retained in hair for up to 3 months after the permanent, which may explain the long-lasting dermatitis that occurs in clients sensitive to glyceryl monothioglycolate.

Pemphigoid: Bullous, gestational, and cicatricial

Abstract The term pemphigoid applies to cutaneous diseases that are characterized clinically (1) by the presence of large tense blisters that lack a Nikolsky sign, (2) by subepidermal blister formation with an inflammatory infiltrate usually rich in eosinophils, and (3) by the deposition of IgG and complement components in affected skin or mucous membrane. Adding to the traditional definition of “pemphigoid” is the definition that considers where the immunoreactants are deposited at the ultrastructural level and the definition of the antigens recognized by the autoantibodies. For example, current data indicate that bullous pemphigoid and herpes gestationis are closely related by all the above criteria. The antigens that are recognized by autoantibodies in both syndromes are discussed. Cicatricial pemphigoid is less well understood, and its relationship to these diseases is unclear at present. These diseases provide a significant challenge for future research and potentially will offer insights into immune dysfunction in select patient populations.

Hemorrhagic Bullae in a 73-Year-Old Man

A 73-year-old man with a history of metastatic colorectal cancer presented with multiple, asymptomatic black, crusted nodules on his arms. Two weeks prior, he had received a diagnosis of deep vein thrombosis and was prescribed low-molecular-weight heparin sodium (LMWH) subcutaneous injections (enoxaparin sodium). Five days after starting to receive anticoagulation therapy, he noted multiple blisters on his arms that grew rapidly, occasionally bled, and dried to become crusted nodules. These were distantly located from his abdominal enoxaparin injection sites. Physical examination revealed multiple crusted nodules on nonerythematous skin ranging in diameter from 4 mm to 2 cm on the patient’s arms, back, and scalp (Figure 1). The abdomen and remainder of skin were not affected. Aside from a low hemoglobin level (10.6 g/dL [to convert to grams per liter, multiply by 10.0]), the results of a complete blood cell count and comprehensive metabolic panel were unremarkable. Coagulation studies had normal results. A 4-mm punch biopsy specimen was obtained for histopathologic examination (Figure 2 and Figure 3). What is your diagnosis?

New-onset oral lichen planus and granulomatous cheilitis in a 66-year-old woman

We report a case of a 66-year-old woman with the simultaneous onset of oral lichen planus (OLP) and granulomatous cheilitis (GC). Both diseases are relatively rare, and further investigation for systemic or environmental causes of disease is warranted in new patients with either OLP or GC. This case and reports of a so-called lichenoid and granulomatous dermatitis in the literature beg the question, is there some unifying etiologic explanation for OLP and GC?1

Red Rashes of the Vulva

Red patches and plaques of the vulva may be manifestations of neoplasms, infections, or inflammatory skin diseases. These diseases can mimic one another clinically; features that generally allow the diseases to be identified on most cutaneous surfaces can be altered in the moist, occluded vulvar environment, making clinical diagnosis difficult. A detailed history and thorough physical examination can point to the likely diagnosis, but biopsy and culture may be needed for diagnosis especially in refractory disease. It is not uncommon for several of these processes to be present concomitantly or complicating other vulvar diseases.