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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 25, Issue 6, 2019

Review

Cutaneous and systemic IgG4-related disease: a review for dermatologists

Immunoglobulin G4-related disease (IgG4-RD) is a chronic inflammatory condition characterized by IgG4+ plasma cell infiltration of the skin and other organs. Cutaneous forms of the disease may be under recognized owing to poorly defined diagnostic criteria and relatively recent recognition in the literature. The aim of this review is to describe the clinical, histological, and serological presentations of cutaneous IgG4-RD, and to provide an overview of its systemic manifestations for dermatologists. Cases of cutaneous IgG4-RD identified in the literature review were compared to control cases. Clinically, plaque morphology and systemic involvement of the orbit, submandibular gland, lacrimal gland, and parotid gland were associated with a diagnosis of cutaneous IgG4-RD. Histologically, lymphoplasmacytic infiltrate and percentage of IgG4+ plasma cells/IgG+ plasma cells > 40% were associated with the diagnosis. Serologically, neither elevated serum IgG4 nor IgE concentrations were associated with the diagnosis. Dermatologists should consider IgG4-RD as part of the differential diagnosis for nodules, papules, and plaques with an IgG4+ plasma cell infiltrate, especially in middle-aged and elderly males with systemic manifestations of the disease. Diagnosis requires thorough investigation of both cutaneous and systemic clinical and histological presentations.

Cutaneous manifestations of genodermatoses and primary immunodeficiency

Immunodeficiency is most commonly related to inherited syndromes, infections, chemotherapy, or aging. As the population of individuals with immunodeficiency continues to grow, physicians are confronted with the task of diagnosing dermatologic disease in this population. Cutaneous involvement in immunodeficiency disorders serves as a useful tool that aids diagnosis and provides prognostic value. Given that cutaneous manifestations often herald an underlying immunodeficiency disorder, understanding the complexities of these diseases is important for appropriate clinical management. Although certain diseases may present with stereotypical cutaneous lesions, others may involve more variable lesions that require specialized consultation for diagnosis and treatment recommendations. In this review, we discuss a number of cutaneous findings associated with primary immunodeficiencies. Awareness of these cutaneous associations may aid in the early detection and prompt treatment of these potentially serious immunologic disorders.

Malacoplakia of the skin: overview of a rare clinical entity

Background: Malacoplakia is a rare acquired, infection-related granulomatous disorder, that may affect many systems, but typically occurs in the urinary tract. Cutaneous involvement is less prevalent, and most commonly presents with a perianal or genital region localization. Cutaneous malacoplakia is believed to be caused by an acquired bactericidal defect of macrophages in the setting of chronic infections and immunocompromised states. A diagnosis of cutaneous malacoplakia should be considered when encountering non-specific granulomatous lesions that are refractory to treatment. Histologic findings are marked by the presence of foamy macrophages containing the pathognomonic Michaelis-Gutman bodies. Objectives. The aim of this review is to discuss the current perspectives on the pathophysiology, clinical features, diagnosis, and treatment of this disease. We would also like to emphasize that the integration of clinical information, microscopic findings, and exclusion of other cutaneous granulomatous processes is necessary to accurately diagnose this exceedingly rare disease and provide opportunity for therapeutic intervention. Patients/Methods. Data for this work were collected from the published literature and textbooks. Results. Combined surgical excision and protracted antibiotic courses appear to have the highest success rate. Antibiotics should be culture specific, but drugs that easily permeate the macrophages appear to be the best choice.

Commentary

Decreased phototherapy effectiveness on lower body

Although phototherapy is an effective treatment for many dermatological conditions on the face, trunk, and proximal extremities, a common issue plaguing whole-body phototherapy is its diminished efficacy on the legs. In this commentary, we elaborate on the factors underlying this phenomenon, as well as potential solutions to improve treatment success.

Variable potential for social media platforms in raising skin cancer awareness

Social media plays an important role in public health outreach, given its ability to connect virtual communities. Examples include campaigns for smoking cessation, breast cancer awareness, and emergency relief [1]. Few studies have investigated the utility of social media in skin cancer awareness, though the potential has been demonstrated [2]. We sought to evaluate current differential social media platform use in skin cancer awareness.

Case Presentation

Discordant molecular tests for acute Graft-Versus-Host Disease after liver transplant: FISHing for the proper diagnosis

We report cutaneous fluorescent in-situ hybridization (FISH) analysis as a useful test for earlier diagnostic confirmation in a case of acute graft-versus-host disease (GVHD) in a sex-mismatched liver transplant patient compared to the gold standard peripheral blood short tandem repeat chimerism analysis.

Apocrine adenocarcinoma in the setting of apocrine hidrocystoma of the leg

Apocrine hidrocystoma is a benign, cystic lesion often presenting in the periorbital region. Apocrine adenocarcinoma is the rare, malignant counterpart occurring mainly in the axilla and anogenital region. There is a paucity of literature on both entities and co-occurrence has been reported in only 5 cases. We present the case of a 48-year-old man with a history of total body irradiation for chronic myelocytic leukemia, diabetes mellitus, and obesity who presented with a calf mass of two years' duration. Epidermal inclusion cyst was presumed and excisional biopsy was carried out. Pathologic analysis revealed apocrine adenocarcinoma in the setting of a precursor apocrine hidrocystoma. Our patient's unique altered immunity and the direct effects of irradiation on the local microenvironment may have resulted in his rare presentation of co-occurrence of apocrine adenocarcinoma within an existing apocrine hidrocystoma. To our knowledge, our patient is the first reported patient with this presentation in the lower extremity.

Amyloidosis cutis dyschromica: report of 3 cases

Amyloidosis cutis dyschromica (ACD) is a rare pigmentary disorder with about 50 cases having been reported in the English literature. Only one case of ACD has been reported from Iran. We present three patients who presented with generalized hyper- and hypopigmented patches, sparing face, hands, and feet in all three cases. The presence of amorphous eosinophilic deposits in the papillary dermis confirmed the diagnosis of ACD; the deposits were stained by crystal violet in the histopathological examination of the lesions. In all three cases, similar lesions were present in some of the family members. ACD should be considered in the differential diagnosis of diffuse hyperpigmentation studded with hypopigmentation, especially when beginning in childhood.

Bullous acral eruption related to secukinumab

Skin reactions related to secukinumab are uncommon. Although the initial phase 3 studies of this medication reported infection and urticaria as adverse cutaneous events, other cases of unique adverse events have since been reported. We are presenting a patient who developed an exuberant hand and foot reaction after starting secukinumab.

Circle hair: report of two cases and brief review of the literature

Circle hair (CH) is an interesting subtype of ingrown hair, characterized by the growing of hair shaft in a spiral or circular morphology underneath a translucent layer of stratum corneum, parallel to skin surface. In contrast to rolled hair (RH), neither perifollicular inflammation nor abnormal follicular keratinization are known to accompany CH. The reason why the hair shaft grows circumferentially and transversely under the skin instead of emerging through an apparently open hair follicle ostium and growing vertically remains to be determined. Although CH is a frequent benign incidental finding in normal skin examination, reports on this disorder are scarce. Herein we report two cases of CH and briefly review the existing literature. We believe that CH develops because of trauma in patients having a genetic susceptibility for this disorder and that CH is more common than the relevant medical literature suggests.

Low-dose naltrexone: a unique treatment for amyopathic dermatomyositis

Gottron papules, a heliotrope rash, scalp and extremity erythema, pruritus, and fatigue are the characteristic signs and symptoms of amyopathic dermatomyositis (ADM). Amyopathic dermatomyositis is considered a distinct entity from dermatomyositis (DM) because the characteristic muscle weakness and muscle enzyme elevations of DM are absent in ADM. With respects to treatment, ADM treatments have traditionally included topical corticosteroids and/or systemic immunosuppressants and immunomodulators. Herein we present a patient with refractory ADM that was responsive to low-dose naltrexone therapy.

Bullous leukemia cutis: a rare clinical subtype

Leukemia cutis represents infiltration of the skin by malignant leukocytes and typically presents as firm, red-brown papules and nodules. The bullous clinical subtype is considered a rare entity and can be a diagnostic challenge. This case describes a patient with bullous leukemia cutis mimicking vesiculobullous skin disease.

Photo Vignette

Warfarin-induced skin necrosis within psoriatic plaques

A myriad of different phenomena exist in the dermatological literature which are based on the concept of locus minores resistentiae. The most commonly described phenomenon is the Koebner phenomenon, which is classically associated with the emergence of psoriatic lesions post trauma. Warfarin-induced skin necrosis (WISN) is a rare but severe side effect that leads to necrosis of the skin, predominantly on areas with increased subcutaneous fat. The presented case reports on WISN within psoriatic plaques.

Hidradenitis suppurativa associated with sorafenib initiation

Sorafenib is a multi-kinase inhibitor approved for the treatment of renal cell and hepatocellular carcinoma. Adverse cutaneous reactions are a very common side effect of the medication. We report the development of hidradenitis suppurativa (HS) in a patient after initiation of treatment with sorafenib. HS is marked by recurrent deep painful nodules, fluctuant abscesses, and draining sinus tracts most frequently occurring in the groin and axilla. To our knowledge, sorafenib-induced HS in the axillary and inguinal skin folds has not been previously reported.

Carcinosarcoma of the hand

Carcinosarcomas are rare malignant tumors derived of both epithelial and mesenchymal elements. Herein, we report an elderly man originally diagnosed with a squamous cell carcinoma of the hand. Upon excision, the tumor was found to be a more aggressive carcinosarcoma. Immunohistochemical stains revealed that the sarcoma component of the lesion was vimentin positive, whereas the primary carcinoma tumor cells were positive for p63 and CK903. Both components were negative for CD34 and D2-40. This tumor was found to have angiolymphatic invasion and eventually metastasized to the axillary lymph nodes and lungs.

Letter

Comment on “Botulinum toxin type-A as an alternative treatment for gummy smile: a case report”

Currently, the search for esthetic excellence has become the main objective in the facial treatment. The gummy smile is one of the complaints from the patients, since this situation may influence their self-esteem and social relationship. The development of new techniques, such as the application of botulinum toxin, may be a conservative therapeutic option in the treatment of gummy smile. The purpose of this letter is to comment some points about a case report publicated in Dermatology Online Journal, of a patient with dentogingival discrepancy and severe gummy smile, who was treated with application of botulinum toxin in order to optimize the harmony of the smile.