Unusual and recently described cutaneous atrophic disorders

Cutaneous atrophic conditions are typically caused by changes in the dermis or subcutaneous tissue, sometimes consisting of the loss of a single fiber type. Since a significant decrease of subepidermal tissue is necessary for these lesions to be macroscopically atrophic, many conditions may not be appreciated as atrophy in the clinical setting. Clinicians should be familiar with the common or classic disorders causing cutaneous atrophy; however, there are a few new or rarely described atrophic conditions which are more difficult to identify and may not be atrophic clinically. This paper serves to describe the salient clinical and histological features of these new or rare disorders.     

Lupus erythematosus: considerations about clinical,cutaneous and therapeutic aspects

Systemic Lupus Erythematosus is a chronic inflammatory disease with multifactorial etiology. Although clinical manifestations are varied, the skin is an important target-organ, which contributes to the inclusion of skin lesions in 4 out of the 17 new criteria for the diagnosis of the disease, according to the Systemic Lupus International Collaborating Clinics. The cutaneous manifestations of lupus are pleomorphic. Depending on their clinical characteristics, they can be classified into Acute Cutaneous Lupus Erythematosus, Subacute Cutaneous Lupus Erythematosus, Chronic Cutaneous Lupus Erythematosus and Intermittent Cutaneous Lupus Erythematosus. Treatment is based on preventive measures, reversal of inflammation, prevention of damage to target organs and relief of adverse events due to pharmacological therapy. The most commonly used treatment options are topical, systemic and surgical treatment, as well as phototherapy. The correct handling of the cases depends on a careful evaluation of the morphology of the lesions and the patient''s general status, always taking into consideration not only the benefits but also the side effects of each therapeutic proposal.     

Never perform laser treatment of skin tumors with clinical “EFG” criteria

Laser treatment is a common procedure for the treatment of cosmetically troubling skin lesions but has the limitation that histopathologic diagnosis is usually not obtained prior to treatment. Laser treatment of melanomas with benign clinical features may delay or make more difficult the correct diagnosis of such tumors. A helpful tool to identify clinically innocent appearing melanomas is the “EFG” rule, summarizing the common clinical features as “elevated, firm skin lesions showing continuous growth”. We report a 42‐year‐old woman who presented with a recurrent and metastatic melanoma after laser treatment of a tumor which was apparently clinically innocent and highlight the clinical features of such benign‐looking melanomas.     

Cutaneous metastases of rectal adenocarcinoma in a herpetiform distribution

We present the case of a 62-year-old male with cutaneous metastases of a rectal adenocarcinoma located on the groin and left thigh. Due to their clinical similarity, the lesions were initially diagnosed and treated as herpes zoster. Cutaneous metastases have variable clinical presentation patterns. They may mimic benign skin lesions like epidermoid cysts, lipomas, erysipelas or, as in our case, herpes zoster.     

Clinical patterns of cutaneous nontuberculous mycobacterial infections

BACKGROUND: Cutaneous nontuberculous mycobacterial infections result from external inoculation, spread of a deeper infection, or haematogenous spread of a disseminated infection. There are two species-specific infections (fish-tank or swimming-pool granuloma, due to Mycobacterium marinum, and Buruli ulcer, caused by M. ulcerans). Most infections, however, produce a nonspecific clinical picture. OBJECTIVES: To define clinical patterns of cutaneous disease in nontuberculous mycobacterial infections. METHODS: Fifty-one patients with cutaneous nontuberculous mycobacterial infections were reviewed. Clinical and histopathological features of normal hosts and immunosuppressed patients were compared. Two subgroups of immunosuppressed patients were distinguished: patients with cutaneous infection and patients with a disseminated infection and cutaneous involvement. RESULTS: In immunosuppressed patients the number of lesions was significantly higher. Abscesses and ulceration were also more frequently observed. Different species were found in normal hosts and immunosuppressed patients. Several clinical patterns of cutaneous infection were defined: lymphocutaneous or sporotrichoid lesions; nonlymphocutaneous lesions at the site of trauma; folliculitis and furunculosis involving the lower extremities; disseminated lesions on the extremities in immunosuppressed patients. Two patterns were observed in patients with a disseminated infection: localized cutaneous lesions and disseminated cutaneous and mucosal lesions. CONCLUSIONS: Cutaneous manifestations of nontuberculous mycobacterial infections may be classified according to criteria such as cutaneous lesions and immune status.     

Necrotic cellulitis. A localized form of septic vasculitis

Cutaneous biopsy specimens from two patients with localized, bacterial, necrotic cellulitis showed a neutrophilic vasculitis with fibrin thrombi, changes indistinguishable from those seen in a third patient with bacterial septicemia. Additional clinical information may be needed before a definitive diagnosis of localized or systemic septic vasculitis can be made with certainty. The clinical and microscopic features of the lesions and their pathologic mechanisms are discussed.     

Sarcoidosis: A great imitator

Sarcoidosis is a chronic multisystemic, inflammatory disease with specific granulomatous cutaneous lesions. The cutaneous form may be considered a “great imitator,” due to its extensive clinical morphology that occurs in upwards of 20% to 35% of patients. Cutaneous lesions may have a variety of presentations including papules, plaques, nodules, infiltrative scars, annular, angiolupoid, psoriasiform, hypopigmented, atrophic, ulcerative lesions, scarring and nonscarring alopecia, erythroderma, and ichthyosiform lesions. The dermatopathology is generally the same for all of the clinical presentations; however, variations in the cutaneous findings cause confusion in following a clinical course, therapeutic approach, or prognosis.     

Ossifying fibroma in Buschke–Ollendorff syndrome

Buschke–Ollendorff syndrome represents an autosomal dominant disorder characterized by connective tissue nevi and osteopoikilosis. Cutaneous lesions may contain either predominantly elastic fibers or predominantly collagen fibers or may show both connective tissue components. The disease results from mutations in LEMD3 (MAN1), which lead to enhanced transforming growth factor‐β (TGF‐β) signaling and resultant changes in fibroblast function. TGF‐β alterations have been implicated in a number of fibrotic disorders, and it is therefore not surprising that a range of cutaneous and skeletal abnormalities have been associated with Buschke–Ollendorff syndrome. Herein, we report a novel association between ossifying fibroma and Buschke–Ollendorff syndrome and discuss how these conditions are likely to be mechanistically linked.     

The color of skin: orange diseases of the skin,nails, and mucosa

Cutaneous disease can present with lesions of all colors of the visible spectrum. Lesions of the skin, nail, and mucous membranes with an orange color can be due to a variety of etiologies. The conditions may appear as purely orange, yellow-orange, red-orange, tan, or brown with an orange hue. The orange color may also present as a transient phase of a disease process. As with all dermatologic pathology, a key way to distinguish orange-colored lesions is by distribution and morphology. The sclera, palate, lips, gingiva, and nails may also be involved. A literature review using PubMed with keywords, including orange, skin, mucosa, cutaneous, xanthoderma, and granuloma, was conducted to gather all dermatologic conditions that can present with an orange color. The relevant diseases were categorized by etiology and include inflammatory, infectious, neoplastic, and exogenous causes.     

Cutaneous gangrene due to hyperparathyroidism secondary to chronic renal failure (uraemic gangrene syndrome)

Cutaneous and subcutaneous gangrene are serious sequelae of secondary or tertiary hyperparathyroidism which may accompany chronic renal failure. Based on analysis of the present typical case and a retrospective survey of similar cases in the literature, we propose the term ‘uraemic gangrene syndrome’ for this association. These skin lesions in chronic renal failure patients represent the most serious clinical manifestation of calciphylaxis, a condition originally described by Selye. The appearance of early skin lesions should be regarded as an indication for subtotal parathyroidectomy.     

Behçet disease

Beh?et's disease is a multisystem inflammatory disorder of unknown origin, characterized by recurrent oral and genital ulcerations, ocular and cutaneous lesions, arthritis, central nervous system, and vascular disease. There is no pathognomonic laboratory test, but there are clinical criteria to assist in establishing the diagnosis. Beh?et's is most common along the Silk Road. It is particularly common among persons who have the HLA-B51 major histocompatibility type. Cutaneous lesions include pustules, erythema nodosum-like lesions, Sweet's-like lesions, pyoderma gangrenosum-ike lesions, and pathergy. The major cutaneous findings may be classified as neutrophilic vascular reactions. There is considerable morbidity resulting from Beh?et's disease, most notably a high risk of blindness from ocular involvement. Mortality may occur as a result of neurologic or vascular disease or gastrointestinal perforation.     

Congenital “self‐healing” Langerhans cell histiocytosis (Hashimoto‐Pritzker disease): A report of two cases with the same cutaneous manifestations but different clinical course

Congenital self‐healing Langerhans cell histiocytosis or Hashimoto‐Pritzker disease is a rare condition present at birth or in the neonatal period characterized by small reddish‐brown crusted papulonodular lesions. In most cases these lesions are not accompanied by systemic findings and tend to involute spontaneously within weeks or months, but in other cases there may be extracutaneous involvement and/or recurrence of the disease. This emphasizes that the clinical course is variable and a long‐term follow‐up is mandatory in order to reveal possible systemic involvement. We describe two cases of congenital self‐healing Langerhans cell histiocytosis with widespread and very similar cutaneous manifestations but different clinical course. The first patient had multisystemic disease (with lymph nodes, bones, liver and lungs affected) that required systemic therapy. The second patient had cutaneous and bony lesions that resolved spontaneously. We think that the adjective “self‐healing” is misleading and should be abandoned. We stress the importance of a complete systemic evaluation and the necessity of a long‐term follow‐up.     

Acitretin combined with NB‐UVB in the treatment of cutaneous CD30‐positive anaplastic large cell lymphoma

Cutaneous CD30⁺ lymphoproliferative disorders represent a spectrum of skin lymphatic reticular proliferative diseases, including lymphomatoid papulosis (LYP), primary cutaneous anaplastic large cell lymphoma (PC‐ALCL), and borderline lesions between them. Although they all express CD30 as a phenotypic marker and share overlapping immunophenotypic features, they differ in clinical manifestations, pathological features, treatment, and prognosis. LYP is a kind of benign disease characterized by recurrent papules and nodules, and may spontaneously regress. PC‐ALCL presents with solitary tumor or local grouped nodules characterized by large T‐cells and may completely or partially resolve in fewer than half of cases. We reported a case of patient with clinical manifestation and pathologic features consistent with LYP in its early stages, which later turned into PC‐ALCL. This patient was treated with acitretin combined with NB‐UVB and had an obvious response.     

Chronic zosteriform cutaneous leishmaniasis

Cutaneous leishmanasis (CL) may present with unusual clinical variants such as acute paronychial, annular, palmoplantar, zosteriform, erysipeloid, and sporotrichoid. The zosteriform variant has rarely been reported. Unusual lesions may be morphologically attributed to an altered host response or owing to an atypical strain of parasites in these lesions. We report a patient with CL in a multidermatomal pattern on the back and buttock of a man in Khozestan province in the south of Iran. To our knowledge, this is the first reported case of multidermatomal zosteriform CL. It was resistant to conventional treatment but responded well to a combination of meglumine antimoniate, allopurinol, and cryotherapy.     

Metastatic tubercular abscesses (tuberculous gumma): an unusual diagnosis in France

INTRODUCTION: Cutaneous tuberculosis is rare in the developed countries. We report a case of tuberculous gumma in an immunocompetent patient diagnosed late. CASE-REPORT: A 33-year-old Moroccan woman was diagnosed with tuberculous gumma after presenting cutaneous nodules and subcutaneous abscesses that had developed during pregnancy and were present for 11 months. Cutaneous and pulmonary tuberculosis was discovered. The patient recovered under antimycobacterial treatment. COMMENTS: Cutaneous tuberculosis presents with a wide spectrum of clinical lesions. It should be considered in the presence of poorly known lesions such as gumma, which require investigation for underlying tuberculosis.     

Subcutaneous sarcoidosis in a melanoma scar

Cutaneous sarcoidosis often has been referred to. as the great imitator because skin lesions can present with various morphologies. Skin lesions may be the only site of involvement or may accompany systemic disease. Occasionally, sarcoidosis also may infiltrate scars from prior trauma, tattoos, or surgery. We report a case of subcutaneous sarcoidosis limited to a melanoma scar without any other cutaneous or systemic involvement. Familiarity with and proper diagnosis of cutaneous sarcoidosis can allow for appropriate systemic screening and timely management of the disease.     

Cutaneous B cell lymphomas: Report of two interesting cases

Cutaneous B cell lymphomas can arise primarily from the skin or may occur due to secondary spread from nodal lymphomas. Primary lymphomas are confined to the skin without systemic spread and they differ from secondary lymphomas in their clinical behavior, treatment and prognosis. Cutaneous lymphomas being relatively rare, lack of precise definition and understanding of their clinical behavior diseases leads to pitfalls in the diagnosis. We report two cases of cutaneous B cell lymphomas who presented with fever of unknown origin initially and later found to have skin lesions. Hence, skin can be a potential diagnostic clue in the evaluation of patients with fever of unknown origin. The distinctions between the primary and the secondary lymphomas become important in choosing the treatment and assessing the prognosis.     

Cutaneous gonococcal infections

Cutaneous gonococcal infections are frequent in the United States and other industrialized countries. This bacterial infection commonly affects the genitourinary tract and may rarely involve the skin. Cutaneous lesions are either primary or secondary to disseminate disease. The diagnosis of gonorrhea is based on clinical ground and is best confirmed by the isolation of the organism. Preventive measures including periodic screening of high-risk individuals and behavioral counseling are effective in controlling the disease.     

Cervico-thoracic lipoma associated with occult syringohydromyelia

Abstract:  Cutaneous lesions in the midline may be hallmarks of an occult spinal dysraphism. They rarely may occur in the cervical and thoracic regions. We report a 9-year-old girl with a cervico-thoracic lipoma associated with an underlying syringohydromyelia. Early detection of potentially complicated conditions can prevent the occurrence of irreversible neurologic damage.     

Cutaneous cryptococcus in an elderly woman with chronic essential dermatitis

Cryptococcus neoformans is a common cause of life-threatening infection. Cutaneous manifestations of Cryptococcus can be primary or secondary, most commonly from immunosuppression. With the global emergence of acquired immunodeficiency syndrome (AIDS), incidence of cryptococcosis is increasing and now represents a major life-threatening fungal infection in these patients. Nonspecific lesions may cause misdiagnosis. Disseminated Cryptocoocus requires early clinical diagnosis and effective management to decrease mortality. We review a case of cutaneous Cryptococcus as a complication of chronic essential dermatitis treated with long-term immunosuppressive agents and discuss updated guidelines on the treatment of Cryptococcus.