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Renal Disease and the Skin

Key Points Skin manifestations of chronic kidney disease (CKD) are commonly encountered and can be due to (1) the cause of the underlying renal disease, either acquired or heritable; (2) conditions unique to uremia; or (3) immunosuppressive therapies and/or immunosuppression itself in renal transplant recipients. Diabetes and hypertension account for most cases of end-stage renal disease (ESRD) in the United States; however, many diseases, such as…

Cardiovascular Diseases and the Skin

Key Points Several systemic disorders can affect both the heart and the skin. Cutaneous findings may serve as a diagnostic clue for many of these conditions. Dermatologists and cardiologists must be aware of the multisystem aspects of such disorders in order to facilitate proper diagnosis and management. A number of cardiac therapies may cause cutaneous complications, and awareness of such potential complications is essential for the…

Sarcoidosis

Key Points Sarcoidosis is a multisystem granulomatous disease of unknown etiology that affects patients of all ages and ethnic groups. Thorough multisystem evaluation of all patients is essential, as sarcoidal inflammation may be clinically quiescent while causing significant damage. Cutaneous sarcoidosis is characterized by a protean array of lesion morphologies, but generally occurs as violaceous infiltrated papules clustered on the face, particularly around the nose. Sarcoidosis…

Acquired Immunodeficiency Syndrome and Sexually Transmitted Infections

Key Points Patients with HIV/AIDS may develop cutaneous manifestations that reflect their immune-compromised state and can be grouped into infections (viral, bacterial, fungal, and parasitic/ectoparasitic), inflammatory, neoplastic, or other conditions. Syphilis, caused by Treponema pallidum , is a sexually transmitted disease that has experienced a resurgence with the HIV epidemic, and it is classically divided into four stages: primary, secondary, latent, and tertiary, with each stage…

Protozoal Diseases

Key Points Trichomoniasis typically presents with vaginal pruritus and a frothy discharge, and responds well to treatment with metronidazole. Systemic therapy for leishmaniasis is recommended for patients who are immunosuppressed or who acquire infection in areas where mucocutaneous disease has been reported. Suramin remains useful to treat early Rhodesian trypanosomiasis, while melarsoprol is the drug of choice for central nervous system (CNS) disease. Pentamidine is still…

Fungal Diseases

Key Points Fungal pathogens affect the skin in many ways: superficial fungi, such as the dermatophytes, rarely cause systemic disease. Subcutaneous mycoses, such as sporotrichosis, mycetoma, and chromoblastomycosis, are typically caused by minor, inadvertent trauma that introduces environmental fungi into the skin and subcutaneous tissues. Host immunologic response toward these typically low-virulence fungi is muted. The classic systemic mycoses (histoplasmosis, coccidioidomycosis, blastomycosis, and paracoccidioidomycosis) are nearly…

Bacterial and Rickettsial Diseases

Key Points Most Gram-positive skin infections can be treated with a semisynthetic penicillin or a cephalosporin. Meticillin-resistant staphylococcal infections typically present as abscess or folliculitis. The primary intervention is drainage. Lyme disease typically presents with erythema migrans and responds to treatment with oral doxycycline. Meningitis or cardiac involvement is treated with intravenous ceftriaxone. Rickettsial diseases often present with a cutaneous eruption, fever, and headache. The treatment…

Viral Diseases

Key Points Measles is generally a benign, self-limited illness that presents with erythematous, confluent macules and papules that spread cephalocaudally and Koplik spots. Hand, foot, and mouth syndrome, caused by various Coxsackie viruses, is a self-limited illness that presents with oval to linear papules and vesicles on the dorsal and lateral fingers and toes as well as painful, oral mucosal vesicles and papules. Epstein–Barr virus infection…

Hepatic Disease and the Skin

Key Points The cutaneous changes of hepatic disease may be related to primary diseases of the liver, cutaneous diseases with associated hepatic abnormalities, disorders with changes in many organs, including the liver and skin, and medications with direct effects on the liver or indirect effects due to hypersensitivity reactions. Pruritus or prurigo nodularis without an identifiable cutaneous eruption warrant an investigation for hepatic disease. The dermatologic…

Cutaneous Diseases Associated with Gastrointestinal Abnormalities

Key Points Gastrointestinal hemorrhage may be associated with pseudoxanthoma elasticum or hereditary hemorrhagic telangiectasia. Adenomatous polyposis may be associated with epidermoid tumors, fibromas, and dermoid tumors. Peutz–Jeghers syndrome and Cowden’s syndrome are associated with hamartomatous polyps. Malabsorption may be associated with dermatitis herpetiformis and celiac disease or acrodermatitis enteropathica and zinc deficiency. Inflammatory bowel disease is associated with neutrophilic dermatoses such as pyoderma gangrenosum, aphthosis, and…

Porphyrias

Key Points Several porphyrias share similar photocutaneous features; sufficient testing to assure correct diagnosis must precede selection of therapies. Most porphyrias can be correctly diagnosed biochemically. Mutation analysis is the gold standard for porphyria diagnosis and family counseling. Clinical and biochemical remissions can be therapeutically induced in most patients with porphyria cutanea tarda. X-linked dominant protoporphyria, recently recognized, can be distinguished from erythropoietic protoporphyria despite many…

Adrenal, Androgen-Related, and Pituitary Disorders

Key Points Classic signs of excess cortisol (Cushing’s) include moon facies, striae, atrophy, and acne. Cortisol deficiency (Addison’s) presents with hyperpigmentation of the skin, nails, and mucous membranes. Excess androgens, as seen in polycystic ovary syndrome and congenital adrenal hyperplasia, cause hirsutism, acne, male-pattern alopecia, and virilization. Androgen insensitivity (testicular feminization) leads to ambiguous genitalia, sparse sexual hair development, and an absence of acne and male-pattern…

Cutaneous Manifestations of Lipid Disorders

Key Points Cutaneous xanthomas present as yellow papules, nodules, or plaques and can signal the presence of an underlying lipid, metabolic, or hematologic abnormality. Xanthelasma is the most common cutaneous xanthoma and can be seen in association with dyslipoproteinemia in about half of patients. Xanthomas can also occur in normolipemic patients and herald an underlying metabolic, neurologic, or hematologic disorder. Treatments are available for many of…

Thyroid and the Skin

Key Points When evaluating cysts and nodules of the head and neck, consider thyroglossal ductal cysts and thyroid carcinoma metastases. There are many syndromes associated with thyroid cancer with dermatologic manifestations (e.g., Cowden’s disease, multiple mucosal neuroma syndromes, Gardner’s syndrome, Carney’s complex, and Werner’s syndrome). Urticaria has been associated with papillary carcinoma and autoimmune thyroid disease although the pathogenesis remains to be elucidated. Pretibial myxedema, Graves’…

Diabetes and the Skin

Key Points Diabetes mellitus (DM) is a highly prevalent, chronic, multisystem disease that affects the skin at some point of the disease in up to 70% of affected individuals. Although the etiology is often unknown, the pathogenesis of many cutaneous findings is thought to be due to microangiopathy, glycosaminoglycan deposition with collagen alterations, and immune complex deposition. There are numerous cutaneous manifestations of DM, which range…

Vascular Neoplasms and Malformations

Key Points A variety of vascular lesions can serve as cutaneous signs of systemic disease. Telangiectasias or angiokeratomas with particular morphologies and distributions raise suspicion for an autoimmune connective tissue disease or a genetic disorder. Vascular anomalies are divided into two major categories: tumors due to endothelial cell proliferation and malformations that result from errors in vascular morphogenesis. Benign vascular tumors and vascular malformations may have…

Cutaneous Manifestations of the Histiocytoses

Key Points Langerhans cell histiocytosis (LCH) is a clonal neoplastic disorder. LCH represents a spectrum of disease from asymptomatic skin-limited self-resolving lesions to systemic multisystem disease with high mortality. The majority of LCH cases harbor the BRAF V600E mutation, suggesting a potential role for BRAF inhibitors as treatment. LCH cells express S100, CD1a, CD207 (Langerin), and Fascin. The LCH-III trial confirmed a combination of vinblastine and…

Dysproteinemias, Plasma Cell Disorders, and Amyloidosis

Key Points The presence of a monoclonal gammopathy may be an incidental finding, but there are many cutaneous disorders that are due to or are strongly associated with the presence of a monoclonal gammopathy. A usual way to organize thinking around the syndromes divides them into diseases directly related to the monoclonal protein, diseases frequently associated with a monoclonal protein without a clear causative role, and…

Cutaneous Lymphomas and Cutaneous Signs of Systemic Lymphomas

Key Points Cutaneous lymphomas represent a heterogeneous group of malignant lymphomas arising primarily in the skin. Most cutaneous lymphomas are listed as separate entities in the World Health Organization classification of hematologic malignancies. Differentiation of primary cutaneous lymphomas from extracutaneous lymphomas with secondary skin manifestations is paramount, as management of patients is different. In some cases, only accurate staging investigations allow differentiation of primary cutaneous lymphomas…

Metastatic Disease

Key Points Cutaneous metastases are not uncommon, and are usually a late finding with poor prognosis. Cutaneous metastases can be the first sign of an internal malignancy or of extranodal disease in a known malignancy. Breast cancer is the most common cause of cutaneous metastases in women, whereas melanoma and lung cancer are the most common causes in men. Cutaneous metastases most often present with solitary…