XI.A.001

Acute hemorrhagic edema of infancy is an acute cutaneous leukocytoclastic vasculitis (LCV) of infants with a violent onset followed by spontaneous recovery. Edema and palpable purpuric skin lesions are present. Light microscopic examination of the skin biopsy specimens revealed LCV as in Henoch-Schönlein purpura. Acute hemorrhagic edema of infancy is observed before 2 years of age and is confined to the skin.

XI.A.002

Amyloidosis, primary. Amyloid deposition in inferior and superior palpebrum. Note waxy surface.

XI.A.003

Amyloidoses, primary. Amyloid deposition in tongue (macroglossia).

XI.A.004

Antiphospholipid antibody syndrome. Anticardiolipin antibody. Ischemic skin changes resulting from thrombotic disease of superficial vessels.

XI.A.005

Antiphospholipid antibody syndrome: lupus anticoagulant. Thrombosis of digital vessels with ischemic changes of the toes.

XI.A.006

Aspergillosis: primary cutaneous lesion initiated from a contaminated arm-board. Primary cutaneous aspergillosis can occur in the absence of disseminated disease. In hospitalized patients, primary cutaneous aspergillosis lesions may arise around the sites of an intravenous catheter or a venipuncture site.

XI.A.007

Disseminated candidiasis. Purpuric nodule and diffuse maculopapular and maculonodular rash in a patient with acute myelogenous leukemia with invasive (disseminated) candidiasis. The rash of candidiasis may be diffuse or only on the extremities. Biopsy, which includes the involved areas down to the dermis will show small oval blastospores with buds and pseudohyphae.

XI.A.008

Chediak-Higashi disease. Five-year-old patient with thin fine hair, which is a dirty blonde with silver streaks, the result of misdistribution of giant melanophores in the hair follicle cells.

XI.A.009

Chediak-Higashi disease. Normal and affected mink. The latter (on the right) has light gray hair and absence of pigment in the irises, which appear translucent. The affected child usually has similar findings. The pigment abnormality is the result of misdistribution of abnormal giant melanophores.

XI.A.010

Cholesterol emboli. Digital necrosis. Small cholesterol crystals or larger cholesterol plaques break off from atherosclerotic plaques and shower into the arterial system, occluding arterioles or larger vessels. Virtually any organ can be affected depending on the size of the crystal or plaque dislodged. The crystals or plaques and an accompanying inflammatory reaction and adventitial fibrosis obliterate the vessel lumen. Vasospastic mediators may contribute to ischemia and necrosis.

XI.A.011

Cholesterol emboli. Splinter hemorrhages the result of very small cholesterol crystal emboli.

XI.A.012

Congenital rubella. Rare in industrialized societies as a result of rubella immunization. Rubella virus transmitted as a result of maternal infection during pregnancy. Cutaneous manifestation characterized by widespread maculopapular lesions of a reddish-blue or magenta color, due to persistent dermal extramedullary erythropoiesis in patients with congenital viral infections. This circumstance is referred to as blueberry muffin baby.

XI.A.013

Coumadin necrosis of breast. Frequently occurs in acral parts and areas of fat deposition such as buttocks or breast (as shown here).

XI.A.014

Coumadin necrosis involving the penis.

XI.A.015

Coumadin necrosis involving the trunk.

XI.A.016

CREST syndrome is a form of systemic sclerosis (scleroderma) that is characterized by Calcinosis (calcium deposits), usually in the fingers; Raynaud; loss of muscle control of the Esophagus, which can cause difficulty swallowing; Sclerodactyly, a tapering deformity of the bones of the fingers; and Telangiectasia, small red spots on the skin of the fingers, face, or inside of the mouth.

XI.A.017

Cryocystalglobulinemia. Skin alterations. Woman with a circulating monoclonal immunoglobulin that crystallized at ambient temperatures resulting in ischemic changes in the vasculture of the skin. She developed chronic necrotic lesions with ulceration. See also VII.C. under “Cryocrystalglobulinemia” for images of intravascular crystals.

XI.A.018

Cryoglobulinemia: purpura in this case. Skin manifestations may include ischemic necrosis; palpable purpura; livedoid vasculitis; cold-induced urticaria; scarring of the tip of the nose, pinnae, fingertips, and toes; acrocyanosis; or nailfold capillary abnormalities. Type I cryoglobulinemia, or simple cryoglobulinemia, is the result of a monoclonal immunoglobulin, usually immunoglobulin M (IgM). Types II and III cryoglobulinemia (mixed cryoglobulinemia) contain rheumatoid factors, which are usually IgM. The rheumatoid factor may be monoclonal (in type II cryoglobulinemia) or polyclonal (in type III cryoglobulinemia) immunoglobulin. Cryoglobulinemia without an associated disease has been known as essential, or idiopathic, cryoglobulinemia. However, the discovery of a close association between hepatitis C virus and mixed cryoglobulinemia has cast doubt on the existence of essential, or idiopathic, cryoglobulinemia. Cryoglobulinemia associated with a particular disease (lymphoproliferative disorder, autoimmune disease, infectious disease) is known as secondary cryoglobulinemia.

XI.A.019

Cryoglobulinemia: subungual purpura.

XI.A.020

Cullen sign. Periumbilical ecchymoses indicating hemoperitoneum. Seen in acute hemorrhagic pancreatitis.

XI.A.021

Dyskeratosis congenita, toenails. Disintegrating nails appear between ages 5-13 years and are eventually destroyed after recurrent episodes of suppurative paronychia.

XI.A.022

Dyskeratosis congenita, fingernails. Disintegrating nails appear between ages 5-13 years and are eventually destroyed after recurrent episodes of suppurative paronychia.

XI.A.023

Dyskeratosis congenita, tongue. Atrophic plaque of leukoplakia.

XI.A.024

Dyskeratosis congenita, fingernails. Disintegrating nails appear between ages 5-13 years and are eventually destroyed after recurrent episodes of suppurative paronychia.

XI.A.025

Dyskeratosis congenita, oral cavity. Small blisters and erosions lead to irregular patches of leukoplakia. Similar changes may occur in the tarsal conjunctiva, anorectum and urethra, and gastrointestinal tract.

XI.A.026

Dyskeratosis congenita, skin of groin. Fine, reticulate grey-brown pigmentation develops early (ages 2-3 years). These changes usually occur on the neck and trunk and later become atrophic with poikilodermatous appearance.

XI.A.027

Ecthyma gangrenosum. A skin infection most often associated with Pseudomonas aeruginosa bacteremia in immunocompromised individuals. Hemorrhagic pustules that evolve into necrotic ulcers surrounded by erythema are characteristic as shown here.

XI.A.028

Ehlers-Danlos syndrome. Purpura on lower extremities. The disorder is one of several resulting from abnormalities in collagen and elastin. The fragile skin can be susceptible to bruising and poor healing of wounds, leaving “cigarette paper” scars.

XI.A.029

Erythema multiforme. Note characteristic target-like lesions. Usually an acute self-limited skin disease, symmetrically distributed on the extremities with typical and often recurrent concentric “target” lesions. May occur as a result of microbial infections.

XI.A.030

Fifth disease (parvovirus B-19 infection). Fifth disease, the result of parvovirus B-19 infection, causes a rash that occurs most commonly in children but can occur in non-immune adults. The manifestation is a “slapped-cheek” rash on the face and a lacy red rash on the trunk and limbs. Occasionally, the rash may itch. Associated with a low-grade fever, malaise, or a “cold” a few days before the rash breaks out. The rash resolves in 7 to 10 days.

XI.A.031

Fanconi anemia. Patient shows characteristic café-au-lait skin pigmentation. Generalized hyperpigmentation, increasing with age, and areas of hypopigmentation may occur.

XI.A.032

Fanconi anemia. Patient displays hypothenar deformity (depression) as a result of bilateral hypoplastic first metacarpal.

XI.A.033

Fanconi anemia. Radial hypoplasia, absent thumbs, and syndactyly. Upper extremity deformities are present in over half of Fanconi anemia patients and radius, wrist, and thumb abnormalities are among the most prevalent.

XI.A.034

Fanconi anemia. Radiographs. Vestigial (hanging) thumbs attached by soft tissue. (A) Arrow indicates thumb soft tissue with absence of metacarpal or phalangeal bones in right thumb. (B) Arrow indicates thumb soft tissue with absence of metacarpal or phalangeal bones in left thumb. (C) Left side shows left hand after excision of vestigial thumb (soft tissue removed). Both hands had thumb soft tissue excised. Right side shows right hand after pollicization. The index finger was swung over and an opposable new thumb fashioned, leaving three fingers and new thumb. Left hand had same procedure to provide an opposable thumb (not shown).

XI.A.035

Graft versus host disease. (A) Grade IV graft versus host disease involving skin with diffuse erythroderma and bullae formation. (B) Resolution of graft versus host reaction after therapy with high-dose glucocorticoids and antithymocyte globulin.

XI.A.036

Grey-Turner sign. Ecchymosis on the side of the abdomen. Usually associated with intraperitoneal bleeding from hemorrhagic pancreatitis, ruptured abdominal aorta, or ruptured ectopic pregnancy.

XI.A.037

Hemophilia arthropathy (factor VIII deficiency). Chronic right knee hemarthrosis with fresh and fading ecchymoses on legs. The most common clinical manifestation of hemophilia is arthropathy secondary to recurrent hemarthroses and chronic synovitis. Joint-surface erosions secondary to chronic synovitis often occur in early childhood and progress to advanced arthropathy by late adolescence. The knee, elbow, ankle, hip, and shoulder are the most commonly involved joints.

XI.A.038

Hemophilia arthropathy (factor VIII deficiency). Acute right elbow hemarthrosis with overlying ecchymosis. The most common clinical manifestation of hemophilia is arthropathy secondary to recurrent hemarthroses and chronic synovitis. Joint-surface erosions secondary to chronic synovitis often occur in early childhood and progress to advanced arthropathy by late adolescence. The knee, elbow, ankle, hip, and shoulder are the most commonly involved joints.