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Photo Quiz

Blue-Gray Centrofacial Hyperpigmentation
Question Which one of the following is the correct diagnosis, given the patient's history, the physical and skin biopsy findings? A. Actinic lichen planus. B. Hemochromatosis. C. Amiodarone-induced hyperpigmentation. D. Ashy dermatosis (erythema dyschromicum perstans). E. Argyria.

A 68-year-old man presented to a university dermatology department with an asymptomatic blue-gray discoloration of the centrofacial region. Physical examination revealed a blue-gray hyperpigmentation of the forehead, cheeks, nose, malar regions and chin (see the accompanying figure). The medical history revealed that the patient was diagnosed with ischemic dilated cardiomyopathy two years previously and was resuscitated from a syncopal episode associated with sustained ventricular tachycardia that was refractory to quinidine, disopyramide phosphate and procainamide. Results of routine laboratory tests were normal. A 3-mm punch biopsy specimen showed normal epidermis and a perivascular deposition of yellow-brown pigment within microphages in the dermis.


The correct answer is C: amiodarone-induced hyperpigmentation. Amiodarone is an iodinated compound used in the treatment of ventricular arrhythmias that are refractory to other medications. Actinic lichen planus is a rare clinical form of lichen planus that occurs in exposed skin areas. The pigmented variant of the disease typically involves only the face, with clinical features resembling melasma. Histopathologic features seen on skin biopsy of actinic lichen planus include a band-like lymphocytic infiltrate with numerous melanophages observed in the dermis.

Hemochromatosis is one of the most common genetic errors of metabolism and is characterized by a diffuse cutaneous slate-gray pigmentation in light-exposed areas. The onset of the disease generally occurs during the fourth or fifth decade of life. Men are more commonly affected than women. Other findings may include diabetes and a high serum iron level.

Ashy dermatosis, also known as erythema dyschromicum perstans, is a rare idiopathic dermatosis characterized by ash-gray skin lesions. The ash-colored macules vary in size and shape and are localized to the arms, neck and trunk. The face is usually involved. The characteristic blue-gray skin color reflects the presence of melanin in the macrophages of the dermis.

Argyria occurs in persons exposed to silver salts and clinically appears as a slate-gray pigmentation on sun-exposed areas of the skin, especially the forehead and nose. The diagnosis of argyria is well established by skin biopsy. Conventional histology shows silver granules in the dermis particularly evident near the basal layer of sweat glands.

Several systemic and dermatologic side effects are attributed to amiodarone, such as pulmonary fibrosis (which has a 10 percent mortality rate), thyroid abnormalities, fulminant hepatitis, keratitis, chronic anxiety reaction, photosensitivity reaction and cutaneous hyperpigmentation (in 2 to 5 percent of patients). The pigmentation is clinically

characterized by progressive blue-gray discoloration of predominantly sun-exposed areas. Lipofuscin is a yellow-brown pigment that accumulates in lysosomes with aging and is a byproduct of cellular degradation. The pathogenesis of amiodarone-induced


This iatrogenic hyperpigmentation is associated with lipofuscin rather than melanin deposits.

hyperpigmentation may be related to the basic action of the drug on the lysosome and to extra phototoxic-induced lysosomal damage. Ultrastructural studies show membrane-bound dense lysosomal bodies within macrophages that probably contain degradation products of the drug bound to lipofuscin. The cutaneous pigmentation slowly fades after discontinuation of

therapy but may persist for months to years.

Drug-induced vasculitis. She had a history of intravenous drug use during adolescence. On laboratory evaluation. Cellulitis. some of which had confluent borders. did not blanch on pressure. Mild swelling of the right ankle was present. although the patient maintained full range of motion. with a creatinine clearance C. B. and had no overlying warmth or tenderness. she was noted to have proteinuria (1. Question Given the patient's history and the results of the physical examination. The patient had no other chronic medical illnesses. A. The patient had been recently diagnosed with hypertension for which she had been taking an angiotensin-converting enzyme inhibitor (ramipril) for two weeks. She had also noted swelling of her right wrist and ankle with associated mild pain.2 g per day). The remainder of the physical examination was unremarkable. Lichen planus. and consisted of erythematous macules. The lesions were located primarily over the shins and medial aspects of both lower extremities. D. The lesions were palpable. Mixed cryoglobulinemia. which one of the following is the correct diagnosis for the disorder shown above? . Porphyria cutanea tarda.Photo Quiz Palpable Shin Lesions A 41-year-old woman presented complaining of the progressive development of lesions on her lower extremities (see accompanying photos). E.

Raynaud's syndrome and purpura. persists for a long time. The answer is C: mixed cryoglobulinemia. The so-called sporadic variety is the most common type. Patients who have no detectable HCV RNA at the end of 4 a course of treatment have significantly improved purpura and serum cryoglobulin levels. bruising. It often may not be possible to distinguish between a drug-induced vasculitis and a cryoglobulinemic rash by physical examination alone. Ramipril has been associated with a rash in a small percentage of patients. Porphyria cutanea tarda presents with photosensitivity. Several studies have established a strong link 1. a multisystem disorder that is characterized by deposition of circulating immune complexes in small and medium-sized blood vessels resulting in arthralgias. although rare familial forms exist. and vesicles and bullae that can become hemorrhagic. Serum aminotransferase levels were mildly elevated: alanine aminotransferase was 123 U per L. skin fragility. However. despite continued treatment with ramipril.93 mL per second). Lichen planus. as well as absence of systemic manifestations such as a fever or elevated white blood cell count. It is important to obtain a thorough medication history in any patient who presents with a rash.of 56 mL per minute (0. Cellulitis was ruled out by the absence of warmth or tenderness on examination of the leg. There is a high prevalence (62 to 91 percent) of HCV antibody positivity in patients with the sporadic form of porphyria cutanea tarda. Her rash is a leukocytoclastic vasculitis caused by cutaneous deposition of cryoimmunoglobulins. a skin rash that occurs in less than 1 percent of the general population. aspartate aminotransferase was 139 U per L. the coincident clinical findings of positive serum HCV antibody. Antibodies to HCV are present in 10 to 38 percent of patients with lichen planus. nerve and brain involvement is possible. Kidney. There is a high incidence of mucosal involvement. . Antiviral 3 therapy with alpha-interferon for the underlying HCV infection can be effective in treating the associated mixed cryoglobulinemia.2 between HCV infection and mixed cryoglobulinemia. most likely contracted during her years of intravenous drug use. In this patient. the rash improved on treatment of HCV infection. violaceous. Discussion. It presents as flat-topped. and it can involve the hair and nails. The patient tested positive for hepatitis C virus (HCV) infection. HCV RNA and serum cryoglobulins make the latter much more likely. because drug-induced etiologies are among the most common causes of leukocytoclastic vasculitis. pruritic papules in a generalized distribution.

Treatment of the underlying chronic infection can ameliorate cryoglobulinemia. hepatitis B. The joint involvement is usually a symmetric. 5 other important extrahepatic manifestations of HCV infection include membranoproliferative glomerulonephritis. It usually presents with palpable purpura and petechiae that involve the lower extremities. Arthralgias and arthritis may be prominent. 4 Although cryoglobulins are found in 19 to 54 percent of patients with HCV infection. nonmigratory polyarthritis involving ankles. 7 The clinical presentation of mixed cryoglobulinemia is quite variable. 6 Several types of cryoglobulinemias are recognized.. The most serious manifestation is renal damage due to deposition of the circulating cryoimmunoglobulins. usually IgM mixed with polyclonal immunoglobulins) and type III (polyclonal only) are also known as mixed cryoglobulinemias. although mixed cryoglobulins are also observed with other chronic infections (e. endocarditis) as well as autoimmune and malignant disorders. porphyria cutanea tarda and lichen planus. Type II (at least one monoclonal immunoglobulin. . Type I (monoclonal immunoglobulin) is associated primarily with malignant processes of the immune system. In addition to cryoglobulinemia. Cutaneous leukocytoclastic vasculitis is the most clinically evident manifestation.g. Duration of HCV infection and severity of 5 histologic hepatic inflammation do not appear to be related. wrists. elbows.however. Corticosteroids and cytotoxic agents are sometimes used in severe cases. hands and toes. symptoms occur in only 25 percent of these. Skin lesions are not 8 always obvious and are sometimes not present. Renal biopsy usually reveals IgM and IgG deposition within subendothelial immune deposits. a rebound phenomenon of clinical and serologic variables has been noted after discontinuation of this therapy. This can present as nephrotic syndrome. HCV has become one of the most common causes of mixed cryoglobulinemia. thyroiditis. glomerulonephritis or acute renal failure.

Question Based on the patient's history and physical examination. On physical examination. Lichen planus. Fine.M. white reticulations on her buccal mucosa. white . P. wrists.D. cyclobenzaprine and tramadol for back pain. furosemide. Discoid lupus erythematosus. M. and shins. violaceous. because there were no eosinophils and no parakeratosis.O. which was established by skin biopsy. Guttate psoriasis. Flat warts. Send photograph and discussion to Marc S. E. carbamazepine and clonazepam for seizures. and potassium replacement. Discussion The answer is D: lichen planus. flat-topped. polygonal papules. B. D. C. Crystal Beach. Contributing editor is Marc S.M. 2 mm to 10 mm in diameter.. lacy. Poison ivy rash. Berger. She also had lacy. FL 34681-0219. C. C. Her medications included triamcinolone inhaler. An inflammatory dermatosis of unknown etiology. Box 219.. estrogen replacement. lichen planus classically presents as shiny. which one of the following is the correct diagnosis? A. FIGURE 1. albuterol inhaler. M. Berger. A 45-year-old woman presented with a pruritic eruption of one month duration. It was not consistent with a drug-induced lichen planus. and beclomethasone nasal spray for asthma.D. there were multiple flat-topped papules with white scale on her hands.Koebner Streak The editors of AFP welcome submission of photographs and material for the Photo Quiz department..

pink. lichen planus occurs in both sexes. and tongue is not uncommon. and secondary syphilis. and discoid lupus erythematosus. but are smooth instead of scaly. Discoid lupus erythematosus typically presents as atrophic or scarring plaques on the head and neck. While some patients remain asymptomatic. lesions may be grouped.scale (Wickham's striae) adhere to well-developed papules. gums. arms. ulcerative and atrophic variants may cause painful erosions and marked distress. or generalized in arrangement. resembling a reticulate network of lichen. instead of flat-topped papules as seen in lichen planus. 2 Lichen planus-like eruptions may occur following exposure to industrial compounds such as color film developer or with use of many commonly prescribed medications. Affecting less than 1 percent of the population worldwide. including . The white buccal lesions of oral lichen planus may necessitate biopsy to exclude leukoplakia. which has a fine. Evolving over several weeks. are flat-topped like the lesions of lichen planus. pityriasis rosea. candidiasis. papule). The initial lesions are usually located on the flexural surfaces of the wrists. Guttate psoriasis is characterized by erythematous plaques with thick white scale. or flat warts. characterized by the four Ps (purple. Trauma during the early stages of evolution may induce the isomorphic (Koebner) phenomenon to produce a linear distribution of papules. thighs. and genitalia also may be involved. These are easier to appreciate if a drop of microscope lens oil is placed on the papule and a glass slide is pressed gently over the lesion. polygonal. the lesions of these granulomatous disorders lack the fine scale of lichen planus papules. 1 Involvement of the oral mucosa occurs in 60 percent of patients and may be the only manifestation of the disease. annular. but the trunk. unlike lichen planus. and legs. or light brown instead of violaceous. pruritic. including guttate psoriasis. Annular lesions may mimic those of granuloma annulare or sarcoid. Involvement of the lips. the eruption is often pruritic with severity related to the degree of involvement. The most common pattern of oral disease reveals a reticular network of white hyperkeratosis on the buccal mucosa. While most cases of mucosal lichen planus are nonerosive and asymptomatic. Contact dermatitis due to poison ivy can also present as a linear distribution of pruritic lesions. often allows a clinical diagnosis of the lesion. Other papulosquamous disorders should be considered in the differential diagnosis. However. Histopathologic 1 examination of a biopsy specimen can confirm the diagnosis in atypical cases. Verruca plana. most often between 30 and 60 years of age. the lesions of contact dermatitis are often vesicular. lacy scale. however. They are usually not pruritic. The appearance of the typical lichen planus papule. and are usually skin-colored.

which one of the following is the correct diagnosis? A. while more severe cases may require systemic corticosteroids. or cyclosporine. papular. Topical or intralesional steroids suppress inflammation and cause regression of lesions in mild cases. erythematous lesion was noted on the medial aspect of the knee. 1 Most localized cutaneous eruptions of lichen planus resolve within 12 to 18 months. and hypoglycemic agents. 2 Photo Quiz Leg Rash A 71-year-old woman presented to the dermatology clinic with a 15-year history of a recurrent erythematous rash on her left knee and lower leg (see accompanying figure). Sarcoidosis. Question Based on the patient's history and physical appearance of the rash. On physical examination. C. The lesions reappeared approximately one year later. this time with mild pruritus and slight hypersensitivity to touch. antihypertensives. leaving residual hyperpigmentation that fades with time. a 5-cm erythematous ring was noted on her left central knee and a serpiginous. Granuloma annulare. . Hansen's disease. Antihistamines can be useful for 3 pruritus with recurrence in less than 20 percent of patients. Exposure and medication history should be reviewed as these lesions resolve after discontinuation of the offending agent. and reported that the lesions resolved at that time. retinoids. B.diuretics. Patients with generalized eruptions or mucosal lesions have a more prolonged course. The patient had been treated with ivermectin four years earlier.

the disease commonly recurs. and actinic. erythematous.3 .3. Selected Differential Diagnosis of Raised Leg Rash Condition Characteristics Hansen's disease Well-defined.2 The condition usually begins with an asymptomatic. self-limited cutaneous condition. travel history Granuloma annulare Circular. the lesion gradually may increase in diameter.3 Among those who experience resolution of lesions. Approximately one half of patients experience spontaneous resolution without scarring. scaly. feet. A skin biopsy showed interstitial lymphocytic and histiocytic infiltrates with collagenolysis.1 The duration of the disease is highly variable.D. clinical symptoms present Thin. hypesthesia. but most often involve the dorsal surfaces of the hands. It is characterized by annular. perforating. Cutanea larvae migrans. travel history Flat.1. including localized. or knees. pruritic. flesh-colored or slightly erythematous plaques and papules that are generally asymptomatic. The patient was treated with clobetasol steroid cream and instructed to follow up in four weeks. with a final size of 0. flesh-colored or erythematous plaques and papules.4 There are several forms of granuloma annulare. generalized. bacilli. hypopigmented papules with or without scale. Discussion The answer is B: granuloma annulare. elbows.1.3 The localized form accounts for approximately 75 percent of all cases. circular lesions with or without raised erythematous border Granuloma annulare is a benign. whereas. usually asymptomatic Sarcoidosis Cutanea larvae migrans Tinea corporis Brown-red plaques that wax and wane. subcutaneous. serpiginous papules.2.2 Some patients may report mild pruritus.5 to 5 cm.3 The lesions of granuloma annulare can occur anywhere on the body. lesions may last for years. as noted in this case.1 Over several months. Tinea corporis. E. flesh-colored papule that undergoes central involution and slowly develops into a ring-shaped lesion. in other patients. consistent with granuloma annulare.

2. scaly spot that later develops a raised advancing border.1.2 Although the etiology of granuloma annulare is unknown. but this is often difficult to demonstrate because of the low fungal load. monocyte activation. which causes moderate to intense pruritus. while the central area becomes hypopigmented and less scaly. hypopigmented papules and may have slight scale.1.1. they are best left untreated.1. but may persist for up to one year.2.1-3 None of these methods are curative.2.. the histology shows collagen degeneration caused by an inflammatory reaction that occurs around the blood vessels. and Nepal). and type IV delayed hypersensitivity. southern Africa. cryotherapy. chloroquine) for patients who have widespread lesions.2 These lesions are typically well-defined.3 Cutaneous larvae migrans is characterized by a thin. Myanmar. these lesions may be treated with intralesional corticosteroid injections.2 Sarcoidosis can present with a number of different skin findings.3 Diagnosis is made by demonstrating leprosy bacilli in the skin. a local inflammatory response is provoked. serpiginous papular eruption caused by hookworm larvae traveling through the skin. In uncertain cases where biopsy is necessary. the larvae usually die within two to eight weeks. .2 Tinea corporis classically begins as a flat. including brownish-red plaques that wax and wane. India. topical corticosteroids. Hansen's disease (leprosy) is a chronic granulomatous disease that affects the skin and nerves. or even systemic agents (e.2 Infection is acquired by skin contact with soil contaminated by dog or cat feces.2 The border may develop red.3 Diagnosis often is obvious from the usual clinical presentation (cough. colchicine. electrodesiccation. If cosmetic appearance is a concern. but plaques also may occur on the trunk or the dorsal surfaces of the arms or legs.2 If untreated.2 The face is most commonly involved.g. ultraviolet light therapy. theories include vasculitis.1 During larval migration.2 Resolution of lesions after empiric antifungal treatment is the most common means by which tineal infection is verified.1 A potassium hydroxide examination of skin scrapings may reveal hyphae.1.The diagnosis of granuloma annulare often may be made by its characteristic clinical presentation. dyspnea) and radiologic findings (hilar lymphadenopathy). trauma. dapsone.1 It occurs most commonly in travelers returning from tropical locations.3 Additional clinical features that help distinguish this diagnosis are perilesional cutaneous anesthesia and exposure to an endemic area (especially Brazil. erythematous. but biopsy of cutaneous lesions may provide confirmatory information in unclear cases. raised papules or vesicles.3 Because lesions are usually asymptomatic.

and no laboratory abnormalities or associations with systemic disease have been established.2 Lichen nitidus may resolve spontaneously at any time without residual atrophy or pigmentary changes.3 They usually are found on the forearms. skin-colored papules appear on the dorsum of his hands and the extensor surface of his upper extremities (see accompanying figure).3 The etiology of lichen nitidus is unknown. He is otherwise in good health and is not taking any medications.Photo Quiz Tiny. they can last for many years and new lesions may continue to appear as old lesions disappear. Discussion The answer is B: lichen nitidus.2 69 percent of eruptions resolved within one year. Lichen nitidus. In one series. Lichen nitidus can be discrete or generalized.3 No racial predisposition or known genetic inheritance pattern has been noted.2 In the generalized (confluent) form. Lichen simplex chronicus. Psoriasis. D. papules coalesce into redyellow to brown plaques. definitive establishment of the epidemiology is difficult. which one of the following is the correct diagnosis? A. however. Question Based on the patient's history and physical examination. This. although the generalized (confluent) form may be more common in women. Hundreds of pinpoint-sized. makes clinical observation a reasonable treatment option. C.3 Biopsy may be helpful because lichen nitidus has a characteristic histologic appearance. Lichen nitidus most commonly presents as an incidental finding on physical examination or after the patient notices an insidious onset of the lesions. they may form or group at sites of trauma or skin pressure (the isomorphic or "Koebner” phenomenon). One study2 of 43 cases demonstrated a male-tofemale ratio of almost pinhead-sized skin-colored papules that may be scaly or have a central depression. B. especially in joint flexures. 1 It is characteristically asymptomatic. trunk.2 .2 Physical examination reveals pinpoint. Because lichen nitidus is rare. or inframammary areas.2 Lichen nitidus primarily affects children and young adults. Skin-Colored Papules on the Arms and Hands A 27-year-old man presents with a 10-year history of asymptomatic lesions on his hands and elbows. Prurigo nodularis. wrist and forearm ventral surfaces. E. In the discrete form. making the clinical diagnosis more challenging. and the glans and shaft of the penis. along with its asymptomatic nature. papules typically do not coalesce. Lichen planus. however.1. with a median age of seven years in males and 13 years in females.

psoralen with long-wavelength ultraviolet light therapy. white scale Thick plaques with increased skin markings that develop after chronic rubbing Skin-colored to hyperpigmented nodules that develop after external manipulation Pruritic.4 Short courses of systemic corticosteroids. Selected Differential Diagnosis of Lichen Nitidus Condition Psoriasis Lichen simplex chronicus Prurigo nodularis Lichen planus Characteristics Erythematous plaques with thick. lichen planus lesions are violaceous and larger than those of lichen nitidus. which may help to distinguish it from lichen nitidus.6 and sun exposure7 have been used to treat generalized disease. Appropriate initial management of this patient may include all of the following except: A. polygonal papules The differential diagnosis includes psoriasis. and lower lumbar area. Psoriasis typically presents as erythematous plaques with thick. Topical steroids may cause lesions to flatten or resolve.5 long-wavelength ultraviolet light/medium wavelength ultraviolet light with systemic corticosteroids. Lichen simplex chronicus is a thickening of the skin caused by chronic rubbing. However. . These papules or nodules develop in areas that the patient can reach. lichen simplex chronicus. violaceous. scalp. FIGURE 1. Prurigo nodularis is similar to lichen simplex chronicus in that the lesions develop because of external manipulation. prurigo nodularis. knees. and typically presents as thick plaques. which was worse in the sun-exposed areas of her skin but involved sun-protected areas as well. Photo Quiz Question A 67-year-old woman presented after three years of progressive hyperpigmentation. lichen planus has a predilection for the ventral wrists and the buccal mucosa. Lichen planus may appear similar to lichen nitidus. Finally. and lichen planus. lichen planus often is pruritic. white scale on the elbows. flat-topped.2 Characteristically. Medication history.The evidence base for other treatments is limited.

particularly the face. Erythema dyschromicum perstans. Finally. are other benign causes of hyperpigmented lesions. such as hydroquinone. and the sequela of resolving lichen planus may cause hyperpigmented lesions. Punch biopsy at border of lesion. A punch biopsy at the border of the lesion is necessary to rule out melanosis secondary to malignant melanoma that manifests as a generalized blue-gray skin discoloration. Certain drugs. commonly known as ashy dermatosis. which occur in otherwise healthy persons. as a cause of hyperpigmented macules. particularly women in the first through third decades of life. thiazides. dapsone. the patient should be examined for the characteristic lacy white patterned and erosive lesions of the oral and genital mucosa to rule out lichen planus. melasma.1-3 The etiology of erythema dyschromicum perstans remains unknown. such as chlorpromazine. particularly a fixed drug eruption. an . which presents in sun-exposed areas. was first described by Ramirez in 1957. Discussion The answer is D: Nd:Yag laser treatment using 532 nm wavelength. C. and obtain a punch biopsy at the border of the lesion to rule out other possible causes of hyperpigmentation. may initially present as erythematous macules that slowly progress to a blue-gray hue. and less frequently over the face and neck. griseofulvin. Thus. slate-gray or violaceous hyperpigmented macules distributed most commonly over the trunk and proximal extremities. General health history. Ashy dermatosis is a chronic condition characterized by asymptomatic. no effective treatment of erythema dyschromicum perstans is available at this time. Oral and genital examination. A general health history can help distinguish diffuse hyperpigmentation caused by internal disease entities. such as Addison's disease and hemochromatosis. tretinoin. and tetracyclines are the most common culprits. This uncommon disorder occurs predominately in dark-skinned Latin Americans. perform an oral and genital examination. and sunscreen. topical steroids. Review of medications may indicate a drug hypersensitivity reaction.2 Unfortunately. Clofazimine. and postinflammatory hyperpigmentation. Melanin complexes in the dermis and epidermis are responsible for the ashen-gray color that occurs in sun-exposed and sun-protected areas. Nd:Yag laser treatment using 532 nm wavelength.1 It is important to obtain a thorough general health and medication history. amiodarone. The macules of erythema dyschromicum perstans vary in size and may occasionally demonstrate an erythematous raised border.B. have been tried but have had poor clinical results. Lesions. Several medications. Several authors speculate that erythema dyschromicum perstans may be a variant of lichen planus. D. E.1. which occurs after inflammatory dermatoses.

FL 34681-0219. P. These lesions developed over the past three years. M. . Necrobiosis lipoidica. Small ulcerations had recently developed on the involved skin. C. A 54-year-old man with type 2 diabetes mellitus complained of asymptomatic. Pretibial myxedema. has recently been studied in six patients who demonstrated marked improvement with treatment. These reddish-brown plaques had depressed shiny centers. C.3 Nd:Yag laser has been attempted for the treatment of postinflammatory hyperpigmentation and melasma with disappointing results and is not recommended in the treatment of ashy dermatosis.M.1 Photo Quiz Skin Rash in a Patient with Diabetes The editors of AFP welcome submission of photographs and material for the Photo Quiz department. and dilated deep subcutaneous vessels could be seen through the atrophic skin. Crystal Beach. which one of the following is the most likely diagnosis? A. C. Granuloma annulare.. and various creams had failed to help. M.D.antileprosy drug. Berger. slowly enlarging. brownish plaques on the anterior surfaces of both lower legs (see accompanying figure).. Send photograph and discussion to Marc S. Contributing editor is Marc S.D. Box 219. Question Given the patient's medical history and the physical appearance of the lesions.. Erythema nodosum. B. D.O.M. Berger.

PAS-positive staining in the areas of necrobiosis. oval reddish-brown plaques over the anterior portion of the legs (bilateral in 75 percent of cases). the lesions of necrobiosis lipoidica are asymptomatic.2 were diabetic).4 Likewise. Clinical variants may be solitary and may be seen on the hands.3 The typical clinical presentation is that of multiple. and it is the cosmetic effect that is of greatest concern to the patient. The female-to-male ratio is 3:1. but lesions often recur within or around the graft.1 Patients may complain of pruritus. Ulceration occurs in about one third of diabetic patients with necrobiosis lipoidica. Surgical intervention may be necessary if these measures are unsuccessful and nonhealing ulcers persist. Resembling bruises.3 Necrobiosis lipoidica usually develops in the third or fourth decade of life. however.4 There is no universally satisfactory intervention for necrobiosis lipoidica. Fever and . Moisturizing of the skin. fibrosis. More frequently. dysesthesia. and nipples. protection against trauma or skin injury. degeneration and thickening of collagen bundles in the dermis. The histologic features of necrobiosis lipoidica include poorly defined histiocytic granulomas with necrobiosis in the middle to deep dermis. but it may be seen in younger or older individuals. Discussion The answer is D: necrobiosis lipoidica. and measures to improve the circulation of the lower limbs are recommended for all patients with this disease.E. and vascular changes consisting of endothelial swelling.3. red nodules most commonly on the anterior shins. inflammatory phase of necrobiosis lipoidica. but it occurs more commonly in patients with well-established metabolic disease. fingers. with the center developing a yellowish sheen and prominent telangiectasias. Plastic surgeons advise excision of the involved area down to fascia and ligation of associated perforating blood vessels followed by split-thickness skin grafts.4 Erythema nodosum is an inflammatory process of the deep dermis and subcutaneous fat characterized by shiny. Diabetic dermopathy.1 but care should be exercised with local steroid use because ulceration may occur. deep.4 The plaques often slowly enlarge.3 percent).4 and spontaneous remission is relatively uncommon (19 percent). The condition may precede the diagnosis of diabetes mellitus. and hyalinization. face. the nodules gradually change from pink to bluish to brown. forearms. injection of triamcinolone in perilesional skin has been used with success. Necrobiosis lipoidica is an unusual skin disorder that is strongly associated with diabetes mellitus. scalp. High-potency topical steroids may be useful in the early.1 but its presence is said to be a strong marker for the disease (65 and 42 percent of patients in two series 1. tender. or pain at the site of lesions. The condition develops only in a small proportion of patients with diabetes (0.5 The admonition to "do no harm" may be well advised in this disorder.

This condition is most commonly associated with Graves' disease. Lesions enlarge slowly over a period of months to years and can be yellowish tan. or fingers. They are thought to be caused by vascular disease. inflammatory bowel disease. Diabetic dermopathy (skin spots) is the most common dermatosis associated with diabetes. Granuloma annulare may be hard to distinguish from early necrobiosis lipoidica. Less common causes include other infections. A 50-year-old white woman presented with a rash that first erupted on her trunk three years earlier and never fully cleared (see accompanying figure). Photo Quiz FIGURE 1. but they are usually much smaller (0.0 cm) in size and greater in number (five to 10. feet. bluish. Erythema nodosum is most commonly seen following upper respiratory infections.arthralgia frequently accompany the rash. it presents with reddish-brown patches on the shins. There are many variants. hands. atrophic scar. Similar to necrobiosis lipoidica. but granuloma annulare is typically characterized by several papules or nodules that spread to form a ring around normal or slightly depressed skin. or the color of the surrounding skin. or more lesions). erythematous. Skin spots gradually resolve to leave a brown. and drug reactions (especially oral contraceptives). and it is thought to be caused by thyroid-stimulating hormone-induced deposition of mucin. sarcoidosis. especially those involving Streptococci. Pretibial myxedema presents as waxy plaques on the shins.5 to 1. The lesions became pruritic after a . They are usually asymptomatic and occur on the distal portion of the legs. with prominent hair follicles that give the peau d'orange appearance. Spontaneous resolution is common and no treatment is required. but there is no correlation with the extent or duration of diabetes.

warm shower or strenuous physical exercise. bronchoconstriction. Physical examination revealed many discrete erythematous papules spread over the abdomen. Lichen planus.Pityriasis rosea. With urticaria pigmentosa. In contrast. Urticaria pigmentosa is the most common form of mastocytosis. E. The mononuclear cells stained strongly for tryptase. such as occurred with this patient. urticaria. and back. Accumulation of mast cells in organs other than the skin occurs primarily in adults. which confirmed the diagnosis of urticaria pigmentosa. gastrointestinal tract. a wheal formed at the site. Histamine-induced hypersecretion may cause gastritis. lymph nodes. Urticaria pigmentosa. C. and bone marrow. Scabies.. B. Release of prostaglandins may cause . a type of cutaneous mastocytosis. Biopsy of a skin lesion revealed superficial infiltrates of mononuclear cells and some eosinophils. termed mastocytosis. abnormal mast cell accumulation is limited to the skin.1 Onset of urticaria pigmentosa in adulthood. toluidine blue O) or enzymatic stains (e. Mast cell degranulation with hormone release and direct organ infiltration lead to the symptoms seen with systemic mastocytosis. but also may involve the liver. malabsorption may occur. and diarrhea. Approximately one half of cases occur before six months of age. spleen.g. or any palpable lymphadenopathy. peptic ulcers. special stains must be ordered when mastocytosis is suspected. and another one fourth before puberty. The patient was given hydroxyzine for pruritus and referred to a hematologist for further testing. These include the metachromatic stains (e. which one of the following is the correct diagnosis? A. and vasopermeability (possibly leading to vascular collapse). Urticaria pigmentosa is part of a group of disorders that shares an abnormal growth of mast cells. Because mast cells often are missed using conventional staining. is less common.g. The patient did not have hepatosplenomegaly. On vigorous rubbing of a papule. gastrointestinal complaints. Urticaria. systemic mastocytosis affects not only the skin. Question Based on the patient's history and physical examination. D. chloroacetate esterase). upper chest. and ultimately. but were otherwise asymptomatic.. Discussion The answer is E: urticaria pigmentosa. Other histamine-induced symptoms include pruritus.

Pityriasis rosea may lead to a truncal macular rash. and finger webs. and cardiovascular abnormalities (e. hypercellular bone marrow. such as cromolyn and ketotifen. The eruption may last up to two or three months. and an associated hematologic disorder. a contagious skin infestation caused by a mite. They usually are less widespread than urticaria pigmentosa and would not wheal when rubbed. and flushing. Finally. Adults with urticaria pigmentosa should undergo bone marrow biopsy to look for evidence of systemic mastocytosis. vascular collapse). Clinical factors associated with poor prognosis include anemia. have been found to have a moderate effect on pruritus. tachycardia. rash. serum tryptase levels greater than 20 ng per mL suggest systemic involvement. Unlike urticaria pigmentosa. In addition to many small macules. is typically highly flushing. Urticaria typically presents with larger areas of erythema. Photo Quiz Generalized Pruritus Without Relief . but also may occur as small erythematous papules.2 In addition. Scabies. but rarely develop hematologic disease. violaceous papules with scale.. herald patch) may be seen. but not years. which may be treated with H1-receptor antagonists. intestinal cramping. cytologic atypia.3 Lichen planus also is a pruritic. Inhibitors of mast cell degranulation. but the lesions are typically flat-topped. Skin lesions may be widespread but tend to cluster in the intertriginous areas. Adults with urticaria pigmentosa usually progress slowly to systemic disease. The most common symptom in urticaria pigmentosa is pruritus. individual urticarial skin lesions wax and wane over a course of hours and would not persist in the same location as chronic papules. hepatosplenomegaly. a large patch (i. whealing. some patients may benefit from topical corticosteroids or psoralens and ultraviolet A (PUVA) therapy.e. such as the axilla.. inframammary area. groin. sometimes with pruritus. often chronic.

Crusting and lichenification also were observed. E. which one of the following is the most likely diagnosis? A. Psoriasis vulgaris. feet. such as elbows. No lesions were present on the face. Discussion The answer is C: severe atopic dermatitis. The most common lesions are papules and nodules like those shown in the photograph. Physical examination revealed numerous 15 to 20 mm papulonodular lesions over all the extremities and trunk with some confluence (see accompanying figure).1 Psoriasis is an inflammatory epidermal proliferative disorder of the skin. . C. knees. Atopic dermatitis is a form of endogenous dermatitis resulting in pruritic inflammation of the epidermis and dermis. which commonly occurs in infants and children but can be found in adults. Atopic dermatitis affects more than 10 percent of children. but in psoriasis they are sharply demarcated and covered by silvery white scale. and the majority of patients are affected during the first five years of life. scalp. Associated findings may include fingernail pitting or thickening (50 percent of cases) and arthritis (up to 10 percent of cases). B. Question Based on the patient's history and physical examination. Pemphigus vulgaris is an autoimmune bullous disease of the skin that usually occurs in adults and is rare in children. Lichen planus. Pemphigus vulgaris. D. This can be a serious and potentially fatal disorder. Sweet's syndrome. The lesions most commonly involve areas of the body that experience repeated minor trauma. The lesions are flaccid vesicles or bullae that are initially localized in oral mucosa and later spread randomly to other parts of the body. which is usually not intensely pruritic.A nine-year-old girl presents with a threemonth history of severe persistent itching and skin lesions throughout most of her body. There is no pruritus. Severe atopic dermatitis. and hands.

Patients with atopic dermatitis often have diffusely dry skin. and genitalia. The pruritus is variable. lumbar area. allergic rhinitis. Pruritic papules with vesicles are typical initially. arthralgias. it is often difficult to differentiate the lesions from the other conditions as outlined above because of the lack of contrast between the lesions and uninvolved skin. detergents. whereas children and adults have predilection for the flexural areas of the extremities. and trunk are involved. sweating. Hot baths or frequent bathing may cause drier skin and should be avoided. although patients may have elevated levels of IgE and peripheral blood eosinophilia. seborrheic dermatitis. Lichen planus is uncommon in children. infection. and upper trunk. and psoriasis. The common sites include wrists. In blacks. No laboratory tests are available to definitively establish a diagnosis of atopic dermatitis. The onset of the skin lesions is usually subacute or chronic. Topical steroid ointments or creams in addition to cool wet dressings are useful in acute flares of the disease as well as in maintenance of healing in the . The diagnosis of atopic dermatitis is based primarily on clinical presentation and history. and approximately 10 percent of patients have an associated underlying malignancy. a subtype of atopic dermatitis occurs frequently where each papule involves a separate hair follicle. Use of H2 blockers in cases of severe pruritus may be helpful. mucous membrane. Common triggers of atopic dermatitis include dry skin. and perfumes. especially with Staphylococcus aureus. cosmetics. these may become scaly and crusty later. Atopic dermatitis is frequently accompanied by a personal or family history of asthma. Secondary infection is common.Lichen planus is characterized by flat-topped. Mild or no soap should be used when bathing. Oral antihistamines can help control itching. and upper extremities. Symptomatic treatment consists of the use of moisturizers to prevent the itching caused by dry skin. and is termed follicular eczema. The histopathologic changes seen in biopsy specimens of atopic dermatitis are nonspecific. These irritants can include soaps. and skin irritants. wool and acrylic clothing. Truncal lesions are uncommon. neck. The distribution of the lesions may become more diffuse in the chronic condition. and peripheral leukocytosis.2 Atopic dermatitis may be confused with a number of the eczematous dermatitides including contact dermatitis. Sweet's syndrome is an uncommon disorder of the skin that generally has associated systemic symptoms like fever. In dark-skinned patients. polygonal. neck. shins. physical or emotional stress. or allergic skin involvement. the extensor surface of the extremities. face. Chronic lesions may have thickened lichenified skin with fibrotic papules and nodules as are seen in this case. linens. In infants. The lesions are red-brown plaques and nodules over the head. violaceous papules. which are much smaller than the confluent papulonodular lesions seen in severe atopic dermatitis. Avoidance of any of the irritants or triggers is the cornerstone of therapy. which are painful rather than pruritic.

Topical or oral antibiotics should be considered when there is suspicion of secondary infection.3 . or recombinant IFN-g. Other alternatives include phototherapy using ultraviolet radiation. cyclosporin. systemic corticosteroids may be used. In cases of severe intractable atopic dermatitis. because this may be a cause of persistent pruritus.chronic phase.