Sweet's syndrome in acute myelogenous leukemia presenting as periorbital cellulitis with an infiltrate of leukemic cells

Sweet's syndrome is characterized by the abrupt onset of fever, neutrophilic leukocytosis, and erythematous, tender pseudovesiculated plaques or nodules that respond readily to corticosteroid therapy. It is usually distinguished by the presence of mature neutrophils on histopathologic examination. We describe a 38-year-old man with acute myelogenous leukemia who had an erythematous vesicular eruption of the left eye develop that resembled cellulitis. A biopsy specimen revealed a dermal infiltrate of mature neutrophils and immature myeloblastic precursors. He later had hemorrhagic pseudovesiculated plaques develop bilaterally on his hands. A biopsy specimen again revealed abundant neutrophils with immature forms. A similar eruption developed at the site of a Hickman catheter placement 4 months later. His skin lesions responded rapidly to oral corticosteroids. This case is unique in that his initial presentation of Sweet's syndrome resembled orbital cellulitis that was characterized by immature myeloid precursors on histopathology.     

A case of recurrent Sweet's syndrome in an 80-year-old man: a clue to an underlying malignancy

An 80-year-old man presented with multiple erythematous papules on the trunk and extremities of a few weeks' duration. He had no past medical or family history of skin diseases or any other medical diseases. A biopsy showed a perivascular lymphohistiocytic infiltrate and sparse neutrophils with several atypical lymphocytes in the deeper dermis. With an initial diagnosis of T-cell pseudolymphoma or unspecified neutrophilic dermatosis, he showed a brisk response to an intramuscular injection of triamcinolone acetonide (40 mg/mL). After 1 month, his skin lesion recurred. Steroid was given with a good clinical response. One month later, however, his skin lesion relapsed. At this time, he presented with disseminated pustulopapular lesions on the trunk and extremities. Examination revealed multiple, variable-sized, erythematous plaques with central pustules on the extremities (Fig. 1). The mucous membranes were not involved. He had no pain or tenderness. He had no systemic symptoms. Laboratory tests showed a hemoglobin level of 10.3 g/dL, a leukocyte level of 6,900/mm(3), with an increased proportion of segmented nuclear neutrophils (83%), and an elevated C-reactive protein. A skin biopsy revealed a dense perivascular and interstitial infiltrate composed of neutrophils with marked dermal edema (Fig. 2). Sweet's syndrome was the final diagnosis and he was treated with oral prednisolone (30-40 mg/day) and dapsone (50 mg/day) for 2 months. As this 80-year-old patient had a recurrent history of similar skin lesions and anemia, an underlying hematologic malignancy was suspected. A bone marrow biopsy showed typical myelodysplastic syndrome (MDS). The hemoglobin level was decreased to 5.3 g/dL during a follow-up period of 5 months. The skin lesions recurred despite oral steroids and dapsone. The patient received only symptomatic treatment, such as a transfusion, for the underlying malignancy MDS.     

Lymphomatoid papulosis and FK 506

A 53-year-old woman underwent an orthotopic liver transplant in Pittsburgh in October 1990. She had been suffering from chronic hepatitis C that had evolved into cirrhosis with ascites, jaundice, and encephalopathy. In April 1991 she required reconstruction of the biliary anastomosis because of stricture, and in October 1991 she underwent a liver biopsy because of mild elevation of alkaline phosphatase. The biopsy revealed mild chronic rejection and changes consistent with viral hepatitis. She was on oral FK 506, 4 mg b.i.d. In November 1992 she developed recurrent self-healing erythematous, papulonodular lesions on the chest and shoulders (Pig. 1). The lesions were purplish red, mildly pruritic, and undergoing vesiculation on the top. The lesions ranged from 3 to 5 mm in size, and a few showed ulceration and necrosis and were healing with hypopigmented scars. The lesions gradually increased in number and size. A trephine biopsy specimen was obtained from the lesional skin. The tissue was prepared for light microscopic study by fixing in 10% formaldehyde solution and staining with hematoxylin and eosin. Immunohistochemistry was carried out for lymphoid markers. In February 1993, a papulonodular lesion near the left axilla enlarged to a size of 10 × 10 cm; it was ulcerated and necrotic. A biopsy was taken and sent for histopathology and immunohistochemistry. The patient continued on FK 506, 4 mg b.i.d. Many of the early lesions had become purpuric, eroded, and healed with scarring; however, fresh lesions continued to appear. A decision about modification of the patient's immunosuppressive medication was left to the Transplant Team; however, there was concern that her lymphomatoid papulosis may be a side-effect of her PK 506 treatment. Laboratory investigation revealed a normal peripheral blood film. Liver enzymes and a renal profile were within normal limits. The results of a bone marrow study, liver and spleen scan, and endoscopic examination of the gastrointestinal tract were normal. A skin biopsy revealed a nodular and wedge-shaped dermal infiltrate involving the entire dermis. The pleomorphic epidermotropic infiltrate in the upper one-third of the dermis was composed of many round or oval cells with hyperchromatic nuclei. A few nuclei were indented or kidney shaped. Mild mitotic activity was seen. The larger immunoblast-like cells were mixed with normal lymphoctyes and histiocytes. The capillaries had thickened walls with prominent endothelial cells. The second biopsy from the ulcerated axillary lesion showed a dense superficial and deep wedge-shaped infiltrate, abnormal pleomorphic cells, and a few large lymphoid cells (Fig. 2) infiltrating the dermis and extending to the subcutaneous tissue. There were scanty histiocytes, neutrophils, and a few eosinophils. Some of the large abnormal cells had kidney shaped nuclei but most had irregularly shaped nuclei with abundant cytoplasm (Fig. 3). There were many abnormal mitoses, and there were also numerous extravasated red blood cells and edema in the dermis. The epidermis was necrotic and ulcerated. The results of immunohistochemical tests showed a strong reaction for LCA and CD8, but a negative reaction for CD20. The patient's general condition and laboratory tests of hepatic and renal functions remained normal. The results of all hematologic studies, including bone marrow smear, lymph node biopsy, and liver-spleen scan, were normal. The patient continued on FK 506, 4 mg b.i.d. Serum levels of FK 506 which were never communicated to us were sent for the first time in May 1993 and showed a serum level of 39 ng/mL (normal 0.5–2 ng/mL)¹ with an instruction to repeat the test. The dose of FK 506 was immediately reduced to 4 mg/day. The patient revisited the clinic for routine check-up on June 26, 1993. The axillary lesion had disappeared leaving a hyperpigmented scar, the small lesions of lymphatoid papulosis continued to come and go.     

Disseminated gonococcemia

A 26-year-old woman presented with a high-grade fever and chills of 2 days' duration. She complained of associated joint pain, especially in the wrists and knees. One day before admission, tender skin lesions began to develop on the fingers, and subsequently spread to the more proximal extremities. The patient recalled having a sore throat and a nonproductive cough before the onset of the fever and eruption. The past medical history was significant for Gardnerella vaginitis and several urinary tract infections. The patient was taking oral contraceptive pills; her most recent menstruation was 3 weeks before admission. She reported having sexual intercourse with her boyfriend 2 weeks before admission. The patient's temperature was 40 degrees C. Dermatologic examination revealed a 6-mm, hemorrhagic pustule on an ill-defined pink base, overlying the volar aspect of the left second proximal interphalangeal joint (Fig. 1a). Scattered on the upper and lower extremities were occasional round, ill-defined pink macules with central pinpoint vesiculation (Fig. 1b). A skin biopsy of the digit revealed a dense neutrophilic infiltrate with leukocytoclasis and marked fibrin deposition in the superficial and deep dermal vessels (Fig. 2a). Gram stains demonstrated the presence of Gram-negative diplococci (Fig. 2b). Laboratory findings included leukocytosis (leukocyte count of 20 x 109/L, with 81% neutrophils). Analysis of an endocervical specimen by polymerase chain reaction was positive for Neisseria gonorrhoeae and negative for Chlamydia trachomatis. Throat and blood cultures grew N. gonorrhoeae. Specimen cultures obtained by skin biopsy yielded no growth. Results of serologic analysis for human immunodeficiency virus, hepatitis, syphilis, and pregnancy were negative. Beginning on admission, intravenous ceftriaxone, 2 g, was administered every 24 h for 6 days, followed by oral cefixime, 400 mg twice daily for 4 days. Oral azithromycin, 1 g, was administered to treat possible coinfection with C. trachomatis. By treatment day 4, the patient was afebrile, with the resolution of leukocytosis and symptomatic improvement of arthralgias.     

Differentiation syndrome during ivosidenib treatment with immunohistochemistry showing isocitrate dehydrogenase R132H mutation

We report a case of differentiation syndrome in a patient receiving the IDH1 inhibitor ivosidenib, with skin biopsy showing isocitrate dehydrogenase (IDH) R132H-mutated leukemia cutis. A 72-year-old man with IDH1-mutated acute myeloid leukemia (AML), status-post allogeneic cell transplantation, on ivosidenib for 6 months, was admitted for culture-negative neutropenic fever, pink and purpuric plaques and patches on the legs, abdomen and back, edema, hypotension, and shortness of breath. Skin biopsy revealed an infiltrate of atypical, immature, myeloperoxidase-positive mononuclear cells compatible with leukemia cutis or Sweet syndrome. Although dermal edema and interstitial neutrophilic infiltrate with karyorrhexis characteristic of Sweet syndrome were not seen, the atypical cells lacked expression of CD117 and CD34, which were expressed in the original leukemia. Additional immunohistochemical staining of suspected blasts was strongly positive for IDH1 R132H, suggesting a diagnosis of leukemia cutis. As the immunophenotype of blasts in skin infiltrates can significantly differ from the immunophenotype seen in blood and bone marrow, this case shows that mutation-specific antibodies such as anti-IDH1 R132H may be useful to help distinguish malignant from non-malignant infiltrates in the skin. Furthermore, differentiation syndrome may show histopathologic features of leukemia cutis on skin biopsy.     

Acute Febrile Neutrophilic Dermatosis (Sweet's Syndrome) Associated with Extreme Infiltration of Eosinophils

A remarkable infiltration of eosinophils was observed in skin biopsy specimens in two cases of Sweet's syndrome. One patient was a 29-year-old woman, whose clinical and histological features included associated asthma, a prior respiratory tract infection, red plaques, blood eosinophilia, and a dense dermal infiltrate of neutrophils and eosinophils, without evidence of vasculitis. The other patient was a 61-year-old man characterized by a fever, red plaques, erythema nodosum-like lesions, hepatic dysfunction, a dermal infiltrate of neutrophils accompanied by eosinophils, and a subcutaneous prominent infiltrate of eosinophils. Clinical and histological evidence supported the diagnosis of Sweet's syndrome in both of these patients. While the literature describes eosinophil infiltration in this disease, extreme eosinophil infiltration could be misleading in the diagnosis.     

Disseminated granuloma faciale

A 40-year-old man presented in January 2001 with multiple purple plaques and nodules, which had been present on the back for approximately 3 years. The lesions had gradually extended over the face, trunk and proximal extremities. He had no symptoms except occasional mild pruritus. The patient was in good health and was on no medications. Physical examination revealed multiple violaceous to brown, indurated, 5-50-mm, round to oval plaques on the face, arms, shoulders, and back (Fig. 1), as well as a solitary lesion on the right thigh. Surface telangiectases were noted, especially on the shoulder lesions. There was no scaling or ulceration. Routine laboratory tests were unremarkable. In April 1999, another medical center performed a biopsy of what they thought was sarcoidosis. The results were reported as "possible angiolymphoid hyperplasia with eosinophilia." With the possibility of granuoma faciale (GF) in mind, another skin biopsy was obtained from a facial lesion. This revealed a diffuse, relatively dense infiltrate of neutrophils, eosinophils and mononuclear inflammatory cells in dermis with an obvious Grenz zone (Fig. 2). Pilar units were intact, and endothelial cell swelling was present (Fig. 3). Retrospective evaluation of the initial biopsy, taken from the back, revealed the same changes, and helped confirm the diagnosis of GF. The patient was treated with liquid nitrogen for 20 s followed immediately by intralesional triamcinolone acetonide (5 mg/ml). This treatment was repeated every 4 weeks for three courses, resulting in partial resolution of the lesions.     

Mycosis fungoides mimicking erysipelas

Mycosis fungoides may rarely simulate facial erysipelas. In a patient with that clinical presentation, a biopsy specimen revealed a diffuse dermal infiltrate with numerous neutrophils that also mimicked erysipelas histopathologically. The diagnosis of mycosis fungoides was made on the basis of atypical lymphocytes with focal epidermo‐ and folliculotropism. It was confirmed by typical findings of mycosis fungoides in a second biopsy from a clinically inconspicuous patch. An identical T‐cell receptor γ‐gene rearrangement was detected in lymphocytes of both biopsy specimens.     

Sterile suppurative folliculitis associated with acute myeloblastic leukaemia

A 20-year-old woman presented with a 4-month history of follicular papules distributed over the trunk and extremities. One month later, routine blood tests were abnormal, showing acute myeloblastic leukaemia (M1 in the French-American-British classification). Skin biopsy demonstrated a dermal infiltrate of a large number of neutrophils with occasional eosinophils and histiocytes in the vicinity of the hair follicle remnants. Intermingled in the infiltrate were atypical cells that were morphologically and immunohistochemically identical to leukaemic myeloblasts. Cultures of the papules and special stains of the biopsy specimen were negative for bacteria and fungi. The follicular eruption improved promptly in response to chemotherapy for the leukaemia. We suggest that this case may represent a rare, follicular variant of neutrophilic dermatosis associated with myelogenous leukaemia.     

Atrophoderma of moulin with preceding inflammation

A 16-year-old Vietnamese man presented to the Dermatology Clinic with a 10-year history of bizarre brown patches, which initially started as red asymptomatic "bumps" on the trunk, upper and lower extremities, and face. His past medical history was significant for hypothyroidism and idiopathic urticaria. He was on Eltroxin for hypothyroidism. The family history was noncontributory. Physical examination revealed two types of lesion: erythematous, well-circumscribed papules in a linear configuration along with linear hyperpigmented atrophic patches following Blaschko's lines were noted on the lower extremities (Fig. 1), right upper extremity, right flank (Fig. 2), and right jawline. Initial biopsies taken from the papular lesions on the right thigh and right elbow revealed the following changes. The first biopsy showed a slightly thinned epidermis with prominent dilated blood vessels in the superficial dermis. There also appeared to be a slight increase in the amount of collagen in the deep dermis. The findings were reported as in keeping with "epithelial atrophy." The second biopsy from the lesion on the right elbow revealed an acanthotic epidermis. The granular layer was absent in several areas and there was marked overlying parakeratosis. In the dermis, there was a heavy perivascular lymphocytic infiltrate. The appearances were consistent with a psoriasiform dermatitis (Fig. 3). A biopsy taken from the left thigh approximately 18 months later showed slight irregular acanthosis with dermal edema, dilated blood vessels, and a patchy lymphocytic infiltrate. The appearances were compatible with mild inflammation.     

Annular atrophic plaques of the skin (Christianson's disease)

A 69-year-old Caucasian man presented to the Gainesville Veterans Administration Medical Center for evaluation of several asymptomatic enlarging lesions on the face and forearms that had been present for 10 to 15 years. They were initially small but had progressively enlarged, especially during the previous 5 years. He reported having sustained a concussive grenade blast injury of the left temple and right forearm during the Korean Conflict in 1951. The injured areas healed uneventfully in 2–3 weeks. The patient was otherwise healthy, and the review of systems was noncontributory. Laboratory work-up (complete blood count, chemical profile, erythrocyte sedimentation rate, rheumatoid factor, hepatitis profile, antinuclear antibodies, urinalysis, and chest X-ray) was within normal limits. Lyme antibody titers were negative. Physical examination revealed similar-appearing lesions located variously on both temples, left preauricular and infraauricular areas, and the right forearm (Fig. 1). The lesions were large (ranging from 4 to 12 cm in diameter), centrally atrophic patches with ivory-colored thickened edges, and had the distinct appearance of healed skin grafts. Blood vessels were easily seen through the atrophic skin. All lesions were located on sun-exposed areas (predominantly on the patient's left side). No similar lesions elsewhere on the body or skin graft donor sites were found. The patient was treated with doxycycline 100 mg by mouth twice a day for 21 days to no avail, Intralesional steroids and clobetasol propionate ointment under occlusion were used without any change in the lesions. At the submission of this paper, the lesions remain stable. Skin biopsies were taken from the edge and center of the lesions, Histologic examination (Fig. 2) of the center of a lesion showed a flattened epidermis without interface changes. Sclerosis of superficial and mid, but not deep, dermal collagen was present, particularly within the specialized connective tissue surrounding eccrine structures. A focus of lymphocytic inflammation with rare plasma cells and edema was present at the dermal-subcutaneous interface. Histologic examination of the edge of the lesion showed a flattened epidermis overlying a perivascular and periadnexal lymphohistiocytic inflammatory infiltrate. The papillary dermis was sclerotic centrally, and sclerosis was present around one eccrine unit. Increased interstitial mucin was noted. Direct immunofluorescence using antibodies to IgG, IgM, IgA, and C3 was negative, A silver stain for borrelia organisms was negative. Electron microscopy of a biopsy from the advancing border of a lesion revealed foci of complete cytoiysis of the basilar epidermis above an interrupted basal lamina (Fig, 3a). Cellular debris was evident even within the subajacent papillary dermis (Fig. 3b). Abundant colloid-like material associated with occasional 8–10-nm straight tubules was also present within the upper papillary dermis. A solitary lymphocyte lay closely apposed to a basilar keratinocyte having large cytoplasmic vacuoles. Electron microscopy of a biopsy from the center of the same lesion revealed a flattened basilar epidermis and focal reduplication and interruption of the basal lamina (Fig. 3c). Abundantly interspersed among the collagen bundles of the upper and lower papillary dermis were aggregates that appeared amorphous at low magnification. Upon higher magnification, however, the aggregates could be seen to be comprised of straight and wavy tubules embedded in a finely granular matrix (Fig. 3d). A dermal blood vessel was surrounded by several basal laminae, just outside of which were presumptive lymphocytes with cerebritorm nuclei and histiocytes. No dermal elastic fibers could be identified. Collagen fibrils appeared normal.     

CHRONIC BULLOUS DERMATOSIS OF CHILDHOOD: DIFFERENTIATION FROM OTHER AUTOIMMUNE BLISTERING DISEASES IN CHILDREN

An 8-year-old girl presented with a 1-week history of intensely pruritic vesicles and bullae on the trunk and extremities. The patient was otherwise in good health and was not taking any medications.
Physical examination revealed multiple large, tense bullae on normal-appearing skin on the trunk, extremities, and pelvic region. Some of the bullae were hemorrhagic, while others were grouped in circular clusters (Fig. 1A, B). The oral cavity and conjunctivae were spared. Complete blood cell count, electrolytes, serum urea nitrogen, creatinine, and liver function studies were normal.
Histopathologic examination of perilesional skin demonstrated a subepidermal blister with neutrophils in the dermal papillae (Fig. 2). Direct immunofluorescence of the specimen showed heavy deposition of IgA in a linear pattern in the basement membrane zone (Fig. 3). Broad but faint deposits of IgM were also present. Blood obtained for indirect immunofluorescence revealed no circulating autoantibodies.
The patient was diagnosed with chronic bullous dermatosis of childhood and treated with dapsone (2 mg/kg) with dramatic improvement. New blister formation and puritis were suppressed within several days. All lesions healed with mild hyperpigmentation but without scarring.     

Cutaneous lymphoma manifesting as pigmented, purpuric capillaries]

INTRODUCTION: A few cases of patients with both purpuric pigmented dermatitis and cutaneous lymphoma have been reported. The aim of this study was to evaluate the prognosis of purpuric pigmented dermatitis. MATERIAL AND METHODS: This is a monocentric retrospective study at the dermatology department of the university hospital of Strasbourg. The records of all patients hospitalized for purpuric pigmented dermatitis between 1967 and 1997 have been reviewed. RESULTS: Eight women and 9 men aged between 17 and 84 years were hospitalized for purpuric pigmented dermatitis during the reference period. Except for one patient, all had had a cutaneous biopsy showing the typical features of purpuric pigmented dermatitis. On the basis of clinical signs and course, one patient was thought to have contact dermatitis and three patients were thought to have a purpuric pigmented dermatitis-like drug eruption (meprobannate, pefloxacine and lorazepam or aspirin). The mean follow-up was one year. During follow-up, two patients developed cutaneous T-cell lymphoma after two and four years respectively and one patient developed Hodgkin's disease with skin and lymph node involvement. Another patient who suffered from purpuric pigmented dermatitis for four years had a monoclonal T cell population within the dermal infiltrate. Two patients died of their lymphoma. DISCUSSION: This study shows that purpuric pigmented dermatitis can be associated to or evolve into lymphoproliferative disease. This course should be suspected when purpuric pigmented dermatitis is extensive, long-lasting (> 1 year), has a reticular arrangement and negative patch-testing. In this situation, a long-term follow-up and treatments indicated in the early patch stage of mycosis fungoides (PUVA therapy, chlormethine) seem adequate.     

A granulomatous dermatitis associated with idiopathic ulcerative colitis

A 22‐year‐old white woman with a past medical history significant for left‐sided ulcerative colitis since June 1996 was in good control until July 1998 when she developed bloody diarrhea treated with oral steroids. In September 1998, she was admitted to Mount Sinai Hospital because of worsening symptoms. Colonoscopic biopsy revealed recurrent ulcerative colitis. During the admission, she developed a painful erythematous plaque with central healing of 3 weeks' duration on the left side of the chest. A 3‐mm punch biopsy was performed from the edge of the plaque. The patient underwent subtotal colectomy and ileostomy because of failure to respond to the medical treatment. Although no change of the skin lesion was observed after the biopsy, the lesion started to become less painful and flat, followed by complete resolution several days to a week after subtotal colectomy. The skin biopsy showed a mild epidermal hyperplasia, papillary dermal edema, and a superficial and deep dermal non‐necrotizing granulomatous inflammation with numerous neutrophils ( Fig. 1 ). The granulomata were seen in perivascular and interstitial areas, with occasional foci at the dermal subcutaneous junction ( Fig. 2 ). The granulomatous infiltrates stained positively with KP‐1 (CD68), confirming the histiocytic nature of the lesion ( Fig. 3 ). As there was a neutrophilic infiltrate associated with the granulomata, an infectious etiology was suspected; however, special stains for acid‐fast bacillus, fungus or bacteria failed to reveal microorganisms. The colonic resection showed universal mucosal colonic involvement without granulomata consistent with fulminant active ulcerative colitis.

Figure 1 Open in figure viewer PowerPoint Low magnification view of the lesion showing superficial and deep perivascular and interstitial non‐necrotizing granulomata with neutrophilic infiltrate     

Subcutaneous fat necrosis of the newborn

The patient in this study was an 18-day-old healthy boy, delivered by caesarean section at 33 weeks gestation, and the product of a pregnancy complicated by gestational diabetes and preterm labor. At 10 days old, three dermal and subcutaneous nodules were noted on the midline of the back (Fig. 1). The nodules measured 1–3 cm in size; they were firm, mobile, and multilobulated in quality. The overlying epidermis was slightly erythematous, but otherwise appeared normal.
A skin biopsy obtained 8 days after the lesions were first noted is shown in Fig. 2(a); necrosis of the fat with giant cell formation and characteristic needle-shaped clefts within residual fat cells can be seen (Fig. 2b).     

Pathergy in atypical eosinophilic pustular folliculitis

A 15-year-old, unmarried female presented to our dermatology department for an intensely pruritic skin rash that had appeared abruptly 3 days earlier. She had a remarkable medical history for a case of allergic rhinitis and several attacks of asthma in her early childhood. The condition waxed and waned initially but had improved in recent years. Physical examination revealed several erythematous plaques, papules studded with scattered pustules having diameters less than 0.3 mm. Conspicuous scratch marks had caused erythematous wheal-like indurations also studded with pustules in a linear distribution across the waist, forearms (Fig. 1), and back (Fig. 2). Discrete papulopustules were present on the face, nape and neck. The patient was otherwise healthy. There were no other symptoms such as fever, malaise, weakness, or lymphadenopathy Laboratory results were normal for hepatic and renal functions, serum electrolytes, glucose, protein, erythrocyte sedimentation rate (8 mm/h), and C-reactive protein (0.355 mg/l). A human immunodeficiency virus (HIV) antibody screen test was negative. Serum was positive for herpes simplex virus (HSV)-1 and HSV-2 IgG (in low titers), but negative for HSV-1 and HSV-2 IgM. White blood cell count revealed leukocytosis (11.2 x 10(3)/l), with a differential count of 68% neutrophils, 27% lymphocytes, and 8% eosinophils. Serum IgA, IgG, and IgM were within normal limits, but the IgE level was elevated (677 mg/dl). Cultures from peripheral blood and pustules were negative. A Tzank smear performed on the pustules showed no multinucleated giant cells. Fungal testing of skin scrapings from the initial lesion site gave negative results. Routine stool tests, including common pathogen and parasite screens, were negative, and urinalysis results were unremarkable. A biopsy specimen obtained from a skin pustule showed subcorneal eosinophilic and neutrophilic pustules in the follicular infundibulum with marked spongiosis of the follicular epithelium. (Fig. 3). There was a moderately dense superficial and deep perivascular mixed inflammatory cell infiltrate comprising eosinophils, neutrophils and lymphocytes. Migration of eosinophils and neutrophils through the vessel wall with variable luminal intramural fibrin deposition, histologically indicative of vasculopathy, was seen. There was concomitant slight perivascular dermal necrosis. (Fig. 4). Based on the clinical presentation and light microscopic findings on biopsy, a diagnosis of eosinophilic pustular folliculitis with pathergy was made. Systemic prednisolone 30 mg in divided doses was given. After 1 week of systemic corticosteroid therapy, the patient's condition was significantly improved and the patient was subsequently discharged. Two months later she had a relapse, upon which corticosteroid therapy was commenced leading to lesional resolution. The foci of eosinophilic folliculitis healed with areas of hyperpigmentation with variable scarring.     

Congenital Annular Erythema in Two Newborns: Similar Morphology, Divergent Etiologies

A 77‐year‐old white male presented to an outpatient clinic with painful, purpuric macules and papules on his right foot. The patient had recently undergone cardiac catheterization via his right femoral artery. The patient had no other complaints, was afebrile, and generally felt well. The clinical impression was either cholesterol emboli versus vasculitis. A 4 mm punch biopsy was obtained. Histopathology showed a vasculitis with many basophilic organisms consistent with bacteria present within the vascular spaces. There was a perivascular infiltrate consisting of lymphocytes and neutrophils. A gram stain revealed gram‐positive cocci. The clinician was immediately notified and blood cultures were drawn and echocardiogram performed. The cultures grew staphylococcus aureus. While no evidence of endocarditis was found, a psuedoaneurysm of the right femoral artery was noted. Despite rapid admission and treatment, the patient subsequently expired.     

AMERICAN CUTANEOUS LEISHMANIASIS: INTERMEDIATE FORM

A man of Portuguese nationality presented with increased volume of the forehead and multiple pustules, which subsequently ulcerated. The pustules appeared progressively on the rest of the face and in the right scapular region during a period of 2 years.
Physical examination revealed multiple rounded ulcers of different sizes with erythematous and infiltrated borders covered by serosanguineous scabs on the face and right scapular region (Figs. 1 and 2).
The otorhinolaryngologic examination showed an infiltrate of granulomatous aspect in the anterior third of the septum. Posterior rhinoscopy was normal.
When the patient was first seen, complete hematology and hepatic and renal tests were normal. Additional tests included: HIV, nonreactive; venereal disease reference laboratory test, nonreactive; chest x-ray, normal; Montenegro skin test, 14 mm; purified protein derivative, 0 mm.
The skin biopsy revealed a granulomatous infiltrate with a dense infiltrate composed principally of plasmocytes, with few lymphocytes (Fig. 3).
A skin smear stained with Giemsa and culture of biopsy material in blood agar were positive for the presence of Leishmania. Antibodies against Leishmania measured in an ELISA test were strongly positive, titer 1:6400. Typing of the isolate using absorbed polyclonal antisera revealed Leishmania braziiiensis .
During hospitalization, the patient received systemic immunotherapy with a combined vaccine containing heat-killed Leishmania mexicana promastigotes and bcg ,^1,2 as well as chemotherapy with meglumine antimoniate in a dose of 50 mg/kg/day IM, two cycles of 20 days with an in-terval of 7 days of rest.
The patient presented clinical healing of his lesions without relapse during a 2 year follow up after completion of therapy.     

Erythema nodosum associated with reactivation tuberculous lymphadenitis (scrofula)

A 73-year-old African American female presented to our clinic with painful lower extremity lesions of 2 weeks duration. She was in her usual state of health until 3 months prior to presentation when she reported symptoms of fatigue and weakness. She also noticed an enlarging mass on the left side of her neck. She denied fevers, chills, night sweats or cough. Her symptoms were unresponsive to a course of oral dicloxacillin. The neck mass enlarged over 8 weeks and she was referred to our institution for evaluation. CT scan of the neck showed an enlarged lymph node. Ten days prior to her presentation in dermatology, a fine needle aspirate of the enlarging lymph node revealed necrotizing granulomas. Tissue was sent for routine mycobacterial and fungal cultures. Routine blood work, chest radiograph, and a tuberculin skin test were also performed. At the time of her dermatology visit she described the development of multiple new painful, non-pruritic lesions, bilaterally on the lower extremities. She also reported a red crusted area that appeared at the site of her tuberculin test that was placed subsequent to the development of her lower extremity lesions. Her past medical history was significant for Parkinson's disease, hypothyroidism and hypertension. Her current medications included l-thyroxine, estrogen and diltiazem. Her travel history was only remarkable for a trip to Jamaica the previous spring. She was born and raised in Haiti. She reported a history of a positive tuberculin skin test 20 years ago, but received no therapy. Physical examination revealed a 2 x 3 centimeter firm, nontender left lateral neck mass (Fig. 1). Her right forearm revealed an erythematous, ulcerated, indurated plaque 1.5 cm in diameter (Fig. 2.). Her lower extremities revealed tender 0.5 to 1 cm erythematous nodules below the knees bilaterally (Fig. 3). A punch biopsy of a lower extremity nodule revealed a mild pervisacular dermal infiltrate. Within the subcutaneous tissue there was septal widening. There was also a lymphohistiocytic infiltrate with a slight admixture of neutrophils within the septa of the fat lobules. There was no evidence of necrotizing vasculitis or collagen necrosis. An acid-fast stain was not performed. The histologic findings were consistent with a diagnosis of erythema nodosum. Her laboratory evaluation including CBC, electrolytes, thyroid studies, angiotensin converting enzyme level and chest radiograph were normal. Approximately 1 week after her dermatological evaluation, the fine-needle aspirate culture grew Mycobacterium tuberculosis. A diagnosis of tuberculous lymphadenitis associated with erythema nodosum was confirmed. The patient was started on quadruple therapy of isoniazid, rifampin, ethambutol and pyrazinamide. Her lower limb skins lesions rapidly resolved over the subsequent month and her neck mass also diminished in size. She completed 6 months of antituberculous therapy with complete resolution of her lymphadenopathy.