Cutaneous leishmaniasis: Texas case diagnosed by electron microscopy

A 78-year-old woman from south-central Texas without a history of travel outside the state presented with an erythematous papule on her right forearm. A biopsy specimen showed non-necrotizing granulomatous inflammation with negative stains for organisms. The lesion was recalcitrant to treatment with topical steroids, and a second biopsy specimen, obtained 3 months later, showed a histiocytic infiltrate in the upper dermis. Numerous, round, 1- to 30-microm bodies were seen within vacuoles in the histiocyte's cytoplasm. Giemsa, Grocott-Gomori methenamine-silver nitrate, and periodic acid-Schiff stains were negative. Electron microscopic examination of the paraffin-embedded tissue showed parasites diagnostic of leishmaniasis. On review of the literature, we found that 29 other cases of autochthonous cutaneous leishmaniasis have been reported in Texas. We emphasize the endemic nature of this condition in Texas.     

Neurotropic T‐cell lymphocytosis: a cutaneous expression of CLIPPERS

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly involves the pons and cerebellum and that improves with immunosuppressive treatment. Only recently described, the etiology is unknown, diagnosis is difficult and long‐term neurological sequelae may occur without aggressive treatment. Herein, we describe a 59‐year‐old woman who presented with subcutaneous nodules affecting her face, trunk, limbs and an indurated annular erythematous lesion on her forearm. This was associated with marked dysesthesia of her skin, refractory to treatment. There was a 4‐year history of dysequilibrium, vertigo, truncal and gait ataxia with progressive neurological symptoms. Skin biopsy of the annular nodular lesion showed a lymphohistiocytic infiltrate in dermis and subcutis with a striking lymphocyte‐dominant infiltrate that was perineural and formed a nodular collection extending along a prominent subcutaneous nerve. Immunophenotyping indicated a marked predominance of T cells that were CD3 positive with a 2 : 1 CD4 : CD8 ratio. Scattered histiocytes were present but no well‐formed granulomas or vasculitis. Magnetic resonance imaging studies showed changes in the pontine, brain stem and cerebellar region, which subsequently were defined as characteristic for CLIPPERS, but no brain biopsy was pursued. The marked neural skin symptoms and the cutaneous histopathological findings indicate that the skin may be an additional target organ in CLIPPERS, and the immune response may be directed against a common neural antigen. In radiologically typical CLIPPERS, identification of clinical skin lesions particularly subcutaneous nodules and biopsy may potentially form a basis for tissue diagnosis in this syndrome.     

Giant Mycobacterium fortuitum abscess associated with systemic lupus erythematosus

A 36-year-old woman with a 1 1/2-year history of systemic lupus erythematosus was first seen in October 1984 with a six-month history of several ulcerated and scarred lesions on the lower extremities. A biopsy specimen showed a granulomatous infiltrate of deep dermis and subcutaneous tissue. Over the next three weeks, the patient developed a violaceous, warm, indurated, tender, fluctuant lesion involving most of the left buttock. A culture showed a rapidly growing atypical acid-fast bacteria, which was later identified as Mycobacterium fortuitum. The patient was treated with surgical drainage of the left hip abscess, followed by a three-week treatment with doxycycline hyclate and amikacin sulfate. She was discharged while receiving oral doxycycline and ethambutol hydrochloride.     

From circumscribed scleroderma (morphea) to subcutaneous panniculitis-like T-cell lymphoma: case report

Subcutaneous panniculitis-like T-cell lymphoma is a rare form of non-Hodgkin lymphoma. It presents clinically as panniculitis, with erythematous, firm subcutaneous infiltrates and recurrent papulonodules. A 45-year-old male Croat presented with a 6-month history of tender erythematous subcutaneous skin lesions without systemic symptoms. Analysis of a lesion biopsy specimen showed circumscribed scleroderma (morphea). During hospitalization, skin biopsies from two subcutaneous lesions on the stomach were repeated, and pathohistologic analysis corresponded primarily to panniculitis in the context of morphea. Pathohistologic analysis and immunohistochemistry of a specimen obtained by tumor excision from the back indicated subcutaneous panniculitis-like T-cell lymphoma. Histologic analysis revealed a dense infiltrate of atypical T-lymphoid cells expressing CD8(+) phenotype located in the subcutaneous tissue with histiocyte-phagocytizing apoptotic cells. Specific hematologic analysis and medical treatment were continued. This case report illustrates the importance of continuous follow-up with repeat biopsy and use of immunohistologic techniques for early diagnosis.     

Multiple myeloma presenting as a novel mucocutaneous eruption

A 71-year-old woman presented with exquisitely tender mucosal erosions, a diffuse polymorphous eruption, and night sweats. Workup revealed multiple myeloma with a monoclonal IgG-kappa paraprotein in the serum. Her severe oral involvement was suggestive of paraneoplastic pemphigus, but direct and indirect immunofluorescence tests were negative. A skin biopsy showed spongiosis and a sparse perivascular lymphocytic infiltrate, with occasional CD8-positive lymphocytes in the epidermis. Her lesions improved with intravenous immune globulin. Immunohistochemical staining on the formalin-fixed biopsy specimen was strongly positive for IgG and IgG-kappa in an epidermal "chicken-wire" pattern, but negative for IgG-lambda. Her pulmonary tissue stained negative for IgG-kappa, suggesting clinical relevance of the myeloma paraprotein in her epidermis. To our knowledge, this is the first report of a multiple myeloma patient with such an eruption.     

Pseudoepitheliomatous hyperplasia - an unusual reaction following tattoo: report of a case and review of the literature

A 59-year-old woman presented with an itchy and uncomfortable raised lesion at a tattoo site (Fig. 1) on the lateral aspect of the left leg, just above the ankle. The tattoo had been placed 2 years before her presentation and the tattoo site was sun exposed. Immediately after she had the tattoo, she noticed redness of the skin. After a week, a pruritic and red scaly nodule developed that continued to gradually enlarge until her presentation. The patient had tried topical vitamin A and D ointment with no relief. The patient also had tattoos on the arms without any noticeable skin changes. The patient reported that the tattoo procedure on her leg was more painful than that on her arms, and was performed by a different (and perhaps inexperienced) tattoo artist. The original tattoo contained red, green, and yellow pigments. A diagnosis of tattoo granuloma was considered; squamous cell carcinoma and fungal infection were included in the differential diagnosis. A punch biopsy was performed, followed by complete surgical excision of the lesion with a split-thickness skin graft from the right thigh. The skin excision specimen showed a 3 x 2.5-cm granular and pitted pink lesion with well-demarcated, somewhat irregular borders. The lesion was raised 0.5 cm above the skin surface. The lesion was present in the center of the original tattoo. Portions of the original tattoo with green and blue-green pigmentation were visible on either side of the lesion. No satellite lesions were identified. Microscopically, the raised lesion demonstrated striking pseudoepitheliomatous hyperplasia, with irregular acanthosis of the epidermis and follicular infundibula, hyperkeratosis, and parakeratosis (Fig. 2). Follicular plugging was present with keratin-filled cystic spaces. There was a brisk mononuclear inflammatory infiltrate in the dermis, composed primarily of lymphocytes, with admixed plasma cells and histiocytes. Giant cells were occasionally identified. Dermal pigment deposition was noted both within the lesion and in the surrounding skin, corresponding to the original tattoo. Variable dermal fibrosis was noted, with thick collagen bundles in some areas. There was no evidence of epidermal keratinocytic atypia, dyskeratosis, or increased suprabasal mitotic activity. Special stains (periodic acid-Schiff and acid-fast) for microorganisms were negative.     

Perforating pseudoxanthoma elasticum associated with chronic renal failure and hemodialysis

A patient with hypertension and chronic renal failure of an undetermined cause who was undergoing hemodialysis developed bilateral crusted, focally eroded plaques on her breasts. A biopsy specimen of the lesional skin revealed typical histologic changes of pseudoxanthoma elasticum, with epidermal perforation. A biopsy specimen of lesion-free skin revealed characteristics typical of pseudoxanthoma elasticum. Perforating pseudoxanthoma elasticum should be added to the growing list of cutaneous disorders that occur in patients with chronic renal failure who are undergoing hemodialysis.     

Palisading granuloma presenting in a patient with systemic lupus erythematosus

A patient with systemic lupus erythematosus who developed a distinctive nodular eruption demonstrating typical features of palisading granuloma is presented herein. A 60-year-old woman was admitted complaining of an elastic-hard, indurated nodule on the right dorsal aspect of the metacarpophalangeal joint of her third finger with a history of several years. She was successfully treated for her lupus nephritis with oral predonisolone. She had experienced erythema on her cheeks after sun exposure and polyarthralgia for a decade. Laboratory examination revealed positive results for antinuclear antibody and rheumatoid factor. Peripheral blood cell counts showed leukocytopenia and lymphocytopenia. Results of hand X-ray were unremarkable. Histological examination of the skin biopsy specimen from the nodule revealed a structure composed of a central area of fibrinoid necrosis, surrounded by a middle zone of palisading cells and an outer zone of chronic lymphocyte infiltrate. These findings led us to the histological diagnosis of palisading granuloma. She was diagnosed as having systemic lupus erythematosus complicated with a rheumatoid nodule. She is currently under treatment with cyclophosphamide (50 mg/day) without exaggeration of her systemic lupus erythematosus and nodule.     

Cutaneous alternariosis

A 77-year-old woman had had a slowly spreading lesion of five years' duration on her left cheek. The lesion consisted of sharply demarcated, dark-red plaques with infiltration. A biopsy specimen from the lesion showed mixed-cell granulomatous infiltration in the upper to middle dermis. Hyphae were observed in the granulomatous tissue. Alternaria tenuissima was isolated from a biopsy specimen. Antimycotic susceptibility test with amphotericin B, ketoconazole, and flucytosine revealed that the isolate was sensitive to the former two drugs. The lesion was treated with intralesional infiltration of amphotericin B.     

A case of cutaneous localized cryptococcosis

A 65-year-old Japanese male with cutaneous localized cryptococcosis, which was developed as an erythematous infiltrated plaque on the right side of the face, was reported. The biopsy specimen taken from the lesion showed a granulomatous change with many spores in the dermis. Culture of the biopsy specimen gave Cryptococcus neoformans serotype D. There were no cryptococcal infections in the other organs. The skin lesion was improved by a systemic administration of miconazole. This patient was unusual since the cryptococcal lesion was limited to the skin, nevertheless he was under a severe cellular immunity deficiency. The reason for this may be that the infected C. neoformans strain belonged to serotype D.     

Lupus erythematosus profundus with unusual skin manifestation: subcutaneous nodules coexisting with eyelid plaques

A 71-year-old Japanese woman presented with erythematous plaques on the eyelids and subcutaneous indurations or nodules with or without overlying erythema on the hands, thigh, and leg. She also had oral ulcers, arthralgia and a low grade fever. Laboratory tests revealed an elevated titer of antinuclear antibody, an increased erythrocyte sedimentation rate and anemia. Skin biopsy specimens from the hand and thigh showed lymphocytic perivascular and periappendageal infiltrates and vacuolar alterations at the basement membrane zone of the skin appendages. Moreover, there was a dense lymphocytic infiltrate deep in the dermis with extension into the subcutaneous fat, which was compatible with the diagnosis of lupus erythematosus profundus. Although the biopsy specimen from the eyelid lesion did not contain the subcutaneous fat, the changes in the dermis were essentially the same as those of the hand and thigh. The eruption as well as the other symptoms promptly responded to oral prednisolone.     

Drugs and Wells' syndrome: a possible causal relationship?

In April 1997, a 28‐year‐old woman presented with a 3‐day history of multiple papules and vesicles on the fingers and wrist of her right hand. She reported that she had been taking nonsteroidal anti‐inflammatory drugs containing tenoxicam and diclofenac sodium, and had no history of an insect bite. The medical history also included pruritic erythema on her trunk 1 month before her initial visit, and oral tenoxicam and diclofenac sodium treatment for ankle arthralgia 2 days before the skin lesions appeared. At that time, her dermatologist had told her that the skin lesions might be due to an insect bite and were secondarily infected. She took amoxicillin for the lesions, and during the following days developed bullous lesions on her hands and feet. The patient was diagnosed with ‘‘drug reaction’' at another hospital based on a histopathologic examination of the affected skin. Treatment with topical corticosteroids and oral antihistamines was ineffective. Physical examination revealed a few crusted, erythematous, papular lesions on the dorsal aspects of the patient's feet, bullous lesions on her right wrist, and vesiculopapules on her fingers. She had no fever or other abnormal clinical findings. Laboratory studies showed a white blood cell count of 6000/mL with 16.5% eosinophils. A bacterial culture of the content of one of the bullous lesions was negative. The results of stool tests for parasites and ova were negative. Clinical and laboratory findings were in line with the diagnosis of drug reaction. The patient was advised not to take nonsteroidal anti‐inflammatory drugs. The skin lesions resolved rapidly on treatment with wet compresses of Burow's solution and oral prednisolone (40 mg/day). The dose of prednisolone was gradually reduced, and was discontinued after 3 weeks. The patient was readmitted in July 1997 for an indurated, erythematous plaque on her left foot, a lesion that resembled cellulitis. Her history at that time included the initiation of amoxicillin treatment for a gingival abscess 2 days before the skin lesion appeared. The lesion regressed after amoxicillin was discontinued and the patient received an intramuscular injection of triamcinolone acetonide. Several months later, in October 1997, she developed a similar cellulitis‐like lesion on her right ankle. This time there was no history of drug intake. We obtained a skin biopsy from the lesion, and histopathology revealed an intense eosinophilic infiltrate in the dermis and extending into the subcutaneous tissue. Flame figures were abundant ( Fig. 1 ). We also observed edema and erythrocyte extravasation in the dermis. Based on these features and her history, a diagnosis of Wells' syndrome was made. The skin lesion resolved spontaneously, and she has experienced no lesion recurrence since.

Figure 1 Open in figure viewer PowerPoint A characteristic ‘‘flame figure’' (hematoxylin and eosin stain; original magnification, × 230)     

Lymphoepithelioma-like carcinoma arising in the scar from a previously excised basal cell carcinoma

We report a case of a primary lymphoepithelioma-like carcinoma of the skin (LELCS) associated with scar from a previous excision of basal cell carcinoma. The patient was a 68-year-old female with a 3.0 mm skin-colored pearly papule on her forehead that developed over 2–3 months. The patient had a history of a basal cell carcinoma in the same location, which was completely excised 1 year earlier. A biopsy and subsequent excision of the tumor were performed. The tumor consisted of small islands of large pleomorphic mitotically active epithelioid cells surrounded by a very dense lymphoplasmacytic infiltrate. The tumor was associated with dermal scar. There was no connection of tumor with the unremarkable epidermis. Immunohistochemical examination showed that the epithelioid tumor cells were positive for pan-cytokeratin and epithelial membrane antigen, supporting the morphologic impression of LELCS. The lesion was negative for Epstein-Barr virus. Retrospective review of the original excision specimen confirmed the diagnosis of an ordinary basal cell carcinoma. Forty-five cases of LELCS have been reported to date. We report the first case of LELCS to arise in the scar from an excision of a cutaneous malignancy     

Neutrophilic Dermatosis-Associated Sterile Chronic Multifocal Osteomyelitis in Pediatric Patients: Case Report and Review

Atypical pyoderma gangrenosum (PG) and Sweet syndrome are neutrophilic dermatoses that share some common features. Sterile chronic recurrent multifocal osteomyelitis is a rare association of these neutrophilic dermatoses that has only been reported in children. We report a 3-year-old girl who initially presented with pain in her left hand and right leg. Roentgenograms and bone scan revealed findings of multifocal osteomyelitis affecting both femurs, the right tibia, left clavicle, right eighth costochondral junction, and left ulna. She was treated with antibiotics without improvement. Bone biopsy of the left ulna revealed histologic changes consistent with osteomyelitis, however, all cultures for bacteria, mycobacteria, and fungi were negative. She subsequently developed an ulcer surrounded by a violaceous, undermined border at the site of the bone biopsy, which also did not improve during antibiotic treatment. A biopsy specimen from this lesion demonstrated a dense perivascular and periappendageal infiltrate of neutrophils within the dermis and edema of the papillary dermis compatible with a neutrophilic dermatosis. She was treated with oral prednisone which resulted in resolution of skin lesions, bone pain, and soft tissue swelling. This case further documents the association between PG or Sweet syndrome and multifocal sterile osteomyelitis.     

Annular epidermolytic ichthyosis

A 21-year-old woman presented to the Charles C. Harris Skin and Cancer Pavilion with a life-long history of palmoplantar keratoderma that was accompanied by episodic flares of polycyclic psoriasiform patches diffusely over the rest of her body. A biopsy specimen of a representative skin lesion showed epidermolytic hyperkeratosis. The particular phenotype that this patient exhibited has been reported in only seven families previously in the literature. Mutations in keratin 1 and 10 have been identified in these patients and are similar to those mutations in patients with classic epidermolytic hyperkeratosis.     

Lichenoid drug eruptions due to nandrolone furylpropionate (Demelon)

A case of lichenoid drug eruption due to nandrolone furylpropionate (Demelon) is reported. A 71-year-old man with aplastic anemia developed widespread erythema and reticulation with violaceous papules and pigmentation after receiving intramuscular injections of Demelon weekly for 2 months. The eruption gradually resolved after drug withdrawal, leaving reticular pigmentation. A biopsy specimen of the skin lesion showed lichenoid-type reactions including hydropic or colloid degeneration of the basal cells, sometimes with satellite necrosis, and a band-like subepidermal lymphocytic infiltrate. Most of the mononuclear cells stained strongly for helper/inducer T lymphocytes (Leu 3a).     

Benign cephalic histiocytosis in a British-African child

A 14-month-old British-African child presented with an asymptomatic eruption localized to her face and upper limbs. At the age of 17 months the eruption had started to spontaneously regress. Histologic examination of a biopsy specimen from a papular lesion on the arm showed a histiocytic infiltrate in the superficial dermis. Immunohistochemical studies showed negative staining for the Langerhans cell markers, CD1a and S-100 protein, while staining for the macrophage/histiocytic markers, CD68 and factor XIIIa, were positive. The clinical and histologic features in this patient are consistent with the rare, non-Langerhans, histiocytic disorder known as benign cephalic histiocytosis. As far as we are aware, this represents the first published occurrence in a child of African origin, emphasizing the widespread nature of this condition.     

Epidermotropic precursor T-cell lymphoma with highly aggressive clinical behavior simulating localized pagetoid reticulosis

We describe a 43-year-old patient with a solitary, eczematous plaque on his right nipple that had developed during the previous 6 weeks. Histopathology revealed a superficial band-like infiltrate of medium to large-sized pleomorphic lymphocytes with striking epidermotropism. The tumor cells expressed a T-phenotype (CD3+, CD20-) and were CD30-, CD4-, and CD8-negative. A diagnosis of localized pagetoid reticulosis (Woringer-Kolopp disease) with possible large cell transformation was proposed. A cervical lymph node excised 2 months later showed features of a highly aggressive blastoid precursor T-cell lymphoma. Reevaluation of the skin lesion and of a tonsil specimen previously interpreted as benign hyperplasia showed features consistent with those observed in the lymph node. The disease was rapidly progressive, and the patient died 15 months after the first skin biopsy. This case represents a unique cutaneous presentation of aggressive precursor lymphoma, showing the protean nature of lymphoproliferative disorders and the overlapping clinical and histopathologic features of different entities.     

Chilblain-like leukemia cutis

We present a 6-year-old girl with an 8-month history of fluctuating chilblain-like lesions on the toes. A full blood count showed slight thrombocytopenia and monocytosis. A skin biopsy specimen showed a dense perivascular and superficial dermal cellular infiltrate which was CD3(-), CD43(+), and lysosyme + on immunocytochemistry, suggesting a monocytic origin. Juvenile myelomonocytic leukemia was diagnosed after a bone marrow biopsy and spontaneous marrow colony growth in culture.     

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.