Idiopathic eruptive macular pigmentation

A 23‐year‐old, dark‐skinned man presented at the dermatology department with pigmented macules over the trunk and proximal thighs of 2 months’ duration. He reported that the number of lesions had increased progressively for a few weeks and then remained stable. Skin examination showed oval brown macules and patches, 5–40 mm in diameter, involving mainly the anterior trunk and proximal thigh, but also the neck and dorsum ( Fig. 1 ). The lesions were asymptomatic. There was no previous history of an inflammatory process, erythema, scaling, or drug intake. The mucous membranes, palms, and soles were clear. The pigmentation was not influenced by sunlight. Darier's sign (urticaria or erythema around the macules after scratching or rubbing of the lesions) was absent. The previous use of emollients, keratolytics, and topical antifungal agents did not alter the aspect of the lesions. The results of physical examination and routine laboratory tests (blood cell count, blood chemistry) were normal. Venereal Disease Research Laboratory (VDRL) test and direct skin examination and culture for fungus were negative. A biopsy specimen was obtained from a pigmented macule. The histologic study showed hyperkeratosis and acanthosis, epidermal basal layer pigmentation with an irregular distribution, focal mild lymphohistiocytic infiltrate in the papillary dermis and dermal–epidermal junction, and discrete pigmentary incontinence ( Figs 2–4 ). The mast cell population was normal. A further biopsy 1 year later showed similar findings.

Figure 1 Open in figure viewer PowerPoint Multiple brown macules involving the trunk     

Atypical acral persistent papular mucinosis.

A 70-year-old woman presented with a 10-year history of cutaneous lesions on her calves that worsened with solar exposure. Her past medical history was irrelevant. Physical examination revealed 10–15 erythematous, firm nodules with a diameter of 2–5 mm, which were slightly painful on palpation. The lesions were located on both calves, although some elements extended to the posterior side of the thighs ( Fig. 1 ). General physical examination did not show any abnormalities. A biopsy specimen from one of the elements showed a normal epidermis and, in the upper dermis, a slight inflammatory infiltrate of mononuclear cells, with the presence of splits between collagen fibers ( Fig. 2 ). Alcian blue stain at pH 2.5 revealed the existence of mucinous material in these spaces ( Fig. 3 ). An electron microscopic study did not show any abnormalities.

Figure 1 Open in figure viewer PowerPoint Clinical appearance of the lesions     

Giant congenital melanocytic nevus with neurofibroma-like changes and spina bifida occulta

A 12‐year‐old Iranian girl presented with a bathing trunk congenital melanocytic nevus. Multiple other pigmented lesions were present. The nevi were distributed over the entire body including the oral mucosa. There were also bilateral, soft, pendulous tumors and nodules in the area covered by the giant congenital melanocytic nevus. The tumors had been present since birth and showed continuous growth during childhood. She was otherwise healthy. Her parents were not consanguineous. There was no family history of similar lesions. Physical examination revealed a large dark‐brown circumferential plaque extending evenly from the upper back and abdomen down to the lumbar region, buttocks, and thighs ( Fig. 1 ). It had a smooth surface, with excessive growth of hair. There were soft, redundant, exuberant folds of skin overhanging the back and buttock, localized to the area covered by the bathing trunk nevus. On palpation, they appeared as deep, multilobulated masses mimicking giant neurofibromas. There were also several smaller dark‐ or skin‐colored, soft, dermal nodules in this area. On other parts of the integument, there were numerous pigmented nevi of different sizes and colors, including speckled nevi, café‐au‐lait spots, and some clinically dysplastic nevi. There was hypertrichosis over some of the nevi. Mucosal examination revealed dark‐brown macules on the hard palate and conjunctiva. General physical examination was otherwise normal. There was no axillary freckling. Ophthalmologic examination was negative for Lisch nodules, and the fundal appearance was normal. Neurological examination revealed no abnormalities. Spinal X‐ray showed spina bifida occulta in the fifth lumbar vertebra. Brain and spinal magnetic resonance imaging (MRI) with gadolinium contrast was performed to detect neurocutaneous melanosis, which was negative. Two deep incisional biopsies were performed of the proliferative nodules over the hips, with the clinical impression of giant neurofibroma. Histologic examination revealed superficial nests of melanocytes with focal involvement of the dermo‐epidermal junction, extending into the dermis ( Fig. 2 ). The melanocytes became spindle shaped within the reticular dermis ( Fig. 3 ). Immunohistochemical techniques showed strong positive staining for both S100 protein and MART‐1 in both the superficial and deep portions of the proliferation, consistent with a melanocytic nevus.

Figure 1 Open in figure viewer PowerPoint Bathing trunk melanocytic nevus with large, pendulous skin lesions mimicking neurofibromas over the buttocks and lower back and multiple other melanocytic nevi of variable size distributed all over the body     

Annular lichen planus involving the esophagus: a rare diagnosis

A 49‐year‐old woman presented with a 2‐year history of a sore mouth, annular, atrophic patches, and chronic ulcerative vulvovaginitis. For 6 years, she had suffered from dysphagia to solids which was increasing in severity. There was no past history of heartburn or other symptoms of esophageal reflux. Mucocutaneous examination showed white erosive patches on the buccal mucosa, cicatricial alopecia, dystrophic nails, and annular atrophic pigmented plaques localized on the trunk ( Fig. 1 ). Genital examination showed atrophic and sclerotic vulvovaginal lesions with synechia. Cutaneous biopsy showed an atrophic epidermis, a dense lymphocytic infiltrate in the upper dermis with degeneration of the basal epithelium, and Civatte bodies. Serologic tests for hepatitis B and C were negative. A diagnosis of cutaneous annular lichen planus with nail, scalp, oral, and genital involvement was made.

Figure 1 Open in figure viewer PowerPoint Annular, atrophic, and pigmented plaques localized on the back     

Erythema multiforme-like lesions associated with lesional infiltration of tumor cells occurring with adult T-cell lymphoma/leukemia

A 66‐year‐old Japanese woman visited our hospital with a complaint of multiple papules on her trunk and extremities. She had a past medical history of appendicitis and blood transfusion 40 years earlier. For the last 10 years, she had noticed multiple, gradually enlarging papulonodular lesions with surrounding erythema on her trunk and extremities. Physical examination revealed multiple, violaceous papules or nodules, less than 10 mm in diameter, with surrounding erythema on her trunk and extremities ( Fig. 1 ). The results of routine laboratory examinations, including blood count, liver function, renal function, serum calcium, and lactate dehydrogenase, were within the normal range. The peripheral blood picture showed a small population of atypical lymphocytes below 1% of the total white blood cells. Human T‐cell lymphotropic virus type I (HTLV‐I) serology was positive. A microscopic examination of a biopsy specimen from a nodule on the abdomen demonstrated diffuse infiltration of large pleomorphic T cells in the upper and middle dermis, although highly atypical lymphocytes, so‐called flower cells, could not be recognized. Infiltrating lymphocytes were positive for CD2, CD3, CD4, CD5, CD7, and CD45, but negative for CD8 and CD20, immunohistologically. Bone marrow biopsy also demonstrated the infiltration of lymphocytes expressing CD2, CD3, CD4, CD5, and CD7, but not CD25. Southern blot analysis of the infiltrating cells in the skin revealed an integration of HTLV‐I proviral DNA in T cells. Clonal T‐cell receptor γ gene rearrangement was detected in skin and bone marrow biopsies. No abnormal mass or bone defect was detected by chest or abdominal computed tomographic scanning, systemic gallium‐67 citrate scintigraphy, or chest radiography. On the basis of these data, the patient was diagnosed with smouldering‐type adult T‐cell lymphoma/leukemia.

Figure 1 Open in figure viewer PowerPoint Clinical features of adult T‐cell lymphoma/leukemia (ATL) skin lesions. Crusted, target‐like, dark‐red plaques on the lower legs     

Chronic disseminated cutaneous histoplasmosis in an immunocompetent individual--a case report

A 45‐year‐old woman from central India reported to clinic with multiple swellings on the face and neck. She had red patches on her forearms and trunk, but there was a predominance of lesions on the face and neck. On examination, her face showed multiple, succulent, erythematous plaques which were mildly pruritic ( Fig. 1 ). There was no discharge. There were also some scattered erythematous papules and nodules on the face ( Fig. 1 ). Examination of the neck revealed multiple erythematous plaques, many of them with a linear orientation and central ulceration and crusting ( Fig. 2 ). The upper extremities showed multiple erythematous plaques, most of which were ulcerated ( Fig. 3 ). Plaques without ulceration had been present for the past 2 years. The patient had been treated in various centers around her village and in Baroda as a case of reactional leprosy.

Figure 1 Open in figure viewer PowerPoint Multiple, succulent, erythematous plaques, papules, and nodules on the face     

Atypical clinical form of cutaneous leishmaniasis: erysipeloid form

A 60‐year‐old woman was admitted to our clinic with a gradually enlarging red papule on her face. Her history revealed that, 9 months previously, a painless, red papule of 1–2 cm in diameter had occurred in the middle of her face and, with time, had enlarged to cover her nose, both cheeks, and eyelids. It was diagnosed as a superficial skin infection, and topical and systemic antibiotics were prescribed; however, no response was obtained. In the last 2 months, a sore had formed in the middle of her nose. The patient lives in the east of Eastern Anatolia, where no case of cutaneous leishmaniasis has been reported in the last 20 years. On dermatologic examination of the patient, an erythematous, indurated, slightly squamous, 10 cm × 5 cm, butterfly‐shaped plaque with sharply defined borders was seen on both cheeks, lower eyelids, and the whole nose ( Fig. 1 ). In smears taken from the lesion, a number of amastigotes belonging to Leishmania were determined in the intracellular and extracellular area ( Fig. 2 ). Histopathologic examination of the cutaneous lesion showed scattered infiltration composed of mononuclear cells, histiocytes, plasma cells, and small epithelioid granulomas. Gram smear and anaerobe–aerobe culture prepared from the lesion were negative. The total blood count and sedimentation rate of the patient were within normal limits. Routine biochemical tests, urine analysis, chest radiography, and intradermal purified protein derivative (PPD) skin test were all normal. Antinuclear antibody and antistreptolysin antibody examinations were negative.

Figure 1 Open in figure viewer PowerPoint Butterfly‐shaped infiltrated erythematous plaque on the face     

Vegetative pyoderma gangrenosum: an unusual presentation

A 44-year-old woman presented with a 6-month history of subcutaneous nodules involving her face and shins. Her past history revealed myomatous uterus with menometrorrhagia, resulting in an iron deficiency anemia, treated by ferrum sulfate tablets. She also had diabetes mellitus type II treated by glibenclamide and metformin tablets. There was no evidence of bromide or iodide ingestion. On initial examination, several firm, tender, erythematous subcutaneous nodules, 2–4 cm in diameter, were present on the face ( Fig. 1 ) and shins ( Fig. 2 ), two with normal overlying skin. Within a few weeks, the nodule on the left shin enlarged and several superficial ulcers appeared, surrounded by purplish borders, secreting sinuses, and a vegetative exophytic surface ( Fig. 3 ). Physical examination was normal. Incisional biopsies from the ulcer margins showed pseudoepitheliomatous hyperplasia, diffuse neutrophilic infiltration with microabscess formation (Fig. .4), and the presence of a dense infiltrate composed of epithelioid cells, giant cells, and a few lymphocytes forming non-necrotizing granulomas within the dermis and subcutaneous lobules ( Fig. 5 ). Leukocytoclastic vasculitis was also present in the lower dermis. Routine blood tests and urine analysis were normal, except for a high erythrocyte sedimentation rate (120/h) and anemia with a hemoglobin level of 10 g%. The results of the following laboratory tests were found to be within normal limits: rheumatoid factor; antinuclear antibody; C3; C4; serum immunoglobulins; serum protein electrophoresis; stool for parasites and occult blood; X-ray of the chest, left shin, hands, and facial bones; isotopic bone scanning; abdominal ultrasonography; chest and

Figure 1 Open in figure viewer PowerPoint Erythematous nodule on the chin     

Lichen nitidus after hepatitis B vaccine

A 27‐year‐old man presented to our clinic with an asymptomatic papular eruption all over his body. The eruption first began after hepatitis B vaccination (Engerix B) and increased with the second vaccination which was given 1 month after the first. His medical history and systemic examination were normal. Physical examination revealed numerous, flesh‐colored or pink, 1–2 mm papules, with a flat, shiny surface, predominantly on the trunk and arms, and grouped in some areas. The face, nails, and oral mucosa were spared. Laboratory investigation revealed normal values of the routine hematologic and biochemical tests. Antistreptolysin O (ASO), C‐reactive protein (CRP), venereal disease research laboratory (VDRL) test, anti‐human immunodeficiency virus (anti‐HIV), chest X‐ray, and ultrasonography were normal. Anti‐hepatitis A virus immunoglobulin M (anti‐HAV IgM), anti‐hepatitis B core total (anti‐HBc total), and anti‐hepatitis C virus (anti‐HCV) were negative. Anti‐hepatitis A virus immunoglobulin G (anti‐HAV IgG) was positive; 10 mm erythema and induration were detected in the purified protein derivative (PPD) test. During the following period, spontaneous resolution of the lesions was observed. When the hepatitis B vaccine was given to the patient again after several months, however, the same types of lesion re‐occurred and spread rapidly all over his body. Again, physical examination revealed numerous, flesh‐colored or pink, 1–2 mm papules, with a flat, shiny surface, on the trunk, arms, and penis ( Fig. 1 ). Laboratory investigations revealed normal hematologic and biochemical values. Anti‐hepatitis B surface antigen (anti‐HBsAg) was > 150 IU/mL. Histopathologic examination of skin biopsy material taken from the regio antebrachii revealed, in the dermis, a granuloma formation of lymphocytes, histiocytes, and plasma cells which was surrounded by rete ridges and covered with focal parakeratotic epidermis ( Fig. 2 ).

Figure 1 Open in figure viewer PowerPoint Numerous, flesh‐colored to pink papules with a flat, shiny surface     

Acute febrile neutrophilic dermatosis (Sweet's syndrome) with nodular episcleritis and polyneuropathy

A 56‐year‐old Japanese housewife presented with multiple erythematous lesions in association with ocular hyperemia and pain in the right upper and lower extremities, including the hands and feet. These symptoms were preceded by a sore throat with persistent fever higher than 38.5 °C for about 1 week. Dermatologic examination showed tender, dull‐red, erythematous lesions, measuring 1–2 cm in diameter, located predominantly on the forehead, cheeks, auricular region, neck, forearm, hands, and feet. A biopsy specimen obtained from an erythematous lesion on the right forearm revealed prominent edema in the papillary dermis and remarkable inflammatory cell infiltration throughout the entire dermis ( Fig. 1 ). The infiltrate predominantly consisted of neutrophils and nuclear dust without signs of vasculitis. In routine examination, the leukocyte count was 15,000/mL (normal range, 4000–8000/mL) with severe neutrophilia (80%). The C‐reactive protein (CRP) level was 17.65 mg/dL (normal range, < 0.5 mg/dL) and the anti‐streptolysin (ASLO) level was 611 IU/mL (normal range, < 166 IU/mL). In human leukocyte antigen (HLA) testing, HLA‐A2, ‐B39, ‐B35, ‐Cw2, and ‐Cw7 were positive, and HLA‐B51, ‐B54, and ‐Cw1 were negative.

Figure Figure 1  Open in figure viewer PowerPoint Histologic picture showing a dermal infiltrate of neutrophils     

CD4/CD8 double-positive, acute type of adult T-cell leukemia/lymphoma with extensive cutaneous involvement

A 67‐year‐old Korean man presented with a 2‐week history of pruritic cutaneous lesions on the trunk and a 2‐month history of cervical neck masses. The cutaneous lesions had been spreading rapidly for the last week. The past history included an unknown liver disease about 40 years previously, and he had never been transfused. The physical examination revealed multiple cervical lymph node enlargements and a two‐finger width of splenomegaly with tenderness. Numerous nodules and plaques were distributed on the trunk, face, and proximal extremities ( Fig. 1 ). The results of laboratory tests were as follows: leukocyte count, 24,200/mm³, with lymphocytes 15,560/mm³; serum lactate dehydrogenase, 943 IU/L (normal range, 263–450 IU/L); alkaline phosphatase, 135 IU/L (normal range, 35–60 IU/L); blood urea nitrogen, 32 mg/dL (normal range, 3–24 mg/dL); serum creatinine, 1.7 mg/dL (normal range, 0.3–1.6 mg/dL); serum calcium, 17.5 mg/dL (normal range, 8.8–10.2 mg/dL). Other laboratory results, including liver function test and urine analysis, showed no abnormalities. The examination of peripheral blood revealed multilobulated atypical lymphoid cells. The radiologic images showed hilar, para‐aortic lymph node enlargement and splenomegaly. A skin biopsy from the nodular lesion revealed massive infiltration of small‐ to medium‐sized atypical lymphoid cells in the dermis ( Fig. 2a ). The infiltrating cells were positive for CD3 ( Fig. 2b ), CD4 ( Fig. 2c ), CD5, and CD8 ( Fig. 2d ), but negative for CD20, CD34, CD56, CD68, and terminal deoxynucleotidyl transferase (TdT) in the immunohistochemical studies on paraffin sections. They also showed high Ki‐67 labeling (80–90%), and this finding reflects their highly proliferative nature. Polymerase chain reaction (PCR) analysis showed a distinct band for the T‐cell γ receptor gene, confirming the T‐cell clonality of the infiltrating neoplastic cells. Specimens from the cervical lymph node and bone marrow showed the same results in immunohistochemical studies. PCR analysis of the DNA from peripheral leukemic cells showed human T‐cell leukemia virus type I (HTLV‐I) proviral integration. The patient was diagnosed as having the acute type of adult T‐cell leukemia/lymphoma (ATLL). The disease course was extremely aggressive, and he died of acute renal failure on the 16th day following diagnosis.

Figure 1 Open in figure viewer PowerPoint Pruritic erythematous nodules and plaques on the face, trunk, and upper arms     

A case of necrobiotic xanthogranuloma without paraproteinemia presenting as a solitary tumor on the thigh

A 82‐year‐old Korean woman had had a 6‐month history of an asymptomatic, flat, hard, red to brown tumor on her right thigh. This lesion had been slowly enlarging with an advancing margin. She had noted gradually developing pain associated with necrosis and ulceration on the lesion. Examination revealed a solitary, 8 × 7.5 cm, yellow to dark red, telangiectatic tumor with multiple areas of punched out ulceration and a peripheral elevated yellowish margin on the right inner upper thigh ( Fig. 1 ). No clinically similar lesions on the periorbital area or other sites were seen. Histologic examination revealed a massive palisading granulomatous infiltration with several layers of extensive bands of necrobiotic zone in the entire dermis and deep subcutaneous tissue ( Fig. 2a ). In the granulomatous infiltrate in the dermis and subcutis, many various‐shaped, some bizarre, angulated, foreign‐body type multinucleated giant cells, many Touton giant cells, and a few Langhans giant cells were found to be scattered ( Fig. 2b ). There were numerous xanthomatized histiocytes. Dense infiltration of lymphoplasma cells was seen in the periphery of the granuloma and perivascularly. Conspicuous granulomatous panniculitis composed of lymphoplasma cells, polymorphonuclear cells, foam cells, and Touton and foreign‐body giant cells was also seen. However, cholesterol clefts and lymphoid follicles were not seen. Subcutaneous septae were widened by necrobiotic change and fibrosis with thrombosed large vessels. Gram, Gomeri‐methenamine silver and acid‐fast stains were negative. The necrobiotic areas were positive to alcian blue. Laboratory investigation revealed elevated white blood cell counts, anemia and elevated erythrocyte sedimentation rate. The following parameters were within the normal range: lipids, glucose, renal and liver function tests, serum complements, serum immunoglobulins, cryoglobulins and antinuclear antibodies. The findings of chest X‐ray, skull X‐ray and ectorcardiography were normal. Serum electrophoresis and serum immunoelectrophoresis revealed no abnormality. The patient was diagnosed as having necrobiotic xanthogranuloma without paraproteinemia. She was treated with oral steroid (0.5–0.6 mg/kg) and NSAIDS for 1 month with partial improvement of pain and the lesion ceased to enlarge. In the following 1 year of follow‐up, with only intermittent NSAIDS, her lesion did not progress and there were no signs of systemic involvement or new skin lesions.

Figure Figure 1  Open in figure viewer PowerPoint (a) A solitary, red to brown plaque with multiple ulcerations and a peripheral elevated yellowish margin on the inner upper thigh     

An unusual association of pemphigus vulgaris with hyperprolactinemia

A 21‐year‐old unmarried woman presented with oral ulcerations and generalized, itchy, fluid‐filled, skin lesions of 10 days’ duration. The lesions ruptured spontaneously, resulting in extensive denuded areas covered by crusts. One month prior to this, she experienced pain and enlargement of both breasts with galactorrhea. Her menstrual cycles were normal initially, but later she developed menstrual irregularities. No past history suggestive of any other systemic or skin disease, including atopy or drug allergies, could be obtained. Her family history was not contributory. Dermatologic examination revealed multiple, flaccid bullae and extensive denuded areas of skin covered with crusts over the scalp, face, trunk, and upper and lower limbs ( Fig. 1 ). Bulla spread sign and Nikolsky's sign were positive. The oral mucosa, including the lips, buccal surface, tongue, and palate, showed multiple erosions covered with necrotic slough. The rest of the mucocutaneous and systemic examination was within normal limits.

Figure 1 Open in figure viewer PowerPoint Extensive erosions and flaccid bullae over the trunk with breast enlargement     

Extensive xanthelasma associated with anaplastic large cell lymphoma and hyperimmunoglobulin E syndrome

A 57‐year‐old woman presented with a 6‐month history of an extensively spreading, yellowish patch on the periorbital areas and cheeks. A diagnosis of hyperimmunoglobulin E syndrome had been made at the age of 22 years on the basis of an eczematous eruption, recurrent furunculosis, and a persistently elevated immunoglobulin E (IgE) level. Her past medical history revealed that she had suffered from numerous recurrent bouts of chronic sinusitis, otitis media, oral candidiasis, orbital cellulitis, acne rosacea, and pneumonia caused by cytomegalovirus since her twenties. In addition, 1 year ago, anaplastic large cell lymphoma of the cervical lymph node (stage IIIb) developed, and she received six cycles of cyclophosphamide–doxorubicin–vincristine–prednisolone (CHOP) chemotherapy with partial remission. None of her family had any of these problems. Cutaneous examination showed extensive, symmetric, noninfiltrated macular areas of distinct yellow discoloration around the eyes and on both cheeks ( Fig. 1 ). There were also erythematous papulonodular eruptions on the nose and both cheeks, which were thought to be acne rosacea. Laboratory findings were normal, except for an elevated IgE level (8157 IU/mL). Serum concentrations of IgG, IgA, and IgM were normal. Serum complement levels were normal, as evidenced by normal C₃, C₄, and CH₅₀. Although she had a previous history of a decreased level (12%) of nitroblue tetrazolium (NBT) test (control, 53%), NBT test at our institute was normal. Neutrophil function tests, including neutrophil chemotaxis, neutrophil phagocytosis, neutrophil respiratory burst, and neutrophil microbial killing test, by flow cytometry, showed normal results. The serum lipid levels, including total cholesterol, triglyceride, low‐density lipoprotein‐cholesterol, and high‐density lipoprotein‐cholesterol, were normal. Serum lipoprotein electrophoresis was normal. A biopsy specimen revealed scattered foamy cells throughout the dermis. The larger clusters of foamy cells tended to group around the blood vessels of the dermis ( Fig. 2 ).

Figure 1 Open in figure viewer PowerPoint Extensively distributed, yellowish, flat xanthelasma on the face     

Cutaneous protothecosis

A 63‐year‐old Korean woman presented with erythematous plaques on her right forearm. The lesion had developed 4 months previously as pruritic papules and scaly patches, and grew slowly coalescing to eczematous plaques. Examination showed thick scaly erythematous plaques with multiple erosions and minute pustules over the right forearm ( Fig. 1 ). Her past history included hypertension and chronic arthritis of her knees requiring intermittent corticosteroid therapy over a period of 15 years. Routine laboratory evaluations (complete blood count, erythrocyte sedimentation rate (ESR), urinalysis, renal and liver function) and chest X‐ray studies were negative or within normal limits. The biopsy specimens of the plaque revealed mild acanthosis and mixed inflammatory infiltrates with some round spores in the dermis, which showed multiple morula‐like sporangia containing many endospores on periodic acid‐Schiff (PAS) and Gomori's methenamine silver (GMS) staining ( Fig. 2 ). Scraping of the lesion with potassium hydroxyde examination showed no specific findings, but the tissue culture revealed creamy yeast‐like colonies on Sabouraud dextrose agar (SDA) in room temperature within 48 h, and the colonies showed characteristic sporangia with morula‐like appearance on lactophenol cotton blue staining. The biochemical evaluation using a Yeast biochemical card (Vitec®, bioMérieux, France) identified the organisms as Prototheca wickerhamii, which assimilated galactose, trehalose, glycerol and glucose on the kit. Oral itraconazole (200 mg/day) for 8 weeks resulted in complete healing with remaining mild pigmentation, and there was no evidence of recurrence over the following 12 months.

Figure 1 Open in figure viewer PowerPoint Scaly erythematous and eczematous plaque on the right forearm     

Multiple eccrine hidrocystomas associated with Graves' disease

A 20‐year‐old Korean woman presented in August 1999 with a 3‐month history of multiple, tiny papules on the periorbital and malar areas ( Fig. 1a ). She had noted hyperhidrosis for the preceding 6 months, even at room temperature. She had been well and had received no medication prior to her first visit to our clinic. Physical examination showed yellow colored, translucent, small papules, as well as finger tremor, exophthalmos, and a goiter. Histologic examination demonstrated cystic structures in the dermis lined with two layers of cuboidal epithelial cells ( Fig. 2 ). The epidermis was normal and the rete ridges were partially effaced. Immunohistochemical studies revealed that the epithelial cells of the cyst wall were carcinoembryonic antigen (CEA) positive but S‐100 protein negative.

Figure 1 Open in figure viewer PowerPoint Multiple tiny papules on the periorbital and malar areas before (a) and after (b) treatment for Graves’ disease     

Extramedullary granulocytic sarcoma of the skin, mediastinum, and pericardium

A 27‐year‐old man, with a past history of developmental delay, presented on 18 November 2005 for the evaluation of an acute onset of multiple red–violaceous nodules on the head, neck, and trunk of 5 days’ duration. The patient had no associated fever, chills, weight loss, night sweats, chest pain, dyspnea, lymphadenopathy, or organomegaly. He had no previous history of malignancies. A biopsy indicated a diagnosis of leukemia cutis ( Fig. 1 ). His initial complete blood count (CBC) was within normal limits. The 2‐week follow‐up revealed enlargement of the previous lesions and the development of new lesions ( Fig. 2 ). By the third week, the patient had developed dyspnea, but with normal breath sounds and oxygen saturation. Chest computed tomography demonstrated a mediastinal mass measuring 16 × 5.2 cm and pericardial thickening ( Fig. 3 ). The diagnosis of granulocytic sarcoma of the skin lesion and mediastinal mass was established on the basis of immunohistochemical stains, with positivity to CD43 and Leder's preparation and negativity to CD3, CD4, CD5, CD8, CD10, CD20, CD23, CD30, CD34, CD56, bcl‐1, terminal deoxynucleotidyl transferase (TdT), and granzyme. The bone marrow was negative for malignant cells. CBC and chemistry panel were all normal. Nevertheless, the patient experienced increased dyspnea and developed a pericardial effusion which required a pericardial window. Cytology of the pericardial fluid was consistent with granulocytic sarcoma. Once the diagnosis of granulocytic sarcoma was established, the patient started a regimen of cytarabine, daunorubicin, and etoposide. Despite this, the skin lesions and mediastinal mass showed minimal response. Repeat computed tomography showed a mediastinal mass measuring 14.5 × 4.4 cm. The patient's respiratory status required intubation and, 2 weeks later, his family requested that he be withdrawn from life support.

Figure 1 Open in figure viewer PowerPoint Immature myelocytic infiltrate in the dermis (hematoxylin and eosin, ×4)     

Probable Werner's syndrome

A 59-year-old woman was first seen in our department for the evaluation of scleroderma-like skin changes and trophic ulcers on her legs. Her past medical history was of a long duration. At 18 years of age, the patient noted graying of the scalp hair, skin thinning, and slight hyperpigmentation. Soon after, she developed a high-pitched, raspy voice. Her growth ceased at about 14 years of age. At the age of 35 years, visual disturbances developed, and she was diagnosed as having bilateral cataracts. Cataract surgery was performed at the age of 40 years. During this time, marked muscular atrophy and joint contractures limited her walking. Around the age of 50 years, she developed leg ulcers unresponsive to treatment. In the ensuing period of approximately 7 years, the cutaneous ulcers worsened, resulting in osteomyelitis. Both her parents were normal and nonconsanguineous. The patient's 50-year-old sister and 34-year-old daughter have no medical problems. On physical examination, the patient's height was 148.5 cm and weight 47 kg. Her voice was high-pitched and childish. The entire skin surface, sparing the palms and soles, had a poikilodermic appearance ( Fig. 1 ). Although the skin was not sclerotic, facial involvement produced thinning and sharpening of the nose giving it a beaked appearance ( Fig. 2 ).

Figure 1 Open in figure viewer PowerPoint Premature aged appearance with atrophic, pigmented, and telangiectatic lesions     

Mucin-poor follicular mycosis fungoides

A 49‐year‐old woman was admitted with generalized pruritus which she had for the last 4 years. Three months ago she developed erythema on her face, alopecia totalis, an erythematous macular eruption with follicular hyperkeratoses on the trunk and limbs ( Fig. 1 ). She had bilateral palpable axillary lymphadenopathy. Histologic examination of the biopsies taken from the erythematous areas on the trunk and scalp revealed a folliculocentric infiltration composed of atypical, small and medium‐sized mononuclear cells, intermingled with reactive lymphocytes, histiocytes, plasmocytes, rare eosinophils, and giant cells without involvement of the epidermis ( Fig. 2 ). The infiltrate surrounded and invaded the hair follicle epithelium without destroying it. With alcian blue staining, only small amounts of mucin were detected within the epithelium of the hair follicles. By the immunohistochemistry performed, folliculocentric infiltration was mainly composed of CD3(+), CD4(+), CD8(–) lymphocytes. A full blood count, peripheral smear, erythrocyte sedimentation rate (ESR), the concentration of nitrogen in the form of urea in the blood (BUN), creatinin, transaminases, serum electrolytes, C‐reactive protein (CRP), IgE, serum lipids, serum lactate dehydrogenase, urinalysis, roentgenogram and CT scan of chest were normal. Beta 2 microglobulin was 3.15 mg/L (normal range 1.2–2.5 mg/L). In the biopsy of the axillary lymph node, there was a focal infiltration of atypical T‐cells in the interfollicular area. She was given psoralen‐UVA (PUVA) treatment for 6 months (85 sessions, in escalating doses with a total 71.8 j/cm²) which resulted in a partial healing of the pruritus, erythematous plaques, follicular hyperkeratoses, and patchy hair regrowth. She then received oral methyl prednisolone, 40 mg daily for 3 weeks and the dose was gradually decreased and eventually reduced to 16 mg daily in 2 months. This resulted in healing of the pruritus, improvement in the erythematous plaques and follicular hyperkeratoses and axillary lymphadenopathy. Beta 2 microglobulin levels decreased to normal range (2.27 mg/L). After taking the 16 mg daily dose of the prednisolone for 6 months, her complaint of itching recurred. Erythematous plaques and follicular hyperkeratoses were noted again. The dosage of the steroids was increased to 40 mg daily and PUVA treatment was restarted. She is currently receiving oral methyl prednisolone and PUVA.

Figure Figure 1  Open in figure viewer PowerPoint Follicular papules are seen on the upper extremities     

A case of simultaneously occurring lichen sclerosus and segmental vitiligo: connecting the underlying autoimmune pathogenesis

A 41‐year‐old African‐American woman reported a 6‐month history of asymptomatic white macules and patches that started on her left foot, and then spread proximally up her leg to her thigh, and then to her buttocks. She also noted that several of her pubic hairs had turned white, but only on the left side of the midline. She also complained of concomitant severe burning and itching in the vaginal area and that her vaginal skin had turned whitish pink. The patient denied contact with industrial or hazardous chemicals, and her medical history included childhood iron deficiency anemia only. On examination, the patient had linear guttate hypo‐ and depigmented macules on the left dorsal foot, extending up the medial calf, the medial and posterior thigh, and coalescing into scalloped patches across her left medial buttock ( Fig. 1 ). There was sharp midline demarcation in the suprapubic region. Her pubic hairs on the left side were predominantly depigmented. Examination of the genital area revealed areas of atrophy, together with slightly hyperkeratotic pink and hypopigmented plaques covering the entire perineal area, with a slightly irregular rim of gray–brown hyperpigmentation almost encircling the introitus. The lichen sclerosus of the labia minora extended directly into the perianal region ( Fig. 2 ). The vulvar area was tender to the touch.

Figure 1 Open in figure viewer PowerPoint Hypo‐ and depigmented macules and patches on the medial left calf, thigh, and pubic area