A case report and review of lymphocutaneous nocardiosis caused by Nocardia brasiliensis reported in China

Primary cutaneous nocardiosis caused by Nocardia brasiliensis is an uncommon disease. The present authors report a Chinese woman presented with lymphocutaneous nocardiosis caused by N. brasiliensis. The lesion begin with her right hand after an unknown insect sting and evolved rapidly and formed painful erythema and two subcutaneous nodules and abscesses on her right forearm in 5 days. N. brasiliensis was isolated from pustule and identified by gene sequencing. The patient received 2 weeks of combination therapy contained infusion of amoxilin potassioum clavinet and oral TMP/SMX and followed by a single therapy of oral TMP/SMX for 1 month and got a marked improvement. The present authors reviewed a case of lymphocutaneous nocardiosis caused by N. brasiliensis reported in China.     

Cutaneous fusariosis caused by Fusarium lichenicola in a child with hyper-immunoglobulin E syndrome

Fusariosis is the second most common mold infection after aspergillosis, and keratomycosis is the most encountered implantation infection. Here, we report a case of a 4-year-old Han Chinese girl presenting with an itchy mass on her right face of almost 2 years’ duration. Direct smear of the lesion sample was positive for fungal hyphae. Biopsy of the lesion showed many fungal hyphae in the epidermis and dermis. The pathogen was identified as Fusarium lichenicola by molecular sequencing and phylogenetic analysis based on the TEF-1α gene. Whole-exome sequencing analysis using her peripheral blood revealed a heterozygous mutation in the STAT3 gene, which is related to autosomal dominant hyper-immunoglobulin E syndrome (AD-HIES). The lesion improved following treatment with i.v. and intralesional amphotericin B, oral voriconazole and topical luliconazole cream. To our knowledge, this is the second reported case of a special localized cutaneous lesion caused by Fusarium species in a child with AD-HIES. Both cases suggest that STAT3 deficiency may increase susceptibility to fusariosis.     

A case of palmoplantar lichen planus in a patient with congenital sensorineural deafness

We report a case of palmoplantar lichen planus in a 7‐year‐old Japanese girl with congenital deafness, who presented with erythematous eruptions and hyperkeratosis, with peeling and fissures on her soles, palms and digits. On histological examination of a skin biopsy from the lesion on her wrist, lichen planus was identified. Using computed tomography of the inner ears, bilateral cochlear dysplasia was found. The patient’s DNA was sequenced; no sequence variants were detected in the GJB2 gene encoding connexin‐26, but she had a missense mutation in SLC26A4 (solute carrier family 26, member 4). Mutations in SLC26A4 are known causes of hearing loss, but this is a novel mutation, which has not been reported previously. In addition, there have been no reports of cutaneous symptoms in previously reported patients with mutations in SLC26A4. To our knowledge, therefore, this is the first report of palmoplantar lichen planus associated with sensorineural deafness accompanied by a mutation in the SLC26A4 gene.     

Pleomorphic mastocytoma associated with loss of chromosome 5, PDGFRA,and HRAS mutations: A case of cutaneous mastocytosis with severe atypia and indolent behavior

A 21‐year‐old female presented with a 5‐year history of an erythematous papule on her right breast. The biopsy showed a dense, dermal nodular infiltrate, extending focally into the subcutaneous tissue. The infiltrate was composed predominantly of pleomorphic cells with bi‐lobed, multi‐lobed, horseshoe, or ring‐shaped nuclei. There was a smaller subset of monomorphous cells characterized by a round, reniform, or elongated single‐lobed nucleus. Accompanying cells included few foamy histiocytes, lymphocytes, and numerous scattered eosinophils. No necrosis, vascular invasion, or ulceration was present. The pleomorphic and monomorphic granular cells were positive for Giemsa stain as well as for tryptase, CD117, CD68, CD2, and CD30 immunohistochemistry and negative for S100, CD1a, myeloperoxidase, lysozyme, and CD56. Clinical examination was negative for any additional similar lesions and serum tryptase was within normal limits. The bone marrow was not biopsied. In addition, fluorescent in situ hybridization revealed multiple clones with loss of number 5 chromosome and PDGFRA and HRAS mutations. The lesion did not recur or progress after a 6‐year clinical follow‐up. To our full knowledge, we report the first case of pleomorphic mastocytoma with loss of chromosome 5 and PDGFRA and HRAS mutations.     

Cutaneous nocardiosis: report of two cases and review of the literature

Background Cutaneous nocardiosis is an uncommon infectious disease that presents as a primary cutaneous infection or as a disseminated disease. It is often misdiagnosed because of its rarity and nonspecific clinical picture. Methods We report a case of each type. The first case is an immunocompetent patient who was infected by Nocardia while gardening and developed a superficial skin infection – one of the three clinical manifestations of primary cutaneous nocardiosis. The second case is an immunocompromised patient with pulmonary nocardiosis that extended to the skin as part of a disseminated disease. Results The immunocompetent patient with primary cutaneous nocardiosis had the classical features of a superficial skin infection. He had a nodular–pustular lesion on the right arm, which appeared 7 days after gardening with bare hands. Nocardia was identified in a skin culture taken from a pustule, unfortunately not to the species level. Treatment with minocycline for 3 months resulted in full remission of the lesion. The immunocompromised patient with disseminated nocardiosis had high fever, productive cough, hemoptysis, and erythematous nodules and pustules on the extremities. N. brasiliensis was isolated from bronchial samples and skin. Treatment with a high dose of trimethoprim and sulfamethoxazole for five months resulted in full recovery from cutaneous and pulmonary complaints. No relapse of the infection was found on follow‐up in either patient. Conclusion These cases demonstrate the need for a high degree of suspicion, focused clinical search, and appropriate laboratory procedures in the diagnosis and management of cutaneous nocardiosis.     

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)     

Mycetoma caused by Nocardia caviae in the first Brazilian patient

Background  Mycetoma is a chronic subcutaneous mycosis caused by exogenous fungi or actinomycetes. This infection has a progressive course and shows a typical clinical characteristic of tumefaction, draining sinuses, and grains. Infection initiation is related to local trauma and can spread to muscle, underlying bone, and adjacent organs. Nocardia brasiliensis is the most frequent actinomycete isolated, while N. caviae is a rare agent. Methods  We present a case of mycetoma in a 37‐year‐old African‐American man on the right hand. The infection had been apparent for four years prior to the consultation. When the infection did not respond to antibiotic therapy, the patient was referred to the Dermatology department. Routine laboratory studies were normal. X‐ray examination of the hand showed an osteolytic lesion on the hand bones. On skin biopsy culture, on Sabouraud Dextrose Agar at 28 °C, a colony was isolated which was further identified as N. caviae by biochemical and hydrolysis testing. Results The patient was treated with oral trimethoprim/sulfamethoxazole (TMP/SMZ) 160/800 mg twice a day for 10 months. Four months after the beginning of the therapy, the subject exhibited clinical improvement and functional recovery of the hand. Five‐year follow‐up X‐ray examination of the hand showed no osteolytic lesion on the hand bones. Conclusion We report the first mycetoma case caused by N. caviae in our country with an unusual location on the hand. The patient showed clinical improvement with oral TMP/SMZ.     

Langerhans cell histiocytosis and Erdheim‐Chester disease,both with cutaneous presentations,and papillary thyroid carcinoma all harboring the BRAFV600E mutation

Langerhans cell histocytosis (LCH) and Erdheim‐Chester disease are two rare histiocytic disorders. Their occurrence in the same patient is more infrequent, but has been described. We report a case of a 38‐year‐old woman who presented with a diagnosis of single system cutaneous LCH. Subsequently, she developed multiple papules on her extremities consistent with a non‐LCH xanthogranuloma type lesion. BRAF^V600E mutation immunostain, VE1 was positive in the skin lesion, which was confirmed by molecular polymerase chain reaction (PCR) studies, initiating a complete systemic workup for Erdheim‐Chester disease. Systemic involvement was confirmed with bilateral sclerotic bone lesions and retroperitoneal and pelvic fibrosing disease. She was also found to have a BRAF^V600E mutation positive papillary thyroid carcinoma. New suspicious cutaneous lesions presenting in patients with a history of LCH need to be biopsied. A BRAF^V600E mutation in a non‐LCH histiocytic lesion with a xanthogranuloma phenotype (CD163/CD68/CD14/fascin/Factor 13a) should prompt an Erdheim‐Chester disease workup. This is a unique case of a woman with BRAF^V600E mutation positive Erdheim‐Chester disease and cutaneous LCH, while also being, to our knowledge, the first reported case in the English literature of it occurring in a patient with a BRAF^V600E mutation positive papillary thyroid carcinoma.     

Co‐infection of Scedosporium apiospermum and Mycobacterium chelonae in an immunocompetent host

A 75‐year‐old man presented with multiple, scaly, erythematous, grouped papules, nodules and plaques with tenderness ranging from the right forearm to hand dorsum and the right lower leg for 2–3 months. Five months prior to presentation, the patient had received an antibiotic skin test on his right forearm. Lesions appeared approximately 2–3 months after the antibiotic skin test, slowly progressing without clinical improvement. Culture for fungus on the right forearm revealed growth of Scedosporium apiospermum. The tissue acid‐fast bacilli (AFB) culture for the right forearm and right leg revealed growth of non‐tuberculous mycobacteria which was Mycobacterium chelonae, and subsequent tissue polymerase chain reaction of both sites reported positive signs of M. chelonae. On diastase periodic acid‐Schiff stain of the biopsy specimen of the right forearm, fungal hyphae were found while rod‐shaped bacilli could be seen in AFB stain for the biopsy specimen of the right leg. The patient was treated with oral clarithromycin and ciprofloxacin along with an oral antifungal agent for 13 weeks. After the treatment, the lesions subsided and left a scar. We report a rare case of co‐infection of S. apiospermum and M. chelonae in an immunocompetent host.     

A congenital smooth muscle hamartoma masquerading as a reticulate vascular naevus

Smooth muscle hamartoma (SMH) is a benign congenital or acquired hamartomatous lesion comprising a dermal proliferation of smooth muscle bundles. We report a case of congenital SMH with an unusual clinical appearance. A 3‐year‐old girl presented with an asymptomatic atrophic linear lesion on the posterior surface of her right thigh, which had been present since birth. The striking resemblance to a vascular lesion initially led to the erroneous clinical diagnosis of atrophic reticulate vascular naevus. However, a skin biopsy showed typical features of SMH. To our knowledge, SMH with linear configuration has only been described in two previous cases, and there are no previous reports of SMH with such a marked resemblance to a vascular lesion.     

An atypical,painful foot ulcer

A 27‐year‐old woman presented with a 2‐year history of a progressively enlarging, painful ulcer on her right foot. Two years earlier, she had noticed an apparent wart on her right foot. The lesion had been treated with liquid nitrogen. An ulcer developed at the site of treatment and enlarged progressively, becoming so painful that she had difficulty walking. Extensive surgical debridement and closure were unsuccessful in healing the ulcer; the ulcer grew larger and more painful. After an amputation was recommended by her local doctors, the patient sought another opinion. At physical examination, the patient had a painful, 9.5 cm × 5 cm ulcer on the plantar aspect of the right foot ( Fig. 1 ). Exuberant, rolled borders were present, and a yellow exudate covered the base of the lesion. The right inguinal lymph nodes were enlarged and firm. A punch biopsy specimen from the ulcer border was examined.

Figure 1 Open in figure viewer PowerPoint A 27‐year‐old woman presented with a 2‐year history of a progressively enlarging, painful ulcer on the right foot. At physical examination, she had a painful, 9.5 cm × 5 cm ulcer on the plantar aspect of the right foot     

Apocrine gland carcinoma of the mammary skin concomitant with pagetoid phenomenon

We reported a 52‐year‐old woman with an apocrine gland carcinoma of the mammary skin concomitant with pagetoid phenomenon. She had a 23‐year history of a small nodular lesion on the lower left part of her right breast with a 1‐year history of the pigmented plaque spreading peripherally around the nodule. Our diagnosis revealed that the nodule was an apocrine gland carcinoma and the intraepidermal neoplastic cells with pagetoid spread in the pigmented plaque were derived from the apocrine gland carcinoma. No Paget’s cells were detected in the right nipple, and no tumor cells were observed in the sentinel lymph node and underlying mammary gland tissue. We also investigated the immunohistochemical changes in this case. They showed that both intraepidermal neoplastic cells with pagetoid spread and tumor cells of the apocrine gland carcinoma were positive with cytokeratin‐7 and human epidermal growth factor receptor‐2 (HER‐2)/neu overexpression. The results of the present study conclude that the intraepithelial spread of tumor cells in the mammary skin distant from the nipple occurred as a pagetoid phenomenon, and that HER‐2 may have a key role in pagetoid phenomenon of an underlying apocrine gland carcinoma, as well as in mammary Paget’s disease.     

The fish tank strikes again: Metachronous nontuberculous mycobacterial skin infection in an immunosuppressed host

An 82‐year‐old woman on long‐term prednisolone for chronic obstructive airways disease presented with a 2‐month history of nodules on her left forearm. This occurred 10 years after nodules on her right forearm caused by a culture‐proven Mycobacterium marinum infection. Histopathological examination, polymerase chain reaction and culture of biopsy specimens were positive for M. chelonae. To our knowledge this is the first case of metachronous nontuberculous mycobacterial skin infection reported, and it highlights the diagnostic and therapeutic challenges of such infections.     

Tinea capitis mimicking tufted hair folliculitis

We report a case of tinea capitis mimicking tufted hair folliculitis in a 56‐year‐old European man, who presented with a 4‐year history of pain and erythema in an area of scarring alopecia of the occipital scalp, with scales and tufts of hair emerging from individual follicles. Histological examination showed hair plugging, and a dense perifollicular infiltrate of plasma cells, lymphocytes, and neutrophils. There was widespread scarring and fibrosis. Bacterial cultures were negative for Staphylococcus aureus, but fungal cultures and periodic‐acid–Schiff stain were positive for Trichophyton tonsurans. Videodermatoscopy of the lesion showed a pattern consistent with folliculitis decalvans. Diagnosis was made on the basis of the clinical, histological, microbiological and videodermatoscopy data. After 30 days of systemic antifungal treatment, there were a substantial clinical improvement and disappearance of pain. After 5 months, a residual cicatricial area was seen with some hair tufts emerging from a single orifice.     

Multiple pilomatricoma with perforation

A 22‐year‐old woman presented to our clinic with a complaint of masses on various parts of her body. A mass on her right forearm had appeared 5 years ago and had enlarged during the past 6 months. Two lesions on the back of her neck had a 3‐year history, one lesion on her eyebrow had a 2‐year history, and one lesion on her left forearm had a 1‐year history. The lesion on her left forearm was discharging purulent material. Dermatologic examination revealed a 15 × 16 mm tumor on the right forearm, 11 × 6 mm and 10 × 5 mm tumors on the back of the neck, and a 20 × 20 mm tumor on the eyebrow; they were flesh‐colored, well‐defined, firm tumors. On her left forearm, there was a 12 × 10 mm, well‐defined, firm, blue–red tumor discharging chalky white granules; purulent material was detected ( Fig. 1 ).

Figure 1 Open in figure viewer PowerPoint Firm, blue–red tumor discharging chalky white granules     

Impetigo‐Like Tinea Faciei Around the Nostrils Caused by Arthroderma vanbreuseghemii Identified Using Polymerase Chain Reaction–Based Sequencing of Crusts

We report a case of Arthroderma vanbreuseghemii (a teleomorph of Trichophyton interdigitale) infection around the nostrils in a 3‐year‐old girl. The culture was negative, so the pathogenic agent was identified using polymerase chain reaction–based sequencing of the crusts taken from the lesion on the nostril. Treatment with oral itraconazole and topical 1% naftifine/0.25% ketoconazole cream after a topical wash with ketoconazole shampoo was effective.     

Unusual hyperpigmented patches,an undeveloped breast and a cataract in a female with incontinentia pigmenti

A 19‐year‐old female and her mother visited our department with a history of unusual reticular brown‐black patches on their trunks and limbs since infancy. Besides the obviously fulsome hyperpigmentation, the daughter also showed other unusual clinical manifestations such as generalized hypohidrosis, especially on the hyperpigmented patches, cicatricial alopecia, a cataract on her right eye, abnormal teeth and a right dysplastic breast. The mother had a median diastema between her maxillary central incisors, hypoplasia of the enamel, hypohidrosis and hyperpigmented patches on her left thoracic region. Analysis of the NEMO (NF‐κB essential modulator) gene in the patient and her mother revealed a deletion of exons 4–10. Their EDA and EDAR genes were normal.     

Cutaneous infection by Scedosporium apiospermum and its successful treatment with itraconazole

We report the case of a 42-year-old man with a 13-year history of bilateral faciocervical infiltrative erythema, which had been misdiagnosed as tuberculoderma and which had failed to respond to treatment with adrenal corticosteroids and antituberculotics. On admission to the department, Scedosporium apiospermum was identified on lesion biopsies and fungus cultures as the causative agent and a diagnosis of cutaneous infection by S. apiospermum was made. This is the first report of chronic skin granuloma caused by S. apiospermum in China. Treatment with oral itraconazole (100–400mg/day) led to clinical cure within 4 months.     

Dermanyssus gallinae (chicken mite): an underdiagnosed environmental infestation

Dermanyssus gallinae is a mite that normally parasitizes small birds but may occasionally bite humans. We report an unusual case of an 82‐year‐old woman who presented with pruritus and bite‐like lesions over her trunk. Other members of the household were also affected. On physical examination, mites < 1 mm in size were found on the patient’s body. The family were residing in the city centre and had no pets, but there were pigeon nests in close proximity to the house. Most dermatologists have difficulties identifying ectoparasitosis. In the case of D. gallinae, the small size of the mites and the fact that they leave the host after feeding means that they may not be seen at presentation, thus such infestations are likely to be underdiagnosed. Physicians should be aware that infection with this mite is possible even in patients from urban areas, and it should be included in the differential diagnosis of conditions causing recurrent pruritus unresponsive to standard treatments.     

Papuloerythroderma‐like cutaneous involvement of a CD62L− subclone of T‐cell prolymphocytic leukemia

We report the case of an 88‐year‐old Japanese man with erythrodermic involvement of T‐cell prolymphocytic leukemia (T‐PLL). He had a history of pharyngeal diffuse large B‐cell lymphoma successfully treated with polychemotherapy including cyclophosphamide and epirubicin, 6 years before the current illness. He presented with numerous reddish, coalescing, flat‐topped papules on the trunk and extremities, sparing the skin folds of the abdomen, the features of which mimicked those of papuloerythroderma. Immunohistochemistry showed perivascular and epidermotropic infiltration of CD3⁺ CD4⁺ T cells in the cutaneous lesion. However, flow cytometric analysis revealed that the skin infiltrating T cells were negative for surface CD4, and that CD3⁺ CD4^? CD8^? cells made up 92% of the T‐cell fraction of peripheral blood. The circulating atypical T cells had a round or oval nucleus and prominent nucleoli, and the deletion of chromosomes 6q, 13 and 17. These cytological profiles were consistent with those of T‐PLL and distinct from those of Sézary cells. The same T‐cell clone was detected in the cutaneous lesion and peripheral blood, but the expression of CD62L was absent in the skin infiltrates and present in the circulating cells. No specific mutation was detected in STAT3 or STAT5B. Although low‐dose oral etoposide had a beneficial effect on the skin rash, a fatal crisis of marked leukocytosis (169 × 10³/μL) occurred 19 months after the illness onset. CD62L‐leukemic cells of T‐PLL may infiltrate the skin to form papuloerythroderma‐like cutaneous lesions.