A Pediatric Case of Atypical Mycobacterium avium Infection of the Skin

We report a case of cutaneous atypical mycobacteriosis in a 12-year-old healthy girl due to Mycobacterium avium. The cutaneous symptoms were three well-defined subcutaneous nodules on both buttocks and on the posterior surface of the left thigh. One had a fistulous opening on the skin surface. Histopathological examination revealed epithelioid cell granulomas surrounded by dense lymphocytic infiltration and acid-fast bacteria were seen with modified periodic acid-carbol fuchsin staining. Using Ogawa's medium at 37°C, acid-fast bacteria were isolated from the biopsied specimen and identified by the DNA-DNA hybridization method as Mycobacterium avium. In drug susceptibility test, these were resistant to all antituberculous drugs. Oral administration of minocycline 100 mg/day for two months had little effect on the two remaining lesions, which were therefore excised. Based upon reported cases of Mycobacterium avium complex, we considered that our pediatric patient with multiple intradermal or subcutaneous nodules on the buttocks and the thigh exhibited the characteristic symptoms of M. avium infection.     

Primary cutaneous infection with Mycobacterium avium intracellulare complex resembling lupus vulgaris

We describe a 56–year–old woman with Mycobacterium avium complex infection of the skin. She presented with a granulomatous plaque studded with pustules and crusts on the cheeks and bridge of the nose. The appearance resembled lupus vulgaris. The lesion responded well to antituberculous treatment within 9 months. An association of Mycobacterium avium compled with squamous cell carcinoma of the cervix is emphasized.     

PAPULONECROTIC TUBERCULID SECONDARY TO DISSEMINATED MYCOBACTERIUM AVIUM COMPLEX

Background. Papulonecrotic tuberculid is a rarely reported cutaneous reaction to the mycobacterium bacillus. It is most often encountered in association with tuberculosis. The clinical and histologic picture of the entity is a distinctive one, but the etiology of the disease process is uncertain. Therapy directed against the causative organism is dramatically successful. Methods. A 35–year-old white man with AIDS was referred to the Dermatology clinic for evaluation of a widespread skin eruption. The skin lesions were biopsied for histopathology and culture. From the cutaneous cultures Mycobacterium avium complex (MAC) organisms were grown. Results. We report the first case of papulonecrotic tuberculid manifestation in an AIDS patient with disseminated MAC. Unusual features seen in this case include the predominance of pruritic eschars rather than asymptomatic papules and the confirmation by special stains of mycobacterium organisms within the skin biopsy. Papulonecrotic tuberculid has not been previously associated with either MAC or AIDS. Conclusions. Papulonecrotic tuberculid should be a diagnostic consideration in immunocompromised patients with MAC whose clinical and histologic features are compatible with this rare entity.     

Secondary syphilis primarily presenting with multiple nodules on the scalp: Case report and published work review

Syphilis, a sexually transmitted disease caused by Treponema pallidum , manifests with a broad spectrum of clinical presentations that usually involves skin. Nodular secondary syphilis occurs rarely, and mostly manifests as disseminated papulonodules or plaques over the trunk and limbs. We report an extremely rare case of nodular secondary syphilis that primarily presented with multiple nodules on the scalp, resembling Rosai–Dorfman disease or cutaneous malignant metastasis. Immunohistochemistry confirmed T. pallidum spirochetes microscopically.     

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.     

Prurigo successfully treated with duloxetine hydrochloride

Prurigo is a common skin disease characterised by erythematous macules and papules/nodules with severe pruritus. We report here two cases with treatment-resistant prurigo that were successfully treated with duloxetine hydrochloride, a serotonin–norepinephrine reuptake inhibitor. In vivo experiments with a mouse model of prurigo-like inflammation showed that duloxetine hydrochloride ameliorated not only scratching behaviours, but also skin inflammation. Duloxetine hydrochloride appears to be useful for treating prurigo via modulating itch signals and immune responses.     

Cutaneous polyarteritis nodosa: a report of a case associated with melioidosis (Burkholderia pseudomallei)

A 22-year-old Thai woman was hospitalized with a 1-month history of high-grade fever and slowly progressing multiple erythematous painful nodules on both legs. A history of arthralgia in the knee and ankle joints was presented. She had been treated with acetaminophen and a non-steroidal anti-inflammatory drug (NSAID) from a provincial hospital before, but without improvement. She denied the histories of upper respiratory tract infection, jaundice, tuberculosis, and taking any drugs before skin eruption. On physical examination, she had a temperature of 40 °C, a pulse rate of 90 beats per minute, a respiratory rate of 22 breaths per minute, and a blood pressure of 110/60 mmHg. She had multiple 1–4 cm erythematous, oval, tender, nodules on the pretibial area of both legs. Some nodules also had fluctuation and hemorrhagic bullae ( Fig. 1 ). Other physical examinations were normal, except for positive splenic dullness on percussion.

Figure 1 Open in figure viewer PowerPoint Erythematous, subcutaneous nodules with hemorrhagic bleb on both legs     

Leprosy type 1 reaction as the first manifestation of borderline lepromatous leprosy in a young native German

While leprosy is usually a chronic disease, leprosy reactions may lead to acute problems. These reactions most often occur after initiation of therapy, but can also develop before treatment. Leprosy rarely presents with a reaction. We describe a German patient who presented in this unusual way in order to demonstrate the various tools used to confirm the diagnosis. A young German woman suddenly developed progressive functional loss of the left hand and within a few weeks an increasing number of erythematous macules and nodules appeared. Histological examination of a skin biopsy revealed tuberculoid granulomas, some located around small nerves: acid-fast bacilli were detected microscopically and DNA from Mycobacterium leprae was identified by polymerase chain reaction in the biopsy and a nasal swab: serological tests were positive. The disease was classified as borderline lepromatous leprosy. The acute neuritis followed by skin lesions represented a leprosy type 1 reaction in which the immune system reacts to bacilli previously unrecognized in nerve tissue, both in nerve and skin.     

Analyses of CDC2L1 gene mutations in keloid tissue

Background. Keloid disease is the result of a deregulated wound‐healing process. Loss of heterozygosity on chromosome 1p36 has been shown to be associated with keloid formation in humans. The cell division cycle 2‐like 1 (CDC2L1) gene is known to be essential for eukaryotic cell‐cycle control, and has also been mapped to 1p36. Aim. To verify the possible association between keloid disease and somatic mutation of the CDC2L1 gene on chromosome 1p36. Methods. Mutations of the CDC2L1 gene in keloid and healthy skin tissues were screened by denaturing high‐performance liquid chromatography, and confirmed by DNA sequencing analysis. Results. Of the 27 patients with keloid assessed, 21 had mutations. The most prevalent exon affected was exon 7, with 15 patients affected: 10 patients (37%) had a base G deletion at codon 247, and 12 patients (44.4%) had a base A insertion at codon 267 (6 patients (25.9%) had both mutations). The remaining six patients had mutations in exons 11 (codon 433; n = 3) and 14 (codon 520; n = 3). Comparing the keloid skin tissues with the healthy control skin tissues, significant differences were seen between the groups for the base G deletion at codon 247 and the base A insertion at codon 267. Conclusions. We have identified a correlation between two exon 7 mutations of the CDC2L1 gene and keloid disease. A further study of protein‐kinase activity should be conducted to confirm the functionality of the CDC2L1 gene in the prevention of scar formation.     

Panarteritis cutanea benigna – an entity limited to the skin or cutaneous presentation of a systemic necrotizing vasculitis? Report of seven cases and review of the literature

Objective In 1931 Lindberg described a limited and benign subcutaneous form of panarteritis nodosa, which, in contrast to systemic panarteritis, only affects the skin. The terms panarteritis nodosa cutanea benigna, cutaneous polyarteritis nodosa, apoplexia cutanea Freund as well as livedo with nodules are used synonymously for this vasculitis which predominantly affects women in the fifth decade of life. Cutaneous lesions characteristically comprise painful subcutaneous nodules or vasculitis racemosa at the lower extremities. The cutaneous panarteritis may be regarded as its own entity or an isolated skin manifestation within systemic panarteritis nodosa. Methods Seven patients (M = 3, F = 4) presented with subcutaneous, painful nodules (n = 2), erythema and edema (n = 2) or livedo racemosa (n = 3) on the lower extremities. Histological examination revealed perivascular lymphocyte inflammatory infiltrates with fibrinoid necrosis and bulging of endothelial cells. In a direct immunofluorescence examination neither immunoglobulin nor complement deposits could be found. Serological autoimmune parameters, abdominal ultrasound examination, and chest X-rays showed no systemic manifestation. Erythrocyte sedimentation rate was slightly raised, and hepatitis B/C serology was negative. Results Topical corticosteroids under occlusion (n = 3), oral methyl-prednisolone (n = 4) in combination with either azathioprine (n = 4) or mycophenolate mofetil (n = 2) showed no relapses [follow up 28.43 (6–82) months]. Conclusion The etiology of panarteritis nodosa cutanea is unknown in detail. Both focal infections and hypersensitivity reactions are discussed. Differential diagnosis includes systemic panarteritis, livedo reticularis, Sneddon's syndrome or erythema nodosum. Despite the prognostically favorable but prolonged chronic course of the disease over decades, patients should be regularly examined to exclude possible transition to systemic disease.     

Glomuvenous malformations with smooth muscle and eccrine glands: unusual histopathologic features in a familial setting

Glomuvenous malformations (OMIM 138000) are hamartomas presenting in childhood as multiple, bluish papules and nodules in the skin, which are characterized histopathologically by irregular vascular spaces surrounded by typical glomus cells. Glomuvenous malformations are caused by autosomal dominant mutations of the GLMN gene. A 34‐year‐old woman and her 16‐year‐old son presented with bluish papules and nodules since childhood. Biopsy specimens from both patients showed histopathologic features of glomuvenous malformations, unusually in consistent and close association with smooth muscle, hair follicles and eccrine glands. Sequencing of the GLMN gene revealed the p.C36X (c.108C>A) mutation in germline DNA from both patients. This is probably the first report describing the hamartomatous features of familial glomuvenous malformations consistently associated with a prominent smooth muscle component and eccrine glands.     

Multiple miliary osteoma cutis is a distinct disease entity: four case reports and review of the literature

Background: Multiple miliary osteoma cutis (MMOC) is a rare nodular skin disease characterized by tiny bone nodules which usually form on the facial skin, typically in middle age. The aetiology of this phenomenon is poorly understood. Objectives: To search for possible bone formation progenitors and to look for a possible association with mutations in the GNAS gene (encoding the G‐protein α‐stimulatory subunit) and related hormonal parameters in patients with MMOC. We also reviewed the literature and discuss the aetiology and pathogenesis of adult‐onset primary osteomas. Methods: We report four cases of MMOC. Histological samples were analysed for bone morphogenetic protein (BMP)‐2, BMP‐4 and oestrogen receptor‐α known to be involved in bone formation. Endocrinological laboratory investigations and hand X‐rays were performed to exclude a systemic disease. The GNAS gene was sequenced from DNA extracted from peripheral blood in all four patients and from a skin sample in one patient to exclude somatic mutations. Results: Histological analyses revealed intramembranous cutaneous bone formation resembling the findings seen in GNAS gene‐based osteoma cutis disorders. However, we did not find any germline or somatic GNAS gene mutations in our patients and all laboratory investigations gave normal results. BMP‐2 and ‐4 were expressed normally in MMOC samples, but oestrogen receptor‐α was not expressed. Altogether 47 MMOC cases, 41 female and six male, have been published between 1928 and 2009. Of these cases, 55% had a history of pre‐existing acne and only 15% had extrafacial osteomas. Conclusions: MMOC is a rare but distinct disease entity of unknown aetiology. Histologically, the tiny nodular osteomas show intramembranous superficial ossification but the aetiology appears to be different from GNAS‐related disorders. The osteomas seem to increase slowly in number after appearing in middle age.     

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.     

Mycobacterium avium complex infection limited to the skin in a patient with systemic lupus erythematosus

We describe a case of Mycobacterium avium infection of the skin in a 51 -year-old woman with systemic lupus erythematosus. Two lesions were treated with a combination of oral tetracycline or minocycline and hyperthermia using a portable warmer. One subsequently healed, leaving an atrophic scar, but the other lesion persisted, and was eventually excised.     

Cutaneous granular bacteriosis,a rarely diagnosed infection of the head and the neck

A young man presented with a large multilobulated and mutilating tumour of the scalp, which had been relapsing for years. Histological examination of a biopsy from the lesion revealed chronic inflammation with granulation tissue and presence of granules with eosinophilic periphery, which was positive for Gram, Grocott and periodic‐acid–Schiff stains. A large excision was performed. Cultures grew Staphylococcus aureus. The patient was treated with penicillin G, but 4 weeks after the start of treatment, new small nodules appeared over the same area. All these new nodules disappeared within 2 weeks the addition of clindamycin and cotrimoxazole. This triple antibiotic treatment was carried on for 18 months, and the patient remained disease‐free after a follow‐up of 4 years. Although the lesions were clinically and histologically suggestive of actinomycosis, culture revealed that they were caused by a completely different organism. We suggest grouping such lesions under a single term “granular bacteriosis” and combining surgery with broad‐spectrum antibiotics covering Actinomyces species and botryomycosis‐causing organisms (mainly Staphylococcus).     

ACQUIRED PERFORATING DISEASE: REPORT OF NINE CASES

Background. A study of nine Guatemalan patients with acquired perforating disease associated with chronic renal failure, diagnosed at the Guatemalan Social Security Institute, General Hospital, during the period of January 1990 to March 1993, is presented (Table 1). Methods. A dermatological examination and skin biopsy were done on all patients. Results. Six of the patients were men and three were women; one case was associated with diabetes mellitus. Three of the patients were diagnosed as Kyrle-like acquired perforating disease, and the other six as perforating folliculitis. The lesions were predominantly located in the lower extremities with hyperkeratotic papules and nodules; some of these were accompanied by pruritus and Koebner's phenomenon. Conclusion. The acquired perforating disease is a clinical cutaneous manifestation of chronic renal failure with or without treatment. The lesions can disappear spontaneously with stabilization of the renal damage.     

Detection of parvovirus B19 DNA in the lesional skin of patients with Behçet's disease

Background. There is disagreement in the current evidence for viral aetiologies in the pathogenesis of Behçet's disease (BD). Objectives. To investigate the presence of B19 DNA in skin lesions of patients with BD, compare with the skin of healthy controls and evaluate its role in the pathogenesis. Methods. In total, 40 patients diagnosed with BD according to the criteria proposed by the International Study Group for Behçet's Disease and routinely followed up at our centre were enrolled into the study. All the patients selected were in the active phase of disease. Skin and blood samples of patients with BD and of the healthy volunteers were examined for B19 serology, histopathology and genome expression. Results. The quantity of B19 DNA in nonulcerative BD lesions of was significantly different from ulcerative lesions in the study group and from the skin of the healthy controls (P < 0.01). For the nonulcerative lesions, real-time PCR analysis for B19 DNA was found to be 64% sensitive (95% CI 42.5–82.0) and 85% specific (95% CI 62.1–96.6) with a cut-off value of > 154 IU/mL (P < 0.001). Conclusions. To the best of our knowledge, this is the first study that provides evidence for a possible causal link between BD and parvovirus B19, and our data suggest the presence of the virus, particularly in intact, nonulcerative skin lesions of BD. Limitations to this study include the limited number of participants, and the fact that the exact source of B19 DNA was undetected.     

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.     

Long‐term results of acitretin therapy for hidradenitis suppurativa. Is acne inversa also a misnomer?

Background Hidradenitis suppurativa (HS) is a distressing chronic inflammatory skin disorder which affects predominantly the groins and axillae. In analogy to acne, oral isotretinoin has been considered in the treatment of HS, although there are strong indications that this drug has only a very limited therapeutic effect. During the past 25 years scattered case reports have described promising results of treatment with acitretin. Objectives To evaluate the long‐term efficacy of acitretin monotherapy. Methods A retrospective study in 12 patients with severe, recalcitrant HS who were treated with acitretin for 9–12 months at one Dermatology Centre in the Netherlands between 2005 and 2007 and were followed up to 4 years. The patients were men and infertile women. The efficacy of the treatment was rated by the patients on global maximum pain of nodules and abscesses on a visual analogue scale (VAS) as well as by physician global assessment. Results All 12 patients achieved remission and experienced a significant decrease in pain as assessed by VAS. In nine patients long‐lasting improvement was observed, with no recurrence of lesions after 6 months (n = 1), 1 year (n = 3), > 2 years (n = 2), > 3 years (n = 2) and > 4 years (n = 1). Conclusions Acitretin appears to be an effective treatment for refractory HS, leading to reduction of pain from painful nodules and reducing the extent of the disease for a prolonged period.     

Leg ulcer caused by Mycobacterium ulcerans ssp. shinshuense infection

Background An 81‐year‐old man presented with a skin ulcer on the left forearm caused by infection with Mycobacterium ulcerans ssp. shinshuense. The patient first noticed the subcutaneous nodule with an undermined ulcer and areola on the left forearm without any episode of trauma. Methods The rod‐shaped and acid‐fast bacteria taken from the ulcerative lesion were positive for Ziehl–Neelsen staining. The bacterial colony was cultured on Ogawa slant egg medium at 28 °C. Results A clinical diagnosis of Mycobacterium infection was made. For therapy, in addition to oral clarithromycin, topical sulfadiazine silver and hyperthermia were used. One month after starting treatment, topical treatment was changed to U‐pasta (sucrose, povidone‐iodine). Four months after the onset of the disease, bacterial colonies composed of scotochromogens were identified as Mycobacterium marinum by the DNA–DNA hybridization (DDH) method. The growth speed and characteristics of the bacterial colonies were different from those of Mycobacterium marinum. Conclusions This pathogenetic bacterium was finally identified as Mycobacterium ulcerans ssp. shinshuense by the polymerase chain reaction method and 16S rRNA gene sequencing. Nine months after the onset of the disease, the ulcer was re‐epithelialized with a residual scar.