Coexistence of Hidradenitis Suppurativa and Steatocystoma Multiplex: Is It a New Variant of Hidradenitis Suppurativa?

Hidradenitis suppurativa and steatocystoma multiplex may coexist in the same patient. The overlap of these 2 conditions could be suggestive of an unrecognized defect in follicular proliferation mutual in the pathogenesis of both conditions. Here we present 5 patients with both hidradenitis suppurativa and steatocystoma multiplex. Recognizing the overlap between these 2 conditions is important for accurate diagnosis, management, and identification of potential surgical candidates, as well as future basic science research.

Numerous asymptomatic dermal cysts: Diagnosis and treatment of steatocystoma multiplex.

OBJECTIVE: To provide family physicians with the information needed to recognize, diagnose, and discuss available treatment options for steatocystoma multiplex (SM). SOURCES OF INFORMATION: A comprehensive PubMed search using steatocystoma multiplex as either a text word or a MeSH term was conducted, and articles reporting on treatment outcomes were included. MAIN MESSAGE: Steatocystoma multiplex is a benign disorder often characterized by numerous asymptomatic dermal cysts on the trunk, arms, axillae, face, thighs, and scalp. Psychological distress due to these undesirable lesions is not uncommon for this condition. A literature review identified the following SM treatments, all of which were associated with limitations: carbon dioxide laser, modified surgical techniques, cryotherapy, and medical management. Steatocystoma multiplex is challenging to treat and, at this time, effective management is most often achieved through patient education. CONCLUSION: Family physicians play a critical role in the early diagnosis and management of SM. Education about treatment options and managing patient expectations might greatly alleviate the psychosocial implications of this disease.

Síndrome de Jackson-Lawler. A propósito de un caso / Jackson-Lawler síndrome. A case report

Se presenta paciente masculino de 27 años de edad, de raza mestiza, con antecedentes de queratodermia plantar, que acude a consulta especializada de dermatología en el hospital clínico quirúrgico docente Celia Sánchez Manduley por exacerbación de las lesiones, acompañadas de dolor intenso al caminar; evidenciándose al examen físico la presencia de lesiones quísticas diseminadas, hipertrofia ungueal y alopecia. Se concluye caso como síndrome de Jackson-Lawler, resaltando su infrecuencia en el medio, la afectación de calidad de vida e impacto psicológico generada al paciente y la importancia del asesoramiento genético al mismo, con el fin de prevenir futuras generaciones afectadas(AU)

We present a 27-year-old male patient of mixed race, with a history of plantar keratoderma, who attended a specialized in the Celia Sánchez Manduley clinical teaching hospital due to exacerbation of the lesions, accompanied by severe pain when walking; the physical examination revealed the presence of disseminatedcystic lesions, nail hypertrophy and alopecia. The case is concluded as a Jackson-Lawler syndrome, highlighting its infrequence in the environment, the impact on the quality of life and the psychological impact generated on the patient and the importance of genetic counseling to them, in order to prevent future generations affected(EU)

Co-occurrence of steatocystoma multiplex, eruptive vellus hair cysts, and trichofolliculomas.

An association between steatocystoma multiplex (SCM) and eruptive vellus hair cysts (EVHCs) has been recognized. Steatocystoma multiplex and EVHC have similar clinical features but distinctive histologic features. Rare cases of co-occurrence of these conditions have been known to occur on the trunk and the forehead. We report a rare case of the simultaneous occurrence of SCM, EVHC, and trichofolliculomas localized to the forehead.

Steatocystoma multiplex is associated with the R94C mutation in the KRTl7 gene.

Steatocystoma multiplex (SM) is an uncommon disorder, characterized by numerous skin­colored subcutaneous cysts. A number of SM pedigrees have been identified with mutations in the keratin 17 (KRT17) gene. The present study examined a four­generation Chinese pedigree with an autosomal dominant mode of inheritance and examined its genetic basis. A review of the literature on KRT17 gene mutations in the SM pedigree was also performed to investigate the KRT17 gene mutation and genotype­phenotype correlation. Exon 1 of the KRTl7 gene was amplified using polymerase chain reaction (PCR) from genomic DNA obtained, which was obtained from 25 family members in the selected Chinese pedigree and from 100 unrelated control individuals. The DNA was then subjected to automatic DNA sequencing. Genealogical investigations demonstrated an autosomal dominant pattern, and direct sequencing of the PCR product revealed a heterozygous mutation, c.280C/T (R94C), which was located in exon 1 of the KRT17 gene in all 10 affected family members. The mutation was not identified in the 15 unaffected family members or in the 100 unrelated control individuals. Therefore, the present study identified a causative mutation in the KRT17 gene in a large Chinese SM pedigree, exhibiting autosomal dominance. A review of the literature suggested that, in addition to the mutation factor, other modifying factors contribute to the phenotype of familial SM.

Carbon dioxide laser perforation and extirpation of steatocystoma multiplex.

BACKGROUND: Steatocystoma multiplex (SM) is a rare condition that presents as multiple dermal cysts, the appearance of which can have a significant impact on the patients' quality of life. Treatment options for SM are limited to surgical excision. OBJECTIVE: To present our experience of treating 8 SM cases using a novel approach that uses the carbon dioxide (CO2) laser and to explore patients' views about the treatment. METHODS: Patients were identified from our records. All patients had multiple lesions treated using the CO2 laser in the super pulse mode that punctured the cyst. This was followed by extirpating the cyst wall and its contents using a small Volkmann's spoon. Treatment efficacy was assessed clinically and feedback from the patients was sought through a telephone interview. RESULTS: All patients showed significant clinical improvement with minimal scarring and low recurrence rates. High levels of patient satisfaction, which helped to significantly improve their quality of life, were reported with the CO2 laser treatment. CONCLUSION: CO2 laser perforation and extirpation is a successful approach for managing SM that results in high patient satisfaction.

Multiple cystic disease: K17 dysfunction?

Our patient is a 29-year-old woman without any previous disease who presented with different kinds of lesions on her face, neck, and chest. She first noticed the lesions 10 years ago and, since that time, they have become more numerous. She has no affected relatives. On physical examination, she had multiple cystic lesions on her neck, chest, and vulva, which were between 0.3 cm and 1 cm and skin-colored or yellowish (Figure 1). She presented with small, white papules on her face measuring approximately 0.2 cm, localized on her forehead and cheeks. Some of these papules had a blueish appearance (Figure 2). She also presented clinically typical eruptive syringomas on her upper and lower eyelids and neck and multiple facial milia. Finally, a sacrococcygeal pilonidal cyst was diagnosed and surgically removed. Her nails and teeth were clinically normal. Biopsies of each kind of lesion were performed, with the following results: (1) neck cystic lesion: steatocystoma; (2) small, white facial papule: eccrine hidrocystoma; (3) blueish facial papule: apocrine hidrocystoma; and (4) small neck papule: syringoma (Figure 3). With these findings, our diagnosis was steatocystoma multiplex with multiple eccrine and apocrine hidrocystomas, eruptive syringomas, and sacrococcygeal pilonidal cyst.

Mammographic and sonographic findings of steatocystoma multiplex presenting as breast lumps.

Steatocystoma multiplex (SM) is an uncommon cutaneous disorder characterised by multiple intradermal cysts distributed over the trunk and proximal extremities. This condition affects both genders and is often inherited as an autosomal dominant trait, although sporadic cases have been described. This report describes the mammographic and sonographic features of the cysts, which presented as breast lumps, for evaluation. The cysts appeared as numerous well-circumscribed, radiolucent nodules with thin radiodense rims on mammography. On sonography, the cysts could be hypoechoic, isoechoic or demonstrate mixed echoes containing debris-fluid levels, depending on the amount of clear oily liquid and keratinous material. SM can be diagnosed based on a clinical setting of multiple asymptomatic small intradermal nodules over the trunk and proximal extremities, positive family history and imaging findings.

Multiple large infected scrotal sebaceous cysts masking Fournier's gangrene in a 32-year-old man.

Extensive large sebaceous cysts on the scotum are rare and present a problem only when infected or when cosmesis is deemed unacceptable by the patient. Fournier's gangrene is an infective condition with a high death rate. We describe a case of Fournier's gangrene in a patient masked by multiple large infected scrotal sebaceous cysts. A 32-year-old man with a history of alcohol dependency, cirrhosis and multiple scrotal sebaceous cysts presented with acute scrotal pain and erythema. Necrosis of the area became evident within 12 h of his admission and an emergency surgical debridement was performed. The wound was left open to heal via secondary intention over 4 weeks without complication. Fournier's gangrene is a rapidly progressive condition and early surgical debridement is crucial to achieve satisfactory outcomes. In this case, prompt intervention allowed a large scrotal defect to heal without the need for skin grafting.

Steatocystoma multiplex.

A 40-year-old man presented with multiple papules on his head and neck. The lesions had been present for about 15 years. The patient was treated for acne for 6 months, but no improvement was noted. A biopsy was performed and microscopic findings were consistent with steatocystoma multiplex.