Lesions resembling malignant atrophic papulosis in a patient with progressive systemic sclerosis

Malignant atrophic papulosis (MAP), or Degos syndrome, is a rare disorder of unknown etiology. It is characterized by a deep subcutaneous vasculopathy resulting in atrophic, porcelain-white papules. We report the case of a 42-year-old woman with a history of progressive systemic sclerosis who presented with painful subcutaneous nodules on her abdomen along with chronic atrophic papules on her upper and lower limbs. Biopsy results of both types of lesions revealed vascular thrombi without surrounding inflammation. We briefly review the literature on MAP and its association with various connective tissue diseases. To our knowledge, there have been no previous reports of a patient with the clinical and histologic presentations described here. Although the histologic appearance of the subcutaneous nodules was very similar to that of the atrophic papules, the clinical characteristics of the 2 types of lesions were strikingly different. It is fair to theorize that Degos lesions do not start as atrophic porcelain-white papules but rather evolve from a primary lesion. We hypothesize that these lesions start as painful red nodules and may represent part of the disease spectrum in the evolution of MAP.     

Subcutaneous granuloma annulare of the scalp: a case report and case review

Subcutaneous granuloma annulare (SGA) is a benign inflammatory disorder that may be alarming in its presentation because of its rapid growth and extensive differential diagnosis. The purpose of our study was to improve the appropriate evaluation and management of pediatric patients with subcutaneous scalp nodules. This article presents the clinical presentation, histopathologic data, evaluation, and management of a patient with subcutaneous scalp nodules diagnosed as SGA. Additionally, the clinical data of all other cases of SGA diagnosed at our institution over a 9-year period were reviewed. The majority (72%) of SGA patients encountered at our facility were children. Most of the SGA lesions were located on the extremities; however, all of the lesions located on the scalp were in children. This article reviews the differential diagnosis and workup of scalp nodules. In evaluating the patient with subcutaneous scalp nodules, we conclude that SGA should be added to the differential diagnosis.     

A Mimic of Self‐Healing Juvenile Cutaneous Mucinosis?

A 14‐year‐old boy presented with a chronic history of atypical papular mucinosis consisting of multiple subcutaneous nodules and confluent papular skin lesions. He initially presented at age 2 years with the rapid onset of numerous subcutaneous nodular lesions that completely resolved over a period of years. Clinical and histologic evidence, together with his clinical course, were suggestive of self‐healing juvenile cutaneous mucinosis (SHJCM), but a few years later, during childhood, he experienced a recurrence of the subcutaneous nodules involving the limbs, trunk, and face, in addition to new findings of multiple flesh‐colored papules coalescing into plaques on his neck and back. Although his early childhood course and histologic picture were suggestive of SHJCM, the progressive nature of his disorder is not like that seen in SHJCM and appears different from other reported disorders involving cutaneous mucinosis.     

Sclerosing segmental neurofibromatosis

Segmental neurofibromatosis is a rare disorder characterized by cafe-au-lait macules and/or neurofibromas limited to a single body segment. The neurofibromas in segmental neurofibromatosis are usually soft, non-tender nodules as in other types of neurofibromatosis. Histopathologically, they are usually non-encapsulated, loosely textured dermal tumors. We report a case of sclerosing segmental neurofibromatosis, in which the patient presented with several grouped, erythematous to brownish, firm tender nodules on the left side of the posterior neck. Histopathologically, the stroma was mostly very fibrotic, especially around hair follicles, in addition to the usual features of neurofibroma. The atypical clinical feature, hardness, and tenderness of the lesions may be associated with the fibrosis.     

Co-existence of lichen amyloidosus and angiolymphoid hyperplasia with eosinophilia

Summary Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon benign vascular tumour. It presents with small, dull red papules or nodules usually on the ears and preauricular areas and only 20% of lesions are multiple. We report a case of multiple scattered lesions of ALHE in a patient who subsequently developed lichen amyloidosus. Cases of lichen amyloidosus in association with Kimura's disease have been reported previously, but there are no reports of lichen amyloidosus with ALHE. The coexistence of these two conditions implies that ALHE is an inflammatory disorder, as an inflammatory process resulting in basal layer damage is necessary for the occurrence of lichen amyloidosus.     

Nodular Lesions of Self-healing Juvenile Cutaneous Mucinosis: A Pitfall!

ABSTRACT:: Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin, which occurs in children in good health. It is characterized by the multiplication of transient cutaneous papules and nodules, mainly located on the head and periarticular areas that spontaneously resolve. Histological features of SHJCM have been well described; therefore, the diagnosis is usually made easily when papules are biopsied. We report a series of 3 new cases of SHJCM presenting mainly with nodular lesions. Histological examination of these nodules showed either lesions consistent with nodular or proliferative fasciitis or nonspecific panniculitis. Mucinous deposits were present but often inconspicuous, so could be disregarded. We wanted to emphasize this misleading presentation because a biopsy for histological examination is always mandatory in cases of proliferating nodules to rule out malignant tumors. Therefore, the diagnosis always requires discussion between pathologists and clinicians to rapidly reassure the parents and avoid inappropriate therapy.     

Scrotal calcinosis: a very rare multiple clinical presentation

Scrotal calcinosis (SC) is a rare benign disease that affects patients in childhood or early adulthood. It is characterized by slow‐growing yellowish‐white nodules consisting of deposits of calcium and phosphates, within the scrotal skin. The nodules vary in number, and can be solitary or grouped. Owing to the age of onset and anatomical location, SC may be a source of embarrassment and lead to social isolation. Because of its rarity, the aetiology of SC is still controversial. We report a very rare case of an SC in a 59‐year‐old white man who presented with multiple nodules with different clinical patterns in the scrotum, which had been present for > 42 years. Despite the rarity and the multiple long‐lasting lesions, surgical excision of the scrotal nodules can offer a very good aesthetic outcome in a single procedure even under local anaesthesia.     

Granuloma gluteale infantum: a case report

Granuloma gluteale infantum is a benign disorder with the clinical appearance of "granulomatous" nodules involving the diaper area. It is an uncommon disorder considered a complication of primary irritant diaper dermatitis. A 6-month-old boy with reddish-purple oval nodules located on the right inguinal fold and right buttock diagnosed as granuloma gluteale infantum is presented. A contributing factor in our patient could be a primary irritant dermatitis or a preexisting candidial infection. The name granuloma gluteale infantum is a misnomer since no granulomas are found in these lesions.     

The neuropathology of tuberous sclerosis.

Tuberous sclerosis, an autosomal dominant disorder associated with hamartomas in multiple organs, prominently affects the central nervous system; principle lesions include subependymal nodules and giant cell astrocytomas, white matter heterotopias, and cortical tubers. Histologic, immunocytochemical, and ultrastructural features of these processes suggest they have their basis in abnormal cell migration and differentiation. Aberrant expression of cystoskeletal proteins appears to be common to the multisystem hamartomas of this disorder and may, in part, underlie their pathogenesis.     

Keratosis lichenoides chronica and eruptive keratoacanthoma-like lesions in a patient with multiple myeloma

We describe a 72-year-old woman with a 13-year history of a lichenoid dermatitis, who developed multiple, papular keratoacanthoma (KA)-like lesions and few crater-like nodules on the extremities over a period of 6 months before our observation. Her medical history also recorded multiple myeloma diagnosed a few years before. The long-standing dermatosis was diagnosed, clinically, as keratosis lichenoides chronica (KLC), although, histologically, a lichenoid tissue reaction pattern was not evident. On the other hand, histology from papular and nodular lesions of recent onset was consistent with a possible early phase of KA and spinocellular carcinoma, respectively. Oral acitretin induced regression of KA-like lesions and improvement of KLC but had no effects on crater-like nodules, which required surgical excision. KLC is a chronic disorder of keratinization characterized by lichenoid hyperkeratotic papules arranged in a linear pattern, erythematosquamous plaques and seborrhoea-like dermatitis. We emphasize in our case the association between KLC and multiple possible KAs, never previously reported, and speculate that these two rare conditions may represent here a 'continuum' from a pathogenetic point of view.     

Multiple elastofibromas

Elastofibroma is an uncommon fibroelastic tumor or tumorlike process that usually occurs between the scapula and the chest wall of elderly patients. This condition is rarely reported in the dermatologic literature, because it infrequently arises in the skin or within the subcutaneous tissue. We present a 78-year-old man with 15 separate subcutaneous nodules on the buttocks and upper extremities together with bilateral subscapular nodules. All specimens taken from different lesions were histologically confirmed as elastofibromas. As far as we know, this case with 17 distinct elastofibromas demonstrates the largest number ever reported in a single patient. Although the pathomechanism of the occurrence of multiple elastofibromas is unknown, it should be included in the differential diagnosis of multiple subcutaneous nodules.     

Congenital self-healing histiocytosis. Report of two cases with histochemical and ultrastructural studies

Congenital self-healing histiocytosis (CSHH) is a rare primary skin disorder. Of the two cases in newborn infants reported here, one had numerous widespread lesions while the other had a solitary ulcerating scalp nodule. Both neonates were otherwise healthy; neither exhibited either systemic involvement or involvement of mucous membranes. The findings drawn from the skin biopsies, including histology, S-100 positivity in the majority of the cells, and the presence of Birbeck granules, were indistinguishable from those described in infantile Letterer-Siwe disease (histiocytosis X). However, the benign clinical course, with rapid regression of the nodules in both cases, was diagnostic of CSHH.     

Immunophenotype and possible origin of nevi with phenotypical heterogeneity

Biphenotypical nevi or nevi with phenotypical heterogeneity consist of phenotypically different cell populations in a pattern other than that observed in classical combined nevi or in various maturation stages of banal nevocellular nevi. Besides several well-known entities such as deep penetrating nevi and plexiform spindle cell nevi, this category of pigment cell lesions also harbors fewer delineated lesions such as nevi with atypical dermal nodules (N-ADN) and nevi with a focal atypical epithelioid cell component (N-FAECC). Their worrisome histology may result in a wrong diagnosis of malignancy. In order to discriminate them from malignant melanoma and to shed light on their histogenesis, we analyzed the immunophenotypical profile of 33 N-FAECC, 6 N-ADN, and 10 giant congenital nevi removed shortly after birth, using antibodies directed to S100 protein, gp100, tyrosinase, NKI-C3, Melan-A and Mib-1. In N-FAECC and N-ADN, the large polygonal cells expressed gp100, S100 protein and Melan-A, and reacted with monoclonal antibody NKI-C3. In addition, there was intense tyrosinase expression but no Mib-1 immunoreactivity. Unexpectedly, we observed similar single or clustered, large epithelioid cells in three out of ten giant congenital nevi; these cells showed a similar phenotype to those observed in N-ADN and N-FAECC. Our histological and immunohistochemical data suggest that N-FAECC and N-ADN may reflect different stages of the same disorder. Moreover, their resemblance to the large polygonal cells in congenital nevi may suggest that the histogenesis of N-ADN and N-FAECC may be related to the persistence and expansion of large epithelioid cells in congenital nevi shortly after birth.     

SLE并发播散性皮肤非典型分枝杆菌感染 1例

报告1例SLE患者并发孢子丝菌病样播散性非典型分枝杆菌原发皮肤感染，患者女性，33岁，水产经营者，既往有SLE病史15年。因“全身散发皮下红斑结节，破溃化脓伴轻痛2月”收住院。皮损散在分布于四肢和躯干。为大小不等的硬性结斑结节，中央破溃，脓性分泌物的黄色结痂。皮损组织液涂片找到抗酸杆菌。组织病理示真皮肉芽肿性炎症。用阿米卡星霉素和复方磺胺甲恶唑（SMZco)治疗1个月，大部分皮损消退好转。     

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).     

Cutaneous involvement of polymorphic post‐transplant lymphoproliferative disorder in a child after liver transplantation

Post‐transplantation lymphoproliferative disorder (PTLD) is one of the most common de novo malignancies in patients who receive immunosuppressive therapy after solid organ transplantation. We report a case of a 5‐year‐old girl who presented with indurated violaceous skin nodules 3.5 years post–liver transplantation, diagnosed as polymorphic PTLD, also involving Waldeyer's ring, spleen, and multiple lymph nodes. Through reduction of immunosuppression, most of the lesions resolved and the liver allograft was preserved.     

Angiolymphoid Hyperplasia with Eosinophilia Mimicking Multiple Cylindromas: A Rare Case Report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare and idiopathic vascular disorder. It is characterized by red to brown papules and nodules, typically localized on the head and neck, particularly around the ear as singular or multiple lesions. Although ALHE is a benign disease, lesions are often persistent and difficult to eradicate. Young to middle age women are more commonly affected. The histological examination corresponds to a florid vascular proliferation with atypical endothelial cells surrounded by a lymphocytic and eosinophilic infiltrate. We describe an elderly male with multiple nodular lesions over the scalp mimicking cylindromas; the histological examination was consistent with ALHE.     

Cutaneous and systemic plasmacytosis in a patient of Asian descent living in the United States

Cutaneous and systemic plasmacytosis is a rare disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. Previous reports have been largely restricted to the Japanese literature. We present the first documented case of cutaneous and systemic plasmacytosis in a patient residing in the United States. This 49-year-old man, who had immigrated from Korea 19 years earlier, developed innumerable persistent pink-to-brown macular lesions over his trunk and face. Initial and repeat skin biopsy specimens revealed dense perivascular and periadnexal infiltrates of mature plasma cells, and polyclonal plasmacytosis noted on two different biopsy specimens of mildly enlarged lymph nodes. Multiple tiny pulmonary nodules were found to be of the same histologic appearance. No evidence of clonal immunoglobulin gene rearrangements or human herpesvirus type 8 infection was noted in these biopsy specimens. Treatment with antibiotics, systemic chemotherapy, and anti-CD20 antibody therapy failed to eradicate these lesions, which have persisted for 6 years. This case demonstrates that cutaneous and systemic plasmacytosis can arise in a patient of Asian ancestry, even many years after emigration to the United States.     

Buschke‐Ollendorff syndrome with LEMD3 germline stopgain mutation p.R678* presenting as multiple subcutaneous nodules with mucin deposition

Buschke‐Ollendorff syndrome (BOS; OMIM 166700) is a rare autosomal dominant disorder characterized by the existence of connective tissue nevus and/or osteopoikilosis. The skin lesions usually present as firm, yellow, or flesh‐colored papules and nodules, which may coalesce into plaques and increase in size and number over time. We present a case of a 26‐year‐old male with multiple subcutaneous nodules on the waist and thigh for more than 20 years. Being deeply seated, his skin lesions were not visible and could only be appreciated by palpation. Accordingly, pathology showed an increase in thick, crossed, or paralleled, elastic fibers arranged between the collagen bundles in the lower part of the reticular dermis and the subcutaneous fat with mucin deposition. Heterozygous point mutation in exon 8 of the LEMD3 gene was detected, which confirmed the diagnosis of BOS. The deeply situated nature of skin lesions noted in our case has not been reported in the literature of BOS. Our case thus expands the clinical and pathological features of the disease.     

Pulmonary Mycobacterium abscessus infection-induced erythema induratum

Erythema induratum (EI) is clinically characterized by recurrent crops of tender nodules on the lower legs and lobular panniculitis with granulomatous inflammation and pathologically characterized by vasculitis and focal fat necrosis. Currently, many authors consider EI to be a multifactorial disorder with diverse causes, including Mycobacterium tuberculosis and hepatitis C infection. Here, we report a case of a 65-year-old female with a 1-year history of recurrent crops of tender nodules and plaques on her bilateral lower legs. In addition, she had suffered from a chronic cough with sputum for 1 year and had contact history with pulmonary nontuberculous mycobacterial infection from her husband. The histopathological findings of the skin biopsies were consistent with the diagnosis of EI. Chest computed tomography revealed multiple lymphadenopathy and two sets of sputum cultures showed M. abscessus. After 2 months of anti-nontuberculous mycobacterial therapy with ciprofloxacin, the skin lesions resolved completely and there was no recurrence within the following year.