Sarcoidosis in a preschooler with only skin and joint involvement

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. It is relatively rare in children less than 15 years of age and especially in those less than 5-6 years of age. Sarcoidosis characteristically involves the skin, eyes, and synovial tissues in patients less than 5-6 years of age. We report a 3-year-old boy with sarcoidosis who had cutaneous findings with joint symptoms. Dermatologic examination revealed lichenoid, erythematous, 2-3 mm papules, some of them grouped, all over his extremities and trunk. There were symmetric swellings on his ankles and wrists without erythema or pain which did not interfere with function. However, until now, no eye involvement had been detected in the patient.     

Granulomatous rosacea-like demodicidosis

Demodicidosis may present as pityriasis folliculitis, papulopustular lesions, rosacea-like eruptions, and granulomatous rosacea-like eruptions. We report a case of demodex granuloma presenting with recurrent granulomatous rosacea-like papules on the face in a middle-aged woman. The diagnosis of demodicidosis was made by finding extrafollicular mites in the perifollicular inflammatory infiltrate. The papules resolved after 3 weeks of systemic and topical metronidazole, and low-dose oral prednisolone therapy. In summary, demodex granuloma may be mistaken for granulomatous rosacea-like papules. Correct diagnosis can be facilitated by finding extrafollicular demodex mites in skin biopsy specimens.     

Lichenoid Sarcoidosis in a 3-Year-Old Girl

We report a case of lichenoid sarcoidosis in a 3-year-old girl. She had numerous discrete skin-colored or erythematous, infiltrated follicular papules on the buttocks and extremities since 2 months of age. Histopathologic examination showed follicular plugging and an upper dermal granulomatous infiltrate of epithelioid cells closely surrounding the follicular ducts. No acid-fast bacilli were seen in the sections examined. Chest radiograph and high-resolution computed tomography (CT) showed no hilar lymphadenopathy or pulmonary parenchymal changes. An angiotensin-converting enzyme level was elevated. The Mantoux reaction was negative and results of ophthalmologic examinations were normal. Treatment was started with triamcinolone 0.2 mg/kg and prednicarbate ointment. Some lesions healed completely and others showed residual pitting.     

Granulomatous pigmented purpura: report of a case and review of the literature

The pigmented purpuric dermatoses (PPD) are a group of diseases characterized by petechiae and bronze discoloration of the skin on the lower extremities. Histopathologically, extravasation of erythrocytes with hemosiderin deposition, a perivascular lymphocytic infiltrate centered on the superficial capillaries and endothelial cell swelling are seen. The granulomatous variant of PPD (GPPD) was described in 1996 and only 10 cases have been reported since in the literature, almost exclusively in patients of East Asian descent only involving the extremities. We present a case of GPPD in a Caucasian, North American Ashkenazi Jewish woman involving the thighs, back, forearms and wrists with concomitant non-granulomatous PPD of the shins. She presented with an asymptomatic, spreading, cayenne pepper-like rash. This rash intermittently involved the lower extremities and back for 15 years, but now involves the thighs with accompanying pink papules on the back, wrists and forearms. Histopathology of the thigh and back lesions revealed superficial lichenoid granulomatous dermatitis with palisading lymphocytes and focal interface changes. Extravasated erythrocytes were seen, but vasculitis was absent. No lymphocytic atypicality was noted and T-cell gene rearrangement studies were non-clonal. This is the second reported case of GPPD in a non-Asian patient and the first case involving sites other than the extremities.     

Granulomatous pigmented purpuric dermatosis

The granulomatous variant of the pigmented purpuric dermatoses (PPDs) is a rare and infrequently described condition, with a total of 16 cases published to date. We report a case of granulomatous PPD in a 59‐year‐old white woman who demonstrated involvement of the arms, legs, chest and back with concurrent hyperlipidaemia. Histopathological examination revealed a lymphohistiocytic infiltrate obscuring the dermoepidermal junction, and loose granuloma formation in the superficial dermis, with extravasated erythrocytes. Other conditions within the differential diagnosis such as atypical infection, papular sarcoidosis and generalized granuloma annulare were excluded on clinical and histological grounds. Our patient represents the ninth patient reported to have granulomatous PPD with coexisting hyperlipidaemia, and the fifth patient with granulomatous PPD and a lichenoid infiltrate.     

Transepidermal elimination in exogenous ochronosis. A report of two cases

Exogenous ochronosis is caused by the long-term application of skin-lightening creams containing hydroquinone. This irreversible disfiguring cosmetic problem assumes epidemic proportions in South African blacks. Mild ochronosis is characterized clinically by coarsening and darkening of the skin; severe ochronosis, by coalescing, caviar-like black papules and atrophy. Histology shows ochronotic collagen fibers with eventual formation of ochronotic colloid milium. A variable cellular infiltrate, which may be granulomatous, is present. We describe two patients with severe exogenous ochronosis who developed superimposed papular lesions. Histology in both cases showed transfollicular elimination of ochronotic fibers. In one patient, gross epidermal hyperplasia, a dense lichenoid infiltrate, and partial destruction of ochronotic fibers accompanied the process of elimination (cell-rich type). In the other, concomitant epidermal hyperplasia and a cellular infiltrate were absent (cell-poor type). Further studies are needed to prove or disprove the existence of such a proposed subdivision. Transepidermal elimination in exogenous ochronosis has been mentioned in a previous report, but to our knowledge this is the first detailed documentation of this phenomenon. The clinical and histopathological spectrum of exogenous ochronosis is thus expanded.     

Lichenoid and granulomatous dermatitis associated with atypical mycobacterium infections

BACKGROUND: Lichenoid and granulomatous dermatitis defines a distinctive pattern of cutaneous inflammation that may be part of the morphologic spectrum of idiopathic lichenoid reactions such as lichen planus and as well may be seen with lichenoid drug reactions, endogenous T-cell dyscrasias and as a feature of certain systemic diseases especially Crohn's disease and rheumatoid arthritis. RESULTS: We encountered three cases of lichenoid and granulomatous dermatitis in which the basis was one of primary cutaneous Mycobacterium infection. In all three cases acid fast stains revealed pathogenic organisms and as well cultures were positive for Mycobacterium kansasii in one case and Mycobacterium marinum in another. Other features included a prominent perineural and periadnexal lymphocytic infiltrate. CONCLUSIONS: The differential diagnosis of lichenoid and granulomatous dermatitis should also encompass primary cutaneous Mycobacterium infection in addition to the other more characteristic entities associated wtih this distinctive reaction pattern. Infection with Mycobacterium induces a TH1 dominant response which would hence produce an infiltrate.     

Giant cell lichenoid dermatitis within herpes zoster scars in a bone marrow recipient

Cutaneous lesions arising in herpes zoster (HZ) scars are rare. We report a 34-year-old woman with acute lymphoblastic leukemia underwent allogenic bone marrow transplant (BMT). Ten days after the BMT, she developed clusters of vesicles over the right neck, scapula, shoulder and chest. She was treated with intravenous acyclovir and foscarnet. One month after the vesiculous episode of HZ she showed 5 mm to 2 cm clustered flat violaceous lichenoid papules and confluent plaques within the HZ scars. Histopathologic examination revealed a inflammatory infiltrate present in the papillary dermis with granulomatous aggregated formed by histiocytes, multinucleated giant cells and lymphocytes. She was treated with topic steroids with significant improvement. Pathologic findings are similar to those of an unusual lichenoid reaction named "giant cell lichenoid dermatitis". We present the first reported case of giant cell lichenoid dermatitis at the sites of HZ scars.     

Photolichenoid plaques with associated vitiliginous pigmentary changes

A 49-year-old man with advanced HIV/AIDS on anti-retroviral therapy (HAART) and trimethoprim-sulfamethoxazole (TMP-SMX) presented with a several-month history of pruritic, erythematous, lichenified papules that coalesced into hyperkeratotic plaques on the trunk and extremities in a sun-exposed distribution. He shortly thereafter developed a progressive depigmentation over more than 80 percent of his body surface area. A biopsy specimen of an erythematous plaque on the trunk showed a superficial and mid-dermal infiltrate of lymphocytes with eosinophils, most consistent with either chronic lichenoid drug eruption or atypical lymphoproliferative disorder (ACLD) of HIV. The patient's lichenoid skin disease has persisted despite discontinuation of TMP-SMX, although it has improved partially with administration of topical glucocorticoids and acitretin. His depigmentation has continued to progress. We discuss the overlapping diagnostic entities which may be comprised by this patient's clinical disease, and highlight a unique presentation of the complex interaction between HIV infection and the skin.     

Papular Acrodermatitis of Childhood (Gianotti-Crosti Disease)

An 11-year-old boy had lentil-sized lichenoid papules, localized to the limbs and trunk, together with acute, nonicteric, hepatitis B surface antigen-positive hepatitis. The clinical picture and course were typical of Gianotti-Crosti disease. Monoclonal antibodies were used to study the lymphocyte subpopulations and surface antigens in the inflammatory infiltrate in frozen sections of a skin biopsy specimen. The results provide data on the pathogenic mechanism of the papular exanthem.     

Monoclonal rearrangement of the T cell receptor gamma-chain in lichenoid pigmented purpuric dermatitis of gougerot-blum responding to topical corticosteroid therapy

Lichenoid pigmented purpuric dermatitis of Gougerot-Blum belongs to a group of closely related disorders which are termed pigmented purpuric dermatoses. It clinically manifests itself with grouped lichenoid papules in association with purpuric lesions. We report a case of lichenoid pigmented purpuric dermatitis of Gougerot-Blum with a heavy band-like CD4-positive lymphocytic infiltrate and clonal rearrangements of the gamma-chain of the T cell receptors as detected by polymerase chain reaction/denaturing gradient gel electrophoresis. Monoclonal expansion of T cells in combination with certain histological features of mycosis fungoides (MF) might support a biological relationship between lichenoid pigmented purpuric dermatitis of Gougerot-Blum and MF. However, prompt clinical response to topical steroid therapy supports the benign clinical nature of our case.     

Papular sarcoidosis of the knees: a clue for the diagnosis of erythema nodosum-associated sarcoidosis

BACKGROUND: In recent years we have systematically explored the skin whenever sarcoidosis was suggested and we have observed with increasing frequency the presence of granulomatous cutaneous lesions of sarcoidosis involving the knees. OBJECTIVE: We sought to evaluate the specific cutaneous lesions of sarcoidosis involving the knees. METHODS: A total of 18 patients with biopsy-proven specific cutaneous sarcoidosis predominantly involving the knees were included in the study. Biopsy specimens were evaluated under polarized light. RESULTS: Of these cases, 4 corresponded to scar-sarcoidosis, 1 to plaque-type sarcoidosis, and 13 were an admixture of papules and minute scars frequently associated with erythema nodosum (papular sarcoidosis of the knees). Foreign particles were observed in 10 of 13 patients with papular sarcoidosis. CONCLUSION: Papular sarcoidosis of the knees can be considered a frequent form of cutaneous sarcoidosis, mainly observed in acute forms of the disease, and frequently associated with erythema nodosum.     

Erythroderma with lichenoid granulomatous features induced by erythropoietin

The increasing use of new drugs in cancer therapy, especially growth factors, hormones, and chemotherapies resulted in several reports of unusual skin eruptions. We studied a patient with erythroderma who had received erythropoietin because of myeloma with tumor anemia. The histological features were characterized by a lichenoid, focally granulomatous infiltrate with predominance of histiocytes. It is important for dermatopathologists to recognize this interesting pattern induced by erythropoietin.     

Cutaneous sarcoidosis in Asians: a report of 25 patients from Singapore

Sarcoidosis is a systemic noncaseating granulomatous disorder of unknown origin involving multiple organ systems. There has been no report so far to describe the epidemiological pattern of cutaneous involvement in sarcoidosis in South-East Asia with diverse ethnic groups. A retrospective study examining the clinicopathological features of all patients diagnosed with sarcoidosis at a tertiary dermatology centre in Singapore from 1980 to 2003 was conducted. Cutaneous sarcoidosis was diagnosed in 25 patients: 13 were Indian, 11 were Chinese and one was Eurasian. Cutaneous manifestations included papules, nodules, plaques and scarring alopecia. Extracutaneous involvement of lymph nodes (four patients), lungs (eight patients) and eyes (two patients) was seen. Eight patients had abnormal chest radiographic findings. Histopathological examination of skin lesions revealed noncaseating, epithelioid granulomatous infiltration in the dermis without evidence of mycobacterial infection, deep fungal infection or polarizable birefringent material. Treatment modalities included corticosteroids, hydroxychloroquine, isotretinoin, methotrexate and surgical excision. Five patients had complete resolution of the cutaneous lesions. Cutaneous sarcoidosis is rare in Asia and indeed in Singapore. Extracutaneous involvement is not uncommon and a thorough clinical evaluation should be undertaken.     

Lichen planopilaris: a clinicopathologic study

Three clinicopathologic variants of lichen planopilaris are described. The first is characterized clinically by individual keratotic follicular papules and histologically by a lichenoid inflammatory cell infiltrate confined to the follicular epithelium. The second variant consists of erythematous to violaceous plaques, some of which show follicular prominence; the histologic appearance is that of a lichenoid inflammatory cell infiltrate that affects both follicular and interfollicular areas. The third variant manifests as follicular papules of the scalp with concomitant or subsequent cicatricial alopecia. In this variant the histologic hallmark is a lichenoid, follicular and interfollicular inflammation, associated with or followed by scarring. Overlap among the three variants exists, and hence the concept of a disease spectrum ranging from pure follicular involvement without evidence of clinical scarring to cicatricial alopecia of the scalp is advocated.     

Giant cell lichenoid dermatitis: A possible manifestation of sarcoidosis

Giant cell lichenoid dermatitis is a recently described dermatosis thought to be an unusual lichenoid drug eruption. It is characterized by a generalized, pruritic, papulosquamous eruption sparing palms, soles, face and mucous membranes. Histopathologic findings include areas of epidermal hyperplasia and atrophy with focal vacuolar alteration of the basal layer, exocytosis and cytoid body formation. The dermis contains a band-like, mononuclear cell infiltrate at the dermoepidermal junction with admixed cosinophils, plasma cells and large multinucleate cells. The histologic differential diagnosis includes infectious processes, sarcoidosis, lichen nitidus, lupus erythematosus and lichen planus. We report 3 patients with giant cell lichenoid dermatitis, one of whom was subsequently diagnosed as having sarcoidosis. Because giant cell lichenoid dermatitis may resemble sarcoidosis both clinically and histologically, and because cutaneous sarcoid is often associated with systemic involvement, the diagnosis of sarcoid should be strongly considered in patients with giant cell lichenoid dermatitis.     

Widespread granulomatous dermatitis of infancy: an early sign of Blau syndrome

BACKGROUND: Pediatric sarcoidosis has traditionally been divided into 2 distinct groups: (1) school-aged children and adolescents with frequent involvement of the lungs and mediastinal lymph nodes (similar to adult sarcoidosis) and (2) infants and preschoolers with the triad of arthritis, uveitis, and a cutaneous eruption of discrete small papules, referred to as early-onset sarcoidosis. Blau syndrome, a rare autosomal dominant genodermatosis caused by mutations in the NOD2 (nucleotide-binding oligomerization domain 2) gene, has been considered as the familial form of early-onset sarcoidosis. OBSERVATIONS: A 9-month-old boy developed an asymptomatic eruption of 1- to 2-mm, red-brown to pinkish tan, flat-topped papules on the face, trunk, and extremities. There was no evidence of ocular involvement or arthritis. The skin lesions were characterized histologically by noncaseating granulomas in a periadnexal distribution within the dermis. A family history of uveitis supported a diagnosis of Blau syndrome, and analysis of the NOD2 gene revealed a heterozygous gain-of-function missense mutation (Arg334Trp) that has previously been detected in Blau syndrome kindreds. CONCLUSION: We draw attention to granulomatous dermatitis as an early manifestation of Blau syndrome and highlight emerging molecular evidence that this heritable autoinflammatory disorder and early-onset sarcoidosis represent a single disease entity.     

A case of facial atrophic sarcoidosis in an adolescent,successfully treated with the combination of prednisone and hydroxychloroquine

Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology. Cutaneous involvement occurs in up to 30% of patients and skin findings are often the initial presenting symptom. The facial atrophic form of sarcoidosis without associated ulceration in adolescents has rarely been described in the literature. We report a case of 13-year-old male patient with a facial atrophic sarcoidosis who was successfully treated with the combination of prednisone and hydroxychloroquine.     

Epidermotropic marginal zone lymphoma simulating mycosis fungoides†

Cutaneous lymphomas can usually be distinguished by architectural features, where most atypical lichenoid infiltrates implicate cutaneous T‐cell lymphoma (CTCL). We report a case of an 80‐year‐old man who presented with asymptomatic golden brown patches and diffuse pink papules on his trunk, buttocks and hips. Biopsies revealed a lichenoid infiltrate and areas of epidermotropism. Although the overall architectural pattern was compatible with mycosis fungoides, the lymphocytes had a more monocytoid and plasmacytoid appearance, and there were interspersed mature plasma cells. Immunohistological studies revealed that the pleomorphic lymphocytes were predominantly B cells (CD20\+ and CD79a\+) with a subpopulation of smaller bland T cells (CD3\+). Moreover, the B‐cell immunophenotype was compatible with marginal zone differentiation (bcl‐2+, bcl‐6?, CD10? and CD5?) and showed a lambda light chain restriction, confirming monoclonality. These findings were diagnostic for cutaneous marginal zone B‐cell lymphoma (MZL) with epidermotropism an entity which has only been reported twice in the literature, once in the setting of primary cutaneous disease, and once as cutaneous involvement of systemic disease. This case illustrates a rare pattern of cutaneous MZL, and underscores the importance of immunophenotypic characterization of cutaneous lymphomas in order to prevent the misdiagnosis of a CTCL.     

Actinic granuloma-like change in exogenous ochronosis: case report

Exogenous ochronosis is caused by the longterm application of skin-lightening creams containing hydroquinone. This irreversible disfiguring cosmetic problem assumes epidemic proportions in South African blacks. Mild ochronosis is characterized clinically by coarsening and darkening of the skin and severe ochronosis by coalescing, caviar-like black papules and atrophy. Histology shows ochronotic collagen fibres with eventual formation of ochronotic colloid milium. A variable cellular infiltrate, which may be granulomatous, is present. We describe a 39-year-old black woman with severe exogenous ochronosis who developed superimposed annular lesions with granulomatous histology bearing great resemblance to lesions of actinic granuloma.