Unmasking Diogenes Syndrome.

Diogenes syndrome is characterized by extreme self-neglect, social withdrawal, and poor personal and domestic hygiene. We report a case of Diogenes syndrome presenting with dermatitis passivata. An unusual "mask" of dirt resembling a carapace, onset of neglect after awareness of a breast lump and resumption of personal grooming and social activities after removal of the lump and counseling were seen.

What was known?

Dermatitis passivata can be a manifestation of the rarely reported Diogenes syndrome.

Introduction

Cessation of skin cleansing due to self-neglect can result in dermatitis passivata.[1] Diogenes syndrome is a rarely reported syndrome of extreme self-neglect, social withdrawal and domestic squalor.[23] It is commonly seen in elderly or demented patients.[1] It is often precipitated by stressful events. Other synonyms for Diogenes syndrome include severe self-neglect syndrome, aged recluse and social breakdown of the elderly.[23] Herein, we report a case of Diogenes syndrome presenting with an unusual “mask” of dirt resembling a carapace.

Case Report

A 55-year-old lady with crusted plaques over head, neck and trunk since 3 months was brought to the hospital by her relatives. She was an illiterate housewife belonging to the lower socioeconomic class. There was history of avoidance of cleansing and domestic squalor. The onset of neglect coincided with the awareness of a lump in her right breast. She had refused to seek help. On examination the patient was withdrawn and reluctant to cooperate for examination. There were adherent crusts over scalp, large dark brown confluent thick crusts forming a “mask” resembling a carapace over face and ears sparing the perioral area [Figure 1a and b]. Dark brown dirt like papules were found on neck, lower back, intermammary and inframammary area. There was poor oral hygiene and halitosis.A 6 × 8cm solitary, firm, non-tender, mobile mass was present in her right breast [Figure 2]. The overlying skin was stretched with engorged veins.

Figure 1

(a and b) “Mask” of dirt resembling a carapace

Figure 2

Breast lump

Complete blood counts, fasting blood sugar, liver and renal function tests were within normal limits. A skin biopsy revealed hyperkeratosis, increase in basal pigmentation and focal vacuolar changes [Figure 3a]. PAS stain showed few budding fungal yeast forms in the stratum corneum [Figure 3b].

Figure 3

(a) Hyperkeratosis with yeasts in stratum corneum (H and E ×40). (b) PAS-positive budding yeasts (PAS ×40)

Right breast lumpectomy specimen had a biphasic pattern composed of glands and cellular stroma on histopathological examination. The glands showed leaf-like projections due to underlying proliferation of cellular spindle-shaped stromal cells. There was no evidence of malignancy. A diagnosis of benign phyllodes tumor of the breast was made. Crusts were removed with saline compresses [Figures 4a and b]. The patient was referred to the psychiatrist where she underwent counseling. Three months after removal of the lump the patient returned for follow up with a clean face and renewed interest in social activities.

Figure 4

(a and b) Removal of crusts with saline compresses

Discussion

The term Diogenes syndrome was first coined by Clark et al., in 1975, to describe elderly patients with severe self-neglect, poor personal and domestic hygiene.[2] It is named after the cynical Greek philosopher “Diogene de Sinope” who advocated minimizing needs and a natural way of life.[4]

It affects men and women equally and may be seen in all socioeconomic groups. Patients usually have normal or above normal intelligence. Most live alone in physical isolation.[3] Hoarding of rubbish may or may not be present.[5] Diogenes syndrome may be primary or secondary. Secondary Diogenes syndrome is associated with underlying mental disorders.[3]

Nutritional deficiencies of iron, folate, vitamin B12, vitamin C, vitamin D and serum proteins may be seen. Mental illness, withdrawal and denial of need in old age may be triggered by various stressful situations.[2]

The cutaneous manifestations of neglect (dermatitis passivata) arise from accretion of keratin and dirt and resemble a carapace. Lesions are commonly found on the upper central chest, back and groin.[1] Our patient had predominant involvement of the face. Sparing of the perioral area could have resulted from her habit of wiping the perioral area after meals. Vigorous rubbing with soap and water can cause complete clearance of lesions arising from neglect.[6]

Terra firma forme dermatosis presents with dirt like patches which can be cleared with isopropyl alcohol but not soap and water. It can be differentiated by the absence of neglect and presence of adequate hygiene.[7]

Though dermatitis passivata may be commonly seen in clinical practice, awareness about Diogenes syndrome with its wider health and psychosocial implications is needed. Specialist psychological therapy and social measures may be needed.[14] Our patient improved with the manual removal of crusts, psychiatric counseling and removal of the lump. Our case is unusual in its “mask” of dirt resembling a carapace and the presence of a breast lump being a possible stressor.

What is new?

Diogenes syndrome presenting with an unusual mask like carapace and responding to clinical and psychological interventions was seen.